Download Corrected Transposition of the Great Vessels without Associated

Corrected Transposition of the Great Vessels
without Associated Anomalies: Report of a
Case with Congestive Failure at Age 45'
John P. Nagle, LTC, MC, USA; Melvin D. Cheitlin, Col, MC, USA, and
Richard 1. McCarty, LTC, MC, USA
The clinical course and laboratory findings in the case of a 45-year-old woman
with corrected transposition of the great vessels and no associated defects are
reported. Spontaneous systemic ventricular failure occurred at the age of 45
without obvious cause. The possibility is raised that this represents failure of the
anatomic right ventricle to sustain systemic pressures for a oormal lifetime. This
patient is discussed in relation to present surgical techniques for correcting
transposition of the great vessels which leave the right ventricle as the systemic
pump.
I
woman with uncomplicated corrected transposition
of the great vessels.
'From the Cardiology Service, Department of Medicine,
Letterman General Hospital, San Francisco.
A 45-year-old. white woman enjoyed good health until
April 1968 at which time she developed shortness of breath
on exertion, orthopnea, increasing fatigability with cough in
the supine position, and paroxysmal nocturnal dyspnea. There
were no symptoms of chest pain. She was in overt left-sided
(anatomic right ventricle) congestive failure with a sinus
tachycardia, presystolic and protodiastolic gallops, a holosystolic regurgitant apical murmur of left-sided atrioventricular
valve insufficiency. Chest roentgenograms confirmed cardiomegaly and pulmonary congestion (Fig 1). This patient
made a full recovery after treatment with bed rest, diuretics
and digitalis. The gallop sounds and apical regurgitant murmur disappeared with cardiac compensation. She was then
referred to Letterman General Hospital for further evaluation.
At the time of evaluation at Letterman General Hospital in
August 1968, she was asymptomatic on digitalis. She was
normal appearing and acyanotic, with a blood pressure of
130/80 mm Hg, and a regular pulse of 76 beats per minute.
Jugular venous pulsations were normal and lung fields were
clear to auscultation. Cardiac examination revealed no abnormal heaves or thrills. The point of maximum impulse was
in the midclavicular line in the fifth intercostal space. The
first sound was of normal intensity. The second sound was
single and loudest in the left second interspace. There was a
short grade II/VI systolic ejection murmur at the lower left
sternal border which did not transmit well in any direction.
There were no diastolic murmurs or gallop sounds. The
remainder of the examination was unremarkable.
solated congenital corrected transposition of the
great vessels is an uncommon anomaly that
results in normal circulating pathways. Although
several types of corrected transposition are theoretically possible, the form of transposition with bulboventricular inversion represents the "classical" form
of corrected transposition in the individual with
situs solitus and is the only type of corrected
transposition with a significant clinical incidence.'
The majority of reported cases of congenital
corrected transposition of the great vessels have had
one or more associated defects.2-fl In addition,
interference with atrioventricular conduction resulting in all degrees of AV block has been a very
common accompaniment with or without other
defects.4~12
Much less frequent is the finding of congenitally
corrected transposition of the great vessels without
significant associated defects.12 The actual incidence of this anomaly is unknown as it does not
frequently cause symptomatology, and, therefore,
an indeterminate number of unrecognized cases
undoubtedly exist.
The following case documents the onset of systemic ventricular failure (anatomic right ventricle)
without an obvious inciting cause in a 45-year-old
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NAGLE, CHElTLlN AND MC CARTY
LAChest roentgenogram revealed a normal cardiac silhouette
and clear lung fields (Fig 1). Cardiac series with barium
swallow revealed no cardiac chamber enlargement and the
barium outlined a normally positioned left-sided stomach.
Electrocardiogram showed extreme left axis deviation with
the initial forces abnormally leftward and posterior (Fig
2).
Diagnostic cardiac catheterization was performed. Catheter passage into the pulmonary artery was medial to the
normal position of the pulmonary outflow tract and actually
overlaid the spine. Right heart pressures and pulmonary
wedge pressures were normal at rest and with exercise in the
supine legs-up position. Retrograde left heart catheterization
was performed, and there were normal left-sided pressures
with no gradient across the aortic valve. The left heart
catheter demonstrated that the aorta was positioned to the
left of the pulmonary artery anterior to its normal position
(Fig 3). Left ventricular cineangiogram demonstrated no
evidence of atrioventricular valve incompetence or left-toright shunting from a ventricular septal defect. The systemic
left-sided ventricle had the configuration of a trabeculated
chamber with an infundibulum which is usually characteristic of an anatomic right ventricle. The aorta arose to the left
of the pulmonary artery anterior to its normal position and
approximately side by side with the pulmonary artery. Selective coronary arteriography, performed by the Judkins'13 tech-
FIGURE
1. A and B top. X-ray
films demonstrating cardiomegaly and pulmonary congestion
during systemic or "left" ventricular failure in April 1968. C and
D bottom. Posterior-anterior and
left lateral chest x-rays at time
of cardiac catheterization in
November 1968. ( I t is noteworthy that the characteristic
sloping left heart border of congenital corrected transposition is
not present in this case.)
