SECTION C

Congenital Heart Disease

10
Congenital Heart Malformations

Key Points

• Congenital heart malformations occur in a small proportion of feline cardiology patients (<10% of cases).
• In general, echocardiography will be needed to diagnose the exact malformation because there is a tremendous amount of
overlap between physical examination, electrocardiographic, and radiographic findings for most malformations.
• Treatment is generally directed at controlling clinical signs that may develop due to heart failure or arrhythmias because
treatment of the primary defect is not usually possible.

INTRODUCTION malformations are patent ductus arteriosus, aortic ste-

nosis, tetralogy of Fallot, atrial septal defect, common
Congenital heart malformations are developmental atrioventricular canal, and pulmonic stenosis (Riesen
defects of the heart that are present at birth. They may et al. 2007).
be inherited or may develop spontaneously without any
known familial pattern.
In general, feline congenital heart disease is much less HISTORY AND CHIEF COMPLAINT
common than acquired heart diseases like the cardiomy- Many kittens and cats with congenital heart malforma-
opathies. Approximately 5% of cats examined by the tions are asymptomatic, but in others, clinical signs may
cardiology service at the School of Veterinary Medicine be intermittent and include exercise intolerance, syncope,
at the University of California-Davis over a 10-year and dyspnea. Animals that have progressed to the point
period were diagnosed with a congenital heart malfor- of development of congestive heart failure may show
mation (MacDonald 2006). A recent study from two other vague clinical signs including loss of appetite,
veterinary cardiology services in Switzerland identified depression, and exercise intolerance.
that 12% of the feline cardiology cases had congenital
heart disease (Riesen et al. 2007). A prospective study of
PHYSICAL EXAMINATION
circulating biomarkers in cats presented to a private
referral practice revealed a ratio of myocardial disease to In many cases, careful evaluation of a juvenile cat with
congenital heart malformations of 23:1 (Ettinger 2010). a congenital heart malformation defect will identify a
The most commonly reported feline congenital heart heart murmur and/or arrhythmia, although some cases
malformations are mitral and tricuspid valve malforma- may not have any auscultatory abnormalities. The loca-
tions and ventricular septal defects, although which tion and timing of the murmur can help to narrow the
defect is actually more common seems to vary depend- differential diagnosis list substantially (see Chapter 1).
ing on the study. Less commonly reported feline heart Cyanosis may be noted in some cases.

Feline Cardiology, First Edition. Etienne Côté, Kristin A. MacDonald, Kathryn M. Meurs, Meg M. Sleeper.
© 2011 John Wiley & Sons, Inc. Published 2011 by John Wiley & Sons, Inc.

85
 86  Section C: Congenital Heart Disease

Innocent Murmurs or as a result of medications. Periodic (2 weeks after

Young kittens (<16 weeks) can have “innocent” murmurs. starting medication, 6 weeks, 3 months) renal profiles to
These murmurs are generally soft (≤3/6), are usually monitor BUN, serum creatinine, and serum electrolyte
heard best at the left base of the heart (rib spaces 3–4) levels are also helpful for cats receiving furosemide and
and may increase or decrease in intensity or tone with an angiotensin converting enzyme inhibitor. Owners
the position of the kitten or an increase in heart rate. should be instructed to watch for the development of
depression, anorexia, or vomiting, which may suggest
Congenital Heart Disease

The presence of loud heart murmurs or heart

murmurs that persist beyond 16 weeks of age is sugges- complications with the medications or progression of
tive of a congenital heart malformation, and additional heart failure.
diagnostic tests are recommended to identify the cause Once a diagnosis is made, echocardiography is usually
of the heart murmur and the severity of the defect if one necessary only annually or if the patient’s clinical status
is present. Although information about the presence or changes dramatically and there is concern for the status
absence of congestive heart failure can be obtained from of myocardial function or development of an embolus.
thoracic radiographs, echocardiography is typically
needed to identify the actual defect. Circulating bio- ATRIOVENTRICULAR VALVE MALFORMATIONS:
markers may help in this respect: a series of 10 cats with TRICUSPID VALVE MALFORMATION
congenital heart malformations uniformly had elevated
Characteristics of tricuspid valve malformation include
plasma NT-proBNP levels compared to healthy controls
a spectrum of tricuspid valve abnormalities made up of
(Ettinger 2010). If an arrhythmia is present, an electro-
focal or diffuse thickening of the valve leaflets, underde-
cardiogram is indicated to determine the type of
velopment of the chordae tendinae and papillary
arrhythmia.
muscles, incomplete separation of the valve components
from the ventricular wall, and focal agenesis of the valve
COMPLICATIONS AND MONITORING tissue (Liu and Tilley 1976).
Some patients can remain asymptomatic and live com- Tricuspid valve dysplasia (TVD) has been recognized
fortably for years with their particular malformation. in cats both as a primary abnormality and commonly in
However, the outcome of many malformations is the coexistance with mitral valve dysplasia or ventricular
development of left and/or right heart failure (e.g., pul- septal defects in the cat (Liu 1977; Chetboul et al. 2004)
monary edema, pleural effusion, or both). Pulmonic stenosis, aortic stenosis, and endocardial
Once the initial diagnosis is made, the best method cushion defect have also been noted in some affected
for long-term monitoring of these patients with cardiac cats (Liu 1977; Kornreich and Moïse 1997; Chetboul
malformations should be carefully considered. Repeated et al. 2004).
visits to the veterinary clinic likely result in more stress
Etiology, Pathophysiology, and
for the cat with cardiac disease (as well as the owner);
Gross Pathology
therefore, it is useful to limit repeated visits to when they
are most needed. Some follow-up can be done with Etiology
simple phone communication with an owner regarding The etiology of tricuspid valve dysplasia is unknown.
the cat’s behavior at home and the owner’s records of Although there is no conclusive evidence that it is famil-
the at-rest respiratory rate. Owners may be instructed to ial, a retrospective study identified that tricuspid valve
monitor the respiratory rate at home once a day when malformations occurred significantly more frequently
the cat is in a resting state. Most resting cats have a in European short-haired cats (odds ratio = 11.37) and
respiratory rate well below 20 breaths/minute. An Chartreux cats (odds ratio = 27.24) compared to the
increase in respiratory rate may be a sign of the develop- general feline hospital population (Chetboul et al. 2004).
ment of or recurrence of pulmonary edema or pleural
effusion and may indicate the need for thoracic radio- Pathophysiology
graphs or an alteration in dosage of medication. The malformation results primarily in tricuspid valve
Some aspects of cardiac disease do require at least a regurgitation, which leads to right atrial and right ven-
brief visit to the hospital for monitoring even in the tricular dilation. Right-sided heart failure (e.g., pleural
absence of overt clinical signs. Cats with tachyarrhyth- effusion) may develop secondary to the dilated right
mias may require physical examination or an electrocar- atrium and elevated right atrial pressure. Supraven­
diogram to be sure that the heart rate is well controlled. tricular arrhythmias may develop and thromboemboli
Blood pressure monitoring may be helpful to make sure may form in the right atrium secondary to the atrial
that the patient is not hypotensive, either spontaneously dilation.
 Chapter 10: Congenital Heart Malformations  87

