Cardiovascular Journal of Africa: Vol 26 No 4 (July/August 2015)

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CARDIOVASCULAR JOURNAL OF AFRICA • Volume 26, No 4, July/August 2015 e6 AFRICA
Unrepaired persistent truncus arteriosus in a 38-year-old
woman with an uneventful pregnancy
Dorra Abid, Emna Daoud, Sahar Ben Kahla, Souad Mallek, Leila Abid, Hela Fourati, Zeineb Mnif,
Samir Kammoun
Abstract
Persistent truncus arteriosus (PTA) is a rare conotruncal
defect, defined as a single arterial vessel arising from the
heart, which gives origin to the systemic, pulmonary and
coronary circulations. It has an extremely poor prognosis
and carries a high mortality rate during the early years
of life unless surgically repaired. A few known cases have
been reported of patients reaching maturity, and exception-
ally, patients suffering from this disease having lived into the
fourth decade.
The purpose of this report was to present a new case of
PTA type 1, diagnosed by echocardiography and MRI, in a
41-year-old woman, with the peculiarity of long survival into
adult life. She had also experienced a full-term pregnancy
and delivery of a normal infant three years prior to her diag-
nosis. Pulmonary vascular disease made her condition inop-
erable but she was doing well with medical management after
a follow up of 15 months. Based on this work, we concluded
that pulmonary arterial hypertension is deleterious for life in
some cardiovascular diseases, but in others, allows survival,
as occurred in these patients with PTA. The patient’s clini-
cal course and anatomical findings are reported, along with
factors that may have contributed to her longevity.
Keywords: persistent truncus arteriosus, adult, echocardiography,
pulmonary artery hypertension, magnetic resonance imaging
Submitted 22/5/13, accepted 11/1/15
Cardiovasc J Afr 2015; 26: e6–e8
DOI: 10.5830/CVJA-2015-005
Persistent truncus arteriosus (PTA) is an uncommon congenital
heart disease (CHD) that was first described by Wilson in 1798.
In 1976, Calder et al. reported it accounted for approximately
0.7 to 1.2% of all congenital heart malformations and occurred
equally in men and women.1
Truncus arteriosus (TA) is defined as a single arterial vessel,
usually arising from both the left and the right ventricle, which
gives rise to the systemic, pulmonary and coronary artery
circulations. 1 It is mostly associated with a large, non-restrictive
ventricular septal defect (VSD) situated below the semilunar
truncal valve.
PTA is also reported to be associated with a high rate of
mortality if uncorrected. Calder et al. stated that about 65% of
patients treated medically did not survive beyond six months of
age, and more than 90% who did not have surgical repair died
before one year of age.1 Surgical intervention is required to avoid
pulmonary vascular disease, which is common in the unrepaired
patient. Because of this extremely poor prognosis, PTA is
uncommonly encountered in adult life.
Here we report on the unusual case of a woman with
unrepaired TA who was evaluated by echocardiography and
magnetic resonance imaging (MRI).
Case report
A 41-year-old housewife initially presented to the Cardiology
Department of Hedi Chaker Hospital in Tunisia in November
2011 with symptoms of exercise intolerance and occasional
palpitations of several months’ duration. She had no family
history of congenital defects. Three years earlier, when she was
38 years old, she gave birth to a normal baby after undergoing
an uneventful full-term pregnancy and delivery.
Cyanosis and dyspnoea on exertion had been present
throughout her life, but despite this, she appeared to have had a
fairly normal life, being able to tolerate daily activities well. Two
weeks prior to admission, she reported having experienced an
exacerbation of dyspnoea.
She was a small-built woman and appeared deeply cyanosed
on examination but not dyspnoeic at rest. Prominent clubbing
of the fingers was noticeable. Congestion of the jugular veins
was also striking. Her oxygen saturation in room air was about
80%. Physical examination revealed a mild systolic murmur over
the left parasternal border and a loud second heart sound in the
right second intercostal space.
A 12-lead resting electrocardiogram revealed sinus rhythm,
right bundle branch block and high QRS voltage suggestive of
Case Report
Cardiology Department, Hedi Chaker Hospital, Sfax,
Tunisia
Dorra Abid, MD,
Sahar Ben Kahla, MD
Souad Mallek, MD
Leila Abid, MD
Samir Kammoun, MD
Department of Radiology, Hedi Chaker Hospital, Sfax,
Tunisia
Emna Daoud, MD
Hela Fourati, MD
Zeineb Mnif, MD