Download Congenital Corrected Transposition of the Great Vessels in a 58

Congenital Corrected Transposition of the
Great Vessels in a 58-Year-Old Man*
A. Benchimol, M.D., S. T w , M.D., and V . Sundararaian, M.B.B.S.
This is the case report of a 58-year-old living man with uncomplicated congenital
corrected transposition of the great vessels proved by cardiac catheterization and
selective cineangiocardiography. Only six patients (3 percent) have lived beyond
the age of 40 years despite the theoretic speculation of a normal life span.
Selective coronary ateriograms revealed that the left coronary artery supplied
the anatomic right ventricle, apd the right coronary artery the anatomic left
ventricle.
he term, congenital corrected transposition of
Tthe great vessels, was introduced by Schiebler
and associates' in 1981 to differentiate from surgically corrected transposition of the great vessels
which can be accomplished after a Mustard procedure.
The true incidence of congenital corrected transposition of the great vessels is not known. Fontana
and Edwards* reported this condition to occur in
1.4 percent of 357 specimens of congenital cardiac
disease. More than 200 cases have been reported
since the first clear anatomic description by Rokitansky in 1875.334 Uncomplicated congenital corrected transposition is rarely recognized in life
because of normal cardiac hemodynamics, while the
majority of the cases with associated intracardiac
defects are recognized in early childhood.3-598
There were only 18 patients who were older than 20
years in several series totaling more than 100
reported cases."." There are six patients who have
reached the age of 40 years, the oldest patient being
a 73-year-old man.b.0
This paper reports the case of a 58-year-old man
with congenital corrected transposition of the great
vessels without any associated intracardiac anomalies.
CASEREPORT
A 58-year-old man, father of five living children, was
admitted to Good Samaritan Hos~italon March 15. 1970.
'From the Institute for Cardiovascular Diseases, Good Samaritan Hospital, Phoenix, Arizona. Supported in part by Nichols' \Ien~orialFund.
with a history of dizzy spells for the past 14 years. These
spells were increasing in number and severity two months
prior to admission. A heart murmur was noted to be present
for a number of years associated with the "electrocardiographic abnormalities." The patient was informed that he
had narrowing of one of the heart valves. His dizzy spells
have been associated with diaphoresis and mild dyspnea
without any definite chest pain. There was no history of
syncopal episodes, paroxysmal nocturnal dyspnea, orthopnea,
or angina pectoris. The dizziness was precipitated by exercise
and relieved by rest of 10 to 20 minutes. There was no history
of rheumatic fever, and the family history was negative for
congenital heart disease.
Physical examination revealed a healthy-appearing white
man who appeared slightly younger than his stated age of 58
years. There was no cyanosis, clubbing of fingers or peripheral edema. The blood pressure was 130/84 on both arms.
Pulse rate was 82 per minute with frequent irregularities due
to extrasystoles. The neck veins showed normal "a" and "v"
waves, and they were not distended. The lungs were clear to
auscultation. The precordium was quiet and there were no
thrills. The first heart sound was normal at the mitral area.
The second heart sound was single and accentuated at the
pulmonic area. There was a soft grade II/VI systolic ejection
murmur best heard at the second left intercostal space, at the
sternal border ( Fig 1 ) A fourth heart sound was heard over
the mitral area. The abdomen was soft, and the liver and
spleen were not enlarged. All peripheral pulses were normal.
The electrocardiogram and vectorcardiogram taken at the
time of admission are shown in Figure 2. The phonocardiogram, shown in Figure 1, confirmed the auscultatory findings.
The chest x-ray picture is shown in Figure 3.
The patient was subjected to right and left heart catheterization and cineangiocardiograms. The hemodynamic data are
summarized in Table 1. The pressure on the venous ventricle
was 20/4 mm Hg, and the pulmonary artery pressures were
20/6/12 mm Hg. With some difficulty, the catheter was
passed across the pulmonary valve, and the "venous" ventri-
Downloaded From: http://publications.chestnet.org/pdfaccess.ashx?url=/data/journals/chest/21516/ on 05/06/2017
.
CONGENITAL CORRECTED TRANSPOSITION OF GREAT VESSELS
FIGURE1. Phonocardiogram at the mitral ( M A ) , tricuspid (TA), pulmonic ( P A ) and aortic
areas ( AA) with the carotid tracing ( C T ) and lead I1 of the electrocardiogram ( see text).
FIGURE2. Electrocardiogram and Frank vectorcardiogram. Note the presence of marked degree
of left axis deviation resembling the pattern of left anterior hemiblock and abnormal T waves.
The QRS-T angle is also increased.