Gross Pathology the morphology of the tricuspid valve should be normal

Tricuspid valve dysplasia is characterized by long, thick- in a cat with ARVC.
ened septal valve leaflets, which can be focal or
diffuse (Liu and Tilley 1976). The leaflets may adhere Diagnostic Testing
directly to the septum with absent or short, abnormal Electrocardiography
chordae tendinae. The papillary muscles may be hyper-
Cats with tricuspid valve dysplasia may have a normal

Congenital Heart Disease

trophied and attach directly to the valve leaflet (Liu
sinus rhythm, a normal axis, and normal QRS morphol-
1977).
ogy. Alternatively, they may also have a splintered
(notched) QRS or evidence of right ventricular enlarge-
Signalment
ment (with a right axis shift of >160° and prominent S
There are no known breed predispositions. The breeds waves in leads I, II, III and aVF) (Liu and Tilley 1976;
reported in the literature include the domestic shorthair, Kornreich and Moïse 1997). Occasional atrial or ven-
Siamese, Birman, Persian, and Chartreux (Lord 1968; tricular premature beats have been observed (Chetboul
Liu and Tilley 1976, Fossum et al. 1994; Kornreich and et al. 2004).
Moïse 1997; Chetboul et al. 2004).

History and Chief Complaint Radiography

There is a spectrum of clinical presentations. Some Depending on the severity of the defect and the age
kittens or even adult cats are diagnosed at the time of a of the cat, the radiographs may be normal or may dem-
routine evaluation when a heart murmur is detected and onstrate signs of right-sided or generalized cardiomeg-
a thorough evaluation is performed. One cat presented aly (Chetboul et al. 2004). A dilated caudal vena cava
with exercise-induced tachypnea and two have been and pleural effusion suggest right heart failure (Figure
reported to present for syncope (Chetboul et al. 2004). 10.1).
Some cats are only diagnosed when they have progressed
to congestive heart failure and present with tachypnea Echocardiography
and dyspnea (Liu 1977). Two-dimensional echocardiography should identify the
abnormal, thickened appearance and movement of the
Physical Examination tricuspid valve and its abnormal attachment to the pap-
Cats with tricuspid valve malformation would be illary muscles or wall of the ventricle. Right atrial and
expected to have a holosystolic murmur over the 3rd– ventricular dilation can be observed as well. In milder
5th intercostal spaces on the right thorax. However, in cases, only a small amount of tricuspid regurgitation
some cases the degree of the abnormality may be so may be noted on color-flow Doppler examination as the
severe that right atrial and ventricular pressures seem to sole abnormality. Ebstein’s anomaly, a malformation of
equilibrate and the heart murmur may be very soft or the tricuspid valve where the basal attachment of the
even not detectable. Additionally some cats have mul- valve is more apically placed than normally, is another
tiple congenital cardiac malformations and have differential for the abnormal appearance of the tricuspid
murmurs that characterize their other defects. valve and may be considered as well. Ebstein’s malfor-
Cats with severe tricuspid valve dysplasia that mation is a specific type of tricuspid valve dysplasia, and
have progressed into heart failure may have distended it appears to be uncommon in the cat. Color-flow
jugular veins and tachypnea associated with pleural Doppler should indicate the presence of tricuspid valve
effusion. In rare cases, cyanosis can develop if a regurgitation in virtually all cases. Valvular stenosis
stenotic tricuspid valve or severe tricuspid regurgitation appears to be uncommon.
leads to elevated right atrial pressure and a patent A staging scheme for tricuspid valve dysplasia based
foramen ovale results in a right-to-left shunt at the on echocardiographic identification of tricuspid regur-
atrial level. gitation was suggested by Chetboul et al. (2004):

Differential Diagnosis • Stage 1: Tricuspid valve is structurally abnormal but

Cats with arrhythmogenic right ventricular cardiomy- the right atrium and right ventricle appear normal.
opathy (ARVC) can have a similar echocardiographic • Stage 2: Tricuspid valve is structurally abnormal and
appearance. However feline ARVC is generally observed right atrial dilation is present.
in older cats and should have a lesser degree of tricuspid • Stage 3: Tricuspid valve is structurally abnormal and
regurgitation noted on echocardiography. Additionally, both right atrium and ventricle are dilated.
 88  Section C: Congenital Heart Disease

cal) procedures are not typically beneficial. Medical

therapy to control signs of right heart failure or signs
caused by arrhythmias should be considered if they are
present (see Chapter 18). Additionally, because right
atrial dilation may predispose a cat to thromboembolic
disease, preventative measures should be considered in
cats with moderate or marked right atrial dilation (see
Congenital Heart Disease

Chapter 20).

Outcome and Prognosis

The prognosis for tricuspid valve dysplasia is likely
dependent on the severity of the valve malformation and
the presence of concurrent defects, and it is best deter-
A mined for each individual case based on echocardiogra-
phy. Extent of valve dysplasia, concurrent heart defects,
arrhythmias, and congestive heart failure appear to be
negative prognostic indicators in the cat (Chetboul et al.
2004).

MITRAL VALVE MALFORMATION/DYSPLASIA

Mitral valve malformation is considered to be one of the
most common congenital malformations in the cat. As
with tricuspid valve dysplasia, this defect may be
observed as an isolated malformation or with other
defects, including tricuspid valve dysplasia.
B
Etiology, Pathophysiology, and
Figure 10.1.  Radiographs from a cat with tricuspid valve mal-
Gross Pathology
formation. Note the enlarged right atrium, auricle, and right ven-
tricle on the ventrodorsal view (A). A dilated and tortuous caudal Etiology
vena cava is apparent on both views (B). The etiology of this malformation is not known,
and there is no evidence of a familial etiology at this
time.

