Br Heart J 1982; 48: 298-300

Congenital atresia of left coronary ostiumLUC G VAN DER HAUWAERT, MONIQUE DUMOULIN, PHILIP MOERMAN

From the Section ofPaediatric Cardiology, University Hospital Gasthuisberg, Leuven, Belgium

SUMMARY A two-year-old girl, who presented with congestive heart failure and an extensiveanterolateral infarction, was thought to have anomalous origin of the left coronary artery from the

pulmonary artery. She improved rapidly and remained symptom free until 13 years of age, when shedied suddenly. At necropsy the right coronary artery was found to be normal but in the left aorticsinus a dimple was the only remnant of the left coronary ostium. The proximal segment of the leftcoronary artery was diminutive and did not connect with the aortic root. This is the first report of along term follow-up of a patient with this rare anomaly.

The majority of left coronary artery malformations areanomalies of its site of origin. The clinical picture inpatients with aberrant origin of the left coronaryartery from the pulmonary artery, leading to myocar-dial infarction and congestive heart failure in infancy,is well recognised.' 2 We describe a patient who ininfancy presented with the same clinical and elec-trocardiographic features, became asymptomatic lateron in childhood, and died suddenly at 13 years of age.At necropsy a normally situated but atretic left coro-nary ostium was found.

Case report

A girl was born by normal delivery after an uneventfulpregnancy, birthweight 3 kg, both parents and anolder sister were healthy, and growth and develop-ment were normal until 22 months of age whenanorexia, tiredness, and tachypnoea were noticed. Achest x-ray film, taken because a respiratory infectionwas suspected, showed gross cardiomegaly andincreased pulmonary vascular markings. Whenadmitted on 24 April 1969, the patient was pale,sweating, and obviously in cardiac distress. The heartrate was 160/min and the respiratory rate was 68/min.The face was puffy and the liver was palpable 6 cmbelow the costal margin. On palpation a heaving leftventricular impulse, displaced to the mid-axillaryline, was felt. A grade 2/6 systolic murmur and a loudgallop were heard at the apex. The electrocardiogram(Fig. 1) showed sinus rhythm and a mean frontalQRS axis of + 300. Deep Q waves and sharplyinverted T waves were seen in I, aVL, and the leftprecordial leads. In V3 and V4 the ST segment was

much raised. The diagnosis was anterolateral myocar-dial infarction, probably caused by anomalous originof the left coronary artery from the pulmonary artery.After two weeks of treatment with digoxin and diure-tics, the tachypnoea, tachycardia, and hepatomegalydisappeared and the patient was discharged.

She was readmitted for cardiac investigation at 2/years of age. The apex beat was displaced and a softsystolic murmur was heard at the fourth left inter-costal space. There were no signs of congestive heartfailure. The electrocardiogram showed the same Qwaves but the ST elevation had disappeared.

Right and left cardiac catheterisation (Table) dis-closed normal pressures, with the exception of aslightly raised pulmonary capillary venous (mean14 mmHg) and left ventricular end-diastolic pressure(12 to 18 mmHg). Oximetry showed no evidence ofleft-to-right shunting. Angiography of the aortic rootshowed absence of a left main coronary artery and alarge, slightly tortuous right coronary artery.Branches of the left coronary artery were seen to fillfrom the right side, but the proximal segment of theleft coronary artery was not seen. There was no pas-sage of contrast material into the pulmonary artery.Though direct proof of the connection was lacking,the angiograms were thought to confirm the diagnosisofanomalous left coronary artery originating from thepulmonary artery.The patient remained symptom free and was

examined at yearly intervals. When last seen in March1980, physical examination was unremarkable. Theelectrocardiogram (Fig. 1) disclosed deep but narrowQ waves and T inversion from V4 to V6. In V4 the STsegment was slightly elevated. On the chest x-ray film

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Congenital atresia of left coronary ostium

A

Iv i

I aVR VI V4

siVL

Fig. 1 The electrocardiogram at 22 months ofage (A) showsdeep Q waves and inverted T waves in I, aVL, V5, and V6.TheST segment is much raised in V3 and V4. At 12years ofage(B) the Q waves persist but the ST alterations are lessconspiuous.

1 min) provoked no chest pain. There was no STsegment depression but the T waves in V4 to V6,which were negative at rest, became positive.On the basis of these results it was felt that cardiac

surgery and a saphenous vein graft could be post-poned for a few years. The patient remained symptomfree. At 13 years of age she died at home suddenly.One morning she was found pale, unconscious, andgasping. She was given mouth-to-mouth respirationand rushed to the emergency department of the hospi-tal but was dead on arrival. The days before her deathshe had had a slight fever, probably unrelated to thefatal outcome.At necropsy the right coronary artery was found to

arise normally from its coronary sinus. In the left aor-tic sinus a dimple was the only remnant of the leftcoronary ostium. The proximal segment of the leftcoronary artery was diminutive. Before the heart wasopened, a coronary angiogram with injection of theright coronary artery (Fig. 2) was performed. After

Table Haemodynamic data (at age 2 years 6 months)

Site Pressure Oxygen saturation (%)(mmHg)

Superior vena cava 68, 67Right atrium a 5 v 4 m 3 66, 69Right ventricle 30/5 66, 66Pulmonary artery 30/15 m 23 67, 65Pulmonary capillary m 14Left ventricle 120/12-16Aorta 120/65 99

the heart was normal. The M-mode echocardiogramshowed a slight increase of the end-diastolic left ven-tricular dimension (43 mm) and the fractional shor-tening of the left ventricle with systole was decreased(23%). The left atrial dimension (23 mm) and thethickness of the left ventricular posterior wall (7 mm)and interventricular septum (8 mm) were normal.Exercise testing (maximum load 600 kpm/min during

Fig. 2 Postmortem coronary artenogram after nljectton of theright coronary artery. Numerous small collaterals produceretrograde filing of the left coronary artery. Its main stem,however, is short and smaU and does not communicate with thecoronary ostium.

