Journal of Cardiology Cases 10 (2014) 2530

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Journal of Cardiology Cases

journal homepage: www.elsevier.com/locate/jccase

Case Report

Truncus arteriosus 5th decade transesophageal and transthoracic

echocardiogram features
Sandra Williams-Phillips (MD, FESC, FASE, FAHA)
Andrews Memorial Hospital, TAI Wing, 27 Hope Road, Kingston 10, Jamaica

a r t i c l e

i n f o

Article history:
Received 9 December 2013
Received in revised form 22 March 2014
Accepted 3 April 2014
Keywords:
Truncus arteriosus
Pulmonary hypertension
Single coronary artery
Pulmonary stenosis

a b s t r a c t
Persistent truncus arteriosus in the 5th decade is a rare entity as the only physical anomaly. Truncus
arteriosus consists of a single great arterial trunk with the systemic, pulmonary, and coronary circulation, with no atretic aortic or pulmonary vessel. The complex cardiac anatomy can be delineated with
transesophageal and transthoracic echocardiography, as is illustrated the case of our patient, who was
diagnosed in infancy, at 5 months of age. This is the oldest documented case report of Type 3 truncus arteriosus with balanced biventricular anatomy, whose survival was secondary to an innate severe branch
pulmonary artery stenosis.
<Learning objective: This case report documents the echocardiographic features of a rare congenital
cardiac anatomy of truncus arteriosus where survival for the majority is only in infancy but those with
pulmonary hypertension can survive to their 4th decade. This is the oldest documented case report of Type
3 truncus arteriosus with balanced biventricular anatomy and one of the eight cases documented survival
after 30 years of age. The transthoracic and transesophageal echocardiographic images are the focused
feature in this paper, conrming clearly dened structural anatomy in complex structural congenital
heart disease.>
2014 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.

Introduction
Persistent truncus arteriosus in the 5th decade is a rare entity.
Truncus arteriosus consists of a single great arterial trunk with the
systemic, pulmonary, and coronary circulation, with no atretic aortic or pulmonary vessel [110]. The index case was diagnosed in
infancy at 5 months of age with truncus arteriosus, after presenting
with cyanosis and congestive cardiac failure requiring antifailure therapy, until late adolescence. Chest pain and palpitations
occurred only on severe exertion, with only one episode of hemoptysis in the past three years. Truncus arteriosus is his only physical
abnormality.
Case report
The index case is a 47-year-old male with a height of 160 cm,
weight of 44.8 kg, and a body mass index of 20 kg/m2 . He has
plethoric mucous membranes, central cyanosis, marked clubbing
of ngers and toes, and a small, short stature with asthenic build.

Tel.: +876 881 7844; fax: +876 978 7540.

E-mail addresses: sandrap@cwjamaica.com, drsandrap@hotmail.com,
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His steady-state oxygen saturation of 77% increased to 84% after

using sildenal for 4 years.
Cardiovascular examination revealed normal pulses, precordial
bulge, parasternal heave, displaced apex beat in the 6th left intercostal space in anterior axillary line, single 2nd heart sound, and a
long systolic murmur 3/6 at mid-left sternal border. There were no
continuous or diastolic murmurs, no additional heart sounds, and
no signs of heart failure.
Hemoglobin was 14.5 g/dl. Packed cell volume was 56 L/L.
Electrocardiogram showed sinus rhythm, right axis deviation,
and biventricular hypertrophy. There were inverted T waves in
V1V6, QR in V1, and normal QRS duration. There was no signicant
ST segment anomaly, but normal P axis and superior T axis.
Chest X-ray showed an increase in the cardiothoracic ratio and
dilated pulmonary arteries with variable vascularity. There was
plethora in right upper lung elds, and oligemia and pruning in the
left lower lung elds thus indicating multifocal pulmonary blood
supply.
Transthoracic echocardiography (TTE) showed situs solitus
(Figs. 1 and 2), atrioventricular concordance (Fig. 3), truncus arteriosus, and persistent [L] superior vena cava (Fig. 4).
There was an intact interatrial septum (Figs. 1 and 3), competent atrioventricular valves (Figs. 3 and 5), with no regurgitation
or stenosis, and normal E and A waves in diastole. There was a

http://dx.doi.org/10.1016/j.jccase.2014.04.001
1878-5409/ 2014 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.

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S. Williams-Phillips / Journal of Cardiology Cases 10 (2014) 2530

Fig. 1. Transesophageal echocardiogram showing closed trileaet truncal valve (arrows 13). RA, right atrium; LA, left atrium; IAS, intact interatrial septum; RAA, right atrial
appendage.