All cats in stage 1 were asymptomatic. The staging did Pathophysiology

not correspond to murmur loudness and it has not yet
Mitral valve malformation results in mitral valve regur-
been studied to see whether stage of disease correlates
gitation, and left atrial and left ventricular enlargement.
with progression (Chetboul et al. 2004).
Elevated pressures in the left atrium can result in pul-
Tricuspid valve dysplasia has been observed to coexist
monary venous congestion and the development of pul-
with additional congenital heart malformations; there-
monary edema. Pulmonary hypertension and right
fore, a thorough echocardiographic evaluation should
ventricular failure can occur as well.
be performed.

Diagnosis Gross Pathology

The physical examination and electrocardiographic and Mitral valve dysplasia is characterized by an enlarged
radiographic findings can overlap with many other mitral valve annulus; short, thickened leaflets; short,
cardiac malformations and diseases, and therefore an stout chordae tendinae; and malposition of the papillary
echocardiogram is needed to confirm the diagnosis. muscles that can result in valve dysfunction, primarily
valve insufficiency (Liu 1977) (Figure 10.2).
Treatment
Because a dysplastic tricuspid valve is not generally ste- Signalment
notic in the cat, interventional (cardiac catheter, surgi- There are no known breed predispositions.
 Chapter 10: Congenital Heart Malformations  89

Arrhythmias including occasional atrial or ventricular

premature beats may be observed.

Radiography
Depending on the severity of the defect and the age of
the cat, the radiographs may be normal or may exhibit

Congenital Heart Disease

left-sided (atrial and/or ventricular) or generalized car-
LA
diomegaly. Evidence of heart failure with patchy pulmo-
nary edema and pulmonary venous congestion may be
observed. In some cases, pulmonary artery congestion
may be observed, suggesting the development of pulmo-
nary hypertension.

Echocardiography
Two-dimensional echocardiography should identify the
abnormal, thickened appearance and movement of the
mitral valve. Left atrial and ventricular dilation can be
observed if the degree of dysplasia and valve regurgita-
Figure 10.2.  Heart from a cat with mitral valve dysplasia. Note
the abnormal, shortened thickened mitral valve leaflets (arrow). tion is substantial. Color-flow Doppler should indicate
LA = left atrium. mitral valve regurgitation. Systolic anterior motion
of the mitral valve resulting in left ventricular outflow
tract obstruction may be present. Due to possible other
congenital defects, a thorough evaluation should be
performed.
History and Chief Complaint
Clinical presentation may include the young, apparently Diagnosis
normal kitten that presents for routine evaluation at
Although the diagnosis may be suggested based on a left
which time a murmur is detected. However, affected cats
apical or sternal murmur in a kitten or young cat, an
in which a heart murmur is initially missed or not evalu-
echocardiogram is needed to confirm the diagnosis and
ated may present later with vague clinical signs sugges-
rule out the presence of concurrent defects.
tive of the development of heart failure including
lethargy, anorexia, and dyspnea.
Treatment
Physical Examination Unless the valve is stenotic (described below), which is
rare, interventional therapy (surgery or cardiac catheter-
Cats with mitral valve dysplasia should have a holosys- ization) is not indicated. Medical therapy to control
tolic murmur over the left caudal sternal border. Mitral signs of heart failure are warranted (see Chapter 19).
valve dysplasia is sometimes identified in conjunction Additionally, cats with moderate or marked atrial
with other cardiac malformations, so heart murmurs enlargement are at risk of developing an atrial thrombus
that characterize those malformations may also be and antithrombotic therapy may be considered as
noted. Cats with severe mitral valve dysplasia that have described for tricuspid dysplasia.
progressed to congestive heart failure may be tachypneic
and tachycardic.
Outcome and Prognosis
The prognosis is dependent on several factors. The
Diagnostic Testing
severity of the defect is important, and small defects with
Electrocardiography minimal valve regurgitation are compatible with few or
The electrocardiogram of a cat with mitral valve dyspla- no clinical signs, whereas markedly dysplastic valves can
sia may have a normal sinus rhythm and a normal be associated with progression to congestive heart failure
electrical axis and QRS morphology. However, they even at a young age. The presence of concurrent defects
may also have evidence of left atrial enlargement defined may be a negative prognostic indicator, particularly if
by a widened P wave (>0.04 seconds) and/or left ven- such defects act synergistically with the hemodynamic
tricular enlargement defined by a tall R wave (>0.9 mV). disturbance created by mitral regurgitation (e.g., aortic
 90  Section C: Congenital Heart Disease

stenosis). Progression to left heart failure is also a likely Physical Examination

negative prognostic indicator, and it appears to be the Cats with primary mitral valve stenosis should have a
most likely outcome of cats with this defect if the cat low-frequency diastolic murmur or diastolic rumble
experiences clinical decompensation. caused by low-velocity turbulence of blood entering the
left ventricle through the stenotic valve. This type of
MITRAL VALVE STENOSIS murmur is often very difficult to hear due to its soft
Congenital Heart Disease

