Journal of Veterinary Cardiology (2015) 17, S10eS52

www.elsevier.com/locate/jvc

REVIEW

Sequential segmental classification

of feline congenital heart disease
Brian A. Scansen, DVM, MS a,c,*,
Matthias Schneider, DVM, Dr med vet, PD b,
John D. Bonagura, DVM, MS a

a
Department of Clinical Sciences, The Ohio State University College of Veterinary
Medicine, 601 Vernon L Tharp St., Columbus, OH 43210, USA
b
Department of Clinical Studies, Small Animal Clinic, University of Giessen,
35392 Giessen, Germany

Received 31 December 2014; received in revised form 1 April 2015; accepted 21 April 2015

KEYWORDS Abstract Feline congenital heart disease is less commonly encountered in veter-
Cat; inary medicine than acquired feline heart diseases such as cardiomyopathy. Under-
Cardiovascular; standing the wide spectrum of congenital cardiovascular disease demands a
Cardiac; familiarity with a variety of lesions, occurring both in isolation and in combination,
Sequential segmental along with an appreciation of complex nomenclature and variable classification
analysis schemes. This review begins with an overview of congenital heart disease in the
cat, including proposed etiologies and prevalence, examination approaches, and
principles of therapy. Specific congenital defects are presented and organized by
a sequential segmental classification with respect to their morphologic lesions.
Highlights of diagnosis, treatment options, and prognosis are offered. It is hoped
that this review will provide a framework for approaching congenital heart disease
in the cat, and more broadly in other animal species based on the sequential seg-
mental approach, which represents an adaptation of the common methodology
used in children and adults with congenital heart disease.
ª 2015 Elsevier B.V. All rights reserved.

* Corresponding author.
E-mail address: brianscansen@yahoo.com (B.A. Scansen).
c
Present address: Department of Clinical Sciences, College of
Veterinary Medicine and Biomedical Sciences, Colorado State
University, Fort Collins, CO, USA.
Introduction
Congenital heart disease (CHD) accounts for a
small subset of cats presenting for veterinary care,
estimated at 5e15% of cardiac disease in this
species.1,2 However, the spectrum of CHD is broad

http://dx.doi.org/10.1016/j.jvc.2015.04.005
1760-2734/ª 2015 Elsevier B.V. All rights reserved.
Feline congenital heart disease S11

Abbreviations

AS aortic stenosis
ASD atrial septal defect
AVSD atrioventricular septal defect
CHD congenital heart/cardiovascular disease
CHF congestive heart failure
CTA computed tomography angiography
CTS cor triatriatum sinister
DCRV double-chambered right ventricle
DORA double outlet right atrium
DORV double outlet right ventricle
EFE endocardial fibroelastosis
LA left atrium/atrial
LV left ventricle/ventricular
MVD mitral valve dysplasia
MVD-S mitral valve dysplasia with stenosis
NT-proBNP amino terminus of the pro-hormone B-type natriuretic peptide
PA pulmonary artery/arterial
PAPVC partial anomalous pulmonary venous connection
PDA patent ductus arteriosus
PFO patent foramen ovale
PHT pulmonary arterial hypertension
PS pulmonary valve stenosis
RA right atrium/atrial
RV right ventricle/ventricular
TAPVC total anomalous pulmonary venous connection
TGA transposition of the great arteries
TOF tetralogy of Fallot
TOF-PA tetralogy of Fallot with pulmonary atresia
TVD tricuspid valve dysplasia
UAPA unilateral absence of a pulmonary artery
VSD ventricular septal defect

and complex, making the diagnosis, treatment,
and prognosis for these animals more challenging.
Accurate diagnosis and successful management of
CHD offers potential benefits and impact on
patient morbidity and survival. For example, cor-
rectly diagnosing and closing a patent ductus
arteriosus (PDA) can add a decade or more to a
cat’s life.
Cats with complex or severe CHD might be
examined within the first days to weeks of life due
to failure to thrive, dyspnea, cyanosis, or syn-
cope.3 However, most kittens with severe car-
diovascular malformations are believed to die
early in life and prior to veterinary examination.
The more common presentation of feline CHD is
that of an asymptomatic pet with an appointment
for a wellness exam. Auscultation of these cats is
often the earliest clue that CHD is present. Much
less often, a cat with CHD is examined later in life
for congestive heart failure (CHF), syncope, or
cyanosis due to previously undetected CHD. For
certain vascular anomalies, clinical signs may
relate to body systems other than cardiovascular,
such as regurgitation in the kitten with a vascular
ring anomaly.
This paper provides an overview of CHD in the
cat. The review first considers etiology and prev-
alence, followed by examination and therapy of
the cat with CHD, and finally specific congenital
defects. The latter are organized by sequential
segmental analysis with respect to their morpho-
logic lesions, diagnostic testing, treatment
options, and prognosis.
Etiology of congenital cardiovascular
disease
The precise etiology of CHD in veterinary species is
seldom known. In humans, CHD is the most
S12
common developmental defect occurring in nine
out of every 1000 live births.4 In roughly 20% of
cases, chromosomal abnormalities, single gene
mutations, or teratogens are implicated in the
genesis of CHD; however, the majority of human
CHD cases also have no identifiable cause.5
In some breeds of dog there is a recognized
familial component to CHD, with chromosomal
localization or isolation of the causative gene
being reported.6e9 The authors are unaware of any
reports proving a genetic basis for CHD in the cat.
Other authors have suggested an increased risk of
CHD for Siamese cats and this breed appears
overrepresented in cases of endocardial fibroelas-
tosis (EFE),10 aortic stenosis (AS), and supra-
valvular mitral stenosis.11 There have also been
reports of a family of Persian cats in which atrio-
ventricular septal defects (AVSD) were reproduced
after several matings.12 In an epidemiologic survey
of tricuspid valve dysplasia (TVD), the Chartreux
breed of cat was found to have an 11-fold higher
risk than other breeds.13 In a more recent study,
the proportion of domestic shorthair and Char-
treux cats with atrial septal defect (ASD) was sig-
nificantly higher than a reference population,
suggesting a possible breed predilection.14
In addition to genetic causes, environmental
factors during fetal growth have been implicated in
the development of human CHD. These conditions
include maternal diabetes, drug use, toxin expo-
sure, or infectious diseases during pregnancy.
Environmental causes of CHD are also poorly
described in cats, aside from scattered reports such
as atrioventricular valve malformation in cats
exposed in utero to griseofulvin15 and multiple
cardiovascular abnormalities in kittens with in
utero exposure to thalidomide.16 It is unknown
whether natural exposure to teratogens contributes
significantly to CHD development in domestic cats.
It is likely that multiple genetic and environ-
mental factors interplay in the development of
CHD, with genetic predispositions triggering car-
diac maldevelopment when the appropriate epi-
genetic factors are present. Determining which
specific genes and external factors lead to CHD in
humans and animals will remain as a focus of
research for the foreseeable future.
Incidence/prevalence of congenital car-
diovascular disease
The prevalence of CHD in the cat is difficult to
quantify due to a lack of routine perinatal care,
occurrence of conditions that are not apparent on
B.A. Scansen et al.
routine physical examination, and hospital biases
in published reports. Reports of feline CHD prev-
alence in the northeastern United States from the
middle of the 20th century vary from 0.2 to 1 per
1000 feline admissions and from 20 to 28 per 1000
feline autopsies.10 In a series of 368 kittens
autopsied over 4 years, 13 cases of CHD were
found for an incidence of 3.5%, although the
selection of cases for autopsy in this study was not
described.17 A recent abstract reported CHD in 47
of 2935 kittens from 2 to 6 months of age, sug-
gesting a feline CHD prevalence rate of 1.6%.d
However, not all 2935 cats were evaluated by
echocardiography, so CHD malformations silent to
auscultation were not included. In a report cur-
rently in press involving 57,025 mixed-breed cats
in a shelter between 1997 and 2003, 79 cats were
diagnosed with 87 CHD defects for a prevalence of
0.14%, which was higher than that reported in a
comparable population of dogs, in which 105
defects were found in 76,301 dogs for a prevalence
of 0.13%.18 Within these 87 feline defects, the
most common was ventricular septal defect (VSD),
comprising 18 cases.18 Importantly, purebred cats
and dogs were excluded from this analysis and not
all animals underwent echocardiography; these
factors may account for the lower rate of CHD
compared to previous reports.
While the true prevalence of CHD in cats is
unknown, there are data available evaluating the
relative incidence of specific malformations. Data
summarized from several studies of feline CHD
were recently reported (Table 1).19 Cases of CHD
from five separate reports were combined for
analysis, making a total of 435 defects.10,20e23
While the figures in Table 1 suggest the relative
incidence of certain defects, there may be tem-
poral factors, geographic variability, or hospital
biases that influence reported CHD prevalence. As
an example, reports of TVD incidence vary from 5%
of feline CHD cases in the 1980s in Boston10 to 23%
of cases in California in the late 1990s.22 Fur-
thermore, the incidence of ASD in the cat appears
rare, comprising 26 of the 435 (6%) summated
cases in Table 1, although a report from France
suggested that 38% of all CHD in both dogs and cats
are ASD, including 43 cases in cats.14 The lack of
high-resolution echocardiography at the time of
the earlier reports may account for some of this
discrepancy, as silent lesions may have been
missed; alternatively, the relative incidence of
d
Dirven MJM, Barendse MA, vanMook MC, Sterenborg JA,
vanden Wildenberg A. Prevalence of heart murmurs and con-
genital heart disease in 2935 young cats. J Vet Intern Med
2012;26:1513.
Feline congenital heart disease S13