CHEST, VOL. 59, NO. 6, JUNE 1971
Downloaded From: http://publications.chestnet.org/pdfaccess.ashx?url=/data/journals/chest/21516/ on 05/06/2017
635
BENCHIMOL, TI0 AND SUNDARARAJAN
636
I
F l c m 3. Chest x-ray films in PA,
lateral, right and left anterior oblique
projections. Note the round contour of
the apex.
FIGURE
4. Ventricular .&$ogram.
The contrast agent was injected in the left-sided arterial
ventricle which connects with the aorta. This ventricle has the morphology of the anatomic right
ventricle. The patient is in a right anterior oblique projection.
CHEST, VOL. 59, NO. 6, JUNE - 1971
Downloaded From: http://publications.chestnet.org/pdfaccess.ashx?url=/data/journals/chest/21516/ on 05/06/2017
CONGENITAL CORRECTED TRANSPOSITION OF GREAT VESSELS
Table 1-Hemdynamic
637
Data
Pressures mm Hg
CI
SVC
a-V-mean
RA
a-V-mean
RV
S/D
PA
S/D/M
P Wedge
a-V-mean
LV
S/D
Aorta
S/D/M
BSA
MZ
L/min/M2
SI
ml/heat/M?
6/3/1
6/3/4
20/4
20/6/12
9/16/9
116/9
116/67/88
1.95
2.5
45
VP
kgM/min/MZ
ET
msec
HR
t~eat/min
Abbreviations:
TTI
mm Hg/sec/min
SP
gm/sec/MZ
SVC:
P Wedge:
HR:
SP:
Superior vena cava
Pulmonary wedge
Heart rate
Stroke power
RA :
LV :
VP :
SER:
RV :
BSA:
ET:
Right ventricle
Basal surface area
Ejection time
PA : Pulmonary art)ery
CI : Cardiac index
T T I : Tension time index
cle was found to be located medially. During n~anipulationof
the right heart catheter, the patient developed an episode of
ventricular fibrillation which subsided spontaneously. The
left-sided "arterial" ventricle had a pressure of 116/7 mm
Hg, and there was no gradient across the tricuspid valve.
Selective dye dilt~tioncurves with multiple injections and
sampling sites showed no evidence of any intracardiac shunts.
Selective cineangiograms revealed that the left-sided arterial
ventricle resembled the morphology of the anatomic right
ventricle with heavy, irregular trabeculations, crista supraventricularis and a conus of the outtlow tract (Fig 4 ) . It
connected with the aorta which arose anteriorly and to the
left of the large pulmonary artery. The right-sided venous
ventricle had the morphology of the left ventricle with
sniooth endocardial surface. The systemic left-sided atrioventricular valve was tricuspid and showed minimal degree of
insufficiency with retrograde opacification of the left atrium
during angiography. Selective coronary arteriograms (Fig 5)
demonstrated that the right coronary artery was not transposed, and it supplied blood to the anatomic left ventricle.
The left coronary artery divided early into the left anterior
descending and circumflex arteries and supplied the anatomic
right ventricle ( Fig 5).
Congenital corrected transposition of the great
vessels is characterized by transposition of the great
vessels with inversion of the ventricles and of the
atrioventricular valves." The right-sided ventricle
receives venous blood from the right atrium
through a bicuspid valve ( "mitral valve" ) .:$."I 1.12
This ventricle has the structure of the left ventricle
which includes a bicuspid atrioventricular valve
resembling the "normal mitral valve," smooth endocardial surface without trabeculation, and the absence of infundibulum or crista supraventricular i s . " . i . K 1 l . l T h e left-sided ventricle received arterial blood through a tricuspid valve, but has the
structure of the normal right ventricle, which includes a tricuspid valve, infundibulum, crista supraventricularis, trabeculations, and a papillary
muscle on its septa1 surface.:{.i.R.".12 Circulation
in this type of congenital heart disease is usually
SER
mr/sec/M2
dp/dt LV
mm Hg/wc
Right atrium
Left ventricle
Ventricular power
Systolic eject,ion rate
normal. Systemic venous i d o w reaches the normal
right atrium and across the "bicuspid" valve (mitral
valve) into the "venous" ventricle. This ventricle
ejects blood into the normal pulmonary artery that
arises posteriorly in relation to the aorta instead of
its normal anterior position. The arterial oxygenated
blood returning from the lungs reaches a normal
left atrium, crosses a "tricuspid" left-sided valve,
and reaches the "arterial" ventricle. Blood is then
ejected into the aorta, which is abnormally placed
anteriorly and to the left of the pulmonary artery.
Therefore, despite the transposition of the great
vessels, the resulting hemodynamic events are normal in that the inverted ventricles "correct" the
transposition so that the systemic venous (unoxygenated) blood flows into the pulmonary artery,
whereas the pulmonary venous ( oxygenated ) blood
flows into the aorta.