intensity, low frequency, and—in the cat—high heart

Mitral valve stenosis, a narrowed annulus of the mitral rate; concurrent pericardial or pleural effusion, or pul-
valve or subvalvular or supravalvular area, is described monary edema, makes a soft diastolic murmur virtually
much less commonly than mitral valve dysplasia in impossible to hear. It was not reported in any of the 6
the cat. Valvular or supravalvular mitral valve stenosis feline cases. However, a left apical systolic murmur may
has been reported in at least 6 cats (Fine et al. 2002; be ausculted (3 of 5 cats in which auscultation was
Stamoulis and Fox 1993; Matsuu et al. 2007; Takemura described) (Stamoulis and Fox 1993; Fine et al. 2002) if
et al. 2003). mitral regurgitation is present (n = 4 of 5 cats in which
color Doppler echocardiographic studies were reported)
Etiology and Pathophysiology (Stamoulis and Fox 1993; Fine et al. 2002; Matsuu et al.
Etiology 2007).
Mitral valve stenosis is an uncommon feline malforma-
tion. The small number of reported cases has prevented Differential Diagnosis
any studies of etiology. Supravalvular mitral valve stenosis can be confused with
cor triatriatum sinister (see below). Differentiation is
Pathophysiology based on the level of the obstruction. In supravalvular
The stenotic area at the supravalvular, valve, or subval- mitral stenosis, the left auricle is proximal to the
vular region impedes normal diastolic (filling) function, obstructing membrane, but with cor triatriatum sinister
resulting in elevated left atrial pressures, increased pul- the left auricle is distal to the dividing membrane.
monary venous congestion, and pulmonary edema. The
valve may be insufficient as well as stenotic. Diagnostic Testing
Electrocardiography
Signalment
The electrocardiogram of a cat with mitral valve stenosis
Reported cases have occurred only in adult cats, would be expected to be similar to those of mitral valve
ranging from 3 years of age (Fine et al. 2002) to 16 dysplasia and could include evidence of left atrial
years of age (Stamoulis and Fox 1993) (median  =  9 enlargement defined by a widened P wave (>0.04
years of age), leading to speculation that mitral valve seconds) and/or left ventricular enlargement defined by
stenosis may be an acquired, rather than congenital a tall R wave (>0.9 mV).
disorder in the cat (Takemura et al. 2003) as it often
is in humans. Alternatively, since the auscultatory find- Radiography
ings of mitral valve stenosis may be subtle or nonex-
istent (see below), the defect may simply escape notice Depending on the severity of the defect and the age
until congestive heart failure occurs (Stamoulis and of the cat, the radiographs may be normal or may
Fox 1993). Four domestic shorthairs (3 male, 1 female) reveal left-sided (atrial or ventricular) or generalized
and 2 Siamese cats (1 male, 1 female), all neutered, cardiomegaly. Signs of heart failure with patchy
have been reported. pulmonary edema and pulmonary venous congestion
are expected when affected cats are dyspneic. In
History and Chief Complaint some cases, pulmonary artery congestion may be
The chief complaint for all 6 cats with mitral stenosis observed as well, suggesting the development of pulmo-
was dyspnea and/or tachypnea referable to pulmonary nary hypertension.
edema, pleural effusion, or both. In 2 cats, signs of aortic
thromboembolism were also present. In a young kitten Echocardiography
or one with a mild malformation, it may be assumed Two-dimensional and M-mode echocardiography may
that overt clinical signs would not be apparent (Fine show abnormal movement of the mitral valve during
et al. 2002; Stamoulis and Fox 1993; Matsuu et al. 2007; diastole if the stenosis is at the valve level. Specifically,
Takemura et al. 2003). tethering of the valve leaflets and concordant movement
 Chapter 10: Congenital Heart Malformations  91

of the leaflets during diastole may occur. The diagnosis Etiology, Pathophysiology, and
is established via Doppler echocardiography, which Gross Pathology
demonstrates increased mitral valve inflow velocities Etiology
demonstrating the existence of a pressure gradient
across the mitral valve during diastole (normal E wave The VSD is not known to be a familial trait in the cat
velocity = 0.7 ± 0.1 m/s in the cat; see the inside covers although extensive etiology studies have not been per-
of this book), with mitral valve E wave velocities typi- formed. There are no known breed predispositions.

Congenital Heart Disease

cally >1 m/s unless severe diastolic dysfunction is also
present. In most cases (4/5), mitral valve insufficiency is Pathophysiology
also noted. In the case of supravalvular stenosis, a ste- Due to the pressure difference between the left and right
notic membrane is observed proximal to the mitral valve ventricles, blood typically shunts across the septal defect
on 2D echocardiography. from the left ventricle to the right. Most of the blood is
immediately pushed out of the right ventricle into the
Treatment pulmonary artery, to the lungs via the peripheral pul-
monary arteries, and returns to the left atrium again via
Depending on the location and severity of stenosis,
the pulmonary veins. The pulmonary arteries, veins and
balloon valvuloplasty may possibly be beneficial. This
left side of the heart (atrium and ventricle) experience
should be considered after careful consultation with a
the majority of the volume load. Left heart failure with
cardiologist. Medical therapy to control signs of heart
pulmonary artery and venous congestion may result
failure if present are warranted (see Chapter 19).
depending on the size of the defect. In some cases, the
Additionally, cats with significant atrial enlargement are
septal defect is so large that the right and left ventricular
at risk of developing an atrial thrombus, and antithrom-
pressure may almost equilibrate with the two ventricles
botic therapy may be considered as described for tricus-
largely openly connected.
pid dysplasia.
Some patients develop elevated pulmonary artery
pressure, likely due to the impact of the increased blood
Outcome and Prognosis volume on pulmonary vasculature (Eisenmenger’s phys-
Reported cats have presented at very advanced stages of iology). The elevated pulmonary pressures result in
disease and have lived between a few hours (critical elevated right ventricular pressures and may become
dyspnea at presentation) to 7 months (good response to high enough to cause a reversal of the direction of the
treatment) (Stamoulis and Fox 1993) after diagnosis. shunt, now becoming right to left. These patients may
Presumably a stenotic defect that is detected before a cat develop polycythemia and cyanosis as the deoxygenated
shows overt clinical signs carries a better prognosis, blood from the right ventricle is shunted to the left and
although physical exam limitations make such early out the aorta. Irreversible changes in the pulmonary
detection difficult as discussed above. vasculature may develop.
Because the defect is most commonly located at the
perimembranous region, directly under the aortic valve,
VENTRICULAR SEPTAL DEFECT the defect may weaken the support structure of the
aortic valve and result in aortic valve insufficiency.
A ventricular septal defect (VSD) is a common feline Finally, in a small number of patients, the ventricular
congenital malformation. It is characterized by an septal defect may become partially or completely covered
opening in the ventricular septum that occurs due to with a thin fibrous membrane, in essence closing itself
failure of the ventricular septum to complete formation. (Thomas 2005).
During ventricular septal development, the septum
develops by growing upward from the apical area of
Gross Pathology
the ventricle. Defects that result from incomplete
development of the ventricular septum are called ven- The defect is generally observed just apical to the aortic
tricular septal defects. The most common location valve within the membranous part of the basal (upper)
for a ventricular septal defect in the cat is the perimem- ventricular septum (Liu 1977). Left ventricular and
branous area. On a long-axis, two-dimensional echocar- atrial dilation may be observed.
diographic view, this area is located immediately
apical to the aortic valve in the left ventricle and adjacent Signalment
to the region of the septal tricuspid valve leaflet in the A specific breed or gender predisposition has not been
right ventricle. noted.
 92  Section C: Congenital Heart Disease