Cardiac murmurs are relatively common in kit-

Table 1 Prevalence of congenital heart disease in
cats.
tens and in most cases are benign or functional in
origin. In a study of cats <6 months of age, 135 of
Defect Number Percentage 2935 kittens (4.6%) had cardiac murmurs, with 47
VSD 80 18.4% of these 135 (34.8%) having CHD and 86 of 135
PDA 49 11.3% (63.7%) having functional murmurs.c The chal-
TVD 47 10.8% lenges that normally arise with feline auscultation,
MVD 44 10.1% including intractability, purring artifact, rapid
AVSD 42 9.7%
heart rate, cardiac rotation, and closely-spaced
AS 31 7.1%
TOF 30 6.9%
auscultatory areas, are exaggerated in kittens.
ASD 26 6.0% There are no specific findings distinguishing a
PRAA 23 5.3% functional murmur from that of a CHD. Soft, brief,
EFE 21 4.8% musical systolic murmurs are more likely to rep-
PS 17 3.9% resent a functional (innocent) murmur in kittens,
Other 11 2.5% but these characteristics are not absolute. Even
DORV 7 1.6% murmurs that come and go can be associated with
CTS 7 1.6% a malformation such as MVD with dynamic outflow
Total ¼ 435 100% tract obstruction. While some of the typical aus-
AS ¼ aortic stenosis; ASD ¼ atrial septal defect;
cultatory features of CHD in cats are summarized
AVSD ¼ atrioventricular septal defect; CTS ¼ cor triatriatum under specific disorders, as a general rule, younger
sinister; DORV ¼ double-outlet right ventricle; cats with moderate-to-loud systolic murmurs
EFE ¼ endocardial fibroelastosis; MVD ¼ mitral valve dyspla- should be investigated by Doppler echocardiog-
sia; PDA ¼ patent ductus arteriosus; PRAA ¼ persistent right raphy. Kittens with faint or soft murmurs might be
aortic arch or other vascular ring anomaly; PS ¼ pulmonary
valve stenosis; TOF ¼ tetralogy of Fallot; TVD ¼ tricuspid
reasonably followed during the vaccine sequence,
valve dysplasia; VSD ¼ ventricular septal defect. and physiologic reasons for murmurs such as fever
and anemia excluded. However, even soft mur-
murs, if persistent, should optimally be evaluated
CHD may vary geographically or be evolving over by echocardiography or, at the least, a thoracic
time. Within the report of Chetboul et al.,14 the radiograph and serum levels of the amino terminus
prevalence of ASD in cats can be calculated as 43 of the pro-hormone B-type natriuretic peptide
cases out of 21,015 cats admitted to the same (NT-proBNP) can be measured to screen for overt
hospital, for a rate of 0.2%; this study was based on cardiac enlargement once the cat has grown.
Doppler echocardiography and not confirmed with When murmurs are not discovered until a cat is
saline contrast studies or post-mortem evaluation. near maturity, the potential for both congenital
and acquired cardiac disease (cardiomyopathy)
must be entertained.
Evaluation of the cat with congenital The general rules applicable to canine auscul-
cardiovascular disease tation apply to cats, but there are some exceptions
to consider. Apical rotation can influence the point
The clinical diagnosis of CHD in kittens and mature of maximal murmur intensity. The small size of the
cats is usually stimulated by the findings of clinical feline thorax causes loud murmurs to radiate
signs that are compatible with heart disease, or the widely. The more gradual onset of pulmonary
identification of a cardiac murmur. There are no arterial hypertension (PHT) in cats with CHD (as
specific historical features of CHD. The finding of compared with dogs) can alter the characteristic
cyanosis in a younger cat without overt pulmonary or intensity of a murmur over time, with some
disease should prompt consideration of a cardiac murmurs becoming quiet or unapparent. The
malformation. When respiratory signs in a cat are murmur of a feline PDA is sometimes more typical
attributed to CHF, the potential for CHD should at of a ‘long systolic murmur’ as opposed to a con-
least be entertained. The authors have evaluated tinuous murmur.
cats with CHD that first exhibited clinical compli- Ancillary physical examination findings such as
cations in middle age, including cats >8 years of abnormalities of the arterial or jugular venous
age. While the signs of CHF are more likely in pulse, palpation for ventricular heaves (prominent
symptomatic patients, the occasional cat is pre- apical impulses) and thrills, and estimation of jug-
sented for arterial thromboembolism, especially in ular venous pressure are qualitatively similar to that
disorders characterized by left atrial (LA) dilata- of dogs or humans with CHD. However, the rapid
tion such as mitral valve dysplasia (MVD). heart rate and small size of cats diminishes the
S14
likelihood that some of these physical findings will
be identified with high sensitivity and specificity.
The diagnostic workup of the cat with suspected
CHD centers on echocardiography with Doppler
studies. Saline contrast echocardiography is
sometimes performed to identify right-to-left
shunting. Thoracic radiography can be instructive
and is essential in cats with respiratory signs or
when moderate-to-severe cardiomegaly is identi-
fied caused by valvular malformation or left-to-
right shunts. Sedation is often required to facili-
tate optimal patient studies and to minimize
stress. Cats with left-to-right shunting lesions
(PDA, VSD, ASD) often have dilation of the pul-
monary vasculature on thoracic radiographs, even
prior to the onset of CHF. The value of multiple-
lead ECG recordings to screen for cardiac disease is
lower in the era of Doppler echocardiography, but
these studies sometimes identify atrial or ven-
tricular enlargement. However, the frequent
finding of a cranial frontal axis often mitigates the
value of the standard limb leads in a cat with a
normal heart rhythm. Aside from a hematocrit,
routine laboratory tests are of little consequence,
except in cats with complications of CHF, arterial
thromboembolism, or other co-morbidities. Hypo-
xemia can be verified by pulse oximetry or arterial
blood gas analysis, but these tests are rarely nee-
ded. The role of biomarkers such as cardiac tro-
ponins or natriuretic peptides in cats with CHD
requires further definition. The feline test for NT-
proBNP is potentially valuable for decision making
in the cat with a soft cardiac murmur when a client
is reluctant to pursue definitive diagnosis.
Advanced imaging studies including computed
tomography angiography (CTA) or magnetic reso-
nance angiography (MRA) and cardiac catheter-
ization with angiocardiography are useful for
complex malformations or when vascular anoma-
lies are suspected. Catheterization is also indi-
cated for catheter-based interventional therapies
such as device closure of a defect or balloon val-
vuloplasty. Considering echocardiography with
Doppler studies has largely supplanted cardiac
catheterization and angiography for diagnosis and
assessment of most lesions, only selected exam-
ples of these studies will be shown.
Therapy for the cat with congenital car-
diovascular disease
There are five general approaches to the man-
agement of CHD that can be taken in isolation or
combination. These include: (1) watchful waiting
B.A. Scansen et al.
for development of clinical signs; (2) medical
therapy; (3) catheter-based interventions; (4)
surgical management; and (5) hybrid approaches
that combine surgery (thoracotomy in most cases)
with catheter-based intervention. Some surgical
procedures can be accomplished without car-
diopulmonary bypass; however, the standard
treatment of complex CHD in children usually
requires extracorporeal circulation, an approach
that is very limited for cats at this time. In this
review, surgical methods for treatment will indi-
cate the standard of treatment for children, unless
specifically noted for cats.
Medical therapy for CHD in cats can include
treatment of CHF, control of heart rate or
arrhythmia, management of dynamic outflow tract
obstruction, and treatment of high pulmonary
vascular resistance in the setting of moderate-to-
severe or symptomatic PHT. Additionally, phle-
botomy (or antineoplastic drugs such as hydrox-
yurea) can be used to treat polycythemia in
cyanotic heart disease. There are no clinical trial
data available, to date, for any of these medical
treatments and, as such, they should be consid-
ered empirical, based on clinical experience only.
Medical treatment of CHF in cats typically involves
furosemide and an angiotensin-converting enzyme
inhibitor. Pimobendan and spironolactone can be
added in refractory CHF or if deemed potentially
useful for the underlying disorder. The treatment
of PHT with sildenafil holds some basis in terms of
clinical reports in cats,24 and this drug should be
considered when moderate-to-severe PHT results
in exercise intolerance, cyanosis or syncope rela-
ted to high pulmonary vascular resistance. Silde-
nafil can also be used as a diagnostic tool to
identify potential reversibility of PHT in cats with
PDA. Control of heart rate (as in MVD with stenosis
(MVD-S)), dynamic ventricular outflow tract
obstruction (as with cases of MVD or pulmonary
valve stenosis (PS)), and ectopic complexes is
often centered on the use of atenolol in cats. For
management of more serious supraventricular
arrhythmias, atenolol, sotalol, diltiazem and,
infrequently, digoxin can be considered for either
suppression of ectopic rhythms or rate control. For
ventricular arrhythmias, most clinicians use sotalol
or atenolol and accept the negative effect of these
drugs on myocardial function.
Catheter-based interventions are increasingly
important and have been used to successfully
manage PDA and PS in cats. The major limitation is
the availability of catheters and devices of
appropriate size for cats and, of course, the
technical challenges of catheterizing such small
animals. In children, hybrid procedures have been
Feline congenital heart disease
used to combine surgery (to provide cardiac
access) and transcatheter delivery of balloons,
stents, and occlusion devices across the ven-
tricular, atrial, or arterial wall. Guidance is ach-
ieved with ultrasound and fluoroscopy. This
approach has been used to a limited degree in
cats.25 Standard thoracic surgery or thoracoscopy
are appropriate for ligation of PDA, treatment of
some vascular rings, and for pericardial defects.
Palliation of cyanotic heart disease by the creation
of systemic to pulmonary shunts is another indi-
cation for surgery, although this is technically
challenging. The lack of cardiopulmonary bypass
seriously limits the definitive treatment of most
complex CHD in cats.
Analysis and classification of feline con-
genital heart disease
Given the complexity that can arise during mal-
development of the mammalian heart, various
classification schemes exist to characterize CHD.
Pediatric cardiology has widely adopted the
sequential segmental approach for analyzing CHD,
which provides a systematic template for
describing the congenitally malformed heart.26e29
Initially, a morphologic approach is taken, in
which each cardiac segment (atria, ventricles,
great arteries) is individually analyzed and mal-
formations in that segment are noted. Next,
abnormalities in the relationship and communica-
tion between two segments (e.g. the atrioven-
tricular relationship or ventriculoarterial
communication) are recorded. Notably, and out-
side the intent of this manuscript, the terminology
chosen for these communications (whether align-
ment30 or connection31) differs between the two
pathologists that have popularized these naming
schemes e Richard Van Praagh30 and Robert
Anderson.31 Regardless of terminology, the seg-
mental analysis has evolved to address a need to
describe increasingly complex lesions. Some forms
of CHD are discrete and simply described (e.g. a
PDA); however, other malformations require a
more prescriptive approach. Analyzing each ana-
tomical chamber independently avoids associating
adjacent connections as hallmarks of that seg-
ment. For example, while the LA is expected to
receive the pulmonary veins, the chamber is not
defined by this arrangement. In an animal with
complex CHD, the pulmonary veins can have an
anomalous connection to the right atrium (RA) or
systemic vasculature. Thus, features that are
largely preserved and specific to that structure
S15
morphologically define each cardiac segment. The
morphologic RA has an auricular appendage that
attaches with a broad base, includes a crista ter-
minalis (the smooth, thick muscular protrusion at
the opening into the RA appendage), and contains
the rim of the fossa ovalis along the atrial septum.
In contrast, the LA has an auricular appendage that
attaches with a more narrowed base, lacks a crista
terminalis, and contains the valve of the fossa
ovalis along its atrial septal surface. Furthermore,
the pectinate muscles of the atrial appendages
extend around the orifice of the tricuspid valve in
the RA, while being confined to the cranial aspect
and auricular appendage in the LA. The right
ventricle (RV) is defined by the coarse trabecula-
tions that line the apex and a prominent moder-
ator band, while the morphologic left ventricle
(LV) has a smooth septal surface and fine apical
trabeculations.29 The morphologic characteristics
of the great vessels are less preserved but, in
general, the coronary vasculature derives from the
aorta and bifurcation after a short main segment is
characteristic of the pulmonary trunk. Van Praagh
has offered the following analogy: the segments
(atria, ventricles, great arteries) are the bricks of
the heart, while each connection (atrioventricular,
ventriculoarterial) is the mortar between the
bricks; maldevelopment or malalignment between
these components leads to variable forms of
CHD.32 Table 2 provides a framework for classifying
CHD in animals using this segmental approach, as
modified from human schemes.28e31 Despite
reports describing this approach in the veterinary
literature,33e35 widespread adoption of sequential
segmental analysis has not occurred. This might be
explained by the high rates of perinatal mortality
for complex CHD limiting the number of complex
cases presented to veterinary cardiologists. How-
ever, complex lesions are occasionally encoun-
tered in feline cardiology; furthermore, the
segmental approach provides a useful framework
for CHD classification, as presented in the
remainder of this review.
Abnormalities in the cardiac position or
thoracic wall integrity
Malformations of the sternum and thoracic
cage
Morphologic lesions
Malformations of the thoracic cage typically
involve the sternum and include pectus excava-
tum, pectus carinatum, sternal cleft, and ectopia
S16 B.A. Scansen et al.

Table 2 Sequential segmental classification of congenital heart disease. Each segment or connection is con-
sidered individually, with several possible variants in morphology, position, alignment, or relationship. Possible
anomalies within each segment or connection are also given.
Segment/Connection Morphology, position, Possible anomaliesa
or relationship
Cardiac position and/or  Ectopia  Ectopia cordisb
Thoracic wall integrity  Dextrocardia  Peritoneopericardial diaphragmatic
 Midline, diaphragmatic, or herniab
thoracic wall defects  Pectus excavatumb
 Pectus carinatumb
 Sternal cleftb
Venoatrial connection  Normal  Anomalous pulmonary venous
 Anomalous connectionb
 Stenotic  Pulmonary vein stenosis
 Cor triatriatum sinisterb
 Anomalous systemic venous
connection
 Budd-Chiari-like lesionsb
 Cor triatriatum dexter
Atria  Solitus (normal)  Atrial septal defectb
 Inversus  Patent foramen ovaleb
 Ambiguous  Supravalvular mitral ringb
Atrioventricular  Normal  Atrioventricular septal defectb
connection  Concordant  Common atrioventricular valve
 Discordant  Accessory pathway/bypass tractb
 Absent
Atrioventricular valves  Normal  Mitral/tricuspid valve dysplasia
 Dysplastic (insufficiency or stenosis)b
 Stenotic  Mitral/tricuspid valve atresiab
 Atretic
Ventricles  Normal  Ventricular septal defectb
 Hypoplastic  Double chamber right ventricleb
 Primitive  Primary infundibular stenosisb
 Inverted  Endocardial fibroelastosisb
 Indeterminate  Uhl’s anomalyb
 Hypoplastic right heartb
 Hypoplastic left heart syndrome
 Univentricular heart
Ventriculoarterial  Normally connected  Subaortic stenosisb
connection  Malposed  Aortic atresia
 Transposed  Tetralogy of Fallotb
 Double outlet  Pulmonary atresiab
 Single outlet  Double outlet right ventricleb
 Transposition of the great
arteriesb
 Truncus arteriosus communisb
Semilunar valves  Normal  Aortic valve stenosisb
 Stenotic  Pulmonary valve stenosisb
 Absent  Absent pulmonary valve

(continued on next page)

Feline congenital heart disease
Table 2 (continued )
Segment/Connection Morphology, position,
or relationship
Great arteries  Equal division
 Maldivision
 Stenosis
 Hypoplasia
 Absence
Peripheral vasculature  Fistula
 Malformation
a
The list of possible anomalies is not exhaustive.
b
Signifies anomalies reported in cats.
cordis. Pectus deformities involve either inward
deviation (excavatum) or protusion (carinatum) of
the sternebrae, resulting in a narrowed (excava-
tum) or excessive (carinatum) dorsal to ventral
thoracic dimension. Based on experience and lit-
erature reports, pectus excavatum appears to be
the more common in cats.36e41 Lateral thoracic
radiographs of both conditions are shown in
Figure 1. A more extreme form of sternal malfor-
mation is a sternal cleft, in which a variable
number of sternebrae (typically the caudal ster-
nebrae) fail to fuse together. This anomaly has
recently been reported in a young Ragdoll kitten.42
Skeletal malformations appear to be more com-
mon in cats with vascular ring anomalies, including
supernumerary or fused ribs and vertebral
anomalies.43
Ectopia cordis is a rare congenital malforma-
tion, reflecting inappropriate cardiac positioning
outside the normal thoracic and intrapleural
space, and results from a failure in midline fusion
during development.44 The heart is partially or
completely displaced outside of the pleural cavity
and may be in a cervical, thoracic, or abdominal
position. Among veterinary species, this defect has
most commonly been described in cattle, with
isolated reports in foals, pigs, and dogs. There are
descriptions of ectopia cordis in kittens with
maternal thalidomide exposure, in which a thor-
acoabdominal fissure and normal intracardiac
anatomy were documented.16
Diagnostic findings
Historical signs typically reflect abnormalities in
respiration, which proportionally vary in severity
S17
Possible anomaliesa
 Persistent right aortic archb
 Retroesophageal subclavian arteryb
 Patent ductus arteriosusb
 Aortopulmonary windowb
 Coarctation of the aortab
 Tubular hypoplasia of the aortic
archb
 Interruption of the aortic arch
 Pulmonary artery stenosisb
 Peripheral pulmonary artery stenosis
 Unilateral absence of right or left
pulmonary arteryb
 Portosystemic shuntsb
 Arteriovenous malformationsb
of the degree of malformation and concurrent
disease. Abnormal pulmonary or cardiac ausculta-
tion is commonly related to displaced or com-
pressed internal thoracic anatomy. Significant
sternal deformities are readily diagnosed by
thoracic palpation, and thoracic radiography is
confirmatory. Computed tomography has also been
used to assess the severity of the defect and the
relationship to internal thoracic anatomy in cats.42
Therapeutic options
Sternal malformations are surgically treated, if
clinical signs mandate repair. Minor sternal defects
are often asymptomatic and do not require
therapy.
Prognosis
Reported outcomes with surgery have been favor-
able,38,39,41 although re-expansion pulmonary
edema after external splint correction for pectus
excavatum has been reported in an 8-week-old
kitten.37
Peritoneopericardial diaphragmatic hernia
and intrapericardial cyst
Morphologic lesions
Congenital peritoneopericardial diaphragmatic
hernia (PPDH) is proposed to occur secondary to
maldevelopment or incomplete closure of the
embryologic septum transversum.45 Clinically,
peritoneal contents (typically falciform fat,
omentum, liver, or intestines) communicate with
the pericardial sac and represent space-occupying
lesions (Fig. 2). Intrapericardial cyst has also been
S18
Figure 1 Right lateral thoracic radiographs of a 10-month-old cat with pectus excavatum (A) and of an 11-year-old
cat with pectus carinatum (B). Sternal deformities may be congenital or acquired, resulting in deviation of the heart
and lungs within the thoracic cavity.
described in a 2-year-old cat, which appeared to
result from incarcerated hepatic capsule through a
PPDH.46
Diagnostic findings
Clinical signs are variable, but typically include
dyspnea/tachypnea, lethargy, vomiting, or ano-
rexia. Common co-morbidities include umbilical
hernia, vertebral anomalies, sternal malforma-
tions such as pectus excavatum previously descri-
bed, or a reduced number of sternebrae. In one
case series, half of the diagnoses were inci-
dental.47 Radiographs (Fig. 2) are diagnostic,
revealing a loss of diaphragmatic integrity and fat,
gas, or soft-tissue opacity within and causing dis-
tortion of the cardiac silhouette. The dorsal border
of the communication, termed the dorsal peri-
toneopericardial mesothelial remnant, is often
evident as a soft tissue density spanning the dis-
tance between diaphragm and pericardium.48 The
caudal vena cava can take a more dorsal course
than normally expected and the pulmonary veins
enter more cranially than anticipated due to cra-
niodorsal cardiac displacement within the peri-
cardial space. Ultrasound may also reveal fat
echogenicity or hepatic parenchyma within the
pericardial space and adjacent to the heart.
Therapeutic options
In asymptomatic cats, treatment is typically con-
servative, with fair-to-good outcomes repor-
ted.45,47 In symptomatic cats, surgery is advised,
with resolution of clinical signs expected in 85% of
cases.45,47,49 In the case of an intrapericardial
cyst, surgical resection and repair of the hernia
have been curative.46
Prognosis
The prognosis for surgical repair of PPDH is good to
excellent. Prognosis is also good for cats with
incidentally diagnosed PPDH that is managed
conservatively. In a series of 66 cats, in which 37
B.A. Scansen et al.
were treated surgically and 29 conservatively, 88%
of the owners who pursued surgical therapy were
very satisfied versus 68% of the owners who pur-
sued conservative treatment.45 Perioperative
mortality as high as 14% was reported in one case
series,45 but much lower in a recent report.49
Complications associated with surgery include
hemorrhage and re-expansion pulmonary edema.
There are case reports of an intrapericardial cyst50
and of constrictive pericarditis51 developing after
PPDH repair in cats, both of which required reop-
eration to address, although these complications
appear to be rare.
Situs inversus and dextrocardia
Morphologic lesions
Abnormalities in left-to-right laterality can occur
during development, resulting in abnormal cardiac
positioning within the thorax. The atrial arrange-
ment or atrial situs present in the normal cat
places the RA cranial and rightward relative to the
LA. If these structures are oppositely positioned,
with the LA on the right, the condition is classified
as atrial situs inversus. As described earlier,
determination of atrial positional arrangement is
based on morphologic features specific to each
chamber and not on which side of midline they
reside. Atrial situs inversus is almost always seen in
conjunction with a mirror-image arrangement of
left-to-right laterality affecting the entire viscera
e both thorax and abdomen. This malformation is
termed situs inversus totalis and has been reported
in two cats.52,53 In addition to situs inversus,
mammals can develop with uncertain atrial
arrangements, termed situs ambiguous. Typically,
such cases are defined by the presence of two
morphologically similar atria in the absence of the
other. Right atrial isomerism indicates two right
atria; LA isomerism indicates two left atria.
Examples of atrial isomerism are missing in the
veterinary literature, aside from reports in cattle54
Feline congenital heart disease S19

