Download Isolated congenitally corrected transposition of the great arteries

Isolated congenitally corrected transposition of the great
arteries with dextroversion discovered incidentally in a
patient with cocaine-induced acute myocardial infarction
Anumeha Tandon, MD, Rahul Bose, MD, Anthony D. Yoon, MD, and Jeffrey M. Schussler, MD
Complex cardiac congenital anomalies can occasionally be found in
adult patients who have no knowledge of their condition. Here we
present the case of a 27-year-old
man with cocaine-induced acute
myocardial infarction in whom an
isolated congenitally corrected
transposition of the great arteries
with dextroversion was discovered
incidentally.
W
hile congenital
anomalies are often left to subspecialists in the adult
congenital arena, familiarity
with them is important for Figure 1. Electrocardiogram demonstrating a reversal of the P-wave axis (inverted P waves in leads I, AVR, and AVF; arrow)
the general cardiologist. When with reversal of progression of R waves across the entire precordial leads (V1–V6).
these patients present in association with other, more common conditions, evaluation can
anterior descending artery, but in a conformation that would be
be more challenging.
considered “opposite” of normal anatomy. The opposite coronary artery appeared similar in conformation to a right coronary
CASE DESCRIPTION
artery, but also had an “opposite” course (Figure 3).
A 27-year-old man presented with chest pain. A loud systolic
A transthoracic echocardiogram (with images obtained primurmur was heard at the right sternal border, and a rightward
marily from right-sided windows) revealed an aorta originating
displaced point of maximal impact was noted. The admission
from a morphologic right ventricle (systemic ventricle) and the
electrocardiogram showed prominent R waves in V1, with an
pulmonary trunk from a morphologic left ventricle (pulmonary
inverted P-wave axis, and reverse R wave progression across the
ventricle). These findings were confirmed by cardiac computed
precordium (Figure 1). A chest radiograph demonstrated a righttomography (Figure 4). Abdominal ultrasound showed normally
ward-pointed heart, prominent right-sided chambers, and the
oriented viscera confirming situs solitus (normal orientation
absence of the usual aortic and pulmonary contours (Figure 2).
of the viscera). The patient was treated medically for cocaine
The urine was positive for cocaine. The patient’s troponin level
intoxication and was ultimately discharged in good condition.
was 55 ng/mL. At urgent cardiac catheterization, the ventriculogram in the left anterior oblique position disclosed the heart
From the Department of Internal Medicine, Division of Cardiology, Baylor University
pointing toward the right hemithorax, suggesting dextrocardia
Medical Center at Dallas (Tandon, Bose, Yoon, Schussler); the Jack and Jane
or dextroversion. Angiography showed no coronary narrowing,
Hamilton Heart and Vascular Hospital, Dallas, Texas (Tandon, Yoon, Schussler);
but demonstrated “mirror image” epicardial coronary arteries.
and Texas A&M College of Medicine (Schussler).
The most anterior coronary artery coursed toward the right side
Corresponding author: Jeffrey M. Schussler, MD, 621 N. Hall Street, Suite 400,
of the body and covered a distribution similar to that of a left
Dallas, TX 75226 (e-mail: [email protected]).
Proc (Bayl Univ Med Cent) 2016;29(2):171–173
171
Figure 2. Chest radiograph demonstrating a rightward-oriented heart, with the
morphologic left ventricle (LV) pointed towards the right. There is loss of the
normal aortopulmonary contours (arrowhead), and this area appears “flat.” The
gastric bubble is noted under the left hemidiaphragm, indicative of situs solitus
(normally oriented viscera).
DISCUSSION
Congenitally corrected transposition of the great arteries
(CCTGA) is a rare (<1% of all congenital heart disease) anomaly where the great arteries are transposed and the ventricles,
ventricular septum, atrioventricular valves, epicardial coronary
arteries, and the conduction system are inverted. Other congenital heart defects such as ventricular septal defect, pulmonary stenosis, anomalies of the systemic atrioventricular valve
(commonly, Ebstein’s anomaly), and conduction defects are
present in approximately 98% of these patients. Most patients
are identified in childhood, usually due to complications from
the associated abnormalities. A minority of patients (particularly those without associated anomalies) may be asymptomatic
for many years and present later in life due to findings on
physical or radiologic exams, or if they experience systemic
ventricular failure (1). Dextrocardia, or a rightward-pointing
heart, is seen in up to 20% of these patients, and CCTGA
should be strongly suspected when dextrocardia is observed
(2). Dextrocardia with situs solitus (normally oriented viscera)
and no other cardiac anomalies is rare, with an incidence of 1
live birth in 30,000 (3).
a
b
c
d
Figure 3. Systemic ventriculogram (morphologic right ventricle) with a rightward-oriented apex. (a) Standard left anterior oblique imaging demonstrates a systemic
ventricle that points towards the right side of the body. (b) Injection of the ascending aorta (Ao) fills the left main (LM) coronary artery. (c) Selective injection of the
LM fills arteries that are equivalent to the left anterior descending (LAD) and left circumflex (LCx) arteries, in that they supply blood to the anterior and lateral portions
of the systemic ventricle. (d) Injection of the posteriorly located coronary artery, equivalent to the right coronary artery (RCA), demonstrates that it supplies blood to
the posterior portions of the systemic ventricle.
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Baylor University Medical Center Proceedings
Volume 29, Number 2
a
b
c
d
cardiac magnetic resonance
imaging, and invasive coronary angiography can all be
used in conjunction to allow
for accurate diagnosis.
Coronary anatomy, in
particular, is variable. In up
to 50% of patients, there
may be associated coronary
anomalies (4). Patients who
present with coronary atherosclerosis or acute coronary
syndromes are uncommon,
as these patients usually present with systemic ventricular
failure early in life. Coronary
revascularization is appropriate and feasible, although it
may be technically challenging due to abnormal conformation of the coronary
anatomy (5, 6).
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Figure 4. (a) Computed tomography of the heart with 3D rendering demonstrating the course of the coronary arteries as
with situs solitus (dextroversion).
they supply blood to the systemic ventricle (SV). (b) A fully rendered 3D representation of the heart shows the relationship
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heart demonstrates that the aorta (Ao) is positioned anterior to the pulmonary artery (PA); (d) rather than being oriented
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once the condition has been identified, should include idenCoronary arterial revascularization in an adult with congenitally cortification of visceral situs, atrioventricular concordance, venrected transposition of great arteries and dextrocardia. J Card Surg
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April 2016
Isolated congenitally corrected transposition of the great arteries with dextroversion discovered incidentally
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