History and Chief Complaint would be expected to allow the ventricular pressures to
In many cases, the diagnosis is made after auscultation equilibrate more and a lower pressure gradient would be
of a murmur in an asymptomatic kitten or adult cat at observed.
the time of a routine evaluation. In other cases, the
diagnosis is made after a cat presents for signs consistent Treatment
with congestive heart failure including tachypnea, Some small, restricted ventricular septal defects are
Congenital Heart Disease

dyspnea, anorexia, or depression. well-tolerated and the cat may remain asymptomatic
for years. Treatment for cats with large ventricular septal
Physical Examination defects is generally thought of as surgical or medical.
Physical examination findings should include the pres- Surgical therapy could include surgical closure of the
ence of a heart murmur typically ausculted at the right defect, which would require cardiopulmonary bypass,
sternum or parasternal area (4th–5th intercostal space) something that is technically possible in a full-sized cat
as blood shunts from the left ventricle to the right. and very difficult in an immature cat with a large defect.
Sometimes a murmur of mitral regurgitation may also Interventional procedures that implant a device across the
be heard at the left apex or the sternum because the left VSD by a catheter would be technically challenging in a cat
ventricle and atrium may be dilated with volume over- because of the small body size. Interventional or surgical
load and lead to valve regurgitation. options for a kitten with a large defect should be discussed
with a cardiologist or surgeon because new techniques and
Diagnostic Testing devices are becoming increasingly available.
Electrocardiography However, because there are currently very limited surgi-
cal interventions feasible for cats with large defects (par-
Cats with a VSD may have a sinus rhythm and normal ticularly small cats), most animals that develop clinical
axis. In cases with larger defects, evidence of left ven- signs are simply managed with heart failure medication
tricular eccentric dilation may be noted based on the as described (see Chapter 19).
identification of a tall R wave (>0.9 mV) in lead II.
Prognosis
Radiography
The prognosis is largely dependent on the size of the
Depending on the severity of the defect and the age
defect. Some cats with a small VSD will remain asymp-
of the cat, the radiographs may be normal or may
tomatic for years and live a normal life; others with
have evidence of left-sided or generalized cardiomegaly.
larger defects and/or concurrent defects develop
Signs of left heart failure with pulmonary venous con-
congestive heart failure very quickly. Finally, in a small
gestion and patchy pulmonary edema may be observed.
number of patients with small defects the ventricular
Cats with large defects that are developing pulmonary
septal defect may become partially or completely
hypertension secondary to their left-sided volume
covered with a thin fibrous membrane; this outcome
overload may show evidence of pulmonary arterial
is thought to be most likely in the first year of life
hypertension with dilated peripheral pulmonary arteries
(Thomas 2005).
as well.

Echocardiography AORTIC STENOSIS

Echocardiography with Doppler is the diagnostic test of Aortic stenosis is a narrowing or stenotic region that can
choice. Two-dimensional echocardiography should occur at the subvalvular, valvular, or supravalvular level
identify the septal defect, most commonly in the peri- (Liu 1977; Stepien and Bonagura 1991; Margiocco and
membranous region immediately below the aortic valve Zini 2005).
on a long-axis 5-chamber view or on a right parasternal
short-axis view. Doppler echocardiography should be
Etiology and Pathophysiology
used to measure the pressure gradient across the defect.
If one assumes that the normal left ventricular systolic Etiology
pressure is 125 mm Hg and the right ventricle is 25 mm The etiology is unknown. A breed predisposition has not
Hg, the pressure gradient or difference between the ven- been noted, although one report described the malfor-
tricles should be approximately 80–100 mm Hg. With a mation in 2 Himalayans and 3 mixed-breed cats (Stepien
small ventricular septal defect that is “restricted” (a small and Bonagura 1991). Several early reports also described
shunt), the pressure gradient should remain in this subvalvular aortic stenosis in the Siamese cat (Liu 1977;
“normal” range (i.e., 80–100 mm Hg). Larger defects Stepien and Bonagura 1991).
 Chapter 10: Congenital Heart Malformations  93

Pathophysiology Systolic anterior motion of the mitral valve may be

The stenosis results in increased left ventricular pressure observed.
and may lead to the development of left ventricular con-
centric hypertrophy, left atrial dilation, and the develop- Treatment
ment of left-sided heart failure. Interventional therapy for aortic stenosis might be con-
sidered depending on the location of the stenosis and

Congenital Heart Disease

History and Chief Complaint other aspects of the individual case. For the many cases
The most common clinical presentations are associated that are not candidates for interventional therapy,
with the development of congestive heart failure and medical therapy is recommended with atenolol (6.25–
include tachypnea, dyspnea, and crackles (Stepien and 12.5 mg orally q 12 hours); such treatment should be
Bonagura 1991). Syncope has also been described initiated only if the cat is not in acute congestive heart
(Margiocco and Zini 2005). failure (which beta-blockade can worsen).

Physical Examination Prognosis

The heart murmur of aortic stenosis might be expected Cats with mild disease can live a normal life span. Cats
to be loudest at the left base over the aortic valve, but in with a more severe defect determined by Doppler echo-
some cases the murmur may be loudest at the sternum. cardiography are at increased risk of sudden cardiac
A gallop rhythm may be ausculted. death, or possibly the development of heart failure. Cats
that have subvalvular aortic stenosis (SAS) could pos-
Differential Diagnosis sibly be at increased risk of developing endocarditis as
in the dog, so prophylactic antibiotics might be war-
Systemic hypertension, hypertrophic cardiomyopathy,
ranted prior to surgical procedures. In the dog, SAS is
and hyperthyroidism can all cause concentric hypertro-
familial, so it should be considered that it also might be
phy of the left ventricle similar to that observed with
in the cat, particularly if a breed or genetic line appears
aortic stenosis.
to have several affected individuals.
Diagnostic Testing
ATRIAL SEPTAL DEFECT
Electrocardiography
A sinus rhythm and normal axis may be observed, but Atrial septal defects are openings between the right and
left atrial enlargement (wide P wave) or left ventricular left atrium that result from abnormal atrial septation
chamber enlargement patterns (tall R waves) may be during development. During this process, the septum
observed. R waves of 2.1 mV were observed in 1 cat forms by growing apically (downward) from the base of
(Margiocco and Zini 2005). the heart and dorsally (upward) from the endocardial
cushion region where the atrioventricular valves develop.
Radiography There are three types of atrial septal defects: sinus
venosus (uncommon; in the dorsal septal region),
Radiographs may appear to be normal or may show
septum primum (at the lower/basal septal region), and
evidence of left atrial or ventricular enlargement. A
septum secundum, which are defects at the level of the
poststenosis dilation bulge may be observed within the
foramen ovale.
aorta. Evidence of left heart failure with pulmonary
venous congestion and patchy pulmonary edema may
Etiology, Pathophysiology,
be observed.
and Gross Pathology
Echocardiography Etiology
Echocardiography from the right parasternal long-axis A specific etiology is not known. The defect may be a
view may reveal an echogenic band below the aortic spontaneous developmental abnormality. A familial
valve (subvalvular), thickened aortic valve (valvular), or nature has not been observed in the cat.
supravalvular stenosis. Secondary findings could include
hypertrophy of the left ventricular walls and papillary Pathophysiology
muscles. The left atrium may appear dilated. Continuous- Because the pressure in the right and left atria are very
wave Doppler echocardiography should be used for similar, shunting from the left atrium to the right atrium
assessing the severity of the stenosis from a position that occurs mainly due to the greater compliance of the right
is parallel to flow of the aortic outflow (see Chapter 7). ventricle and sucking of blood from the right atrium
 94  Section C: Congenital Heart Disease

