46
Tricuspid Valve Problems
JOHN F. KEANE AND DONALD C. FYLER
Tricuspid valve problems (a somewhat vague title that
Dr. Nadas would have likened to “a banker’s aneurysm”)
may be anatomic, physiologic, primary or secondary, alone
or in various combinations thereof, or even of no apparent
significance (the largest group in our data base of tricuspid
valve problems consists of more than 900 patients with the
sole finding of echocardiographic mild or less regurgitation;
see Chapter 19). Our most common primary anatomic abnor-
mality is Ebstein anomaly, just over half of whom had other
cardiac defects (Exhibit 46-1). Another group includes
patients with tricuspid regurgitation secondary to annular
dilatation in a variety of postoperative lesions with free
pulmonary regurgitation and/or stenosis. Although tricuspid
valve hypoplasia/stenosis/regurgitation is part and parcel of
pulmonary atresia with intact septum (see Chapter 42),
isolated tricuspid stenosis was absent in our population.
Stenosis or regurgitation due to rheumatic fever is extremely
rare in this country (see Chapter 24) and carcinoid heart
disease occurs only in adults.
As a general rule, moderate degrees of tricuspid or
pulmonary valve deformity (whether stenosis or regurgi-
tation) are well tolerated; surgery is necessary. However
if symptoms occur when both valves simultaneously are
involved, symptomatic combinations of these lesions require
surgery. Transient tricuspid incompetence does occur in
newborns: The problem is presumed to result from major
metabolic and hypoxic insults to the myocardium and usually
results in death or complete recovery.
Recognition of tricuspid incompetence depends on the
presence of a pansystolic (usually of high frequency) murmur
audible at the lower left or right sternal border, and varies
with respiration. Two-dimensional echocardiography readily
establishes the diagnosis.
Tricuspid stenosis is clinically recognizable because of
the presence of a diastolic rumbling murmur at the lower
left sternal border, seeming to occur somewhat earlier in
diastole than a mitral diastolic murmur. The diastolic murmur
of tricuspid stenosis has been observed in rare patients
with myxomas, rhabdomyomas, and extension of a Wilm
tumor up the inferior vena cava.
EBSTEIN DISEASE
Definition
Ebstein disease characteristically involves the septal and
posterior leaflets of the tricuspid valve. The leaflets are
deformed, displaced, and variably adherent to the ventricular
septum below the atrioventricular junction.
Prevalence
Ebstein disease is rare, with an incidence range from
0.012 to 0.06/1000 live births.1,2 At Children’s Hospital
Boston, 245 patients were seen between 1988 and
2002 of whom 133 had other cardiac abnormalities
(Exhibit 46-1).
Pathology
Although there is great variation in valve anatomy,3
both septal and posterior leaflets are always involved to
some degree. These leaflets are displaced toward the apex,
are adherent to the ventricular septum, and may be redun-
dant, contracted, or thickened, and even rarely atretic.
761
762 Congenital Heart Disease

Exhibit 46–1
Children’s Hospital Boston Experience
Ebstein Disease
1973–1987 1988–2002
Pt. N. Deaths Pt. N. Deaths
Ebstein, primary 75 14 112 7
Ebstein, secondary 45 133
Dx
Ventricular septal defect 4 0 12 2
Tetralogy of Fallot 4 1 16 4
Pulmonary stenosis 4 1 14 0
Malposition 4 0 12 4
Pulmonary atresia, intact ventricular septum 4 2 9 1
L-transposition 8 2 63 3
Other 17 9 63 3
TOTAL 120 29 245 24

(A) (B)

There were 245 patients with Ebstein seen 1988 to 2002 (compared to 120 from 1973 to 1987): 112 were uncomplicated
whereas Ebstein was listed as a secondary diagnosis in the other 133. In 1988 to 2002 group (a) Wolfe-Parkinson-White
syndrome was present in 36 (15%) (19% in primary 11% in secondary group), (b) overall mortality was 10% (vs. 24% for the
1973 to 1987 group), (c) only eight patients underwent tricuspid valve surgery (four plasty procedures; four replacements).
Life tables comparing survival between 1973 to 1987 and current 1988 to 2002 period, for (A) Primary Ebstein diagnosis
and (B) Ebstein as a secondary diagnosis: there has been a modest improvement in survival in both (A) and (B).