nique, showed widely patent and unobstn~ctedvessels ruling
out angiographically demonstrable occlusive disease. The major coronary artery arose posteriorly and to the left, and
branched into three main vessels. One vessel was directed posteriorly along the atrioventricular groove and subsequently
gave rise to the posterior descending vessels, supplying the diaphragmatic surface of the heart. The other major branch of the
left coronary artery branched into three vessels which supplied
various aspects of the systemic ventricle. The second major
artery, or right coronary artery, was not entered selectively,
but filled adequately on systemic ventricular and aortic root
angiograms. This vessel arose to the right and anteriorly and
supplied the anterior iurface of the heart. It was a much smaller vessel as compared to the left and was unobstructed.
In summary, the catheterization revealed corrected transposition with bulboventricular inversion in situs solitus and no
associated defects. At the time of catherization, the patient
was well-compensated with no evidence of abnormal pressures.
Subsequent to this study the patient remained compensated on digitalis and diuretics for over a year but has
recently redeveloped evidence of mild left-sided failure. In
addition, several episodes of paroxysmal atrial tachycardia
have been documented and are presently controlled on quinidine.
CHEST, VOL. 60, NO. 4, OCTOBER 1971
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CORRECTED TRANSPOSITION OF GREAT VESSEL.S WITHOUT ASSOCIATED ANOMALl ES
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FIGURE2. Electrocardiogram of a 45-year-old woman with
uncomplicated corrected transposition of the great vessels
demonstrating left axis deviation and altered initial forces
which are directed leftward and posteriorly.
The preceding case documents the spontaneous
onset of systemic ventricular failure (anatomic right
ventricle) during the natural course of uncomplicated congenital corrected transposition. No incitjng cause such as myocarditis, coronary atheroscler-
FIGURE3. Postero-anterior projection of heart with arterial
catheter retrograde from aorta into systemic ventricle (large
arrow) and venous catheter from superior vena cava through
right ah-iom, venous ventricle and into left pulmonary artery
( s d arrou:). This demonstrates the aorta to the left of the
pulmonary artery and the medial position of the venous ventricular out8ow tract.
osis, valvular defects, or arrhythmias could be found
to explain this break in cardiac compensation.
Complete transposition of the great vessels as
opposed to corrected-transposition, accounts for
about 8 percent of all congenital cardiac defects
recognized at birth and 90 percent of these children
die by the age of one if no surgical intervention is
undertaken.14 A multitude of surgical procedures
have been attempted at both palliating and correcting this lesion. Of the "corrective" procedures, the
Mustard procedure, an intra-atrial pericardial baffle
redirects arterialized pulmonary venous return to
the tricuspid valve, right ventricle and hence the
aorta. The systemic venous return is directed to the
mitral valve, left ventricle and to the pulmonary
artery. This results in physiologic correction of
blood flow but does not correct the basic anatomy
and the right ventricle remains the systemic pump.
This procedure has gained wide application with an
acceptable surgical mortality rate.15
The question to be answered is whether or not
the right ventricle can maintain its job as a systemic
pump indefinitely. The naturally occurring counterpart of the surgically corrected transposition of the
great vessels is the congenital defect demonstrated
by the present case, namely, that of congenital
corrected transposition of the great vessels due to
bulboventricular inversion. In this defect, the anatomic right ventricle is the systemic pump. Greater
than 90 percent of the reported cases of corrected
transposition have associated defects and are not
analogous to this situation. However, there are a
small number of cases with isolated corrected
transposition reported where the natural history
might allow spme insight to the durability of the
right ventricle as the systemic pump.12
It has been claimed that when corrected transposition is not associated with other anomalies, a fairly
normal longevity is to be expected. In a few
reported cases the patients have lived into the fifth
and sixth decade. One patient, age 60, in a series
reported by Schiebler and associates,4 had an
isolated corrected transposition which was an incidental finding at autopsy. A 73-year-old man reported by Lieberson and co-workers16 had associated mild left atrioventricular and aortic regurgitation. Moss and colleagues17 have recently reported a case of uncomplicated corrected transposition in a 54-year-old man who had exertional
breathlessness and nodal tachyarrythmia and, although exercise pressures are not mentioned, he
apparently had normal hemodynamics when he was
studied. These cases point out that in certain
instances, the anatomic right ventricle can act as a
CHEST, VOL. 60, NO. 4, OCTOBER 1971
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NAGLE, CHElTLlN AND MCCARTY
systemic pump for long periods, even in the presence of valvular incompetence. However, these
cases may indeed be the exception. Lieberson and
associates"' in reviewing previously reported cases
of corrected transposition, could find only six persons who had survived to age 40.
