R i g h t Ve n t r i c u l a r Fa i l u re

and Congenital Heart

Disease
Payton Kendsersky, MDa,*, Cary Ward, MDb

KEYWORDS
 Right ventricle  Congenital heart disease  Tetralogy  Transposition

KEY POINTS
 The overall mortality of ACHD patients with heart failure is significantly higher than patients with
ACHD without heart failure.
 The significance of right ventricular physiology in the development of heart failure in ACHD patients
is increasingly recognized.
 Congenital malformations, palliations, residual defects, and their resultant physiology impact the
RV through both pressure and volume loading.

INTRODUCTION and neurohormonal coupling that exists between

Survivorship into adulthood of patients with
congenital heart disease (CHD) lesions has been
due in large part to improvement in prenatal detec-
tion, novel surgeries and procedural interventions,
and the development of specialized adult CHD
(ACHD) care.1 As patients survive further into
adulthood, the long-term complications of
congenital and repaired physiology have been
more clearly elucidated. A major source of health
care use in ACHD patients is a result of the treat-
ment for heart failure.2 The overall mortality of pa-
tients with ACHD with heart failure stands at
around 4%, which is significantly higher than pa-
tients with ACHD without heart failure, even after
controlling for age and comorbidities.2 Historically,
the contribution of left ventricle (LV) dysfunction
has received more attention, owing in part to a bet-
ter understanding of its morphology and contribu-
tion to normal hemodynamics, resulting in the right
ventricular (RV) moniker “the forgotten ventricle.”3
However, it is now well-understood that the LV and
RV, which share a pericardium, septum, and
myocardial fibers, cannot be considered singu-
the 2 ventricles has subsequently provided insight
into the altered biventricular mechanics of CHD.4
Congenital malformations, palliations, residual de-
fects, and their resultant physiology impact the
RV, and this relationship influences morbidity and
mortality. LV mechanics and ventricular–
ventricular interactions are adversely affected in
both the pressure loaded and volume loaded
RVs—isolated volume overloading being associ-
ated with decreased LV ejection fraction, and
pressure overloading being associated with pre-
served LV ejection fraction.5 For this discussion,
we therefore categorize right-sided congenital
heart defects into RV pressure and volume over-
loading lesions, focusing on atrial septal defects,
Ebstein anomaly, tetralogy of Fallot (TOF), trans-
position of the great vessels, and single RV
physiology.
RIGHT VENTRICULAR VOLUME OVERLOAD
There are several congenital lesions that influence
cardiac function by overloading the RV with vol-
ume, including atrial septal defects, atrialization
cardiology.theclinics.com

larly. Growing understanding of the mechanical

of the RV (Ebstein anomaly), and corrected TOF.

a b
Duke University Medical Center, Box 2819, Durham, NC 27710, USA; Division of Cardiovascular Medicine,
Duke University Medical Center, Box 2819, Durham, NC 27710, USA
* Corresponding author.
E-mail address: payton.kendsersky@duke.edu