The part of the right ventricular wall proximal to the
adherent valve leaflets is described as the atrialized
portion of the right ventricle. The distal right ventricular
chamber size ranges from small to large, the average length
in one autopsy series was 0.9 that of the left ventricular
value4 whereas the right atrium is almost invariably
enlarged, sometimes grossly so (Fig. 46-1). The tricuspid
valve is usually incompetent, occasionally stenotic, with the
orifice being displaced anterosuperiorly to varying degrees.4
The proximal atrialized part of the ventricle may be paper
thin with the distal portion unaffected.
There is virtually always a patent foramen ovale or an
atrial septal defect. Pulmonary stenosis, pulmonary atresia,
tetralogy of Fallot, ventricular septal defect, and other
lesions are sometimes associated with Ebstein deformity.
When the ventricles are inverse (as in corrected transposi-
tion), the left-sided tricuspid valve may have some of the
features of Ebstein disease (see Chapter 51).
 Tricuspid Valve Problems 763

Children
After infancy, the child may or may not be cyanotic and
is usually otherwise asymptomatic. Poor growth is rarely an
issue. Older children may suffer the limitations of cyanosis
and some may develop congestive heart failure. The cardiac
impulse may be undulating. Both first and second heart
sounds may be widely split and there may be a diastolic
rumble. Triple and quadruple gallops and a systolic murmur
are common.6,7

Electrocardiography
FIGURE 46–1 Chest radiographs of two patients with Ebstein Just as the anatomic deformity varies greatly, so too does
disease showing the great variation in heart size that may be
the electrocardiographic findings, which are normal in those
encountered.
From Fyler DC (ed) Nadas’ Pediatric Cardiology, Hanley &
with the mildest involvement and very abnormal in the most
Belfus, Philadelphia 1992. severely affected. Often there is right atrial enlargement
and a bizarre right ventricular conduction delay (Fig. 46-2).
The P-R interval is sometimes prolonged and 10% to 25%
have Wolff-Parkinson-White syndrome.8 Arrhythmias are
Physiology
Whether there is tricuspid insufficiency or stenosis or
both, the practical effect is limitation of passage of blood
through the right heart. The right atrial pressure is higher
than normal, and right-to-left shunting through a patent
foramen ovale is usual. Occasionally, with minimal Ebstein
disease, there is left-to-right shunting through an atrial
defect. At birth, cyanosis may be extreme, gradually improv-
ing as pulmonary arterial resistance and right-to-left atrial
shunting decrease over the first days and weeks of life.
Attempts to define relative stenosis or regurgitation are
unsuccessful when limited amounts of blood pass through
the right ventricle, although magnetic resonance imaging
may resolve this issue. The atrialized ventricular muscle and
the remainder of the ventricle by contracting simultaneously
(a) probably disrupt forward flow of blood and (b) likely
oppose each other. It has been proposed that the distal
attachments of the tricuspid valve subdivide the right ventri-
cle into two chambers with deleterious effect on function.5
Replacement of the valve at or above the valve ring, or
valve plasty, in association with closure of the atrial opening
does help, although much of the symptomatic relief is due
to elimination of the right-to-left shunt.