Figure 2 Images of two cats with peritoneopericardial diaphragmatic hernia. Panels A and B are lateral (A) and
dorsoventral (B) thoracic radiographs from a 9-year-old Persian cat with a markedly enlarged cardiac silhouette and
fat opacity obscuring the diaphragmatic border consistent with herniation of falciform fat into the pericardial space.
Panel C is a post-mortem image of a different cat showing an accumulation of omentum and small intestine in the
pericardial space. In addition, marked left auricular enlargement can be seen (arrow); the cat was also diagnosed with
mitral valve dysplasia. Image in panel C courtesy of Christopher S. Kovisto, DVM, The Ohio State University, Columbus,
Ohio.

and mice.55 It is unclear if this reflects a lack of
their occurrence or of recognition.
Dextrocardia is a term used to describe a heart
with a rightward apex-to-base axis, in contrast to
the normal leftward axis.56 Although often used to
refer to a mechanical displacement of an other-
wise normal heart into the right hemithorax (more
correctly termed cardiac dextroposition), true
dextrocardia is rare and implies that cardiac
development was abnormal. A case report34
described dextrocardia in a cat with discordant
atrioventricular connection and double-outlet RV
(DORV), while Figure 3 provides an example of
dextrocardia on the dorsoventral thoracic radio-
graph of a 13-week-old kitten with complex cya-
notic CHD.
S20
Diagnostic findings
Cats with visceral or atrial situs abnormalities,
particularly dextrocardia or situs ambiguous, are
likely to have significant additional cardiac mal-
formations, as noted in the few case reports of this
condition.34 Situs inversus totalis can be clinically
silent and only detected once imaging is per-
formed. Imaging of an animal with situs inversus
totalis can be complicated by the appearance of
atypical echocardiographic imaging planes or
unexpected radiographic or surgical findings.53
Therapeutic options
Treatment is directed at concurrent cardiac
defects or non-cardiac malformations. In cases of
abnormal laterality such as situs inversus, no
therapy is required.
Prognosis
Complex abnormalities in cardiac arrangement
carry a grave prognosis in cats, while situs inversus
totalis should be well tolerated, as long as addi-
tional developmental malformations are not
present.
Figure 3 Dorsoventral thoracic radiograph of a 13-
week-old kitten with central cyanosis, complex con-
genital heart disease, and dextrocardia. Right (R) and
left (L) are labeled. Note that the base-to-apex axis of
this heart is directed to the right, rather than the normal
leftward direction. This is in contrast to cardiac dex-
troposition, in which chamber enlargement, atelectasis,
or a mass effect deviates the heart or mediastinum
towards the right thorax.
B.A. Scansen et al.
Abnormalities in the venoatrial
connections
Anomalous pulmonary venous connection
Morphologic lesions
The pulmonary veins arise within the embryo dur-
ing development of the lung buds from the fore-
gut, and initially drain to the developing systemic
venous system e the cardinal veins.57 As develop-
ment proceeds, the primitive common pulmonary
vein develops from an out-pouching of the LA.57
This common pulmonary vein grows outward and
eventually fuses with the primitive pulmonary
veins; at this time, the connections from the pul-
monary veins to the systemic venous system
decline.57,58 If the embryologic communications
from pulmonary veins to systemic veins persist,
either partially or completely, the animal is born
with partial (PAPVC) or total anomalous pulmonary
venous connection (TAPVC), respectively. Alter-
native and historical descriptors for these con-
ditions included anomalous pulmonary venous
‘return’ or ‘drainage’; however, connection is the
preferred word for this condition as it is the ana-
tomic connection that is anomalous, while drain-
age can be aberrant, even in the setting of a
normal anatomic connection (e.g. pulmonary
venous drainage through an ASD). Functionally,
these conditions can be comparably viewed to an
ASD, as they result in a left-to-right shunt at the
atrial level. Classification of TAPVC is based on the
original description by Darling, in which the
abnormal connection is classified as supracardiac
(Type I), cardiac (Type II), infracardiac (Type III),
and mixed (Type IV).59,60 In humans, such con-
nections are also classified as obstructive or non-
obstructive, with constriction of the anomalous
pulmonary venous connection causing increased
pulmonary venous pressure and CHF. Cases of
TAPVC are reported in the dog61e64 and a foal,65
and PAVPC in two miniature Schnauzers has been
reported.66 Although not yet reported, to the
authors’ knowledge, it is likely that both PAPVC
and TAPVC occur in cats.
Diagnostic findings
The few reports in animals with TAPVC have indi-
cated signs of cyanosis and failure to thrive
developing within the first days to weeks of life.
Features of clinical presentation for these defects
that are unique to cats have not been reported. In
other species, echocardiography is supportive, and
cross-sectional imaging such as CTA or MRA pro-
vides a detailed three-dimensional understanding
Feline congenital heart disease
of the pulmonary venous anatomy. Cardiac cathe-
terization and angiography may also be employed
to evaluate the angiographic appearance of the
pulmonary venous connections and any concurrent
alterations in intracardiac hemodynamics.
Therapeutic options
Surgical reconstruction of the pulmonary veins,
establishing normal drainage to the LA, is the
preferred treatment strategy in humans with
TAPVC or PAPVC. To the authors’ knowledge, such
repairs have not been described in animals.
Prognosis
The prognosis for TAPVC is presumed to be grave in
cats, while PAPVC might be tolerable in the post-
natal period, but the natural history is unknown.
Cor triatriatum sinister
Morphologic lesions
As described above in the setting of TAPVC, the
common pulmonary vein originates as an out-
pouching of the LA and connects with the primi-
tive pulmonary veins before incorporating into the
body of the LA and creating a smooth walled LA
chamber.57 If the connection of the common pul-
monary vein to the LA is stenotic, the LA chamber
appears as two discrete compartments with a
dividing membrane. This clinical entity is termed
cor triatriatum, denoting three atria within a sin-
gle heart. In veterinary medicine, the modifying
term sinister is added to distinguish this condition
from a divided RA (cor triatriatum dexter). Of the
several classification schemes proposed for cor
triatriatum sinister (CTS), the system proposed by
Lucas is the most complete.60 Type-A CTS is cate-
gorized by an accessory atrial chamber that
receives all pulmonary veins and communicates
with the LA; Type B receives all pulmonary veins
and does not communicate with the LA; and in
Type C, the LA receives some of the pulmonary
veins with the others connecting normally or
anomalously to the heart.60 Among veterinary
species, CTS is most frequently seen in the cat,
with several reports in the literature.25,67e71
Diagnostic findings
Obstruction of pulmonary venous drainage results
in respiratory signs of left-sided CHF. Thoracic
radiographic findings include LA dilation, pulmo-
nary venous congestion, and patchy interstitial to
alveolar infiltrates of pulmonary edema. Echo-
cardiography is used to visualize the divided LA,
with color and spectral Doppler interrogation
S21
defining the location and degree of stenosis across
the dividing membrane. An important echo-
cardiographic distinction involves precisely deter-
mining where the level of obstruction lies within
the LA relative to the pulmonary veins and left
auricular appendage (Table 3), as well as the
coexistence of an AVSD to separate CTS from
supravalvular MVD-S or double-outlet RA.
Therapeutic options
Surgical dilation of the CTS membrane has been
reported on the beating heart of a 7-month-old
kitten using a valve dilator delivered through a
stab incision in the proximal LA chamber and
resulting in resolution of CHF.68 Recently, a hybrid
approach using cutting balloon dilation catheters
with echocardiographic guidance was reported in a
cat with good success in resolving CHF and reduc-
ing the pressure gradient across the LA
membrane.25
Prognosis
Without dilation of the obstructing membrane, the
prognosis is guarded, with death reported at 2
months in a cat that did not undergo medical
therapy70 and at 7 months of age in a cat on
medical therapy for CHF.69 With either surgical or
transcatheter intervention, complete resolution of
clinical signs and good long-term survival have
been reported.25,68
Hepatic venous obstruction
Morphologic lesions
Obstruction of the hepatic venous outflow tract (or
the Budd-Chiari syndrome) in humans typically
stems from an acquired cause such as caudal vena
caval thrombosis.72 However, stenosis of hepatic
venous outflow also can lead to this syndrome and
has been reported in one cat, though it was
unclear if this case represented a congenital
stenosis.73 Another potential cause is cor tria-
triatum dexter, a malformation in which the RA is
divided into a cranial and a caudal chamber, with a
restrictive membrane between resulting in ele-
vated caudal vena caval pressures. Although
reported in dogs,74e77 cor triatriatum dexter has
not yet been reported in a cat.
Diagnostic findings
In the one reported feline case of hepatic venous
stenosis,73 the cat presented at 10 years of age
with vomiting and ascites; ascites is also the typi-
cal clinical finding in cor triatriatum dexter.
Obstruction of the middle hepatic vein was
S22
Table 3 Anatomic characteristics of congenital heart defects resulting in pulmonary venous congestion and
obstruction to left ventricular inflow.
Lesion
Anomalous pulmonary venous connection
Pulmonary vein stenosis
Cor triatriatum sinister
Double outlet right atrium
Supravalvular mitral stenosis
Mitral valve stenosis
AVSD ¼ atrioventricular septal defect; PAPVC ¼ partial anomalous pulmonary venous connection; TAPVC ¼ total anomalous
pulmonary venous connection.
diagnosed by ultrasonography and angiography.
Although the cat was older, no acquired etiologies
could be identified. While an uncommon diagnosis,
ultrasound evaluation of the caudal vena cava and
hepatic veins in combination with angiographic
imaging (either selective angiography, CTA, or
MRA) might delineate the hepatic venous anatomy
and localize the stenosis. In cases of cor tria-
triatum dexter, echocardiography reveals a divi-
ded RA, with a caudal and a cranial chamber;
contrast echocardiography can be performed to
visualize the separate atrial chambers and confirm
the diagnosis using agitated saline administered
alternately from a cranial (cephalic) and caudal
(saphenous) vein.78
Therapeutic options
Medical therapy for hepatic venous obstruction
includes diuretic therapy, with abdominocentesis,
as needed, to control ascites. Catheterization-
B.A. Scansen et al.
Anatomic characteristics
The pulmonary veins do not attach to the left atrium.
Obstruction to pulmonary venous return occurs if the
anomalous vein is constricted during its atypical course.
Congestion can involve all lung lobes (TAPVC) or within
segmental lobes (PAPVC).
A narrowing is apparent at the pulmonary venous ostium,
with a normal anatomic connection to the left atrium.
Congestion occurs in those lung lobes draining into the
stenotic pulmonary ostium.
There is stenosis of the common pulmonary vein resulting
in an obstruction in the body of the left atrium proximal to
the left auricular appendage. Pulmonary congestion occurs
in all lung lobes and left auricular appendage size is normal
to small.
Occurs in the setting of AVSD as a result of ventriculo-atrial
malalignment. The apical atrial septum is deviated to the
left and fused to the lateral aspect of the endocardial
cushions resulting in obstruction to pulmonary venous
inflow. In published feline reports, the level of obstruction
appears to be distal to the left auricular appendage,
resulting in dilation of this structure.
Results from maldevelopment of the mitral apparatus and
development of a fibrous or fibromuscular obstruction
above the mitral annulus. The obstruction is located distal
to the left auricular appendage, resulting in enlargement
of that structure.
The mitral valve leaflets or chordae are abnormally formed
or fused together, resulting in an obstruction to left
ventricular inflow and causing left atrial enlargement,
including dilation of the left auricular appendage.
based therapy to dilate a focal stenosis can be
considered,73 but this option proved unsuccessful
in the reported feline case due to venous perfo-
ration and hemorrhage. In cases of cor triatriatum
dexter, surgical resection,76 conventional balloon
dilation,77 and cutting balloon dilation79 have all
been reported with successful outcomes in dogs.
Prognosis
The prognosis for these defects in cats is unknown.
Abnormalities in the atria
Atrial septal defect and patent foramen
ovale
Morphologic lesions
An ASD is a persistent communication between the
LA and RA. It appears to be a relatively rare defect
Feline congenital heart disease S23

in cats. In the case of isolated ASD, blood shunts

from the LA to the RA due to higher right ven-
tricular compliance. The subsequent left-to-right
shunt leads to volume overload of the right heart
and increases pulmonary blood flow. The degree of
shunting depends on the size of the defect. In
moderate-to-large ASDs, RA enlargement, RV
enlargement, and pulmonary overcirculation
develop because the right heart directly receives
both the normal systemic venous return as well as
the excess volume traversing the shunt. Although
defects in the fossa ovalis (ostium secundum
defects) are the only ‘true’ ASD, as they are the
only defects in which the deficiency of tissue is
localized to the interatrial septum, four principal
forms of ASD are recognized and they are classified
by location relative to the interatrial septum
(Fig. 4). The ostium primum ASD is a defect in the
ventral interatrial septum directly above the AV
valves and is recognized as a form of AVSD (Figs. 5
and 6). Ostium secundum defects result from
insufficiency of tissue at the fossa ovalis in the
central interatrial septum (Fig. 5). Secundum ASDs
are the most common form in other species and
appear to also be the most frequent form of ASD in
cats.14 Sinus venosus defects occur in the dorsal
interatrial septum and can be located either cau-
dally or cranially, which often allows pulmonary
Figure 4 Schematic representation of the anatomic
venous return to enter the RA. A rare form of ASD
locations of atrial septal defects (ASD). The right atrial
is the unroofed coronary sinus, which is reported in free wall has been removed to reveal the interatrial
humans and partially characterized in a recent septum. Defects in the ventral septum above the tri-
case report of a dog.80 In this defect, there is a cuspid valve are termed primum ASD (1); those in the
communication between the LA wall and the great area of the fossa ovalis are secundum ASD (2); those high
cardiac vein, often associated with persistence of in the dorsal atrial septum near either cranial or caudal
the left cranial vena cava. These lesions permit vena caval inflow are sinus venosus ASD (3) and may be
shunting of blood from the LA to the RA through associated with anomalous pulmonary venous drainage;
the coronary sinus. To the authors’ knowledge, and an unroofed coronary sinus ASD (4) represents the
sinus venosus and unroofed coronary sinus ASDs last location for these defects. Reproduced by permission
of The Ohio State University.
have not been reported in the cat.
Flow across the interatrial septum can continue
if high RA pressure persists following birth. This
situation can prevent the fusion of the septum Less common than either ASD or PFO is an atrial
primum and septum secundum that normally septal aneurysm, in which a saccular deformity of
occurs in the first week of life. Shunting can occur the interatrial septum is observed and is asso-
even in the presence of otherwise normally formed ciated with excessive tissue in the area of the fossa
atrial septal components. Right-sided heart dis- ovalis, with varying degrees of endocardial fibrosis
eases that elevate RA pressure, such as PS or TVD, and septal thickening.81 Reported in a mature cat,
can prevent this normal atrial septal closure this condition can develop with either congenital
maintaining the right-to-left shunting found in the or acquired heart disease.81
fetus. This form of interatrial communication is
referred to as a patent foramen ovale (PFO). Diagnostic findings
Although less common, left-to-right shunting Cats with left-to-right shunting ASD typically have
across a PFO is also possible when the LA is a mild-to-moderate systolic murmur, often most
markedly stretched (as with MVD) and if this prominent at the left heart base and caused by
stretch prevents fusion along the rims of the increased flow into the pulmonary trunk (so-called
foramen ovale. ‘relative pulmonary stenosis’). The flow velocity
S24 B.A. Scansen et al.