Of the reported cases of corrected transposition
that are alleged to be uncomplicated, the majority
have had either varying degrees of left AV valvular
insufficiency, complete heart block, or both. l 2 With
the onset of complete heart block, congestive failure
is often superimposed and may be the cause of
death. Berben and Adicoffls have reported two
cases of corrected transposition without associated
defects who developed complete heart block in
their mid 30's. In one of these cases, despite an
artificial pacemaker, an inexorable course of congestive heart failure ensued over the next 1%years
and finally death. This suggests that the systemic
ventricle was unable to handle the pressure load for
a sustained period of time.
The present case was not associated with either
mitral insufficiency or complete heart block and yet
the patient developed systemic or "left" ventricular
failure without an inciting cause. It is conceivable
that the patient's congestive failure was precipitated
by unrecognized myocarditis or arrhythmia, but the
possibility exists that the congestive failure was due
to the inability of the anatomic right ventricle to
sustain the pressure load. If so, this certainly is
important knowledge for the cardiac surgeon who,
in performing corrective or palliative procedures for
complete transposition of the great vessels, has
assumed that the right ventricle can sustain.systemic pressures for a long period of time. While it is
tempting to talk of the Mustard procedure as being
a "total correction," it should be remembered that
the correction is physiologic only. Whether or not
this is a truly corrective procedure or just another,
albeit highly successful, palliative procedure depends largely upon whether the right ventricle can
maintain a systemic workload for a normal life
span. It has been said that subtle differences
between the ventricles fit them for their respective
high and low pressure loads. In lesions where the
RV functions at high pressure and high resistance,
life expectancy has been greatly shortened with but
few exceptions.
1 Paul MH, Van Praagh S, Van Praagh R: Corrected
transposition of the great arteries, Chap 41, in Paediatric
Cardiology, by Watson, H. St Louis, C. V. Mosby Co.,
1968
2 Anselmi G, Munoz S, Machado I, et al: Complex cardiovascular malformations associated with corrected type of
transposition of the great vessels. Amer Heart J 66:614,
1963
3 Honey M: The diagnosis of corrected transposition of the
great vessels. Brit Heart J 25:313, 1963
4 Schiebler GL, Edwards JE, Burchell HB, et al: Congenital corrected transposition of the great vessels. A study of
33 cases. Pediatrics 27:851, 1961
5 Morgan AD, Drovetz LJ, Bartley TD, et al: Clinical
features of single ventricle with congenitally corrected
transposition. Amer J Cardiol 17:379, 1966
6 Anderson RC, Lillehei CW,Lester RG: Corrected transposition of the great vessels of the heart. Review of 17
cases. Pediatrics 20:626, 1957
7 Helmholz H F Jr, Daugherty GW, Edwards JE: Cardiac
Clinic CXLV. Congenital "mitral" insufficiency in association with corrected transposition of the great vessels:
report of probable clinical case and review of six cases
studied pathologically. Mayo Clin Proc 31 :82, 1956
8 Hallman CL, Gill SF, Bloodwell RD, et al: Surgical
treatment of cardiac defects associated with corrected
transposition of the great vessels. Circulation (Suppl 1 )
35 and 36: 133, 1967
9 Walker WJ, Cooley DA, McNamara DG, et al: Corrected
transposition of the great vessels, atrioventricular heart
block and ventricular septa1 defect. A clinical triad. Circulation 17:249, 1958
10 Verry WB, Roberts WC, Morrow AG, et al: Corrected
transposition of aorta and pulmonary trunk. Amer J Med
36:35,1964
11 Cardell BS: Corrected transposition of the great vessels.
Brit Heart J 18: 186, 1956
12 Rotem CE, Hultgren HN: Corrected transposition of the
great vessels without associated defects. Amer Heart J
70:305, 1965
13 Judkins MP: Percutaneous transfemoral selective coronary arteriography. Radiol Clin N Amer 6:467, 1968
14 Campbell M: The incidence and later distribution of
malformations of the heart, Chap 5, in Paediatric Cardiology, by Watson, H. St Louis, C. V. Mosby Co., 1968
15 Ankeney JL, O'Grady TJO: The treatment of transposition of the great vessels. Ann Thorac Surg 5:262, 1968
16 Lieberson AD, Schumacher RR, Childress RH, et al:
Corrected transposition of the great vessels in a 73 year
old man. Circulation 39:96, 1969
17 Moss AJ, Hutter AM, Lipchick EO, et al: Congenital
corrected transposition of the great vessels without cardiac anomalies. Amer J Med 47:986, 1969
18 Berman DA, Adicoff A: Corrected transposition of the
great arteries causing complete heart block in an adult.
Amer J Cardiol24: 125, 1969
Reprint requests: LTC Nagle, Box 137, Letterman General
Hospital, San Francisco 94129
CHEST, VOL. 60, NO. 4, OCTOBER 1971
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