Congenital atresia of the left coronary ostium and hypoplasia of the left main coronary artery
Craig J. Byrum, M.D. Marie S. Blackman, M.D. Bernard Schneider, M.D. Henry M. Sondheimer, M.D. Rae-Ellen W. Kavey, M.D. Syracuse,
N. Y.
Anomalies of the coronary arteries are not common, comprising less than .46 per cent of congenital malformations of the heart.‘. ” The case to be reported is a rare congenital malformation of the coronary arteries, atresia of the left coronary ostium with hypoplasia of the left main coronary artery. A single coronary system results which is not a true anatomical single coronary artery. In addition, the clinical and angiographic findings may be confused with anomalous origin of the left coronary artery from the pulmonary artery. Case report A six-month-old female infant was admitted to the Pediatric Unit of Crouse-Irving Memorial Hospital with a two-day history of progressive respiratory distress. Prior to this illness the patient had been completely asymptomatic with normal growth and development. Birth and family history were unremarkable. The infant appeared acutely ill and on physical examination was mottled, gray, with shallow respirations, pulse 180, respiratory rate 80, blood pressure faintly audible at 100/60. Examination of the head and neck was unremarkable. The lungs revealed rhonchi; no riles were heard. The heart was enlarged to percussion: the heart tones were of poor quality with a gallop rhythm at the apex. A faint ejection systolic murmur was heard at the left sternal border. There was hepatomegaly 3 cm. below the right costal margin. All peripheral pulses were diminished. The pertinent laboratory findings revealed: an elevated white blood count of 16,000 with a slight shift to the left. The
From the Division of Cardiology, Department of Pediatrics, Upstate Medical Center, Syracuse, New York, and the Department of Radiology, Grouse-Irving Memorial Hospital, Syracuse. Received for publication Nov. 16, 1978. Accepted for publication Dec. 8, 1978. Reprint requests: Dr. M. Blackman, Pediatric Avenue, Syracuse, N. Y. 13210.
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arterial blood gases were: pH 7.35; PCO, 38; PO, 79 and arterial saturation of 79 per cent. The cardiac enzymes 12 hours after admission revealed a CPK of 9,630, SGOT of 88, and an LDH of 996. The electrocardiogram taken on admission (Fig. 1) demonstrated the pattern seen in anterior lateral wall myocardial infarction. There was inversion and marked ST segment depression in Lead I and in the left precordial leads. A deep Q was present in Lead aV,.. The x-ray examination (Fig. 2) revealed marked cardiomegaly and the vascular pattern was consistent with pulmonary venous congestion. The patient was digitalized, placed in oxygen, and cardiac catheterization was performed on the first hospital day (Table I). The data were consistent with poor myocardial performance. There was no evidence of left-to-right shunting. The right atria1 and pulmonary artery pressures were moderately elevated. There was a marked elevation of left atria1 pressure and left ventricular end-diastolic pressure. Angiograms were interpreted as consistent with the diagnosis of anomalous origin of the left coronary artery from the pulmonary artery. The left atria1 angiogram (Fig. 3) revealed dilated left atria1 and left ventricular chambers. There was akinesis of the anterior and lateral walls as well as the apex of the left ventricle. An aortic root angiogram (Fig. 4) showed filling of a large right coronary artery arising from the anterior aortic cusp. There was delayed opacification of a small, delicate, left main coronary artery via collaterals from the right coronary artery. The anterior descending and circumflex arteries both filled from the collaterals. The high posterior position of the reconstituted main left coronary artery was consistent with the location encountered with anomalous origin of the left coronary from the posterior cusp of the pulmonary artery. However, the main pulmonary artery did not opacify on the aortic root injection and a pulmonary artery arteriogram did not demonstrate filling of a coronary vessel. Open-heart surgery was performed. The left coronary artery did not arise from the pulmonary artery as expected. There was atresia of the left coronary ostium and marked hypoplasia of the left main coronary artery. The left circumflex artery appeared normal. The left anterior descending artery was extremely small and would not accept a graft. An attempt was
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Orifice atresia
Fig.
1. Electrocardiogram
2. X-ray on admission demonstrating pulmonary venous congestion, Fig.
American
Heart
demonstrating
cardiomegaly
and
an anterior
lateral
wall
mvocardial
and CA hypoplasia
infarction.
Fig. 3. Frontal film of left atria1 angiogram with the left ventricle in end systole. There is anterior, apical, and anterior septal akinesis.
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Fig. 4, A and 6. The frontal (A) and lateral (B) aortic root angiogram demonstrates normal origin of a large right coronary artery. There are septal collaterals from the right to left coronary artery. Observe the reconstituted anterior descending, circumflex, and main left coronary artery. Sequential films showed delayed filling via collaterals. Note high posterior position of main left coronary artery.
Table
I. Cardiac catheterization
data
-* Superior vena cava Right atrium Right ventricle Main pulmonary artery Left atrium Left ventricle Aorta
15/6 50/20 46/13 31/19 84126 85/41
10 30 21 72
56 55 48 43 87 87 94 (on 0:)
made at dilatation of the left coronary ostium. The patient did not survive the surgery. At autopsy, the heart was markedly enlarged. The right coronary artery appeared normal. The left circumflex artery was normal, arising from the atretic left main coronary. The left anterior descending branch was small, measuring 1 mm. The left coronary ostium admitted a probe with difficulty. There was a large left ventricular myocardial infarct of recent origin involving the apex, lateral wall, and a portion of the ventricular septum. The microscopic examination of the left main coronary artery revealed a focal area of diminished elastica on the wall adjacent to the myocardium.
