C

Te

SA

a

ARRA

KTPSP

I

Tstiwuota

C

wpo

d

h1

Journal of Cardiology Cases 10 (2014) 25–30

Contents lists available at www.sciencedirect.com

Journal of Cardiology Cases

journa l h om epage: www.elsev ier .com/ locate / j ccase

ase Report

runcus arteriosus 5th decade transesophageal and transthoracicchocardiogram features

andra Williams-Phillips (MD, FESC, FASE, FAHA) ∗

ndrews Memorial Hospital, TAI Wing, 27 Hope Road, Kingston 10, Jamaica

r t i c l e i n f o

rticle history:eceived 9 December 2013eceived in revised form 22 March 2014ccepted 3 April 2014

eywords:runcus arteriosusulmonary hypertensioningle coronary arteryulmonary 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. Truncusarteriosus consists of a single great arterial trunk with the systemic, pulmonary, and coronary circula-tion, with no atretic aortic or pulmonary vessel. The complex cardiac anatomy can be delineated withtransesophageal and transthoracic echocardiography, as is illustrated the case of our patient, who wasdiagnosed in infancy, at 5 months of age. This is the oldest documented case report of Type 3 truncus arte-riosus with balanced biventricular anatomy, whose survival was secondary to an innate severe branchpulmonary artery stenosis.<Learning objective: This case report documents the echocardiographic features of a rare congenitalcardiac 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 Type3 truncus arteriosus with balanced biventricular anatomy and one of the eight cases documented survivalafter 30 years of age. The transthoracic and transesophageal echocardiographic images are the focusedfeature in this paper, confirming clearly defined structural anatomy in complex structural congenitalheart disease.>

© 2014 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.

ntroduction

Persistent truncus arteriosus in the 5th decade is a rare entity.runcus arteriosus consists of a single great arterial trunk with theystemic, pulmonary, and coronary circulation, with no atretic aor-ic or pulmonary vessel [1–10]. The index case was diagnosed innfancy at 5 months of age with truncus arteriosus, after presenting

ith cyanosis and congestive cardiac failure requiring antifail-re therapy, until late adolescence. Chest pain and palpitationsccurred only on severe exertion, with only one episode of hemop-ysis in the past three years. Truncus arteriosus is his only physicalbnormality.

ase report

The index case is a 47-year-old male with a height of 160 cm,

eight of 44.8 kg, and a body mass index of 20 kg/m2. He haslethoric mucous membranes, central cyanosis, marked clubbingf fingers 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,

rsandrap2@gmail.com

ttp://dx.doi.org/10.1016/j.jccase.2014.04.001878-5409/© 2014 Japanese College of Cardiology. Published by Elsevier Ltd. All rights re

His steady-state oxygen saturation of 77% increased to 84% afterusing sildenafil for 4 years.

Cardiovascular examination revealed normal pulses, precordialbulge, parasternal heave, displaced apex beat in the 6th left inter-costal space in anterior axillary line, single 2nd heart sound, and along systolic murmur 3/6 at mid-left sternal border. There were nocontinuous or diastolic murmurs, no additional heart sounds, andno 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 inV1–V6, QR in V1, and normal QRS duration. There was no significantST segment anomaly, but normal P axis and superior T axis.

Chest X-ray showed an increase in the cardiothoracic ratio anddilated pulmonary arteries with variable vascularity. There wasplethora in right upper lung fields, and oligemia and pruning in theleft lower lung fields thus indicating multifocal pulmonary bloodsupply.

Transthoracic echocardiography (TTE) showed situs solitus(Figs. 1 and 2), atrioventricular concordance (Fig. 3), truncus arte-

riosus, and persistent [L] superior vena cava (Fig. 4).

There was an intact interatrial septum (Figs. 1 and 3), compe-tent atrioventricular valves (Figs. 3 and 5), with no regurgitationor stenosis, and normal “E” and “A” waves in diastole. There was a

served.