Figure 5 Images from cats with atrial septal defects (ASD). Panel A: Right parasternal long axis echocardiographic
image from a 3-year-old Angora cat with a large primum ASD as a component of an atrioventricular septal defect. Note
the massive right-sided heart enlargement as well as the minimal portion of dorsal interatrial septum that remains
(arrows), nearly resulting in a common atrium. Panel B: Transesophageal echocardiographic four-chamber image from
a 4-year-old domestic longhair cat with a large secundum ASD. The defect is centered in the interatrial septum, with
portions of the dorsal and ventral interatrial septum still visible (arrowheads). Panel C: Gross pathologic image from a
cat with two ASDs. The smaller, more dorsal defect (#) is in the area of the fossa ovalis and represents a secundum-
type defect, while the ventral defect (*) overlying the atrioventricular valves represents a primum-type defect.
CaVC ¼ caudal vena caval entrance; LA ¼ left atrium; LV ¼ left ventricle; RA ¼ right atrium; RAA ¼ right auricular
appendage; RV ¼ right ventricle.

Figure 6 Gross pathologic image from a 4-year-old Persian cat with a partial atrioventricular septal defect and
double outlet right atrium resulting in left heart failure. Severe dilation of the left auricular appendage (LAA) is seen
in Panel A. In Panel B, the right atrium is opened, revealing a large ostium primum defect (*) above the septal tricuspid
valve leaflet. The opened left atrium in Panel C shows the narrowed orifice (arrow) created by the fusion of the apical
atrial septum to the lateral endocardial cushion and resulting supravalvular mitral obstruction. From this image alone
the lesion cannot be distinguished from a supravalvular mitral ring, but the concurrent presence of partial atrio-
ventricular septal defect argues for double outlet right atrium.
Feline congenital heart disease
across the atrial defect is too low to generate a
murmur. Thoracic radiographs show enlargement
of the right heart and pulmonary trunk, along with
pulmonary overcirculation when the shunt is left-
to-right and significant in volume. Echocardiog-
raphy confirms the diagnosis of ASD and any con-
current defects (Fig. 5). As with other left-to-right
shunts, pulmonary vascular disease can develop as
a response to chronic pulmonary overcirculation.
Cyanosis in animals with ASD may develop as a
consequent of PHT or, alternatively, tricuspid
regurgitation associated with RV enlargement, or
concurrent TVD may direct deoxygenated blood
across the ASD. The latter appears to be the more
common cause of cyanosis in animals with ASD.
Therapeutic options
Treatment is focused on medical therapy to alle-
viate clinical signs if they develop. Newer trans-
catheter occluding devices can be considered.
Although catheter-based interventions have been
reported in dogs,82 these have not been deployed
in a cat (to the authors’ knowledge). Open-heart
surgery provides a definitive repair and has been
reported in a cat,83 but the expertise for this sur-
gery is not widely available. The value, if any, of
pulmonary artery banding to minimize shunting
and protect the pulmonary vascular tree is
uncertain.
Prognosis
There are no prospective studies evaluating the
natural history of ASD in cats. However, a retro-
spective study14 evaluating ASDs in both dogs and
cats found that 38 of the 45 cats (84%) had no signs
of heart disease when diagnosed as adults, sug-
gesting a good-to-excellent prognosis for many
cats that survive to maturity. Conceivably, the
prognosis for this defect depends on the degree of
shunting, cardiac remodeling, pulmonary vascular
resistance, and concurrent defects.
Abnormalities in the atrioventricular
connection
Atrioventricular septal defect
Morphologic lesions
An atrioventricular septal defect (AVSD), formerly
called endocardial cushion or atrioventricular
canal defect, results from maldevelopment of the
atrioventricular septum. This tissue is the unique
septum that connects the atrial and ventricular
septae and separates the dorsal LV from the
S25
ventral RA. The differing insertion points of the
mitral annulus relative to the more apically loca-
ted tricuspid annulus form this septum. The dif-
ferent forms of AVSD are characterized by a loss of
this normal offset and place the tricuspid and
mitral annuli along the same anatomical plane.
Additional maldevelopment of the ventral atrial
septum, dorsal interventricular septum, and por-
tions of the AV valves result in defects classified as
partial, transitional/intermediate, or complete
AVSDs. Partial AVSD implies there is only an atrial
communication (Fig. 6) or only a ventricular com-
munication, and that both AV valve annuli appear
as discrete orifices. A complete AVSD is a defect in
which there is a large primum ASD as well as an
interventricular communication; furthermore, the
AV valve is singular and covers both the right and
left sides of the heart with a bridging leaflet in
between. Based upon the chordal attachments of
the AV valve, complete AVSDs can be subdivided
into three types.84 The transitional or inter-
mediate form of AVSD can be defined as one in
which both an ASD and a VSD are present, but
separate and distinct mitral and tricuspid valve
annuli remain.85 In all cases e partial, inter-
mediate, and complete e there can also be
defects present in the AV valves, resulting in var-
iable degrees of regurgitation, most notably a cleft
in the septal leaflet of the left AV valve that
facilitates regurgitation into the LA and often the
RA across the ASD. Originally described in 1968,86
various forms of AVSD are relatively common in
cats, and comprise approximately 10% of cases of
feline CHD.
Another unique feature of AVSD described in
humans and also recognized in the cat is the
development of a double outlet RA (DORA).87,88 In
this condition, the apical interatrial septum fuses
with the lateral endocardial cushion, and the
leftward deviation and malalignment of the atrial
septum results in a supravalvular obstruction to LV
inflow (Fig. 6; Table 3).89,90
Diagnostic findings
The largest series of feline AVSD published to date
involved 26 cats, of which 17 had an isolated AVSD
and nine had concurrent CHD.88 Of the isolated
cases, 13 had partial and four had complete AVSD;
transitional or intermediate forms were not tabu-
lated. Eight of the 17 isolated cases were asymp-
tomatic at presentation, eight had respiratory
distress or tachypnea, and one was syncopal.
Electrocardiographic findings include a variable
frontal plane axis and can include a RV hyper-
trophy pattern or a left cranial axis with increased
QRS voltages, depending on the type of AVSD. In
S26
some cats, the P-waves are markedly increased in
voltage or duration, presumably from atrial dila-
tation. Thoracic radiographs reveal cardiomegaly,
dilated pulmonary vasculature, and pulmonary
edema, if CHF has developed. All cats that have
been described with DORA were reported to have
CHF. Echocardiography establishes the diagnosis in
all cases.
Therapeutic options
Treatment is symptomatic, with medical therapy
for CHF, as required. Open-heart repair is the
standard in human medicine, but to the authors’
knowledge is not yet reported in a cat.
Prognosis
Survival is variable for cats with AVSD. In the case
series, a 5-year survival rate of 53% was reported
for the isolated defects, although three cats lived
longer than 10 years.88 Although cardiac remod-
eling can appear to be very advanced, a surprising
number of cats with AVSD do well, as evidenced by
this report and the authors’ experience.
Abnormalities in the atrioventricular
valves
Mitral valve dysplasia (regurgitation, steno-
sis, and supravalvular mitral ring)
Morphologic lesions
Mitral valve dysplasia (MVD) has variably been
reported as the most common CHD in cats.20 Mal-
formations of the mitral valve complex include
shortened and thickened chordae, direct papillary
to valve attachments, papillary muscle fusion or
displacement, and altered leaflet geometry
(Fig. 7). These malformations often lead to mitral
valve regurgitation, while MVD-S is less common. In
addition, a supravalvular ring of fibrous or fibro-
muscular tissue directly above the mitral annulus
that impairs LV inflow falls within the spectrum of
MVD and has been reported in at least 16
cases.11,91,92 Table 3 provides further descriptions
of valvular and supravalvular MVD-S in comparison
with other congenital causes of impaired LV inflow.
Mitral valve dysplasia is also a common feature of
AVSD, as previously discussed.
Dynamic LV outflow tract obstruction is a
recently described variant of MVD.e,93 Dynamic LV
e
Dirven MJM, Barendse MA, vanMook MC, Sterenborg JA,
vanden Wildenberg A. Dynamic left ventricular outflow tract
obstruction in 13 young cats. J Vet Intern Med 2012;26:1513e4.
B.A. Scansen et al.
outflow tract obstruction is typically caused by
systolic anterior motion of the mitral valve, and
this is a common echocardiographic finding in
acquired heart diseases leading to concentric LV
hypertrophy such as hypertrophic cardiomyopathy.
In a report of 13 cats, aged 12e21 weeks of age at
the time of first evaluation, systolic anterior
motion of the mitral valve resulting in outflow
tract obstruction and a variable amount of con-
centric LV hypertrophy were observed.d In five of
these cats, abnormal architecture of the mitral
valve complex was also appreciated by echo-
cardiography. Notably, the dynamic obstruction
and concentric LV hypertrophy were absent on re-
evaluation in nine of the 12 cases, and resolved
with atenolol in two of the three cases that had
persistent obstruction and LV hypertrophy.d Reso-
lution of concentric LV hypertrophy was also
reported in a 6-month-old cat.93 This observation
is consistent with the human experience, in which
abnormalities of the mitral valve apparatus
including an elongated anterior leaflet, direct
papillary muscle insertion, and tethered chordae
tendineae are frequently observed in humans with
hypertrophic cardiomyopathy.94 It is therefore
possible, given the high prevalence of hyper-
trophic cardiomyopathy in the feline population,
that some cases diagnosed as an acquired car-
diomyopathy are in fact a congenital malformation
of the mitral apparatus and that the true preva-
lence of MVD in the cat is underdiagnosed (Fig. 8).
This might also explain the discrepancy between
recent reports of CHD, in which the incidence of
MVD is less frequently reported compared with
older pathology-based surveys.
Diagnostic findings
Cats with MVD can present without clinical signs,
aside from a cardiac murmur, or be examined for
signs of respiratory distress secondary to left-
sided CHF. Hemoptysis has been observed by the
authors, presumably from flash pulmonary edema
or rupture of thin-walled bronchial venous-
pulmonary venous connections. In cases of
supravalvular MVD-S, a male predominance and
overrepresentation of the Siamese breed have
been reported.11 In cats with predominately MVD-
S and without regurgitation, a murmur might not
be present, or a subtle diastolic murmur may
rarely be detected. Mitral valve regurgitation
results in a systolic murmur along the left para-
sternal border. Gallop sounds are also reported in
cats with MVD. Abnormalities on ECG can include
atrial fibrillation, abnormal P-wave amplitude or
duration, and criteria of right heart enlarge-
ment.11,91 Thoracic radiographs may show LA
Feline congenital heart disease
Figure 7 Echocardiographic images from a 1-year-old
Savannah cat with mitral valve dysplasia causing
regurgitation and stenosis. The mitral valve appears
tethered (A) with a ‘hockey stick’ appearance to the
anterior leaflet in diastole (arrowhead). Color Doppler
imaging (B) shows a large jet of mitral regurgitation,
while a spectral Doppler recording (C) of the mitral
inflow shows a prolonged pressure half-time to the early
filling wave consistent with mitral stenosis. A ¼ late
transmitral filling wave; E ¼ early transmitral filling
wave; LA ¼ left atrium; LV ¼ left ventricle; RV ¼ right
ventricle.
enlargement and signs of pulmonary edema if CHF
has developed. Definitive diagnosis requires
echocardiography to characterize the structural
morphology of the mitral valve complex and
Doppler studies to evaluate the severity of
stenosis and regurgitation (Fig. 7).
Therapeutic options
Treatment is directed at resolution of pulmonary
edema in cases of CHF, or beta-blockade for cases
S27
of dynamic outflow tract obstruction. Beta-
blockade is potentially useful for cats with MVD-
S, considering the potential for tachycardia to
suddenly increase LA pressure, but no clinical tri-
als of treatment are available. Therapy for PHT
might also be necessary if resting tachypnea,
cyanosis, or syncope is observed. Open-heart sur-
gery has been reported in two cases of supra-
valvular MVD-S, with both cats dying during the
perioperative period. Catheterization-based or
hybrid procedures to dilate valvular or supra-
valvular MVD-S might be a consideration in the
future, but will require introducers and devices
appropriate for the size of a cat.
Prognosis
Cats have been observed to live for >8 years with
documented MVD. The prognosis is guarded if
respiratory signs predominate, although some cats
examined by the authors have survived for years
with medical therapy for CHF. Progressive and
refractory CHF is common, however, it can be
complicated by pulmonary hypertension or sys-
temic arterial thromboembolism. In cases of
moderate-to-severe LA dilatation, the authors
recommend antithrombotic therapy with clopi-
dogrel, accepting the potential risk for bleeding.
The risk for thrombosis associated with general
anesthesia should also be considered, in as much
as LA function is probably impaired by anesthetics.
Cases of dynamic LV outflow tract obstruction
carry a good-to-excellent prognosis, with regres-
sion of hypertrophy common during maturation or
after beta-blockade (Fig. 8).
Tricuspid valve dysplasia (regurgitation and
stenosis)
Morphologic lesions
Dysplasia of the tricuspid apparatus results in
morphologic changes comparable to that seen with
MVD e thickened and elongated leaflets, short-
ened or absent chordae, direct papillary to leaflet
attachment, papillary muscle fusion and hyper-
trophy, and variable degrees of regurgitation or
stenosis, the latter being rare in cats (Fig. 9).
Concurrent defects are relatively common,
including MVD, VSD, ASD, and PS.13,95,96 As with
MVD, TVD is also seen in cases of AVSD. Ebstein’s
anomaly is a common eponym directed to cases of
TVD. This descriptor implies specific meaning from
human medicine, namely that the leaflets of the
tricuspid valve fail to delaminate from the ven-
tricular myocardium, resulting in the appearance
S28 B.A. Scansen et al.

Figure 8 Echocardiographic images from a domestic shorthair cat at 10-months (A, B, C) and 4-years of age (D, E, F).
At 10-months of age, the long-axis (A) and short-axis (B) images show moderate left ventricular hypertrophy with
severe left ventricular outflow tract obstruction and mitral regurgitation appreciated on color Doppler imaging (C)
secondary to systolic anterior motion of the mitral valve. After 3 years and therapy with a beta-adrenergic receptor
blocker (atenolol), the left ventricular walls appear normal in thickness with no outflow tract obstruction apparent.