RA
Congenital Heart Disease

LA

Figure 10.3.  Heart from a cat with an ostium primum atrial sep- Figure 10.4.  Right-sided long-axis 4-chamber echocardiogram
tal defect (arrow), left-sided view. The proximal attachment of the from a cat with an ostium primum atrial septal defect (arrow). The
septal leaflet of the mitral valve is seen along the ventral border location is characteristic of ostium primum defects. Echo drop-
of the defect. The left atrium (opened) appears enlarged. out artifact is unlikely due to location: typically, dropout occurs
more dorsally, in the mid-interatrial septum (in the location of the
fossa ovalis). Marked right ventricular eccentric hypertrophy and
a small amount of pericardial and pleural effusion are also pres-
ent. LA = left atrium; RA = right atrium.
into the right ventricle and, therefore, from the left
atrium to the right atrium.

Gross Pathology
A defect of the atrial septum should be noted at the top
of the septum (sinus venosus), at the foramen ovale level murmur had a significantly larger atrial septal defect
(ostium secundum), or at the level of the atrioventricu- than those without a heart murmur (Chetboul et al.
lar valves (ostium primum) (Figure 10.3). 2006).

Signalment Diagnostic Testing

A specific signalment is not known, although one study Electrocardiography
found certain breeds to be overrepresented (domestic Cats with an atrial septal defect generally have a normal
shorthair, Persian, and Chartreux) (Chetboul et al. sinus rhythm and normal axis. However, changes associ-
2004). ated with right atrial and ventricular enlargement can
be observed.
History and Chief Complaint
In the majority of cases, the atrial septal defect is an Radiography
incidental finding observed at necropsy without any Evidence of increased volume in the right atrium and
clinical signs. However, in one study 1/14 cats presented ventricle may be observed by increased chamber size and
with a history of exercise intolerance and 2/14 had an overcirculation pattern of the pulmonary vascula-
syncope (Chetboul et al. 2004). ture. With smaller atrial septal defects the radiographs
may appear normal.
Physical Examination
A heart murmur is frequently not detected. If a heart Echocardiography
murmur is present with an atrial septal defect it is typi- Echocardiography can be used to image the defect
cally ausculted at the left base of the heart. It may be a (Figure 10.4). Doppler echocardiography can be used
systolic murmur resulting from relative pulmonic steno- to identify the abnormal flow crossing the atrial
sis associated with increased blood volume (due to the septum. Diagnosis without Doppler can be challenging
interatrial shunt) crossing a normal-sized pulmonic though due to natural echo dropout of the atrial septum
valve. One study of both dogs and cats with atrial septal with two-dimensional echocardiographic imaging of
defects found that those animals with a left systolic heart this area.
 Chapter 10: Congenital Heart Malformations  95

Treatment Differential Diagnosis

Because the majority of cats with atrial septal defects The clinical, radiographic and echocardiographic find-
remain asymptomatic, treatment is not generally needed. ings of supravalvular mitral valve stenosis are very
Cats with large defects and volume overload may benefit similar to those of cor triatriatum sinister. The position
from angiotensin-converting enzyme inhibitors such as of the obstructing membrane relative to the left auricle
enalapril or benazepril. Large defects may result in the as noted on a two- dimensional echo, 4-chamber, right

Congenital Heart Disease

development of heart failure and should be treated as parasternal view may help differentiate the two. Cor
described (see Chapter 19). triatriatum should be suspected if the left auricle is
located distal to the dividing membrane and supraval-
Prognosis vular stenosis should be suspected if the left auricle is
located proximal to the dividing membrane (Fine et al.
The prognosis is typically dependent on the size of the
2002).
defect. Cats with small atrial septal defects may remain
asymptomatic for life. Diagnostic Testing
Electrocardiography
COMMON AV CANAL (ENDOCARDIAL A right axis shift was observed in one case (Koie et al.
CUSHION DEFECT) 2000).

This developmental malformation is characterized by Radiography

absence of the lower/basal atrial septum, higher/dorsal Radiographs may indicate signs of left heart failure
ventricular septum, and malformation of the atrioven- including pulmonary venous congestion and pulmo-
tricular valves (mitral and tricuspid). nary edema.

Pathophysiology Echocardiography
The resultant abnormality leads to communication Echocardiography should identify a double chamber left
between all the chambers and results in heart failure atrium with a dilated proximal chamber (Figure 10.5).
usually by 1 year of age. The atrium should appear to be divided by a membrane
with a small communication between the two chambers.
The left auricle should be associated with the distal
COR TRIATRIATUM SINISTER chamber as noted on a two-dimensional, 4-chamber,
Cor triatriatum sinister is an uncommon defect in the right parasternal view echocardiogram.
cat (Gordon et al. 1982; Wander et al. 1998; Koie et al. Treatment
2000; Heaney and Bulmer 2004). It is characterized by
the presence of a band of tissue that divides the left Kittens with cor triatriatum often present with clinical
atrium resulting in 2 left atrial chambers, which com- signs of congestive heart failure that should be addressed
municate through a small opening. One chamber as described (see Chapter 19). Surgical correction of cor
receives the pulmonary venous flow and the second
chamber communicates with the mitral valve.

Pathophysiology
Because of the small communication between the two,
obstruction of blood flow occurs between the two cham-
bers. The pressure in the proximal chamber becomes
elevated, the chamber dilates, and increased pressure is
reflected to the pulmonary veins. Left-sided heart failure
usually results.