Cardiol Clin 38 (2020) 239–242

https://doi.org/10.1016/j.ccl.2020.02.002
0733-8651/20/Ó 2020 Elsevier Inc. All rights reserved.
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240 Kendsersky & Ward
The RV is able to tolerate an excess of volume for a
long time, mainly owing to the compliance of a
thin-walled RV, a more distensible right atrium as
compared with the left atrium, and a roomier
tricuspid valve as compared with the left-sided
mitral valve.4 Despite this adaptation of the right
heart to volume overloading, it is well-understood
that long-standing volume overload of the RV
leads to adverse ventricular interdependence and
LV dysfunction resulting from myofibril rearrange-
ment, decreased myofiber preload, and altered
chamber geometry.6
Atrial Septal Defects
An atrial septal defect is a communication be-
tween the atria resulting from a deficiency of tissue
in the septum and can be subdivided according to
anatomic location. The 3 most common types of
atrial septal defects (ASDs) include ostium secun-
dum, ostium primum, and sinus venosus. Of these
3 defects, ostium secundum is the most common
type, accounting for about 70% of all cases.7–9
Ostium secundum defects classically involve the
fossa ovalis and can be associated with anoma-
lous pulmonary venous return to the right atrium.
Defects of the septum inferior to the fossa ovalis
include ostium primum defects, which occur
immediately adjacent to the tricuspid and mitral
valves in a portion of the atrioventricular canal.
The least commonly occurring sinus venosus de-
fects, around 6% of cases, straddle an otherwise
intact septum, but represent a biatrial connection
of the superior vena cava.7 Common to all sub-
types of ASD is volume overloading of the RV, right
atrium, and pulmonary vasculature. In a small per-
centage of cases of unrepaired ASD, Eisenmenger
physiology can develop, characterized by reversal
of the intracardiac shunt, cyanosis, and RV hyper-
trophy and failure. RV failure can also develop in
unrepaired ASDs later in life, even in the absence
of Eisenmenger syndrome, as the left-to-right
shunt magnitude increases with progressive LV
stiffness associated with aging.10 In this case, it
is both volume and pressure loading that begets
right heart failure. ASDs do not close spontane-
ously, but surgical or percutaneous closure of
the ASD results in varying degrees of reversal of
the increased mortality and morbidities (ie, heart
failure, arrhythmia, and thromboembolic events)
associated with unrepaired defects. Current
guidelines recommend closure of an ASD in pa-
tients with RV enlargement, regardless of symp-
toms.11 Patients who do not undergo ASD
closure have worse long-term outcomes, including
decreased functional capacity, greater degrees of
pulmonary arterial hypertension, and more atrial
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arrhythmias.12 Studies by Kodaira and col-
leagues12 and Takaya and colleagues13 have
also demonstrated that in patients with moderate
or severe tricuspid regurgitation in the setting of
an unrepaired ASD, tricuspid regurgitation
severity, and heart failure symptoms were
improved for the vast majority of patients who un-
derwent percutaneous closure.
Ebstein Anomaly
Ebstein anomaly has a wide range of clinical pre-
sentations, but is uniformly characterized by ab-
normalities of the RV and tricuspid valve. The
anterior leaflet of the tricuspid valve is attached
normally to the annulus, whereas varying portions
of the posterior and septal leaflets are displaced
downward attaching to the RV wall below the
annulus.6,13 In other words, there is an atrialization
of the RV and varying degrees of tricuspid regurgi-
tation. Often there is an associated atrial commu-
nication in the form of a patent foramen ovale. For
patients surviving into adulthood with an Ebstein
anomaly, mortality is largely due to heart failure
and arrhythmias.13 In adulthood, a combination
of severe tricuspid regurgitation, severe right
atrium dilation, and reduced effective RV size
can result in inadequate antegrade flow and pro-
gression to RV failure. In addition to the impact
of volume overload, the natural history of RV failure
in Ebstein anomaly also owes to increased
arrhythmia burden, as RV cardiomyocytes pre-
serve ventricular specificity despite anatomic atri-
alization.14 One study confirmed an association
between myocardial fibrosis and supraventricular
arrhythmias in this population and suggests that
chronic RV volume overload contributes to the
development of said fibrosis.15
Tetralogy of Fallot
Characterized by a large ventricular septal defect
with overriding aorta, obstruction of pulmonary
blood flow, and RV hypertrophy, TOF is among
the most common cyanotic congenital cardiac de-
fects.6 Among the first lesions to be corrected sur-
gically, TOF represents a model not only of the
impact of abnormal physiology of the defect, but
also of repair physiology. Classically, infundibular
pulmonary stenosis owing to hypertrophy and de-
viation of the infundibular septum, as well as mal-
formation and stenosis of the pulmonary valve
result in obstruction of pulmonary blood flow.4
Currently, severe pulmonary regurgitation (PR) is
the most common residual lesion seen in adult-
hood after repair; significant pulmonary annular
hypoplasia requires a transannular patch, which
disrupts the valve architecture.16 Although the