Clinical Manifestations
Infants
Infants with Ebstein disease are recognized because
of cyanosis in the first days of life, the diagnosis in some
being made by echocardiography even before birth.
Cyanosis may vary from slight to severe and may be asso- FIGURE 46–2 An electrocardiogram from a patient with Ebstein
ciated with tachypnea in direct proportion to the degree of disease. Note the large and bizarre P-waves and the wide and
cyanosis. The liver may be enlarged. There are no charac- bizarre QRS complexes.
teristic auscultatory findings and often there is no systolic From Fyler DC (ed) Nadas’ Pediatric Cardiology, Hanley &
murmur. Belfus, Philadelphia 1992.
764 Congenital Heart Disease
FIGURE 46–3 Apical echocardiogram in Ebstein malformation
of the tricuspid valve. The displacement of the tricuspid valve
(arrows) into the right ventricle (RV) is apparent. Note that this
is a systolic frame (the mitral valve is closed) indicating that the
size of the atrialized RV is nearly the same as the size of the left
ventricle (LV).
common, increase with age, and include supraventricular
tachycardia, atrial flutter, and atrial fibrillation.7,9 Accessory
conducting pathways when present are single in most
(62%) and located in the right free wall, are right septal
A B
FIGURE 46–4 Magnetic resonance images from patient with Ebstein anomaly showing (A) in four-chamber view, dilated right
atrium (RA) apically displaced tricuspid valve (arrows) into right ventricle (RV) and (B) short-axis view, dilated RV much larger
than left ventricle (LV) in this view (courtesy of Dr. T. Geva).
in 34%, atrioventricular nodal in location in a few, and
multiple in 29%.10
Echocardiography
Using two-dimensional echocardiography, the degree of
tricuspid valve deformity and right heart chamber sizes are
readily seen and quantified (Fig. 46-3).11 Non-Ebstein lesions
causing regurgitation such as tricuspid valve dysplasia or
prolapse or right ventricular dysplasia can usually be easily
differentiated.12 The degree of regurgitation and or steno-
sis is also readily evident on Doppler color flow and pulsed
Doppler.
Magnetic Resonance Imaging
Magnetic resonance imaging, if necessary, provides
excellent anatomic details and also functional information
(Fig. 46-4).
Cardiac Catheterization
Because the two-dimensional echocardiographic and
Doppler details are so informative, catheterization for
anatomy and function has been unnecessary for some
decades. Indications for catheterization are (a) electro-
physiologic, namely to study arrhythmias and ablate if
feasible underlying accessory pathways10; and (b) to device
 Tricuspid Valve Problems
close atrial septal defects if temporary balloon occlusion is
hemodynamically tolerated and especially if a significant
left-to-right shunt is present.
Management
In the newborn period, all efforts are made to support
the infant through the period of transitional circulation,
including prostaglandins and/or nitric oxide to increase
pulmonary blood flow when the patient is dangerously
cyanotic. Oxygen and correction of anemia may be helpful
for those severely ill. Complex surgical procedures, includ-
ing tricuspid valve plasty, atrial reduction and other defect
repair and even tricuspid or pulmonary closure with a shunt,
in small recent series seem to have some promise,13–15
although in one large group over some decades mortality
was some 47%.16
Older children generally can be followed medically.
Rhythm problems however are common (≈30%), most are
due to accessory connections (single or multiple), some are
considered life threatening, and indeed sudden death is
not uncommon.17 Ablation of such pathways at catheteri-
zation is feasible in about 80% although recurrences are
encountered in almost 30%.10 Surgery is generally reserved
for patients who are very symptomatic, deeply cyanosed, in
heart failure, or with other associated anomalies. Increasing
numbers are undergoing valve plasty (many with wall
plication in addition, some including bidirectional Glenn
procedures) with survival rates of 82% at 20 years.18–20
In a large series of valve replacement only, at 10 years
survival was 93% and freedom from reoperation 81%.21
Some patients have undergone bypass tract ablation at the
time of surgical valve plasty with a satisfactory outcome to
date.22,23
Cyanosis, particularly if it is marked, or the appearance
of congestive failure is ominous. The earlier these symptoms
appear, the more likely life expectancy will be limited.
Course
Because modern echocardiographic techniques have
revealed patients who formerly would have been unrecog-
nized, a cadre of patients with mild forms of Ebstein disease
has been added to the patient pool. These patients are
expected to have a nearly normal life expectancy: Indeed
survival at age 87 years has been reported.24 Nevertheless,
most of those who survive infancy are likely to have serious
difficulties within a few decades, with survival rates of 59%
at 10 years in one large series,17 81% in another, 25 and 89%
of medically managed patients at 14 years in yet another.9
The predictors that we and others feel are indicators
of a poor outcome include heart failure, deterioration,
cyanosis, other defects, and arrhythmias25; in the neonate
765
echocardiographic right atrial and atrialized size exceeding
that of the functional right ventricle and left heart cham-
bers has been reported as ominous.16 Brain abscess and
endocarditis remain a threat.
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766 Congenital Heart Disease
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