of an apically displaced tricuspid annulus.97 As Diagnostic findings

such, the diagnosis requires demonstration of RV
electropotentials, with concurrent recordings of
RA pressures as has been described in dogs.98 In
feline cases of TVD, the leaflets of the tricuspid
valve are malformed, but the characteristic
delamination and apical displacement of the tri-
cuspid annulus are seldom, if ever, apparent. In
the authors’ experience, true Ebstein-like malfor-
mations are exceptionally rare in cats, although
they have been reported.99 Clinical signs can
develop from right-sided CHF or from right-to-left
shunting across a septal defect.
A breed predilection for TVD has been reported in
Chartreux cats.13 Auscultation typically reveals a
systolic murmur, which may radiate widely and
with maximal intensity on the right hemithorax.
The electrocardiographic features described in
cats with TVD are splintering of the QRS complex
and a RV enlargement pattern in the frontal plane
leads.96,100 Thoracic radiography might reveal
cardiomegaly and dilation of the caudal vena cava,
with cavitary effusions in advanced cases.13,96
Echocardiography confirms the diagnosis and
evaluates the severity of cardiac remodeling (e.g.
Feline congenital heart disease
Figure 9 Pathologic specimen from a cat with tricus-
pid valve dysplasia viewed from the opened and under-
developed right ventricle. Fusion of the papillary mus-
cles to the tricuspid valve orifice and malformed tri-
cuspid valve leaflets results in a stenotic ring (arrow).
RAA ¼ right auricular appendage; RV ¼ right ventricle.
RA and RV dilation), as well as the degree of
valvular regurgitation and stenosis. Concurrent
defects such as ASD, PFO, or MVD are also char-
acterized on echocardiographic study. Differential
diagnosis includes PHT, isolated ASD, and RV car-
diomyopathy. A three-stage classification system
has been proposed for animals with TVD and
regurgitation, in which: Stage 1 is characterized by
a structural alteration without volume overload;
Stage 2 is characterized by an altered valve and
atrial volume overload; and Stage 3 is charac-
terized by both RA and RV volume overload being
apparent with a structurally malformed valve.13
Due to the high prevalence of physiologic tricus-
pid regurgitation by Doppler studies in cats,101 the
precise delineation of valvular malformations in
cats without a right-sided murmur would be
potentially problematic (as currently encountered
in dogs).
Therapeutic options
Medical therapy for CHF is indicated. Catheter-
based interventions for TVD with stenosis might be
considered, but have not yet been reported in the
feline literature. In children, surgical options are
S29
performed to repair or replace a dysplastic valve,
but have not been reported in the cat.
Prognosis
The prognosis is good for cats with mild-to-
moderate disease (Stages 1 and 2 in the above
classification system). Cats with signs of cyanosis
or cavitary fluid accumulation have a poor prog-
nosis. In a study of 11 cats with TVD, only one
death attributed to TVD was reported in a 7-
month-old cat that also had MVD, although long-
term follow-up was unavailable for most of the
animals.13 In a series of cases diagnosed at post-
mortem, including cats with more-advanced dis-
ease, the prognosis was considered worse, with
average mortality at 5e7 months of age.96
Abnormalities in the ventricles
Ventricular septal defect
Morphologic lesions
Ventricular septal defects are defined as commu-
nications between the ventricles allowing for the
exchange of blood between the LV and RV. The
interventricular septum is made up of four com-
ponents: an inlet portion between the tricuspid
and mitral valves; an apical or trabecular portion;
a smooth-walled outlet portion between the LV
and RV outflow tracts; and a membranous portion
at the cranial aspect of the septal tricuspid valve
leaflet separating aortic from tricuspid valve.
Classification and nomenclature of VSDs is
unfortunately variable due to multiple descriptors
for the same lesion, but they are broadly named by
which portion of the septum is deficient when
viewed from the perspective of the RV (Fig. 10).
Defects can be classified as: (1) muscular, if
entirely surrounded by muscular septum; (2) jux-
taarterial, if located in the outlet septum imme-
diately below the pulmonary and aortic valves; and
(3) perimembranous (or paramembranous), when
surrounded by or encompassing part of the mem-
branous septum, which lies at the cranial aspect of
the septal tricuspid leaflet. Defects that result in
fibrous continuity of the aortic and tricuspid valve
are perimembranous, while defects that result in
fibrous continuity between the aortic and pulmo-
nary valves are juxtaarterial. Based on the relative
location of the VSD, modifiers may then be applied
to these descriptors (e.g. apical muscular defect
or perimembranous outlet defect). Juxtaarterial
defects, also termed doubly committed or sub-
arterial VSDs, are always associated with a loss of
S30
the muscular outlet septum. In addition to the
above naming conventions, historical terminology
relates the position of the VSD relative to the
supraventricular crest, such that perimembranous
defects are infracristal and juxtaarterial defects
are supracristal. The term ‘inlet VSD’ is a source of
some confusion, as both muscular and perimem-
branous defects can extend into the inlet part of
the ventricular septum. Historically, an inlet VSD is
directly ventral to the septal tricuspid valve leaflet
and associated with an AVSD.102 Defects that are
present with ventriculoarterial malalignment (e.g.
tetralogy of Fallot (TOF) or pulmonary atresia
(TOF-PA)) can be more challenging to classify as
Figure 10 Schematic representation of the anatomic
locations of ventricular septal defects (VSD). The right
ventricular free wall has been removed to reveal the
interventricular septum. Defects associated with atrio-
ventricular septal defects lie within the inlet portion of
the right ventricle under the septal tricuspid valve
leaflet (1). Perimembranous VSDs (2) occur at the cranial
aspect of the septal tricuspid valve leaflet, just below
the supraventricular crest, resulting in fibrous continuity
of the aortic and tricuspid valves and may extend toward
the inlet, outlet, or apex. Juxtaarterial VSDs (3) occur
above the supraventricular crest and immediately below
the pulmonary valve leaving the aortic and pulmonary
valves in fibrous continuity. Muscular VSDs (4) can
develop occur anywhere within the interventricular
septum and are completely surrounded by muscle; these
can be located within the inlet, trabecular/apical, or
outlet septum. Reproduced by permission of The Ohio
State University.
B.A. Scansen et al.
they result in loss or malposition of the supra-
ventricular crest and portions of both the mem-
branous and muscular outlet septum. Taken as a
whole, such defects are sometimes termed mala-
lignment or conotruncal VSDs, reflecting their
etiology. However, if the defect is scrutinized for
fibrous continuity between aortic and tricuspid
valve (perimembranous) and between aortic and
pulmonary valve (juxtaarterial), an appropriate
descriptor can be applied. In some cases of TOF,
the defect is perimembranous, in others, it is
juxtaarterial, and in others, the VSD has charac-
teristics of both.103
As the interventricular septum develops from
the apex to the crux of the heart, the inlet and
membranous portions are the last to close.104
Presumably as a consequence of this pattern of
development, defects in the membranous septum
are the most common in humans105 and animals.106
However, muscular defects might occur with sim-
ilar or higher frequency, with a prevalence of 2e5%
of all human infants. However, most muscular VSDs
spontaneously close within the first year of life.107
Spontaneous closure of VSDs has also been repor-
ted in the dog.108 While spontaneous VSD closure
has not been documented in a cat, aneurysms of
the interventricular septum have been described
in both dogs and cats that may represent sponta-
neously closed defects,109 and the authors have
observed nearly closed feline VSDs at necropsy.
Excessive pulmonary blood flow associated with a
large VSD can increase pulmonary vascular resist-
ance, leading to PHT and shunt reversal with
hypoxemia, cyanosis, and erythrocytosis. This
clinical scenario is termed Eisenmenger’s syn-
drome. Right-to-left shunting can also stem from
PS or a double-chambered RV (DCRV).
Diagnostic findings
Cats with VSD typically present for murmur eval-
uation at a young age. Alternatively, cats with
large defects or multiple malformations can pres-
ent with signs of CHF due to LV volume overload, or
signs of cyanosis related to shunt reversal. The
systolic murmur is typically loudest on the right,
although it often radiates across the thorax.
Thoracic radiographs reveal left-sided or general-
ized cardiomegaly and a variable degree of pul-
monary overcirculation. The ECG is variable and
shows voltage criteria or axis deviations compat-
ible with cardiomegaly or ventricular conduction
disturbances. Echocardiography confirms the
diagnosis, allows for the evaluation of concurrent
defects, and determines the anatomic location of
the VSD (Figs. 10 and 11).
Feline congenital heart disease
Therapeutic options
Many cats with VSD do not require therapy. The
defect is often small or restrictive, suggesting that
there is preservation of normal RV and LV pressures.
This can be suspected when Doppler echocardiog-
raphy records high velocity left-to-right shunting
flow of w5 m/s or greater. If CHF or PHT are docu-
mented, medical therapy is targeted to minimize
clinical signs. As discussed for ASD, devices are
available for transcatheter occlusion of VSDs and
this procedure has been performed in dogs,110 but
not in cats, to the authors’ knowledge. Open-heart
repair is an additional treatment option, allowing
for direct patch closure of the defect, but also has
not yet been reported in cats. Palliative banding of
the pulmonary artery or branch pulmonary arteries
might be considered to reduce pulmonary blood
flow and lessen the degree of pulmonary over-
circulation. This has been reported in several cats,
with fair-to-good results111e113; however, aside
from overt left-sided CHF, specific clinical criteria
to recommend this procedure are undetermined.
Prognosis
There are no published studies evaluating the nat-
ural history or post-therapeutic outcome in cats
with VSD, although a recent abstract reported on
109 animals (56 dogs, 53 cats) and suggested that
isolated VSD is associated with low rates of cardiac-
related mortality in companion animals.f Anecdotal
and clinical experience suggests that the prognosis
for smaller, uncomplicated defects is excellent. If
additional cardiac defects are present or the defect
is large, the prognosis becomes guarded.
Double-chambered right ventricle and pri-
mary infundibular stenosis
Morphologic lesions
Double-chambered right ventricle refers to the
presence of anomalous muscle bundles traversing
the middle of the RV, separating it into a proximal,
hypertrophied chamber and a distal, low-pressure
chamber. The muscular proliferation results in a
demonstrable pressure gradient across the mid RV.
This lesion is often associated with VSD in humans,
with 69% of people having concurrent VSD.114 In
cats, the association appears to be less strong,
with two of 10 reported cats having concurrent
VSD,115,116 although the possibility that some
defects might have closed at the time of
f Therapeutic options
Bomassi E, Misbach C, Tissier R, Gouni V, Trehiou-Sechi E,
Petit AM, Damoiseaux C, Pouchelon JL, Chetboul V. Ventricular
septal defect in dogs and cats: A retrospective study of 109
cases (1992e2013). J Vet Intern Med 2014;28:1011.
S31
evaluation must be considered. The etiologic basis
of DCRV is uncertain, although the association with
VSD leads to speculation that the anomalous
muscle bundles could represent a defect in inter-
ventricular septal development such as incomplete
fusion of the bulbar and endocardial cushions.117
Alternatively, some cases may develop or pro-
gress as a secondary response to the high-velocity
VSD jet impacting the RV wall.118
Primary infundibular stenosis is a condition
similar to DCRV. While DCRV is defined by anom-
alous muscle bundles from the supraventricular
crest to the RV apex, and is often associated with
VSD, primary infundibular stenosis is characterized
as a fibromuscular band of tissue at the junction of
the RV cavity and pulmonary infundibulum (prox-
imal RV outflow tract).119 Primary infundibular
stenosis can also occur as a fibromuscular thick-
ening of the infundibulum.119 Primary infundibular
stenosis has been described in 13 cats, 12 of which
were combined in a case series.120,121 Practically,
DCRV and primary infundibular stenosis can be
difficult to distinguish ante mortem by echo-
cardiography. If a VSD is present or the obstruction
appears to be predominately muscular and extends
to the apical RV, DCRV is the more likely diagnosis.
Diagnostic findings
In cats with DCRV, the most common presenting
complaint is an asymptomatic animal with a heart
murmur. In two cases, the presenting complaint
was chylothorax, resulting in respiratory difficulty,
while in another cat, premature ventricular com-
plexes were the reported reason for referral.115,116
The murmur in cats with DCRV and primary infun-
dibular stenosis is typically loud, with maximal
intensity at the left or right parasternal bor-
der.116,121 Electrocardiographic changes are varia-
ble, with increased QRS duration, right axis
deviation, junctional tachycardia, and uniform
premature atrial and ventricular complexes all
having been reported.116,121 Thoracic radiographs
show cardiomegaly, predominately of the right
heart,116 although a normal cardiac silhouette was
reported in cases with mild-to-moderate dis-
ease.121 Echocardiography defines the stenosis,
degree of RV hypertrophy and RA enlargement,
and the presence or absence of a concurrent VSD.
In cats with primary infundibular stenosis, con-
current LV hypertrophy consistent with hyper-
trophic cardiomyopathy has also been reported.121
Medical therapy can include consideration of beta-
adrenergic antagonists (atenolol) in asymptomatic
cats with dynamic intraventricular obstruction.
S32 B.A. Scansen et al.

Figure 11 Compilation of echocardiographic images from a young cat with a large perimembranous ventricular
septal defect (VSD). Panels A and B show 2-D imaging in long-axis and short-axis, respectively. The defect (asterisk)
lies below the non- and right-coronary cusps of the aortic valve (Ao) and enters the right ventricle (RV) under the
septal tricuspid valve leaflet. Panels C and D show the aliased and turbulent color Doppler signal in the same planes as
A and B, indicating left-to-right systolic flow. LA ¼ left atrium, LV ¼ left ventricle.

Heart failure therapy is appropriate for cats with
cavitary effusion. Balloon dilation of DCRV115 and
of primary infundibular stenosis121 has also been
reported, with minimal long-term improvement in
the cat with DCRV and mixed results in the cats
with primary infundibular stenosis. Improvement is
more likely in cats without CHF at the time of
intervention.121 Surgical palliation of DCRV with a
patch graft and ventriculotomy under inflow
occlusion has been reported in two cats, with one
experiencing resolution of chylothorax and the
other dying intraoperatively.116
Prognosis
The prognosis is variable and depends on the
severity of the RV obstruction. In asymptomatic
cats with severe disease (instantaneous gradient
70 mmHg), the prognosis appears to be guarded
in primary infundibular stenosis and mixed in
DCRV. Once CHF is present, medical therapy can
manage disease for months to 1e2 years.
Endocardial fibroelastosis
Morphologic lesions
Endocardial fibroelastosis is a pathologic thicken-
ing of the endocardium that can be primary or
secondary to other cardiac disease. The primary
condition has been reported in Siamese122 and
Burmese cats,123,124 but has also been recognized
in a closed colony of domestic shorthair cats.125
Both the LV and LA are primarily affected,124
although the disease has also been noted to
solely affect the LA.17 Pathogenic studies have
suggested a familial or genetic disorder, poten-
tially related to obstruction of cardiac lymphatics,
although the exact etiology remains unknown.124
The prevalence of this condition appears to be in
decline, as few cases have been reported since the
1970s and 1980s, at which time it was suggested to
account for 11% of all cases of feline CHD.126 It
might be speculated that some suspected cases
were actually due to taurine deficiency and dilated
cardiomyopathy with secondary subendocardial
fibrosis of the LV.
Diagnostic findings
Based on the literature, presentation is typically
within the first 2e4 months of life, with signs of
inappetence and dyspnea,126 although less severe
changes have also been reported in related cats of
older age.125 Pleural effusion is common, with car-
diomegaly also noted on thoracic radiographs.
There are no echocardiographic reports of the
Feline congenital heart disease
condition in cats, although LV and LA dilation would
be anticipated given gross pathologic findings. The
differential diagnosis would likely include causes of
primary or secondary myocardial disease.
Therapeutic options
Treatment is supportive for signs of CHF. There is
no therapy available to directly target the under-
lying pathophysiology responsible for the thick-
ened endocardium.
Prognosis
The prognosis is grave, with most reports describ-
ing rapid progression of disease and death within
days of presentation.
Uhl’s anomaly
Morphologic lesions
Uhl’s anomaly is a rare congenital defect asso-
ciated with almost total absence of the RV myo-
cardium. It bears similarity to arrhythmogenic RV
cardiomyopathy/dysplasia, with the distinction
that in Uhl’s anomaly, there is direct apposition of
the RV epicardium and endocardium without
intervening myocardium, infiltration, or inflam-
mation.127,128 While considered to be CHD, due to
its appearance in childhood and in antenatal
diagnoses, the embryologic basis remains unre-
solved.128 There are two case reports129,130 of a
comparable condition described in cats.
Diagnostic findings
The reported feline cases presented at 2 and 4
years of age, and both were domestic short-
hairs.129,130 Physical examination findings included
right-sided CHF with dilated and pulsating jugular
veins and ascites. Thoracic radiographs should
reveal right-sided cardiomegaly with pleural effu-
sion if CHF is evident. Electrocardiography has
been reported as showing P pulmonale with small
QRS complexes and a right axis deviation in the
frontal plane. Echocardiography confirms severe
right-sided heart enlargement with a paper-thin
and hypokinetic RV free wall. The final diagnosis
requires histopathologic demonstration of direct
epicardial and endocardial apposition.
Therapeutic options
Treatment is directed at medical therapy to
resolve CHF.
Prognosis
The prognosis appears poor, as the first case pro-
gressed to refractory CHF and anorexia 5 months
S33
after the diagnosis,129 while the second case that
presented at 2 years of age died naturally from
CHF, despite medical therapy, 1 month after
diagnosis.130
Abnormalities in the ventriculoarterial
connection
The term ‘conotruncal defect’ is conventionally
used to describe lesions associated with abnormal
partitioning of the LV and RV outflow tracts, as
seen in TOF, TOF-PA, and truncus arteriosus com-
munis.131 This term was adopted because it rec-
ognized lesions that were in proximate
relationship to the ‘conus’ e broadly considered to
be the muscular infundibulum that supports the
semilunar valves e and the ‘truncus’ e broadly
considered to be the embryologic structure giving
rise to the arterial trunks (pulmonary and aortic).
However, as suggested in a recent editorial by
Robert Anderson,132 the term conotruncal has no
broadly accepted definition that is scientifically
accurate and the term might logically be applied
to abnormalities of the semilunar valves that lie
between the conus and the arterial trunks. It
therefore seems appropriate, despite the histor-
ical importance of the term conotruncal defect in
veterinary medicine, that it be abandoned in favor
of more precise descriptions of proximal (ven-
tricular outlets), intermediate (arterial roots and
valves), and distal (arterial trunks) lesions involv-
ing the outflow tracts.132 Within this context are
the complicated defects of TOF, TOF-PA, double-
outlet-right-ventricle (DORV), truncus arteriosus
communis, and transposition of the great arteries
(TGA).
Tetralogy of Fallot
Morphologic lesions
This constellation of defects is named after the
French pathologist who described the four com-
ponents in children, namely: a large VSD, pulmo-
nary stenosis (infundibular narrowing, annular
hypoplasia, þ/ valvular fusion), RV hypertrophy,
and malalignment (overriding) of the aortic root
(Fig. 12). The main functional consequence of TOF
is right-to-left shunting across the VSD from ele-
vated RV pressure caused by the pulmonary
stenosis. While considered as four lesions, TOF
might be better viewed as a singular defect
explained by malrotation and improper division
(septation is displaced cranially) of the outflow
tracts. Some pathologists actually refer to this
S34 B.A. Scansen et al.

