Case ReportsBritish Heart journal, I971, 33, 409-4II.

Unusual left ventricular aneurysm in a patientwith anomalous origin of left coronaryartery from pulmonary artery

P. Kafkas1 and G. A. H. MillerFrom the Cardiac Department, Brompton Hospital, London S.W.3

A case is described of a I3-year-old girl with anomalous origin of the left coronary artery fromthe pulmonary artery, in whom an unusual left ventricular aneurysm was demonstrated angio-graphically.

Anomalous origin of the left coronary arteryfrom the pulmonary artery is a rare conditionaccounting for some 0o5 per cent of cases ofcongenital heart disease (Keith, 1959). At thepresent time some i6o case reports have beenpublished, and in many of these apical leftventricular 'aneurysms' have been reported(Lang, Phillips, and McAfee, I958; Sabiston,Neill, and Taussig, I960; Kaunitz, I947;Fisher and Lloyd, I95I). In the majority, theaneurysm takes the form of thinning andakinesis of the apical portion of the leftventricle, while in the case reported bySabiston et al. (I960) paradoxical expansionwas observed at the time of operation. Thisreport describes a patient with anomalousorigin of the left coronary artery from thepulmonary artery in whom the aneurysm wasunusual in that it appeared as a localizeddiverticulum of the left ventricle which wasobserved to contract during ventricularsystole.

Case reportThe patient was first seen at the age of 3 yearswhen, after an attack of bronchitis, a chest x-rayrevealed cardiac enlargement. She was asympto-matic. Abnormal findings were confined to thecardiovascular system. The cardiac impulse washyperdynamic, the apex being in the anterioraxillary line. Auscultation revealed a single secondheart sound and a grade 2/6 ejection systolic

1 Present address: 2nd Propedentic Medical Unit,University of Athens, Evangelismos Hospital, Ipsilan-ton St., Athens, Greece.

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murmur maximal at the base. Electrocardiogram:sinus rhythm, T wave inversion in leads I and aVL,and Q waves with ST elevation and T wave in-version in V4-V6 (Fig. iA). Cardiac catheteriza-tion showed slight increase in pulmonary arterypressure (32/15 mmHg) and indirect left atrial(wedge) pressure (a=io, x=8, V=I7, Y=7,mean=II mmHg). No intracardiac shunt wasdetected. Venous angiography showed generalizedenlargement of the left ventricle with a triangularapical aneurysm (Fig. 2). A presumptive diagnosisof anomalous origin of the left coronary arteryfrom the pulmonary artery was made.The patient was reinvestigated at the age of 13

years. She remained symptom free with a normaleffort tolerance. Chest x-ray no longer showedcardiac enlargement. Electrocardiogram: Q waveswith T wave inversion in leads I and aVL withnormal QRS voltage in VI-V3 but low amplitudesintered QRS complexes in V4-V6 (Fig. iB). Car-diac catheterization showed raised pulmonaryartery (45/20 mmHg) and left ventricular end-diastolic (20 mmHg) pressures. There was also arise in right ventricular end-diastolic pressure (i8mmHg) with a commensurate rise in right atrialpressure (a=20, x=13, v=I9, y=13 mmHg).Cineaortography showed a large and tortuousright coronary artery which was seen to drain tothe pulmonary artery via anastomoses with ananomalously arising left coronary artery. A pul-monary arteriogram did not opacify the leftcoronary artery. An injection of contrast mediuminto the left ventricle indicated generalized en-largement of the left ventricle with reducedemptying. There was mild mitral insufficiency.There was an aneurysm of the apex of the leftventricle (Fig. 3A) which, during ventricularsystole, was seen to contract, forming a tongue-like 'diverticulum' of the left ventricle (Fig. 3B).

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410 Kafkas and Miller

FIG. I Electrocardiogram (A) at age of 3years, (B) at age of 13 years.