26 S. Williams-Phillips / Journal of Cardiology Cases 10 (2014) 25–30

Fig. 1. Transesophageal echocardiogram showing closed trileaflet truncal valve (arrows 1–3). RA, right atrium; LA, left atrium; IAS, intact interatrial septum; RAA, right atriala

ltwtlt(wir

ta

oT

ppendage.

arge nonrestrictive ventricular septal defect (VSD), extending fromrabecular to outlet interventricular septum (Figs. 3 and 4). Thereas a balanced biventricular anatomy with marked hypertrophy of

he right and left ventricles (Figs. 3–5). There was normal right andeft ventricular function. Although there was bilateral flow acrosshe VSD, it was predominantly left to right. Trileaflet truncal valveFigs. 1 and 6–8) was observed with no stenosis or regurgitation,ith peak velocity of 1.3 m/s. The truncal valve originated predom-

nantly from the right ventricle (Fig. 4). There were normal left andight atrial appendages (Figs. 1 and 2).

There was a left-sided aortic arch and normal ventricular func-ion with shortening fraction of 38%. Definitive ostium coronary

rtery location was not seen on TTE.

Four miniscule systemic pulmonary (S-P) collaterals were notedn anterior aspect of descending aorta in suprasternal view.here was no flow on color Doppler, confirming pulmonary

Fig. 2. Transesophageal echocardiogram showing left atr

hypertension in segments of lungs receiving blood supply fromthese S-P collaterals. This was then confirmed during cardiaccatheterization.

Transesophageal echocardiogram (TEE) showed additional fea-tures not seen on TTE; a single coronary artery originating fromthe right coronary sinus with normal distribution of the right andleft coronary arteries (Figs. 1, 6, 7 and 9). There was stenosis oforigin of left pulmonary artery originating from the left posterioraspect of truncal trunk and stenosis of origin of right pulmonaryartery originating from the right posterior aspect of truncal trunk(Figs. 9 and 10).

Diagnostic cardiac catheterization with no contrast confirmed

severe right and left pulmonary artery stenosis, which preventedthe catheter from entering either arteries. Three systemic pul-monary collaterals had systemic pressures. Systemic pressureswere in right and left ventricles.

ial appendage (LAA) and left atrium (LA). AO, aorta.

S. Williams-Phillips / Journal of Cardiology Cases 10 (2014) 25–30 27

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

Fig. 4. Transesophageal echocardiogram, parasternal long-axis view in diastole showing mitral valve (MV), balanced right ventricle (RV) and left ventricle (LV), prominentcoronary 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).

28 S. Williams-Phillips / Journal of Cardiology Cases 10 (2014) 25–30

Fig. 6. Transesophageal echocardiogram showing flow in single right coronary artery (RCA) beside right coronary (arrow 1) sinus and trileaflet valve (arrows 1–3).

Fig. 7. Transthoracic echocardiogram parasternal short-axis view showing closed trileaflet truncal valve (arrows 1–3) with origin of single right coronary artery (RCA).

Fig. 8. Transthoracic echocardiogram parasternal short-axis view shows open trileaflet truncal valve (arrows 1–3).

S. Williams-Phillips / Journal of Cardiology Cases 10 (2014) 25–30 29

Fig. 9. Transesophageal echocardiogram showing single right coronary artery, origin of right pulmonary artery (RPA), and origin of left pulmonary artery (LPA) from truncalaorta. 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),t eratri

D

sSnvbmafiadna

ttm

rileaflet truncal valve (TV) (arrows 1–3), right atrium (RA), left atrium (LA), and int

iscussion

Survival in truncus arteriosus has a direct correlation to thetructural anatomy and also the degree of pulmonary stenosis.urvival without pulmonary stenosis is usually not beyond theeonatal period, but those with pulmonary hypertension can sur-ive to their 4th decade. Type 4 truncus arteriosus with multifocallood supply from bronchial arteries, and naturally occurring pul-onary stenosis protects the lungs from pulmonary hypertension,

nd is associated with increased longevity, as this index case con-rms. [1–5,9,10]. The index case has competent atrioventricularnd truncal valves and a single right coronary artery with normalistribution of the right and left coronary vessels. The normal coro-ary artery distribution does not affect his morbidity or mortality,nd hence is of no clinical significance [1–11].