lesion as the monology of Stensen,133 after the
original descriptor of the malformation. Con-
ceptually, an error in development occurs,
wherein the muscular outlet septum is deviated
cranially, resulting in extension of the aorta to the
right and narrowing of the pulmonary infundibulum
(RV outflow tract below the pulmonary valve),
resulting in RV hypertrophy. Because the muscular
outlet septum is deviated cranially, the interven-
tricular septum is malaligned and cannot close,
which results in the VSD. Significant right-to-left
shunting with deoxygenated blood mixing within
the LV leads to hypoxemia and cyanosis. With long-
standing arterial desaturation, increased red-
blood-cell mass often develops, which is medi-
ated via the release of erythropoietin, and hema-
tocrits in the range of 60e80% can be encountered.
Some cases with less severe RV outflow tract
obstruction are often acyanotic and maintain a
normal hematocrit. When erythrocytosis does
occur, abnormal rheological properties develop,
with increased viscosity and reduced blood flow in
small vessels. This can clog arteries in the ocular
and central nervous systems and result in throm-
bosis and ischemic injury. Several case reports of
this condition exist in cats and it has been reported
to occur in conjunction with AVSD.88,134e141
Diagnostic findings
Cats with TOF display moderate-to-loud heart
murmurs, which are related to pulmonary stenosis
or secondary tricuspid valve regurgitation. When
cyanosis is present, it can be appreciated in both
the cranial and the caudal mucous membranes.
Although rare, right-sided CHF has been repor-
ted.137 Thoracic radiographic findings include right
heart enlargement and underperfusion of the
pulmonary vasculature. The aorta often appears
more prominent and projects cranioventrally on
the lateral view, especially in cases of severe RV
outflow tract obstruction or TOF-PA. The ECG
usually demonstrates a right axis deviation due to
RV hypertrophy, but cranial and even left axis
deviations have been observed. Two-dimensional
echocardiography confirms the anatomic lesions
and Doppler studies document the presence and

Figure 12 Echocardiographic images from two cats with tetralogy of Fallot. The four components of the tetralogy
can be appreciated, including a large ventricular septal defect (arrow), infundibular pulmonary stenosis (arrowhead)
with a fused pulmonary valve (PV), right ventricular hypertrophy, and rightward malposition of the aorta (Ao), which is
seen best in Panel B. In panel D, the direction of shunt flow can be seen by color Doppler as left-to-right in systole. In
addition, subvalvular narrowing with flow turbulence in the right ventricular outflow tract is evident. The left ven-
tricle of the cat in panels A and B cat was also concentrically hypertrophied due to systolic anterior motion of the
mitral valve.
Feline congenital heart disease
direction(s) of shunting, as well as the severity of
the pulmonary stenosis (Fig. 12).
Therapeutic options
Definitive surgical correction has not been repor-
ted in the cat, and medical therapy is typically
employed. Beta-blockade might be beneficial to
prevent hypercontraction of the hypertrophied RV
and increased right-to-left shunting associated
with sympathetic activity or exercise. The authors
prefer the nonselective beta-blockader proprano-
lol, as beta-2 receptor antagonism will theoret-
ically increase systemic vascular resistance and
lessen right-to-left shunting. Chemotherapeutic
medications (such as hydroxyurea) have been used
to reduce red blood cell production if eryth-
rocytosis is extreme. While the drug can be
administered to cats, adverse effects have been
reported and efficacy data are lacking for TOF. A
safer approach might involve periodic phlebotomy,
accepting that sedation is likely required for this
procedure in cats.142 Drugs that reduce systemic
vascular resistance, such as acetylpromazine,
should probably be avoided in TOF and other right-
to-left shunts. Palliation of TOF involves diverting
a major systemic artery (classically the left sub-
clavian) to the pulmonary trunk to augment pul-
monary blood flow and reduce cyanosis. This has
been performed in dogs with success; it can also be
performed in cats, although the procedure
requires a surgeon who is skilled in microvascular
techniques.135 In cases of valvular fusion, balloon
dilation can help to reduce the degree of right-to-
left shunting and cyanosis.
Prognosis
The prognosis is guarded for cats with TOF. Survival
of >10 months after aortopulmonary shunt surgery
has been reported.135 Without surgery, natural
death at 18 months of age has been reported,134
although survival to >5 years of age has also
been described in the cat137 and observed by the
authors.
Pulmonary atresia
Morphologic lesions
Pulmonary atresia can be considered to be an
extreme form of TOF, in which the muscular outlet
septum is severely deviated toward the free wall
of the RV, with the aorta receiving nearly all of the
primitive outflow tract and the pulmonary trunk
persisting as an atretic stalk (Fig. 13). A VSD is
common in this defect, though an intact inter-
ventricular septum can also be observed in human
S35
infants. Severe cyanosis develops early in life, and
this lesion can be considered ‘ductal-dependent’,
meaning that pulmonary blood flow and, even-
tually, systemic oxygenation relies upon patency
of the ductus or other major aortopulmonary col-
lateral vessels (Fig. 13). As ductal closure usually
occurs within the first week of life, severe cyanosis
or hemodynamic collapse can develop. This defect
was formerly termed pseudotruncus arteriosus,
given the similar imaging findings with truncus
arteriosus communis; however, TOF-PA is pre-
ferred, as it reflects the comparable etiopatho-
genesis between this defect and TOF. This
condition is rare, but has been observed in a
cat.143
Diagnostic findings
The findings are comparable with TOF, but with
decompensation developing at an earlier age. On
echocardiography, it is difficult-to-impossible to
visualize the atretic pulmonary trunk or source of
pulmonary blood flow, such that a diagnosis of
truncus arteriosus also must be considered. Dem-
onstration of a ductal pulmonary blood supply,
aortopulmonary collaterals, and the atretic stalk
on CTA, MRA, angiocardiography, or post-mortem
examination is needed to confirm the diagnosis
(Fig. 13).
Therapeutic options
Open surgical repair is advised in children, but to
the authors’ knowledge has not been reported in a
veterinary species. As with TOF, a systemic-to-
pulmonary anastomosis can be considered as a
potential surgical palliation to improve pulmonary
blood flow and arterial oxygenation.
Prognosis
The prognosis is grave, although survival to 2 years
of age has been described in a dog with this
defect.144
Double outlet right ventricle
Morphologic lesions
Double outlet RV is a cardiac malformation in which
both great vessels arise entirely or predominately
from the RV. Clinical signs greatly depend on the
magnitude of pulmonary blood flow, with cyanosis
related to insufficient pulmonary flow and CHF
associated with excessive left-to-right shunting. A
great deal of controversy exists over the precise
terminology of this condition. Characteristics of
DORV are shared with TOF and TGA.145,146 As noted
in the Congenital Heart Surgery Nomenclature and
S36 B.A. Scansen et al.

Figure 13 Angiographic (A) and post-mortem (B) images from a cat with pulmonary atresia. The pulmonary blood
supply is primarily derived from major aortopulmonary collateral arteries arising from the descending aorta (arrow).
The pulmonary artery arises as an atretic stalk (arrowheads) and a large ventricular septal defect (asterisk) is present.
AAo ¼ ascending aorta; BT ¼ brachiocephalic trunk; LSA ¼ left subclavian artery; LV ¼ left ventricle.

Database Project, the degree of aortic positioning
over the RV required for the description DORV is
variable, with some authors requiring 50% of the
aorta arising from the RV, while others use a stand-
ard of 90%; others confine the term DORV to sit-
uations in which aortic-mitral fibrous continuity is
lost.147 There are several forms of DORV, which are
predominately related to the position of the inter-
ventricular communication relative to the aorta and
pulmonary trunk: the defect can be subaortic, sub-
pulmonary, doubly-committed, or remote/non-
committed.145,148 These differences have important
surgical considerations for repair, but are currently
less important in veterinary medicine because sur-
gical repair is rarely, if ever, pursued. There are at
least seven cases of feline DORV reported in the
veterinary literature.17,20,34,99,149e151 Although not
identified in all papers, both subaortic151 and sub-
pulmonic20,34,149 VSD positions have been described
in cats with DORV.
degree of pulmonary blood flow150,151; pleural
effusion may also be apparent if the cat is in CHF.20
Electrocardiographic changes have been reported
as compatible with a right axis deviation in the
frontal plane150; ventricular arrhythmias have also
been described.151 Echocardiographic reports are
limited in cats,34 but human descriptions demon-
strate the VSD, and the association of both great
vessels with the morphologic RV and taking a par-
allel course, as opposed to crossing great vessels.
The degree of pulmonary arterial obstruction and
other concomitant defects can also be established
by echocardiography and Doppler studies.
Therapeutic options
Treatment with propranolol has been described in
a cat, with fair results.34 Surgical closure of the
VSD and tunneling of the aortic outflow tract to the
LV is the preferred therapy in humans and was
successfully accomplished in a kitten.151