Figure 10.5.  Right-sided long-axis 4-chamber echocardio-

Physical Examination
graphic view from a cat with cor triatriatum sinister. Note the
A heart murmur is typically detected on the left side. double chamber left atrium (LA in 2 locations, separated by the
Both systolic and diastolic murmurs have been reported partitioning membrane [arrow]), with a dilated proximal chamber.
(Wander 1998; Heaney and Bulmer 2004). LV = left ventricle; RA = right atrium; RV = right ventricle.
 96  Section C: Congenital Heart Disease

triatriatum has been reported but is challenging (Wander Radiography

et al. 1998). Right-sided enlargement is a common finding. Pleural
effusion may be observed.
Prognosis
The prognosis for cor triatriatium is generally poor, Echocardiography
with kittens generally succumbing to the signs of A fibromuscular bundle should be noted crossing the
Congenital Heart Disease

congestive heart failure. Pulmonary hypertension may right ventricle, most commonly at the midventricular
develop. level. Right ventricle hypertrophy should be noted prox-
imal to the lesion but not below it. The pulmonic valve
DOUBLE CHAMBER RIGHT VENTRICLE should appear normal. Doppler echocardiography
should reveal an increased pressure gradient across the
Double chamber right ventricle is a congenital defect
fibromuscular band (high velocity of blood flow across
characterized by the presence of anomalous muscle
the communicating aperture in the band). Flow across
bundles that spread from the septal wall of the right
the pulmonic valve should be normal. In some cases a
ventricle to the parietal wall, dividing the right ventricle.
small perimembranous ventricular septal defect was
A breed predisposition has not been noted. One report
noted as a concurrent finding (Koffas et al. 2007).
noted it in 4 domestic shorthairs, 1 domestic longhair,
and 1 each Birman, Bengal, Maine coon, and Manx
(Koffas et al. 2007). Treatment
Treatment for heart failure, if present, should be initi-
Pathophysiology ated as described (see Chapter 19). A beta blocker such
as atenolol (6.25–12.5 mg PO q 12 hours) might be ben-
The right ventricle is divided into two smaller compart-
eficial if heart failure is not present. Surgical treatment
ments by the muscle bundles. The proximal compart-
with a patch graft was successfully performed in one case
ment is exposed to higher pressure due to the obstruction
(Koffas et al. 2007). Balloon valvuloplasty was attempted
caused by the muscle bundles and may have right ven-
in one case, not successfully, likely due to the fibrous
tricular hypertrophy. The distal chamber has normal
bundles not being responsive to valvuloplasty (i.e., they
pressures.
stretch and deform rather than breaking or tearing)
(MacLean et al. 2002).
History and Chief Complaint
Reported clinical signs are fairly nonspecific and have Prognosis
included lethargy, exercise intolerance, and chylothorax,
but some cats remain asymptomatic (MacLean et al. Many cats remain asymptomatic for years, but others
2002; Koffas et al. 2007). may progress to the development of heart failure, with
chylothorax a possible outcome.
Physical Examination
A systolic heart murmur is most commonly heard on PATENT DUCTUS ARTERIOSUS
the left hemithorax, but it may be loudest on the right. Patent ductus arteriosus (PDA) is an uncommon defect
Progression of the disease can include the development in the cat. A patent ductus arteriosus occurs when the
of right heart failure, so jugular venous pulses may be embryologic ductus that allows shunting of blood
observed. between the pulmonary artery and the ascending aorta
fails to close (Figure 10.6).
Differential Diagnosis
Because pulmonic stenosis can also lead to the develop- Etiology and Pathophysiology
ment of right ventricular hypertrophy, it should be Etiology
considered.
Although the patent ductus arteriosus is well known for
being a familial trait in the dog, heritability has not been
Diagnostic Testing
demonstrated in the cat.
Electrocardiography
A sinus rhythm is expected, but a right axis shift or an Pathophysiology
arrhythmia could be observed. Atrial tachycardia has As long as the ductus is patent, blood will shunt from
been noted as well. the descending aorta to the pulmonary artery since the
 Chapter 10: Congenital Heart Malformations  97

History and Chief Complaint

In one study, the most common clinical presentation
included abnormal respiration, exercise intolerance, and
AO
poor growth (Hitchcock et al. 2000).

Physical Examination

Congenital Heart Disease

PA Although the heart murmur of a patent ductus arterio-
sus is typically continuous, in about 1/3 of the feline
cases in one study, only the systolic component of the
murmur was heard (Hitchcock et al. 2000). In some
cases, the continuous murmur is heard best ventrally.
Hyperkinetic pulses were identified in about 1/3 of
feline cases.

Diagnostic Testing
Electrocardiography
Figure 10.6.  Heart from a cat with a patent ductus arteriosus A normal sinus rhythm and normal axis may be
(PDA). The arrow indicates the patent ductus connecting the de- observed, but left ventricular chamber enlargement pat-
scending aorta (Ao) and the pulmonary artery (PA). Moderate terns may be observed.
right ventricular enlargement is also seen.

Radiography
The most commonly reported radiographic abnormal-
pressure in the aorta is naturally always higher ity is cardiomegaly. Left-sided enlargement would be
(120/80 mm Mg) than the pulmonary artery (25/12 mm expected, with pulmonary overcirculation also com-
HG). Shunting of blood occurs both though systole and monly expected. The cardiac apex may be displaced to
diastole and is, therefore, a continuous shunt. The shunt- the right.
ing of blood from the aorta to pulmonary artery results
in left-sided volume overload as the blood circulates Echocardiography
through pulmonary artery and veins, back to the left Echocardiography is needed to confirm the diagnosis.
atrium and ventricle, and through the aorta and pulmo- Doppler echocardiography should in the region of the
nary artery again. Left heart failure may develop. pulmonic valve and ductus should show continuous
Some cats develop elevated pulmonary artery pres- flow shunting into the pulmonary artery from the
sure, likely due to the impact of the increased blood ductus (Figure 10.7). It can be quite challenging to
volume on pulmonary vasculature (Eisenmenger’s phys- image the actual ductus in a cat, particularly a kitten.
iology), as described for ventricular septal defects. The Cats that develop pulmonary hypertension may show
elevated pulmonary pressures may become high enough signs of right ventricular hypertrophy.
to result in a reversal of the direction of the shunt, now
shunting from pulmonary artery to the descending
aorta. These patients may develop polycythemia and dif- Treatment
ferential cyanosis as the deoxygenated blood from the Because patients with patent ductus arteriosus may
pulmonary artery is shunted to the descending aorta. develop left heart failure and pulmonary hypertension,
Irreversible changes in the pulmonary vasculature may repair is almost always advised. Surgical ligation of the
develop. defect is most common, but transvenous embolization
with a detachable coil has also been reported in 2 cats
Signalment (Schneider and Hildebrandt 2003; Summerfield and
Specific breed predispositions or genders with increased Holt 2005). Uncommonly an older cat may present with
predisposition have not been noted. a patent ductus arteriosus that was not diagnosed at a
The patent ductus arteriosus is an uncommon defect young age. In some cases, if the patent ductus arteriosus
in the cat; therefore, specific signalment prevalences is small with little volume overload, surgical correction
have not been defined. may not be needed.
 98  Section C: Congenital Heart Disease

sented for exertional dyspnea (Schrope and Kelch

2007). Syncope and exercise intolerance could also be
expected.