Systemic pulmonary collaterals indicate multifocal blood flowo lung segments and have produced pulmonary hypertension inhe segments of lungs supplied by them, which has been docu-

ented by Chiaw et al. [9]. The index case is the longest balanced

al septum (IAS).

biventricular survivor with truncus arteriosus Type 3 without inter-vention [7,8,10]. The longest documented survivor with Type 4truncus arteriosus with multifocal pulmonary blood supply and nopulmonary arteries was 54 years of age [11]. There are seven othertruncus arteriosus survivors beyond 30 years of age, excluding theindex case. These include Types 1 and 4 truncus arteriosus and fourothers with no pulmonary stenosis and Eisenmenger syndrome[10–14].

The exact structural delineation of the heart of the index casewas obtained by TTE and TEE, defining more clearly the type oftruncus arteriosus by showing the origins of the right and left pul-monary arteries and an anomalous single right coronary artery. Inthe absence of computed tomography and cardiac magnetic reso-nance imaging, TTE and TEE can clearly define complex structuralcardiac anomalies [15].

Conflict of interest

The authors declare no conflict of interest.

3 f Card

R

[

[

[

[

[

0 S. Williams-Phillips / Journal o

eferences

[1] Hicken P, Evans D, Heath D. Persistent truncus arteriosus with survival to theage of 38 years. Br Heart J 1966;28:284–6.

[2] Morgan JR, Kearney DJ, Hagan AD. Type 1 truncus arteriosus in an adult. Chest1972;62:501–4.

[3] McCue CM, Lester RG, Bosher LH, Mauck HP. Persistent truncus arteriosus, aclinical correlation with the pathologic anatomy. Chest 1964;46:507–23.

[4] Kirklin JW, Barrett-Boyes BG. Truncus arteriosus. Cardiac surgery, vol. 2. NewYork: Churchill Livingstone Inc.; 1993. p. 1131.

[5] Anderson RH, Macartney FJ, Shinebourne EA, Tynan M. Truncus arteriosus.Paediatric cardiology, vol. 2. New York: Churchill Livingstone Inc.; 1987. p. 913.

[6] Gutierrez PS, Binotto MA, Aiello VD, Mansur AJ. Chest pain in an adult with

truncus arteriosus communis. Am J Cardiol 2004;93:272–3.

[7] Porter I, Vacek J. Single ventricle with persistent truncus arteriosus as two rareentities in an adult patient: a case report. J Med Case Rep 2008;2:184.

[8] Guenther F, Frydrychowicz A, Bode C, Geibel A. Cardiovascular flashlight. Per-sistent truncus arteriosus: a rare finding in adults. Eur Heart J 2009;30:1154.

[

iology Cases 10 (2014) 25–30

[9] Chiaw TH, San TR, Le TJ. An adult with truncus arteriosus and unilateral pul-monary hypertension. Congenit Heart Dis 2007;2:433–7.

10] Williams-Phillips S. Truncus arteriosus: surviving at 46 years with no interven-tion. West Indian Med J 2013;62:273–6.

11] Bodi V, Insa L, Sanchis J, Ibanez M, Losada A, Chorro FJ. Persistent truncusarteriosus type 4 with survival to the age of 54 years. Int J Cardiol 2002;82:75–7.

12] Carter JB, Blieden LC, Edwards JE. Persistent truncus arteriosus. Report of sur-vival to age 52 years. Minn Med 1973;56:280–2.

13] Fernández Fernández FJ, Mesías Prego A, González González C, Sesma P, GarcíaRodríguez JF. Truncus communis-type congenital cardiopathy: survival up to53. An Med Interna 2000;17:566–7.

14] Niwa K, Perloff JK, Kaplan S, Child JS, Miner PD. Eisenmenger syndrome in

adults: ventricular septal defect, truncus arteriosus, univentricular hearts. J AmColl Cardiol 1999;34:223–32.

15] Verhaert D, Arruda J, Thavendiranathan P, Cook SC, Raman SV. Truncus arterio-sus with aortic arch interruption: cardiovascular magnetic resonance findingsin the unrepaired adult. J Cardiovasc Magn Reson 2010;12:16.