surgical approach to the repair of TOF has shifted
recently to a transatrial–transpulmonary approach,
more than 60% of TOF repairs still use transannu-
lar patch enlargement according to the Society for
Thoracic Surgery database.17 Over time, the
resulting severe PR precipitates RV dilation and
tricuspid regurgitation, likely in combination with
a component of tricuspid valve trauma. Electrical
remodeling of the heart after TOF repair also im-
pacts the synchronicity and function of the RVs
and LVs.14
In a 1993 review of patients who had under-
gone repair of TOF from 1955 to 1960, 22 late
deaths were discovered: 10 of these were due
to sudden cardiac death and 3 of heart failure.
Since then, significant advances in the care of pa-
tients with repaired TOF have revealed the con-
nections between PR, RV dilation, longer QRS
duration, and ventricular arrhythmias.18 There is
now recognition that it is important to correct sig-
nificant PR before the RV dilatation and dysfunc-
tion becomes too advanced. The 2018 American
College of Cardiology/American Heart Associa-
tion Adult Congenital Heart Disease guidelines
recommend regular surveillance of the RV by car-
diac MRI to evaluate for size and RV fibrosis.11
Pulmonary valve replacement is suggested for
patients with severe PR and symptoms, as well
as for patients with severe PR and mild to moder-
ate RV dysfunction and/or severe RV dilation,
even in the absence of symptoms.11 The option
of a transcatheter pulmonary valve is also avail-
able and thousands have been implanted since
they became available in 2000.19 However, the
transcatheter pulmonary valves have specific
anatomic requirements that limit their use in a
native RV outflow tract, including a uniform diam-
eter from the RV outflow tract to the pulmonary
artery bifurcation and adequate pulmonary artery
length. They are most commonly used in dysfunc-
tional RV to pulmonary artery conduits or as a
second procedure in a preexisting but dysfunc-
tional bioprosthetic pulmonary valve.19
RIGHT VENTRICULAR PRESSURE OVERLOAD
Surgical palliation of several different forms of
CHD, including D-transposition of the great ar-
teries and hypoplastic left heart syndrome requires
reassignment of the RV from its role in the low-
resistance pulmonary circuit to supporting the
systemic circulation. The same is true for the phys-
iology of L-transposition of the great arteries or
congenitally corrected transposition of the great
arteries. These systemic RV systems represent in-
stances of RV pressure overload, leading to RV
dysfunction and failure.
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RV Failure and CHD 241
Transposition of the Great Arteries
Discordant ventriculoarterial connection underly-
ing D-transposition of the great arteries results in
displaced aorta and pulmonary artery, so that the
morphologic RV supports the systemic circulation
and the morphologic LV supports the pulmonary
circulation. These parallel circuits are incompatible
with life and require atrial or arterial switch opera-
tions.20 The long-term outcomes of the newer
arterial switch operations remain to be elucidated,
but the complications of the older atrial switch op-
erations, the Mustard and Senning procedures,
include RV dysfunction in adult survivors of the
correction.21 The RV coupled to the high-
pressure systemic circulation, leading to an
increased risk of RV dilatation and failure.22 In
these patients, there may also be a contribution
of myocardial perfusion defects and RV fibrosis.4
The role of medical therapy in a patient with sys-
temic RV dysfunction related to an atrial switch
procedure is unclear. Currently, there is not suffi-
cient evidence to recommend the use of
angiotensin-converting enzyme inhibitors, beta-
blockers, or aldosterone inhibitors in this popula-
tion. Although patients with a systemic RV are at
risk for ventricular arrhythmias, there is also not
enough evidence to recommend primary preven-
tion implantable cardioverter-defibrillators in this
population.11
Single Right Ventricle Physiology
The most common of the functional single
ventricle palliations is the Fontan procedure, which
reroutes systemic venous return directly to the pul-
monary artery, eliminating the need for a functional
pulmonary ventricle. This palliation comes at the
expense of a chronic increase in the central
venous pressure. Many of the long-term problems
associated with Fontan physiology are a result of
chronically increased central venous pressure,
including liver disease, coagulation factor abnor-
malities and resultant thromboembolic events,
and protein-losing enteropathy. In instances of hy-
poplasia or total absence of the LV, the Fontan
palliation also results in a morphologic RV sup-
porting systemic circulation.16,23 The high resis-
tance systemic circulation pressure overloads
the RV, leading to higher likelihood of RV dilation,
tricuspid regurgitation, and RV failure in these pa-
tients over time as compared with patients with a
systemic LV. As in Ebstein anomaly, in a cohort
of late Fontan survivors, myocardial fibrosis was
associated with arrhythmia and adverse ventricu-
lar mechanics.22 The medical management of pa-
tients with a single ventricle is the same regardless
of the morphology of that ventricle.11
242 Kendsersky & Ward
DISCLOSURE
The authors have nothing to disclose.
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