Diagnostic findings Prognosis

The clinical presentations reported in cats with
DORV include: fatigue and respiratory distress at
30 days of age in a Persian cat34; lethargy and
sudden death in a 1-day-old Siamese cat and a 6-
day-old Persian cat17; retarded growth and cya-
nosis in two 6-month-old kittens151; lethargy and
dyspnea in a 5-month-old cat20; and an asympto-
matic 6-month-old cat with a murmur.150 The
murmur has been described as grade 4/6-6/6 hol-
osystolic,20,34,150 or with a machinery quality.151
Thoracic radiographs reveal right-sided car-
diomegaly,150 with variable pulmonary arterial
tortuosity or hyperperfusion depending on the
The prognosis is considered to be guarded to poor,
as sudden death within days of life has been
described.17 However, survival to 3 years of age has
also been reported, prior to a fatal thromboembolic
event.34 With surgical repair, survival of >2 years
with no signs of disease has been reported.151
Transposition of the great arteries
Morphologic lesions
Transposition of the great arteries refers to ven-
triculoarterial discordance, such that the aorta
arises from the RV and the pulmonary trunk from
Feline congenital heart disease
the LV. If compared to an electrical circuit, TGA
creates two circulations in parallel, rather than in
series (the normal arrangement). Without a com-
munication between the systemic and pulmonary
circulations, such as a VSD, ASD, or PDA, circu-
latory collapse occurs shortly after birth. The
embryologic basis of TGA remains uncertain,
although new research suggests that abnormalities
in left-right lateralization and improper spiraliza-
tion of the heart during development may con-
tribute to it.152 This defect has been reported in
several calves and foals.153,154 The authors are
aware of a single feline case report describing
TGA.155 Toxicity studies also report development
of TGA in some kittens with maternal thalidomide
exposure.16
Congenitally corrected TGA is a rare defect, in
which ventriculoarterial discordance is present
along with atrioventricular discordance. Aside from
the ventricular ‘inversion’, this combination
maintains the normal series path of blood flow (RA
to LV to pulmonary trunk to lungs to pulmonary
veins to LA to RV to aorta). However, congenitally
corrected TGA is often associated with concurrent
CHD, and chronic exposure of the RV to systemic
pressures leads to tricuspid regurgitation and ven-
tricular failure. Although this lesion should be sur-
vivable beyond the neonatal period, the authors
are aware of no reports in the veterinary literature.
Diagnostic findings
In the one reported clinical case of TGA, the cat
was asymptomatic with a grade 5/6 continuous
heart murmur. Thoracic radiographs showed car-
diomegaly, pulmonary overcirculation, and mild
pulmonary edema. The diagnosis of TGA with PDA
was made by selective angiography and oximetry,
and confirmed by post-mortem examination.
Echocardiographic reports and angiographic stud-
ies in children suggest that the diagnosis can be
established if fibrous continuity between the
mitral and pulmonary valves can be demonstrated,
with a subaortic conus arising from the RV. As with
DORV, the great vessels appear to arise parallel
rather than their normal intertwined appearance.
Determination of the morphologic aorta is made by
demonstration of coronary ostia and cranial vas-
culature (e.g. brachycephalic trunk and left sub-
clavian artery), while the pulmonary trunk is
suggested by bifurcation and a sharp angulation
toward the lungs.154,156
Therapeutic options
In infants, atrial septostomy is performed to per-
mit mixing between pulmonary and systemic cir-
culations, particularly if a VSD is absent or
S37
restrictive. Definitive repair requires open-heart
surgery, the arterial switch procedure, but has not
yet been reported in an animal with TGA.
Prognosis
The prognosis without surgical repair is grave.
Without a shunt, survival beyond the first day of
life is unlikely. With a mixing lesion, survival to
several months of age may be possible, as descri-
bed in the reported case of a cat.155
Truncus arteriosus communis
Morphologic lesions
Truncus arteriosus communis (common arterial
trunk) is characterized by a single great vessel
leaving the heart through a common valve to
provide systemic, pulmonary and coronary circu-
lations (Fig. 14).157 The malformation results from
a lack of septation within the embryologic outflow
tracts resulting in no division between the aortic
and pulmonary trunks. The left and right pulmo-
nary arteries arise from the truncus at variable
locations, as classified by Collett and Edwards.158
While uncommon in animals, it seems that Type I
(single posterior pulmonary trunk) is most often
reported across species (Fig. 14). The defect has
been reported in three cats.159e161 A more useful
classification was recently proposed, in which the
truncal vessel is simply classified as having aortic
dominance or pulmonary dominance.162 This
classification avoids the paradox of previous sys-
tems, in which a heart can fit into two separate
types158; the newer classification also highlights
the most important differences dealt with during
surgical repair. Pulmonary circulation in truncus
arteriosus is reduced if there is a restriction or
stenosis at the origin of the PAs or high-resistance
pulmonary vascular disease. Pulmonary blood flow
is increased if entry into the PAs is unrestricted
because the pulmonary circulation originates
from a high-pressure vessel. Although there is
mixing of blood in the truncus, clinical signs of
cyanotic disease could potentially be reduced by
relatively higher contributions of oxygenated
blood from the LV. Conversely, marked pulmonary
overcirculation could lead to pulmonary
congestion.
Diagnostic findings
The first report of truncus in a cat described a 3-
week-old cat with signs of weakness and thoracic
deformity, and the diagnosis was made on post-
mortem evaluation.159 The more recent report
described a 6-year-old cat with a lifelong history of
S38
Figure 14 Pathologic image from a cat with Type I
truncus arteriosus communis. Note the pulmonary artery
(PA) arising from the proximal aspect of the truncus (TA)
with the descending aorta (DAo) continuing caudally.
RAA ¼ right auricular appendage. Image courtesy of
Philip R. Fox, DVM, MS, DACVIM/ECVIM, DACVECC, The
Animal Medical Center, New York, New York.
tachypnea and cyanosis and a grade 5/6 systolic
murmur on thoracic auscultation.161 Electro-
cardiography showed ventricular ectopic beats and
radiography indicated severe cardiomegaly with a
rightward shift to the cardiac apex, along with
pulmonary edema. In this case, the diagnosis was
made by echocardiography through the demon-
stration of a solitary great vessel, from which arose
a common pulmonary trunk consistent with Type I
truncus arteriosus.161 As previously discussed, the
ante mortem imaging findings in truncus arteriosus
are similar to TOF-PA; however, TOF-PA is asso-
ciated with atretic remnants of the pulmonary
annulus and pulmonary trunk, and the single vessel
exiting the heart is solely the aorta. Pulmonary
blood flow in TOF-PA is derived from a ductus
arteriosus or major aortopulmonary collaterals,
and is typically limited. Thus, the key to diagnosis
is identifying the pulmonary blood supply, which
can be a challenge without CTA, MRA, or conven-
tional angiography.
Therapeutic options
Treatment of this condition is targeted at relieving
the clinical signs that depend on the degree of
B.A. Scansen et al.
pulmonary blood flow. As indicated above, both
CHF and cyanosis can be seen. In the only reported
cat to undergo treatment, therapy for CHF and
thromboprophylaxis stabilized clinical signs for at
least 1 month after presentation. Definitive ther-
apy would require open surgical correction to close
the interventricular communication and re-route
the pulmonary arteries to the RV outflow tract.
Prognosis
In general, the prognosis is guarded, with most
humans dying in the neonatal period if surgical
correction is not performed. Interestingly, survival
to middle age has been reported in the cat, indi-
cating survival to maturity is possible in this species.
Abnormalities in the semilunar valves
Aortic valve stenosis
Morphologic lesions
Various forms of aortic stenosis (AS) have been
reported in cats, including
subvalvular,99,163,164 valvular,163 and supra-
valvular165 locations. Although the numbers are
sparse, subvalvular AS appears to be the most
common form. Pathologic reports describe fixed,
fibrotic tissue below the aortic valve and encir-
cling the LV outflow tract. This tissue may take
the form of a discrete thin band, or wide
plaque-like proliferation throughout the LV out-
flow tract. Supravalvular AS has been reported
as a thickened aortic valve with fusion of the
leaflet tips to the sinotubular junction creating a
supravalvular ring in a 1.5-year-old Siamese
cat.165 Post-stenotic dilation of the aorta can
also be present.
Diagnostic findings
Presenting complaints include asymptomatic mur-
mur evaluation, respiratory difficulty, or syncope.
Cardiac auscultation detects a loud systolic mur-
mur, reported as grade 4/6e5/6 in most cases,
with or without a gallop sound. In contrast to dogs,
the quality of the femoral pulse is reported as
normal in cats with AS.163 Left-sided cardiomegaly
is observed on thoracic radiographs, with the
development of pulmonary edema in advanced
disease. Electrocardiography is reported to show
increased voltage R waves that are consistent with
LV hypertrophy in most cases, variably tall P
waves, and possible left cranial axis deviation in
the frontal plane. Echocardiography with Doppler
confirms the diagnosis, assesses the degree of
Feline congenital heart disease
secondary LV hypertrophy, screens for con-
comitant defects (e.g. mitral regurgitation), and
provides an estimate of the systolic pressure gra-
dient across the stenosis.
Therapeutic options
Reported empirical treatments involve medical
therapy for CHF, if present, thromboprophylaxis, if
moderate-to-severe LA enlargement is identified,
or beta-blockade for the asymptomatic cat. Bal-
loon aortic valvuloplasty might be contemplated,
but has not been reported in cats.
Prognosis
The prognosis is variable and dependent on the
degree of obstruction and LV hypertrophy. Some
cats have concurrent defects that can influence the
prognosis. In severe cases, the prognosis is repor-
ted as guarded-to-poor, with a third of cats dying
within the first year of life and two-thirds of cats
dying within a year of the onset of clinical signs.163
Pulmonary valve stenosis
Morphologic lesions
Pulmonary valve stenosis is a rare disease in the
cat and, as with AS, appears to take variable
forms, with reports of valvular fusion166e169 and
subvalvular obstruction.120,170 Additional descrip-
tions of subvalvular PS are more consistent with
DCRV or primary infundibular stenosis.169,171 Eight
cases of feline PS are mentioned in a textbook, but
the details of these cases are not provided.172
Valvular fusion and PS in a snow leopard have
also been described.173 Concentric hypertrophy of
the RV is common, with variable degrees of RA
enlargement and post-stenotic dilation of the
pulmonary trunk. Cavitary effusion associated with
right-sided CHF has been observed.
Diagnostic findings
No breed predilection is described, though pure-
bred cats appear to be more common in the lit-
erature, including the Devon Rex, Abyssinian, and
Siamese breeds. Auscultation reveals a loud,
reported as grade 4/6e5/6, systolic murmur along
the left thorax. Additionally, the murmur of tri-
cuspid regurgitation, jugular venous distension,
and jugular pulsations may be appreciated. Elec-
trocardiography reveals right axis deviation
(prominent S waves in leads I, II, III) and possible
evidence of RA enlargement (P pulmonale).
Thoracic radiographs show dilation of the pulmo-
nary trunk and right heart enlargement with pos-
sible undercirculation of the pulmonary
S39
vasculature. Echocardiography with Doppler is the
diagnostic of choice and characterizes the nature
of the obstruction (valve morphology) as well as
the pressure gradient across the stenosis. Secon-
dary dynamic RV outflow tract obstruction might
also be observed.
Therapeutic options
If the cat is in CHF, medical therapy is initiated
along with paracentesis, as required. The impor-
tance of thromboprophylaxis in this condition is
unknown, but the authors prescribe clopidogrel if
severe RA enlargement is apparent. In the
asymptomatic cat, beta-blockade (atenolol) might
be prescribed to attenuate dynamic muscular
obstruction from forceful RV contraction. If valve
fusion or dysplasia is the primary lesion, definitive
therapy requires alleviation of the stenosis and
this is performed with balloon pulmonary valvulo-
plasty (Fig. 15). Reports of improvement in activity
level after balloon pulmonary valvuloplasty have
been described in cats.168,173
Prognosis
There are no studies evaluating the natural history
of PS in cats, and several of the reported cases
were euthanized at the time of diagnosis with CHF.
In general, the prognosis is considered fair to good
if a reduction in the stenotic gradient can be
achieved with balloon valvuloplasty; if CHF has
developed, the prognosis is considered guarded,
although it may be manageable for months with
medical therapy.
Abnormalities in the great arteries
Patent ductus arteriosus
Morphologic lesions
Persistent patency of the ductus arteriosus, also
referred to as the duct of Botalli, is commonly
regarded as the most frequent congenital heart
defect of dogs in North America and in Australia. It
is less commonly seen in the cat, with a variable
relative incidence, but is cumulatively appearing
as the second most common CHD in the cat (Table
1) with approximately 10% of diagnoses. However,
PDA has not been reported in other studies.174
Within the same institution, the prevalence of
PDA in the dog was reported as 4.7 per 1000 cases,
while in the cat it was 0.2 per 1000 cases.175 Ductal
patency is maintained by prostaglandins, but
within the first 12e24 h after birth the vascular
smooth muscle of the ductus begins to constrict in
S40 B.A. Scansen et al.

Figure 15 Fluoroscopic images taken during balloon pulmonary valvuloplasty in a cat with pulmonary valve stenosis
and right-sided congestive heart failure. During right ventriculography (A), a thickened and doming pulmonary valve
can be seen (arrow) together with right ventricular hypertrophy and post-stenotic dilation of the pulmonary trunk (as
well as dilatation of the caudal vena cava, without contrast). After passage of a guide wire to the branch pulmonary
artery, a balloon dilation catheter is passed across the valve and initially inflated, showing indentation from the
stenotic valve (B). With increased inflation pressure in the balloon dilation catheter, the stenotic waist has resolved (C).

response to increased oxygen tension and inhib-
ition of local autacoids. It is closed within the first
week of life. Failure of the canine ductus to close
has been shown by Buchanan and Patterson176 to
result from a deficiency of muscular tissue in the
walls of the ductus. A similar mechanism is sus-
pected in the cat. Left-to-right shunt volume var-
ies with ductal anatomy and pulmonary vascular
resistance. The development of progressive pul-
monary vascular disease is associated with PHT and
reduced velocity of left-to-right shunting on Dop-
pler echocardiography.g,24,177 Cats with PDA might
carry a greater liability for development of PHT
when compared with dogs,g,h and a more gradual
development of PHT seems typical. This is relevant
because ductal closure can still be accommodated
in some of these cases, especially if shunting
remains left-to-right. In rare cases, a bidirectional
shunt or exclusively right-to-left shunt may be
present.178 It is unknown whether these cases have
primary PHT or hypertension secondary to volume
overload.
component is apparent due to PHT. In a third of
cases, the arterial pulse in a cat with a left-to-
right PDA is hyperkinetic.f In cases of PHT, jug-
ular venous distension or ascites might be detec-
ted, even in the setting of predominately left-to-
right shunting.g,24,178 The ECG in cats with PDA
shows increased R wave amplitude associated with
LV enlargement.177,179 Cardiomegaly is typically
identified by thoracic radiography, most cases
show pulmonary overcirculation, and in severe
cases, pulmonary edema is evident.g,179 Cats with
PHT have dilated and tortuous pulmonary arteries
on thoracic radiography.24,177,178 Echocardiog-
raphy confirms LA and LV enlargement in most
cases, but with PHT, mild-to-moderate RV hyper-
trophy may be seen.f,g,24 Turbulent, high-velocity,
continuous flow can be visualized entering the
pulmonary trunk, which is often dilated. In cases
of PHT, left-to-right flow velocity is reduced,24,177
and in severe cases, the diastolic component can
disappear, explaining the lack of a continuous
murmur (Fig. 16).

Diagnostic findings Therapeutic options

The cat with PDA can be asymptomatic or show
signs of abnormal respiration, exercise intoler-
ance, lethargy, or retarded growth. In about two-
thirds of cases, auscultation is characterized by a
continuous machinery heart murmur at the dorsal
left heart base.f,g In the other third, only a systolic
g
Hildebrandt N, Schneider M, Wehner M, Schneider I. Echo-
cardiography in 9 cats with patent ductus arteriosus. Proceed-
ings of the 17th ECVIM-CA Congress, 2007, Budapest, Hungary.
h
Hitchcock LS, Lehmkuhl LB, Bonagura JD, Eyster GE. Patent
ductus arteriosus in cats: 21 cases. J Vet Intern Med
2000;14:338.
Medical therapy is the initial treatment for PDA
associated with CHF. Severe PHT might be
responsive to sildenafil if there is residual pulmo-
nary vascular reactivity. Definitive therapy is
either surgical ligation175 or transcatheter closure
(Fig. 16). Successful occlusion has been achieved
with transvenous180 or transarteriali delivery of
i
French A, Martin M, Van Israel N, Tometzki A, Wilson N.
Gianturco coil implantation for the treatment of patent ductus
arteriosus in 37 dogs and one cat. Proceedings of the 11th
ESVIM-CA Congress, 2001, Dublin, Ireland.
Feline congenital heart disease
thrombogenic coils. In the authors’ experience,
cats with PDA and PHT often have a relatively
large, tubular ductus that is difficult to treat by
transcatheter techniques. Independent of closure
technique, cats should be monitored for hypoxia
after surgery due to reactive pulmonary vascular
changes.177 In cases of moderate-to-severe PHT,
cardiac catheterization can be considered to help
optimally guide therapy by determination of pul-
monary vascular resistance and reactivity. The
ductus can usually be catheterized retrograde via
jugular venous access, and the aortic to pulmonary
artery pressure gradient measured with the cat
breathing 100% oxygen. Angiography can document
the direction of shunt flow. These findings can
identify cats that might still benefit from ductal
occlusion. An alternative, noninvasive approach
involves the measurement of left-to-right shunt
velocity before and after sildenafil treatment.
While cats with PHT and ascites have been suc-
cessfully treated by ductal closure showing reso-
lution of CHF, care must be taken prior to any
attempted closure to confirm that the PHT is due
to volume overload and is reversible rather than
fixed and irreversible. For the cat with fixed PHT,
ductal closure is not possible.
Prognosis
Without closure, severe volume overload leading
to CHF and death is likely. The prognosis after
successful shunt closure is good,180 even for cats
with reversible PHT.24,177
Aortopulmonary window
Morphologic lesions
Aortopulmonary window is a rare CHD that is
pathophysiologically comparable to PDA. Rather
than persistence of the fetal circulation, as occurs
with PDA, aortopulmonary window results from
failure to close the aortopulmonary foramen in the
developing aortic sac.181 It has alternatively been
described as a failure to close the aorticopulmo-
nary or spiral septum, although recent editorials
have suggested that the term ‘aortopulmonary
septal defect’ is anatomically inaccurate, as both
the developing aortic and pulmonary pathways
have discrete arterial walls.181 The consequence of
this failed closure is a communication between the
proximal aorta and pulmonary trunk that results in
a shunt just distal to the semilunar valves and
proximal to the origin of the ductus arteriosus.
Classification of aortopulmonary window is based
on the description of Mori et al., which includes
three types: small proximal defects (Type I), origin
S41
of the right pulmonary artery from the aortic
aspect of the window (Type II), and a large defect
that extends from the sinotubular junction to the
origin of the left pulmonary artery (Type III).182
There is probably a greater potential for develop-
ment of pulmonary vascular disease and PHT with
this defect, but too few cases have been reported
in animals to be certain. Aortopulmonary window
has been reported in a 1-year-old cat with con-
current pulmonary infundibular stenosis.170
Diagnostic findings
In the reported feline case, a continuous machi-
nery murmur at the right third intercostal space
was auscultated, and the cat was weak and cya-
notic at presentation.170 Diagnostic testing results
are comparable with PDA, with the exception of
the location of the left-to-right shunt, which is
more proximal in the great vessels than the PDA
ostium and just beyond the sinotubular junction.
The differential diagnosis should include a coro-
nary artery to PA fistula.
Therapeutic options
No successful therapies have been described in
cats with aortopulmonary window. Medical ther-
apy would be considered for CHF or for PHT, with
few options for safe closure of the defect without
open-heart surgery.
Prognosis
The prognosis in cats is considered guarded to
poor, as the only reported case died within 8 h of
presentation.170 Presumably, small shunts could be
tolerated, with large shunts leading to more rapid
development of clinical signs.
Vascular ring anomalies
Morphologic lesions
Several malformations of the arterial system have
been described in cats; the most common clinical
presentation is a kitten or juvenile cat with dys-
phagia. The basic pathophysiology of these mal-
formations is constriction of the esophagus at the
level of the heart base, dilation of the cranial
esophagus, and regurgitation (Fig. 17). To appre-
ciate the pathophysiologic alterations that lead to
a vascular ring anomaly, it is important to under-
stand the normal sequence of arterial develop-
ment. Concurrent with and following cardiac
looping, the great vessels and their principal
branches form from a set of paired arches, derived
from the aortic sac. These vessels surround the
esophagus and trachea, and connect to paired
S42 B.A. Scansen et al.