Physical Examination
The heart murmur of pulmonic stenosis should be a
Congenital Heart Disease

systolic murmur ausculted loudest at the left base over

the pulmonic valve but it may radiate widely (Johnson
and Martin 2003; Schrope and Kelch 2007). Tricuspid
regurgitation associated with right ventricular hypertro-
phy and pressure overload may result in a systolic
murmur ausculted over the right hemithorax.

Differential Diagnosis
A double chambered right ventricle may produce similar
Figure 10.7.  Doppler echocardiograph from a cat with a patent clinical findings. An echocardiogram with Doppler
ductus arteriosus (PDA). Continuous flow is noted to be shunting
should differentiate the two defects.
into the pulmonary artery from the ductus.
Diagnostic Testing
Electrocardiography
A normal sinus rhythm and normal axis may be
Prognosis
observed, or a right axis shift consistent with right ven-
The prognosis for patent ductus arteriosus, particularly tricular hypertrophy may be present. Right atrial enlarge-
if diagnosed early, is very good. Once cats develop pul- ment (tall P wave) may be noted.
monary hypertension and reversal of shunting, the
prognosis becomes guarded to poor. Radiography
Radiographs may appear to be normal in many cases.
PULMONIC STENOSIS, PULMONARY Alternatively, evidence of right atrial or ventricular
ARTERY STENOSIS enlargement may be noted. A bulge due to poststenotic
Pulmonic stenosis is a narrowing or a stenosis that can dilation may be observed within the pulmonary artery.
occur at the subvalvular, valvular, or supravalvular level
(Keirstead et al. 2002; Johnson and Martin 2003). Echocardiography
Additionally, pulmonary artery stenosis, a stenosis of the Two-dimensional echocardiography may demonstrate
main or branched pulmonary artery, has also been some degree of right ventricular and/or papillary muscle
recently reported in several cats (Schrope and Kelch 2007). hypertrophy, flattening of the interventricular septum,
A breed predisposition has not been noted. These right atrial enlargement, and/or dilation of the pulmo-
defects have been reported in 1 Devon Rex, several nary artery above the stenosis. Doppler echocardio-
domestic shorthairs, and 1 Persian (Johnson and Martin graphic studies should demonstrate an increased velocity
2003; Schrope and Kelch 2007). across the stenotic area. The severity of the stenosis is
typically based on Doppler velocity across the narrowing
Pathophysiology and the calculated pressure gradient.
Pulmonic stenosis or stenosis of the pulmonary artery,
(main pulmonary artery or a peripheral pulmonary Treatment
artery) results in similar hemodynamic effects. Increased Interventional therapy for pulmonic stenosis might be
right ventricular systolic pressure resulting in right ven- considered on a case-to-case basis. Balloon valvuloplasty
tricular concentric hypertrophy, septal flattening, right has been successfully performed in a cat with pulmonic
atrial dilation, and the development of right heart failure stenosis (Johnson and Martin 2003). Surgical palliation,
can all be observed. including placement of a patch graft, might also be con-
sidered in some cases. Medical therapy with atenolol
History and Chief Complaint (6.25–12.5 mg orally q 12 hours), a beta blocker, may
Clinical signs appear to be rare, with many animals also be considered if the patient is not a candidate for
remaining asymptomatic for many years. One cat pre- valvuloplasty and is not in congestive heart failure.
 Chapter 10: Congenital Heart Malformations  99

Prognosis shunting across the ventricular septal defect (Fruganti et

The prognosis may depend on the severity of the defect, al. 2004).
but some cats appear to do quite well and remain asymp-
Additional Testing
tomatic or with only mild symptoms for some time.
Polycythemia is expected when substantial right-to-left
TETRALOGY OF FALLOT shunting of blood is present.

Congenital Heart Disease

Tetralogy of Fallot is an uncommon congenital defect in Treatment
the cat. It is characterized by a ventricular septal defect, Surgical therapy including the development of a shunt
obstruction of the right ventricular outflow tract (pul- from the systemic to the pulmonary system has been
monic stenosis), an overriding (dextropositioned) aorta successfully performed but is challenging in a cat (Miller
(arising from both ventricles), and secondary right ven- et al. 1985). Medical therapy with a beta blocker, par-
tricular hypertrophy. Shunting of blood through the ticularly propranolol (2.5–5 mg/cat PO q 8h; begin low
ventricular septal defect is typically right to left or mixed and titrate up to desired effect of preventing tachycar-
due to the obstruction of the pulmonary artery and dia) may be helpful. In this case, propranol may be more
elevated right ventricular pressures. helpful than atenolol, which is cardioselective.
Phlebotomy may be needed periodically to try to main-
History and Chief Complaint
tain the PCV below 68% if polycythemia occurs.
Affected cats have been reported to be small and possibly
stunted. They may present with dyspnea, exercise intol- Prognosis
erance, cyanosis, or syncope. Tetralogy of Fallot generally carries a poor prognosis,
though the specific prognosis depends on the combina-
Physical Examination tion of size of ventricular septal defect, degree of pul-
Cyanosis associated with a right-to-left shunting ven- monic stenosis, and degree of dextroposition of the
tricular septal defect is generally noted, but not always. aorta. Therefore, the diagnosis of tetralogy of Fallot does
A systolic murmur consistent with pulmonic stenosis not by itself indicate a definitive prognosis; the extent of
may be ausculted at the left base of the heart, but it may the primary lesions, and their effects (overt clinical
radiate generally and be heard over the right thorax signs, polycythemia, etc.) must be considered in order
as well. to provide an accurate prognosis.

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