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opacification of the large and slightly tortuous rightcoronary artery, the branches of the left coronaryartery were seen to fill retrogradely via collaterals.Even at the point of maximum filling the branches ofthe left coronary artery were distinctly smaller thanthose of the right. The main stem of the left coronaryartery was very short and narrow and did not com-municate with the normally situated but atretic coro-nary orifice. Transverse sections of the heart showed alarge (2 x 3 cm) fibrotic scar in the anterior and apicalwall of the left ventricle. The entire left ventricularendocardium appeared thickened and fibrotic. Themajor histological finding was a zone of fresh infarc-tion in the interventricular septum, characterised byinterstitial oedema, eosinophilic coloration of the sar-coplasma, and pyknosis of cell nuclei.

Discussion

Atresia of the left coronary ostium is a rare congenitalanomaly. To our knowledge, five similar cases havebeen reported.37 The clinical picture in our case andin two previously reported infants34 was indistin-guishable from that observed in anomalous origin ofthe left coronary artery from the pulmonary artery. Ascharacteristically seen in the latter condition, ourpatient presented with congestive heart failure, mas-sive cardiac dilatation, and anterolateral infarction ininfancy, with Q waves and ST elevation in I, aVL,and the left precordial leads.' 2A unique feature of our case is the duration of

follow-up from an episode of myocardial infarction ininfancy to adolescence. Between three and 13 years ofage the patient was symptom free and had a normalexercise tolerance. The cardiac volume on the chestx-ray film had become normal.

Cardiac catheterisation may contribute to the diffe-rential diagnosis if oximetry discloses a left-to-rightshunt in the pulmonary artery. By contrast with olderchildren with anomalous origin of the left coronaryartery, however, infants with this anomaly rarely havea significant increase of oxygen saturation in the pul-monary artery.2 Only angiography, carefully inter-preted, permits an accurate diagnosis. In our case

opacification of a large right coronary artery and fail-ure to fill the left coronary artery after aortic rootinjection were thought to corroborate the diagnosis ofanomalous origin of the left coronary artery. Thenon-opacification of the main stem of the left coronaryartery was attributed to poor collateral circulation.

Van der Hauwaert, Dumoulin, Moerman

The same mistake was made in the two reportedinfants.35 We therefore suggest basing the diagnosisof anomalous origin of the left coronary artery on thevisualisation of the anomalous connection afterretrograde filling, which is possible in most cases,2and not only on the non-opacification of the left coro-nary artery after injection of the aortic root. Failure toshow the left main coronary artery after retrogradefilling from the right coronary artery strongly suggestsatresia of the left coronary ostium.

Judging from the postmortem angiograms, theanomaly in our patient was probably not amenable tosurgery. The small calibre of the main stem and majorbranches of the left coronary artery precluded theconstruction of a saphenous vein graft. Successfulsurgical correction and hence creation of a two coro-nary system have been reported in one adolescent4and one adult7 who both presented with long standingangina pectoris.

References

1 Wesselhoeft H, Fawcett JS, Johnson AL. Anomalousorigin of the left coronary artery from the pulmonarytrunk. Its clinical spectrum, pathology, and pathophysiol-ogy based on a review of 140 cases with seven furthercases. Circulation 1%8; 38: 403-25.

2 Askenazi J, Nadas AS. Anomalous left coronary arteryoriginating from the pulmonary artery. Report of 15 cases.Circulation 1975; 51: 976-87.

3 Vemey RN, Monnet P, Arnaud P, Loire R, Salle B,Saubier E. Infarctus du myocarde chez un nourrisson decinq mois. Annales de P&diatrie 1%9; 16: 263-71.

4 Mullins CE, El-Said G, McNamara DG, Cooley DA,Treistman B, Garcia E. Atresia of the left coronary arteryostium. Repair by saphenous vein graft. Circulation 1972;46: 909-94.

5 Lurie PR. Abnormalities and diseases of the coronary ves-sels. In: Moss AJ, Adams FH, Emmanouilides GC, eds.Heart disease in infants, children and adolescents. Baltimore:Williams & Wilkins, 1977: 489-90.

6 Vidne BA, Nih M, Aygen M, Levy MJ. Congenital atresiaof the left main coronary artery ostium. Scand J ThoracCardiovasc Swrg 1979; 13: 37-40.

7 Dymond D, Camm J, Stone D, Rees S, Rees G, SpurrellR. Dual isotope stress testing in congenital atresia of leftcoronary ostium. Applications before and after surgicaltreatment. Br Heart J 1980; 43: 270-5.

Requests for reprints to Dr L G Van der Hauwaert,Section of Paediatric Cardiology, University HospitalGasthuisberg, 3000 Leuven, Belgium.

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