Figure 16 Echocardiographic and angiographic images from cats with patent ductus arteriosus (PDA). Panel A:
Spectral Doppler image from a 13-year-old European shorthair cat with PDA showing continuous high velocity left-to-
right ductal flow (systolic 4.5 m/sec; diastolic 3.5 m/sec) indicating normal pulmonary pressure. Panel B: Spectral
Doppler image from a 9-month-old European shorthair cat showing low-velocity left-to-right ductal flow (systolic
2.8 m/sec; diastolic 0.5 m/sec) due to severe pulmonary arterial hypertension. Panel C: Lateral angiography with a 4-
French pigtail catheter placed from the femoral vein retrograde through a PDA into the descending aorta in a 5-
month-old European shorthair cat. There is a large amount of contrast flowing through the tubular ductus into a
dilated pulmonary artery. A marker catheter is present in the esophagus for radiographic calibration.

dorsal aortae (only one dorsal aorta will persist).
Not all aortic arches are present at the same time;
they develop and atrophy at various stages of
development. There are six primary arch vessels,
with the intersegmental arteries sometimes
referred to as the seventh arch. The third arches
form the common carotid arteries and proximal
segments of the internal carotid artery. The seg-
ment of the dorsal aorta between the third and
fourth arches is called the carotid duct, which
atrophies and declines. The right fourth arch forms
the proximal segment of the right subclavian
artery, while the left fourth arch forms the defin-
itive aortic arch. The fifth arches are present in
reptiles but not in mammals. Rare reports of vas-
cular anomalies involving these arches have been
reported in humans, involving systemic-to-
systemic and systemic-to-pulmonary con-
nections.183e185 The proximal segment of the left
sixth arch forms the pulmonary trunk and the dis-
tal segment forms the ductus arteriosus. The
proximal segment of the right sixth arch forms the
proximal right pulmonary artery. The left and right
pulmonary arteries grow distally as branches from
the proximal aspects of the left and right sixth
arches. The seventh intersegmental arteries arise
from the dorsal aorta and supply blood to the
forelimbs cranial to the first rib. The paired sev-
enth intersegmental arteries are pulled forward
along the wall of the aorta by tension produced
between the cranially developing forelimbs and
first rib, and the caudal expansion of the growing
heart (known as the ‘caudal descent of the
heart’). The right fourth arch separates from the
aorta after the right seventh intersegmental
arrives and the two arterial segments form the
right subclavian artery. The left seventh inter-
segmental artery stops partway down on the arch
and remains as the left subclavian artery arising
from the left fourth arch. In the normal mammal,
all of the mature aortic arch structures lie on the
left side of the esophagus and trachea (birds nor-
mally have a right aortic arch).
The most common form of vascular ring com-
pression in dogs, and likely in cats, is persistence
of the right aortic arch (PRAA) with absence of the
left arch. Persistence of the right aortic arch has
been described in the cat (Fig. 17).186e192 A second
Feline congenital heart disease S43

Figure 17 Images from cats with persistent right aortic arch (PRAA). Panels A and B are gross pathologic specimens
of a cat with PRAA. Panel A is the left lateral perspective with cranial to the left showing the dilated cranial
esophagus, no visible aortic arch, and the constricting ligamentum (black arrow). Panel B is the right lateral per-
spective from the same cat, now with cranial structures viewed on the right, showing the right-sided aortic arch with
brachiocephalic trunk and left subclavian artery. Panel C is a ventrodorsal radiograph from a cat showing leftward
deviation of the trachea at the heart base (arrowheads) consistent with a right-sided aortic arch. Panel D is a 3-D
reconstruction from a CT angiogram in the same cat as panel C, showing the aortic arch ascending to the right of
the trachea, the dilated esophagus (asterisk), and a retroesophageal left subclavian artery (white arrow). R and L
represent the right and left sides of the cat, respectively.

form of vascular ring, often reported as the most
common form in humans, is the double aortic arch.
In this malformation, both the left and right fourth
arches remain attached to the dorsal aorta, which
causes additional constriction of the esophagus
and trachea, in addition to that caused by the
ligamentum. This anomaly has been described in
the domestic cat193 as well as a lion.194 A right
ductus arteriosus with left aortic arch is another
form of vascular ring. The normal ductus arises
from the distal left sixth arch; however, if this
segment regresses and the right sixth arch persists,
the consequence is a right ductus arteriosus or
ligamentum arteriosum. In the presence of a nor-
mal left fourth arch, this combination would cause
constriction of the esophagus and trachea. This
anomaly has also been reported in the cat.195,196
Partial tracheoesophageal compression can also
occur with aberrant subclavian artery anatomy. A
retroesophageal left subclavian artery develops
when the left seventh intersegmental artery fails
to reach the left fourth arch before the latter
separates from the dorsal aorta. The resultant
retroesophageal left subclavian artery causes
dorsal compression of the esophagus (Fig. 17) in
addition to the constriction caused by the PRAA
and left-sided ductus arteriosus or ligamentum
arteriosum. A retroesophageal right subclavian
artery, in the presence of a normal left arch, can
also cause compression of the dorsal esophagus.
This abnormality occurs when the right seventh
intersegmental artery, being pulled forward along
the dorsal aorta, fails to reach the right fourth
arch before the latter separates from the dorsal
aorta. The surgeon enters the thorax expecting to
find a typical vascular ring and discovers the aortic
arch on the left side along with a left ligamentum
arteriosum. Further dissection reveals the right
subclavian artery arising on the dorsal aorta and
crossing over the esophagus. This anomaly has
been reported in dogs197 and has been observed by
one of the authors (BAS) in a cat with regurgitation
and cranial esophageal dilation.
Diagnostic findings
The clinical history and signalment are often highly
suggestive of a vascular ring anomaly involving a
young animal with frequent regurgitation begin-
ning at the time solid food is introduced. Physical
examination might include poor body condition or
an abnormal growth pattern; a cardiac murmur is
appreciated if the anomaly includes a patent
ductus arteriosus. The work-up for these patients
is variable. A standard thoracic radiograph is sup-
portive and Buchanan198 has suggested that a
dorsoventral or ventrodorsal projection is suffi-
cient for the diagnosis in dogs, as leftward devia-
tion of the trachea at the heart base is considered
S44
pathognomonic; this is often seen in cats as well
(Fig. 17). Contrast radiography provides con-
firmatory evidence of the location of the esoph-
ageal compression and barium swallows are
frequently performed for this purpose. The
authors’ preference for confirming the diagnosis
and fully delineating the venous and arterial
anatomy is contrast-guided cross-sectional imaging
(Fig. 17).199
Therapeutic options
The treatment of choice for vascular ring anoma-
lies is surgical division or rerouting of the offending
vascular structure. In most cases, the constriction
is relieved by dividing the ductus (or ligamentum
arteriosum) via a left fourth intercostal thor-
acotomy. A thoracoscopic approach to divide the
ligamentum has also been described.200 If the cat
has a double aortic arch, the surgical approach is
to ligate and divide one of the aortic arches,
whichever is least developed. In the case of
aberrant subclavian anatomy, the offending ret-
roesophageal artery can be surgically divided and
released from the cranial border of the esophagus;
arterial perfusion to the ipsilateral limb will be
accomplished via retrograde perfusion through the
vertebral artery. Surgical intervention for a vas-
cular ring anomaly should be pursued as soon as
the cat is of sufficient size for intrathoracic surgery
and after any concurrent aspiration pneumonia has
resolved.
Prognosis
The prognosis for cats after surgical intervention
for vascular ring anomalies is generally good. Older
age at the time of intervention may lead to higher
risk for persistent esophageal dysfunction.
Coarctation of the aorta, tubular hypoplasia,
interrupted aortic arch
Morphologic lesions
Aortic coarctation is a common affliction of chil-
dren, but rarely seen in veterinary species. The
coarctation is a ridge of tissue at the aortic isthmus
e the junction of the arch and the descending
aorta e adjacent to the site of the ductus or lig-
amentum attachment and immediately distal to
the left subclavian artery. In addition to a focal
constriction, narrowing of the aortic arch may
occur over a longer segment and this is termed
tubular hypoplasia. In the most severe case, the
ascending aorta is separated from the descending
aorta, a condition referred to as an interrupted
aortic arch. Causes of coarctation, tubular
B.A. Scansen et al.
hypoplasia, and interruption are unclear, but have
been theorized to occur secondary to ectopic
ductal tissue or abnormal preductal flow.201 If
severe, the coarctation can cause poor perfusion
of the descending aorta and eventual hypertension
in the cranial limbs. Left ventricular hypertrophy is
a response and severe collateral arterial circu-
lation development can occur. A discrete coarc-
tation has been described in a 13-week-old cat
with concurrent PRAA and right ligamentum arte-
riosum.202 Tubular hypoplasia in association with
PDA has been reported in a Sumatran tiger cub.203
Although reported in the dog,204 the authors are
unaware of any cases of interrupted aortic arch in
the cat.
Diagnostic findings
In the reported feline cases, the diagnoses were
made from angiographic studies performed to
investigate a vascular ring anomaly202 or on post-
mortem examination following unobserved
death.203 In humans, findings may include a gallop
sound, systolic murmur over the spine, weak or
absent femoral pulses, or symptoms of CHF. Pelvic
limb weakness and respiratory distress have been
reported in dogs with aortic coarctation.205,206
Echocardiography is supportive or definitive for
the diagnosis in people, but was not suggestive of
this defect in the reported canine case.206 This is
likely because the optimal echocardiographic
imaging window for coarctation assessment in
humans is the suprasternal view, which is not
typically feasible in animals. The diagnosis is
optimally made by angiography or cross-sectional
imaging with CTA or MRA.201
Therapeutic options
In the only reported case of a domestic cat, no
therapy was required, as the coarctation did not
result in clinical signs. In people, therapy involves
surgical resection and anastomosis of the con-
striction, or transcatheter balloon or stent
implantation to dilate the narrowing.
Prognosis
Prognosis is variable, depending on the degree of
aortic narrowing, although limited reports of the
condition in cats results in uncertainty of the
natural history of the defect in this species.
Pulmonary arterial stenosis
Morphologic lesions
In cats, stenosis of the pulmonary trunk or branch
pulmonary arteries appears less frequently than
Feline congenital heart disease
stenosis of the pulmonary valve or infundibulum.
However, seven cases of pulmonary artery stenosis
were reported in one case series,207 and a single
case report of juxtaductal stenosis of both branch
pulmonary arteries in a 4-year-old cat has also
been described.208 This lesion comprises obstruc-
tive tissue in the lumen of the pulmonary trunk or
branch pulmonary arteries. In humans, it is
reported to occur in four forms: Type I lesions are
confined to a single constriction of the pulmonary
trunk or the right or left pulmonary arteries; Type
II lesions begin at the bifurcation of the pulmonary
trunk and extend into the ostia of the left and right
pulmonary arteries; Type III lesions involve multi-
ple segmental pulmonary arteries; and Type IV
lesions involve multiple stenoses of both periph-
eral and central pulmonary arteries.209 The lesions
reported in all cats in the initial case series were of
Type I, with a focal constriction in the pulmonary
trunk,207 while the case report of juxtaductal
narrowing was classified as Type II.208
Diagnostic findings
Six of the seven cats with focal stenosis in the
pulmonary trunk were asymptomatic at the time of
initial evaluation, with one showing reduced
activity and another developing exertional dysp-
nea later in their disease course.207 The cat with
bilateral pulmonary artery obstruction showed
recurrent dyspnea that was responsive to furo-
semide and oxygen therapy.208 On physical
examination, systolic heart murmurs were appa-
rent in all cats, basilar in location and graded 2/
6e4/6; a diastolic murmur was also apparent in
one cat. One cat in the series was reported to have
right deviation of the mean electrical axis with a
widened QRS complex on ECG. Thoracic radio-
graphs are often unremarkable with normal heart
size, although the cat with bilateral obstruction
showed generalized cardiomegaly and a dilated
caudal vena cava. Echocardiography confirms the
diagnosis by direct visualization of obstructive
tissue in the affected pulmonary vessels, with
Doppler studies being used to visualize color flow
and measure the systolic pressure gradient and
diastolic pressure half-time across the stenosis.
Therapeutic options
In the cat, no effective therapy for this condition
has been reported. However, balloon angioplasty
has proven variably effective in children with
peripheral pulmonary artery stenosis and improved
results with intravascular stent placement have
led to adoption of transcatheter stenting as the
preferred treatment option.209 This was attemp-
ted in the symptomatic cat with bilateral
S45
obstruction, but the cat died during the inter-
vention.208 As cats appear to tolerate this defect
into maturity, and given the risk of intervention
and uncertain outcome of transcatheter therapies
in this species, transcatheter intervention might
be reserved for those cases with clinical signs
referable to the pulmonary arterial stenosis.
Prognosis
The prognosis appears to be good to excellent in
many cases, with few cats developing signs refer-
able to the disease if a Type I lesion is present.207
Worsening signs may be expected with bilateral
obstruction, including development of CHF and
intermittent dyspnea.
Unilateral absence of a pulmonary artery
Morphologic lesions
Unilateral absence of a pulmonary artery (UAPA) is
a rare defect, having been reported in three
cats208,210,211; with an additional two cases seen by
one of the authors (BAS). In this form of CHD, one of
the branch pulmonary arteries is absent from birth;
in humans, this is most commonly the right pul-
monary artery.212 Embryologically, the left and
right branch pulmonary arteries derive from the
proximal portions of the left and right sixth aortic
arches. With involution of the proximal sixth arch
on either side, UAPA is created and the distal
(intrapulmonary) pulmonary arteries on that side
are supplied by the ductus arteriosus until closure
occurs in the post-natal period. Systemic-to-
pulmonary collateral vessels are common and help
to provide some perfusion to the intrapulmonary
arteries in the lung ipsilateral to the UAPA. Notably,
in at least two of the possible reported feline
cases,208,211 constriction of the origin of the con-
tralateral pulmonary artery was also reported,
suggesting that these may be more consistent with
juxtaductal pulmonary artery stenosis Type II, as
described above, rather than UAPA.
Diagnostic findings
In humans, one third of cases are asymptomatic
until adulthood, and this appears to be a frequent
observation in cats as well, with reported cases
presenting at the age of 6 months, 2 years, and 4
years.208,210,211 The two cases examined by the
author presented at 3 and 5 years of age. Clinical
signs relate to respiratory difficulty, most com-
monly exertional dyspnea and tachypnea. Non-
specific systolic murmurs are common and cyanosis
is variably present. In humans, recurrent respira-
tory infections, hemoptysis, or exercise
S46 B.A. Scansen et al.

Figure 18 Images from a cat with unilateral absence of the right pulmonary artery. Marked asymmetry in the size of
the branch pulmonary arteries is observed, with a dilated left pulmonary artery (asterisk) seen on the ventrodorsal
thoracic radiograph (A) and gross pathologic photograph (B). Also note the discrepancy in lung volume between the
right (R) and left (L) lung lobes and the complete absence of an extrapulmonary right pulmonary artery (arrow).

intolerance are the most common presenting
complaints.212 Radiography can suggest loss of lung
volume on the affected side and a mediastinal
shift, as well as marked asymmetry in the size of
the branch pulmonary arteries (Fig. 18). Inter-
stitial opacities in the parenchyma of the hyper-
perfused lung contralateral to the UAPA are
common. Echocardiography might indicate PHT
(enlargement and hypertrophy of the RV and RA,
increased tricuspid or pulmonary regurgitant
velocity), and suggest the absence of one of the
branch pulmonary arteries. Definitive diagnosis
requires angiography e either nonselective,
selective into the right heart or pulmonary trunk,
or dual-phase CTA to evaluate the pulmonary
arterial anatomy as well as any systemic-to-
pulmonary collateral vessels.
clinical signs in middle age appears to be frequent.
Management of clinical signs is possible, with
amelioration of signs for years in some cases.
Pulmonary hypertension appears to be an indicator
of a more negative prognosis.
Conclusions
Many forms of CHD have been described in cats,
and inconsistent reporting biases precise estimates
of the relative prevalence of each defect. A cat-
egorization scheme for CHD in veterinary medicine
is proposed, which aligns with the sequential seg-
mental analysis system widely adopted in pediatric
cardiology.

Therapeutic options Conflicts of interest

Therapy is symptomatic, with treatment directed
at resolution of pulmonary congestion and CHF, if
present, or toward increased pulmonary blood flow
and management of PHT. Response has been vari-
able, with some cats showing progressive respira-
tory decline and others stabilizing on medications
for more than 3 years.
Prognosis
The prognosis appears to be variable. Presentation
later in life is common, although development of
No conflict of interest is declared by any author.
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