DiscussionProbably some 85 per cent of patients withanomalous origin of the left coronary arterydie during infancy (Keith, I959), and in suchpatients findings include generalized enlarge-ment of the left ventricle with patchy fibrosis,particularly involving the apex which maybe pathologically thin. There is also somedegree of endocardial fibroelastosis. In a smallproportion of patients who have a good col-lateral circulation between the left and rightcoronary arteries survival into adult life ispossible. In such patients there is generalizedenlargement of the left ventricle, and mitralincompetence may occur which may be dueto dilatation of the mitral valve ring (Burchelland Brown, I962) or to involvement of thepapillary muscles by myocardial fibrosis. Aswas first postulated by Brooks (i885), flow inthe left coronary artery is retrograde and suchretrograde flow was first shown angiographic-ally by Lampe and Verheugt in I960. Thepatient presented here had all these featuresand, in addition, an apical left ventricularaneurysm. Thinning ofthe apical myocardium(Lang et al., 1958) or actual aneurysm forma-tion with paradoxical expansion (Sabiston etal., I960) has been reported. The patient re-ported here had an apical aneurysm but thiswas shown to contract during ventricularsystole. Congenital diverticula of the leftventricle occur and may exhibit contractionduring ventricular systole (Skapinker, 195I;Gasul, Arcilla, and Lev, I966); however, suchdiverticula are usually associated with defectsin the pericardium, diaphragm, or abdominalwall (Potts, DeBoer, and Johnson, I953); inthe case described by Swyer, Mauss, andRosenblatt (I950) there were no such defects,but the diverticulum arose from the posterioraspect of the ventricle, and in this case maytruly have represented an out-pouching in anarea of muscle weakness. In other cases ofdiverticulum of the left ventricle the defect ispresent from birth. Thus, the development ofthe aneurysm over a period of years in the casereported here, its location at the apex of theleft ventricle - the usual site of thinning andaneurysm formation in patients with anomal-

FIG. 2 Left ventricle opacified by venousangiocardiogram at age of 3 years showinglarge (and poorly functioning) left ventricularcavity with early aneurysm formation at apex.

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Unusual left ventricular aneurysm 411

A BFIG. 3 Left ventricular angiocardiogram at age of I3 years. (A) Diastolic phase showinglarge left ventricular cavity and single large (right) coronary artery; the apical aneurysmis not obvious during diastole. (B) Systolic phase showing 'diverticulum-like' aneurysmobvious during ventricular systole; there is some opacification of the left atrium indicating thepresence of mitral incompetence.

ous left coronary - and the absence of defectsin the abdominal wall suggest that theaneurysm developed at the site of myocardialfibrosis. Such obvious aneurysm formationhas not been described before and its apparentcontraction during systole was a surprisingfeature.

We are indebted to Dr. R. V. Gibson for per-mission to publish details of this case.

ReferencesBrooks, H. St J. (i885). Two cases of an abnormal

coronary artery of the heart arising from the pul-monary artery; with some remarks upon the effectof this anomaly in producing cirsoid dilatation ofthe vessels. Journal of Anatomy and Physiology, zo,26.

Burchell, H. B., and Brown, A. L. (I962). Anomalousorigin of coronary artery from pulmonary arterymasquerading as mitral insufficiency. AmericanHeart_7ournal, 63, 388.

Fisher, H., and Lloyd, 0. C. (i95i). A case of anomal-ous left coronary artery. British HeartJournal, 13,406.

Gasul, B. M., Arcilla, R. A., and Lev, M. (I966).

Heart Disease in Children, p. I025. Lippincott,Philadelphia.

Kaunitz, P. E. (I947). Origin of left coronary arteryfrom pulmonary artery. Review of the literatureand report of two cases. American Heart Journal,33, I 82.

Keith, J. D. (1959). The anomalous origin of the leftcoronary artery from the pulmonary artery. BritishHeartJournal, 21, 149.

Lampe, C. F. J., and Verheugt, A. P. M. (I960). Casereports. Anomalous left coronary artery. Adult type.American Heart_Journal, 59, 769.

Lang, E. K., Phillips, L. A., and McAfee, J. G. (1958).Angiocardiographic features of the Bland-White-Garland syndrome. American Journal of Roent-genology, 80, 38I.

Potts, W. J., DeBoer, A., and Johnson, F. R. (I953).Congenital diverticulum of the left ventricle. Casereport. Surgery, 33, 301.

Sabiston, D. C., Neill, C. A., and Taussig, H. B. (I960).The direction of blood flow in anomalous leftcoronary artery arising from the pulmonary artery.Circulation, 22, 591.

Skapinker, S. (195I). Diverticulum of the left ventricleof the heart. Review of the literature and report ofa successful removal of the diverticulum. Archivesof Surgery, 63, 629.

Swyer, A. J., Mauss, I. H., and Rosenblatt, P. (I950).Congenital diverticulosis of left ventricle. AmericanJournal of Diseases of Children, 79, III.

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