large nonrestrictive ventricular septal defect (VSD), extending from

trabecular to outlet interventricular septum (Figs. 3 and 4). There
was a balanced biventricular anatomy with marked hypertrophy of
the right and left ventricles (Figs. 35). There was normal right and
left ventricular function. Although there was bilateral ow across
the VSD, it was predominantly left to right. Trileaet truncal valve
(Figs. 1 and 68) was observed with no stenosis or regurgitation,
with peak velocity of 1.3 m/s. The truncal valve originated predominantly from the right ventricle (Fig. 4). There were normal left and
right atrial appendages (Figs. 1 and 2).
There was a left-sided aortic arch and normal ventricular function with shortening fraction of 38%. Denitive ostium coronary
artery location was not seen on TTE.
Four miniscule systemic pulmonary (S-P) collaterals were noted
on anterior aspect of descending aorta in suprasternal view.
There was no ow on color Doppler, conrming pulmonary

hypertension in segments of lungs receiving blood supply from

these S-P collaterals. This was then conrmed during cardiac
catheterization.
Transesophageal echocardiogram (TEE) showed additional features not seen on TTE; a single coronary artery originating from
the right coronary sinus with normal distribution of the right and
left coronary arteries (Figs. 1, 6, 7 and 9). There was stenosis of
origin of left pulmonary artery originating from the left posterior
aspect of truncal trunk and stenosis of origin of right pulmonary
artery originating from the right posterior aspect of truncal trunk
(Figs. 9 and 10).
Diagnostic cardiac catheterization with no contrast conrmed
severe right and left pulmonary artery stenosis, which prevented
the catheter from entering either arteries. Three systemic pulmonary collaterals had systemic pressures. Systemic pressures
were in right and left ventricles.

Fig. 2. Transesophageal echocardiogram showing left atrial appendage (LAA) and left atrium (LA). AO, aorta.

S. Williams-Phillips / Journal of Cardiology Cases 10 (2014) 2530

27

Fig. 3. Transesophageal echocardiogram showing four-chamber view with left ventricle (LV) and right ventricle (RV), large ventricular septal defect, right atrium (RA), and
left atrium (LA).

Fig. 4. Transesophageal echocardiogram, parasternal long-axis view in diastole showing mitral valve (MV), balanced right ventricle (RV) and left ventricle (LV), prominent
coronary sinus (CS), and truncal valve (TV) over RV. LA, left atrium.

Fig. 5. Transthoracic echocardiogram short-axis view showing balanced right ventricle (RV) and left ventricle (LV), and open mitral valve (MV).

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S. Williams-Phillips / Journal of Cardiology Cases 10 (2014) 2530

Fig. 6. Transesophageal echocardiogram showing ow in single right coronary artery (RCA) beside right coronary (arrow 1) sinus and trileaet valve (arrows 13).

Fig. 7. Transthoracic echocardiogram parasternal short-axis view showing closed trileaet truncal valve (arrows 13) with origin of single right coronary artery (RCA).

Fig. 8. Transthoracic echocardiogram parasternal short-axis view shows open trileaet truncal valve (arrows 13).

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29

Fig. 9. Transesophageal echocardiogram showing single right coronary artery, origin of right pulmonary artery (RPA), and origin of left pulmonary artery (LPA) from truncal
aorta. RA, right atrium; LA, left atrium and intact interatrial septum; RCA, right coronary artery; TA, truncal aorta.

Fig. 10. Transesophageal echocardiogram showing origin of right pulmonary artery (ORPA), main right pulmonary artery (MRPA), main left pulmonary artery (MLPA),
trileaet truncal valve (TV) (arrows 13), right atrium (RA), left atrium (LA), and interatrial septum (IAS).

Discussion
Survival in truncus arteriosus has a direct correlation to the
structural anatomy and also the degree of pulmonary stenosis.
Survival without pulmonary stenosis is usually not beyond the
neonatal period, but those with pulmonary hypertension can survive to their 4th decade. Type 4 truncus arteriosus with multifocal
blood supply from bronchial arteries, and naturally occurring pulmonary stenosis protects the lungs from pulmonary hypertension,
and is associated with increased longevity, as this index case conrms. [15,9,10]. The index case has competent atrioventricular
and truncal valves and a single right coronary artery with normal
distribution of the right and left coronary vessels. The normal coronary artery distribution does not affect his morbidity or mortality,
and hence is of no clinical signicance [111].
Systemic pulmonary collaterals indicate multifocal blood ow
to lung segments and have produced pulmonary hypertension in
the segments of lungs supplied by them, which has been documented by Chiaw et al. [9]. The index case is the longest balanced

biventricular survivor with truncus arteriosus Type 3 without intervention [7,8,10]. The longest documented survivor with Type 4
truncus arteriosus with multifocal pulmonary blood supply and no
pulmonary arteries was 54 years of age [11]. There are seven other
truncus arteriosus survivors beyond 30 years of age, excluding the
index case. These include Types 1 and 4 truncus arteriosus and four
others with no pulmonary stenosis and Eisenmenger syndrome
[1014].
The exact structural delineation of the heart of the index case
was obtained by TTE and TEE, dening more clearly the type of
truncus arteriosus by showing the origins of the right and left pulmonary arteries and an anomalous single right coronary artery. In
the absence of computed tomography and cardiac magnetic resonance imaging, TTE and TEE can clearly dene complex structural
cardiac anomalies [15].
Conict of interest
The authors declare no conict of interest.

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S. Williams-Phillips / Journal of Cardiology Cases 10 (2014) 2530

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