Download diagnosis of a congenitally corrected transposition of the great

AFRICA
CARDIOVASCULAR JOURNAL OF AFRICA • Vol 22, No 4, July/August 2011
203
Diagnosis of a congenitally corrected transposition of
the great arteries in a 50-year-old multiparous woman
R JALALIAN, S MASOUMI, A GHAEMIAN
Abstract
Congenitally corrected transposition of the great arteries
(cc-TGA) is a rare congenital heart disease. In the literature,
few patients with this anomaly have been reported to be
asymptomatic until after the fifth decade. We describe a 50year-old female with five pregnancies and successful deliveries, who was unrecognised until late in her fifth decade.
Keywords: congenitally corrected transposition of great arteries,
cc-TGA, congenital heart disease, pregnancy
Submitted 15/35/10, accepted 1/7/10
Cardiovasc J Afr 2011; 22: 203–204
www.cvja.co.za
DOI: 10.5830/CVJA–2010–053
Congenitally corrected transposition of the great arteries (cc-TGA)
accounts for less than 1% of all congenital heart diseases.1 This
anomaly is characterised by atrio-ventricular and ventriculoarterial discordance.2 Associated anomalies occur in up to 95%
of patients and consist of ventricular septal defect (VSD) (75%),
pulmonary artery or subpulmonary artery stenosis (75%), and leftsided valve anomaly (> 75%) (tricuspid and often Ebstein like).
About 5% of patients with cc-TGA are born with a congenital
heart block and acquire complete atrio-ventricular block at a rate
of 2% per year.3 In addition, they may have an abnormality in
the anatomy of their coronary artery, including a left coronary
artery ostium over the commissure, a single left coronary artery
from the right anterior sinus, the anterior descending artery
coming off the right coronary artery, a circumflex ostium over
the commissure, both ostia from the posterior sinus, an eccentric
left coronary artery, a conal branch of the left coronary artery, or
the conal artery near the left coronary artery.4
Although patients with no associated defects theoretically
have a normal life span, few with this lesion survive 40 years
because of associated congenital defects or the subsequent development of atrio-ventricular (AV) valvular insufficiency and/or
heart block.5 Patients without any associated defects (< 5%) may
be asymptomatic until late in adulthood. Dyspnoea and exercise
intolerance resulting from systemic ventricular dysfunction and
left-sided AV valve regurgitation most often appear from the
fourth decade of life.
of diabetes mellitus was referred to us to evaluate for coronary
artery disease. She was married and had had five children without any major problems during her pregnancies. The patient was
in NYHA class I to II and had not been on any medical treatment for her new symptoms. On physical examination, systolic
murmurs (grade II/VI) were heard.
Electrocardiography (ECG) showed an initial Q wave in the
right precordial leads without any Q wave in the left precordial
leads. Also, our patient did not have any evidence of pre-excitation on surface ECG. An exercise tolerance test (ETT) was
performed, but the patient had exertional dyspnoea in the early
phase of stage II, so it was terminated before reaching the optimal heart rate (ETT with Bruce protocol, 4 METS).
With transthoracic echocardiography (TTE), prominent
trabeculations and a moderate band in the left ventricle, with
apical displacement of the left AV valve was seen (Figs 1, 2). The
great vessels were in parallel and side by side. The aorta originated from the left ventricle (morphological right ventricle), being
left and anterior to the pulmonary artery (Fig. 3). The systemic
ventricle (morphological right ventricle) had moderate dysfunction and the left AV valve had mild-to-moderate regurgitation.
Transoesophagial echocardiography (TEE) was performed for
optimal evaluation and showed drainage of all pulmonary veins
into the left atrium (AV discordance) and ventriculo-arterial
discordance. A 3-mm patent foramen ovale with right-to-left
shunt was seen after injecting agitated saline via the right arm.
We also performed computed tomography (CT angiography)
for assessment of the coronary arteries and to confirm the diagnosis. Two separate right and left coronary arteries without any
apparent lesions or anomalies and an anteriorly located aorta
arising from a trabeculated ventricle were visualised (Figs 4, 5).
Discussion
Life expectancy of patients with cc-TGA without associated
Case report
A 50-year-old woman with exertional dyspnoea and a history
Cardiovascular Department, Mazandaran University of
Medical Sciences, Sari, Iran
R JALALIAN, MD
A GHAEMIAN, MD
Mazandaran University of Medical Sciences, Artesh BLVD,
Sari, Iran
S MASOUMI, MD, [email protected]
Fig. 1. Hypertrabeculated left ventricle (physiological
right ventricle) apically displaced, and left AV valve.