Discussion
Non-patency of the left coronary ostium associated with atresia of the proximal left main 356
coronary has been infrequently described in the literature as an isolated finding. Only recently has it been included in a classification of primary congenital anomalies’. ’ of the coronary arteries. It has been reported’ in association with other congenital malformations of the heart and in some systemic diseases. It is absent from most reviews of coronary artery occlusive disease and myocardial infarction in infancy and childhood, including Stryker’s frequently quoted review,” which outlines eight categories of occlusive coronary disease. No description of atresia of the left main coronary artery and ostium specifically appears in his discussion. More recent discussions of occlusive congenital coronary artery disease, and myocardial infarction in infants and children, also contain no mention of the lesion.“‘!’ A case very similar to the patient presented is reported in a textbook discussion by Lurie” and represents the first inclusion of this lesion in a formal categorization of congenital anomalies of the coronary arteries producing occlusion and myocardial infarction. Levin and associates,’ in the most recent series of casesof congenital coronary artery disease,also formally documents and separately classifies this entity. In addition to the above two cases,there have been five case reports in the literature which fit March,
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the description of the case of Lurie3 and our own case. Mullins and co-worken? describe a case in a ten-year-old male who underwent successful aortocoronary bypass -grafting. The case of Fortuin and Roberts” in a 60-year-old male who was diagnosed at autopsy following sudden death, attests to the fact that the lesion spans a wide age range and has unpredictable morbidity and mortality. MacMahon and Dickinson” describe the case of a six-week-old with myocardial infarction who at autopsy revealed non-patency of the right coronary ostium and stenosis of the proximal segments of both the right and left coronaries. The case of Goormaghtigh and colleagues’.’ in a nine-year-old female was discussed in the context of an unproven infectious process and labeled as “idiopathic.” The autopsy findings demonstrated non-patency of the left coronary ostium associated with atresia of the first 1 cm. of the left main coronary artery and stenosis of the right coronary ostium. This case is similar to ours and we believe represents a primary congenital anomaly of coronary arteries. There has been confusion in the literature in the distinction between the entities of congenital atresia of the coronary ostium and proximal coronary arteries and true single coronary artery. This is typified by the report of Murphy” entitled “Single Coronary Artery.” He describes a 71-year-old male in whom the pathologic findings are actually identical to the cases of atresia of the left coronary ostium and hypoplasia of the proximal portion of the left coronary artery described above. The paper by Fortuin and Roberts” considers the possibility that atresia of the coronary ostium and hypoplasia of the proximal coronary artery segment may be one of the causes of true single coronary artery. However, reviews’“, In of the subject of congenital single coronary artery in man contain no cases where an atretic proximal coronary artery was found, Some of the cases described did have dimples at the site of the absent coronary ostium. Embryologically it is suggested that single coronary arteries arise by failure of development or displacement of one coronary anlage.“-” The developmental defect in congenital atresia of the coronary ostia and hypoplasia of the proximal main coronary artery segments may be similar to that producing true single coronary artery, but the resultant malformations are anatomically and clinically distinctly different. Cardiac angiography should be a useful diagAmerican
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and CA hypoplasia
nostic tool to delineate the lesion in this report. However, both our case and the case described by Lurie” were diagnosed preoperatively as anomalous origin of the left coronary artery from the pulmonary artery. The criteria by cardiac catheterization for the diagnosis of anomalous origin of the left coronary artery from the pulmonary artery has been outlined’. ” and include: retrograde filling of the left coronary artery through collaterals with opacification of the pulmonary artery on aortic root injection; the presence of a negative jet in systole or forward flow in diastole into the anomalous left coronary on pulmonary root injection; and left-to-right shunting by oximetry at the main pulmonary artery level. The absence of these findings in a suspected case of anomalous origin of the left coronary artery from the pulmonary artery should suggest the possibility of the lesion under discussion. But it should be recognized that anomalous origin of the left coronary artery from the pulmonary artery with poor collateral flow may not meet these criteria. Additionally, the presence of a short left main coronary artery filling retrograde and ending blindly should be diagnostic of congenital atresia of the ostium and hypoplasia of the left main coronary. Differentiating between the two conditions is of surgical importance but may not be possible clinically or angiographically. In summary, the entity of congenital atresia of the coronary ostium and proximal left main coronary artery should be added to formal classification of primary congenital lesions of coronary arteries which cause occlusive coronary disease and/or myocardial infarction in infancy and childhood. As an isolated event, the lesion can be unilateral or bilateral and probably represents a failure of development of the coronary artery anlage. Pathologically it has been confused with congenital single coronary artery and clinically it can mimic anomalous origin of the coronary artery from the pulmonary artery. Absence of the usual catheterization and angiographic findings for anomalous origin of the left coronary from the pulmonary artery should raise the diagnostic possibility of congenital atresia of the coronary ostium and hypoplasia of the proximal main left coronary artery. Summary
The clinical and pathological findings are described in a six-month-old female with an unusual congenital malformation, atresia of the 357
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orifice and hypoplasia of the left main coronary artery. The literature is reviewed and the clinical findings are discussed. A comparison has been made with the anatomic findings in true single coronary artery and with the angiographic findings in anomalous origin of the left coronary artery from the main pulmonary artery.
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