The important feature in this case is the recognition of this congenital lesion in the older age group.
This emphasizes the fact that this form of corrected
transposition of the great vessels is compatible with
long-term survival provided that there are no associated intracardiac defects or significant valvular
insufficiency. Cardiac catheterization with selective
cineangiocardiography is mandatory to establish the
correct diagnosis. Of additional interest in this case
is that the coronary arteries were not transposed
except for the minor abnormality of the early take
off of the left anterior descending artery and the
circumflex arteries (Fig 5 ) . The left coronary a r t e r y
supplied the anatomic right ventricle and the right
coronary artery the anatomic left ventricle. Despite this unusual distribution of blood supply,
the patient had no evidence of coronary insufficiency. The usual coronary artery distribution in this
condition has been previously described.4,!'.1° In
the majority of cases, the right coronary artery
usually arises above the right aortic sinus, gives rise
CHEST, VOL. 59, NO. 6, JUNE 1971
Downloaded From: http://publications.chestnet.org/pdfaccess.ashx?url=/data/journals/chest/21516/ on 05/06/2017
FIGURE5. Selective coronary arteriograms. A-Right coronary artery injection in a left anterior oblique projection. This vessel and its branches s u p
ply blood to the anatomic left ventricle. B, C-Left coronary artery injection in a right anterior oblique projection. This vessel bifurcates early into
the left anterior descending artery
(LAD) and left circumflex artery
( LC ) and both arteries supply blood
to the anatomic right ventricle.
to the anterior descending branch and then contin-
ues in the right atrioventricular groove. The left
coronary artery usually arises from the left aortic
sinus and proceeds into the left atrioventricular
groove with a left circumflex distribution, giving off
a marginal branch and a posterior descending
branch. However, there are many variations of the
above pattern.4.9.10 The anterior aortic cusp is
usually the noncoronary cusp.
ACKNOWLEDGMENTS: We wish to acknowledge the
assistance of Catherine Avianantos, Cheri Bingham, Nancy
Copeland, Virginia Jum, Larry Kuriger, Bettie Jo Massey,
Deanna Moeller and Sydney Peebles.
REFERENCES
I Schiebler CL, Edwards JE, Burchell HB, et al: Congenital corrected transposition of the great vessels: a study of
33 cases. Pediatrics 27 (5, Pt 11) 851-888, 1961
2 Fontana RS, Edwards JE: Congenital Cardiac Disease: A
Review of 357 Cases Studied Pathologically. Philadelphia,
WB Saunders Company, 1962, page 41
3 Ruttenberg HD: Corrected transposition of the great
vessels; splenic syndromes ( asplenia, polysplenia ) , I n
Heart Disease in Infants, Children and Adolescents.
(hloss and Adanis, ed), Baltimore, Williams & Wilkins,
1968
4 Rr~ttenhr~rg
HD, Elliott LP, Anderson RC, et al: Congeni-
tal corrected transposition of the great vessels. Arner J
Cardiol 17:339-354, 1966
5 Moss AJ, Hutter AM Jr, Lipchik EO, et al: Congenitally
corrected transposition of the great vessels without cardiac anomalies. Arner J Med 47:986, 1969
6 Lieberson AD, Schumacher RR, Childress RH, et al:
Corrected transposition of the great vessels in a 73-yearold man. Circulation 3996, 1969
7 Van Praagh R, Van Praagh S: Isolated ventricular inversion. A consideration of the morphogenesis, definition
and diagnosis of nontransposed and transposed great arteries. Amer J Cardiol 17:395-406, 1966
8 Keith JD, Rowe RD, Vlad P: Heart Disease in Infancy
and Childhood. New York, Macmillan 1967
9 Shaher RM, Puddu CC: Coronary arterial anatomy in
complete transposition of the great vessels. Amer J Cardiol 173.55-361,1966
10 Elliot LP, Amplatz K, Edwards JE: Coronary arterial
patterns in transposition complexes. Amer J Cardiol 17:
382-378,1966
11 Morgan AD, Krovetz LJ, Bartley TD, et al: Clinical
features of single ventricle with congenitally corrected
transposition. Amer J Cardiol 17:379-388, 1966
12 Raghib C, Anderson RC, Edwards JE: Isolated bulbar
inversion in corrected transposition. Amer J Cardiol 17:
407-410, 1966
Reprint requests: Dr. Benchimol, 1033 East h.lcDowell Road,
Phoenix 8506.
CHEST, VOL. 59, NO. 6, JUNE 1971
Downloaded From: http://publications.chestnet.org/pdfaccess.ashx?url=/data/journals/chest/21516/ on 05/06/2017