Ann Thorac Surg 1999;67:254-256
© 1999 The Society of Thoracic Surgeons
Case Reports
Incipient left ventricular rupture complicating anomalous left coronary artery
Andrew D. Cochrane, FRACSa,
Tiow H. Goh, FRACPa,
Conal Austin, FRCSa,
Tom R. Karl, MDa
a Victorian Pediatric Cardiac Surgery Unit and Department of Cardiology, Royal Children’s Hospital, Melbourne, Victoria, Australia
Accepted for publication June 29, 1998.
Address reprint requests to Dr Cochrane, Victorian Pediatric Cardiac Surgery Unit, Royal Children’s Hospital, Flemington Rd, Parkville, 3052, Victoria, Australia
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Abstract
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A 4-month-old girl presented with 2 weeks of symptoms and physical signs of heart failure. Echocardiography demonstrated marked left ventricular dilation, thinning of the myocardium with anterolateral akinesis, mitral regurgitation, a moderate pericardial collection, and an anomalous left coronary artery from the pulmonary artery. At operation there was a tense hemopericardium and a site of imminent rupture through a transmural anterior infarction. The anomalous artery was reimplanted in the ascending aorta, and an extensive infarct resection and ventricular repair performed. Support with a left ventricular assist device was required for 3 days, but the infant subsequently made a satisfactory recovery. Left ventricular rupture is a very rare complication of this lesion, but should be considered if there is evidence of a pericardial collection.
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Introduction
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Anomalous origin of the left main coronary artery from the pulmonary artery (ALCAPA) is an uncommon congenital cardiac lesion, usually presenting in infancy with heart failure, evidence of ventricular infarction or ischemia, dilation of the left ventricle (LV), and mitral regurgitation [1]. We report a case complicated by cardiac rupture. Resection of the infarct, repair of the ventricle, and reimplantation of the anomalous coronary artery were successfully performed. This case is the second report in the literature of this rare complication of ALCAPA [2].
A 4-month-old girl was referred with a 2-week history of poor feeding and a cardiac murmur. She had been born at 3.3 kg. and had initially gained weight well to reach 5 kg. On examination there was sinus tachycardia at 150 beats/min and tachypnea at 50 breaths/min. The cardiac apex was displaced with a precordial systolic murmur, and there was 3 cm of hepatomegaly.
The electrocardiogram demonstrated an acute anterolateral infarction, with Q waves in leads I, aVL, V4, V5, and V6, and typical ST-segment elevation. Chest roentgenogram showed moderate to severe cardiomegaly, but no significant pulmonary congestion. On transthoracic echocardiography there was a very dilated LV (LV end-diastolic dimension of 5.6 cm), severely impaired LV function with a fractional shortening of 11%, and a moderate pericardial collection (Fig 1). There was marked thinning of the anteroseptal wall and akinesis of the anterolateral wall of the ventricle. There was increased echogenicity of the anterolateral papillary muscle and the endocardium of the lateral wall, consistent with ischemic injury, and moderate mitral regurgitation with a posteriorly directed jet. There was dilation of the proximal right coronary artery, and the left coronary artery was clearly demonstrated to arise from the main pulmonary artery (MPA), with retrograde diastolic flow into the MPA (Fig 2).
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Fig 1. Transthoracic echocardiogram; subcostal view of the left ventricle. There is marked dilation of the left ventricle, thinning of the septum, a moderate pericardial collection, and increased echodensity of the papillary muscle. (PE = pericardial collection; LV = left ventricle; RV = right ventricle; PM = papillary muscle.)
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Fig 2. Transthoracic echocardiogram; high parasternal short axis view. This demonstrates the anomalous left coronary artery arising from the main pulmonary artery (left side), and retrograde flow into the main pulmonary artery in diastole on color Doppler assessment (right side). The diastolic timing of the color Doppler is indicated by the electrocardiographic tracing (PA = pulmonary artery; LC = left coronary artery.)
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At operation a tense hemopericardium was drained, and the left atrial pressure was measured at 17 mm Hg. Cardiopulmonary bypass was rapidly commenced, followed by snaring of the pulmonary arteries to maintain pressure in the MPA. On lifting the heart there was extensive infarction of the anterior and lateral LV wall, with a large site of subepicardial hemorrhage in the middle of the infarct (Fig 3). The LV was opened at the site of bleeding, and the LV wall was very thin with loss of all myocardial tissue. There was clearly infarction of the anterolateral papillary muscle. Extensive resection of the infarct tissue was performed back to relatively healthy myocardium. The ascending aorta was then clamped and cardioplegia run into both the aortic root and the MPA. The left coronary ostium arose from the left posterior pulmonary sinus, and was reimplanted into the aorta. The posterior commissure of the pulmonary valve required detachment to mobilize the coronary button. No intervention on the mitral valve or the papillary muscle was attempted. The left ventricle was then repaired with continuous 4.0 Prolene in two layers, air evacuated and the cross-clamp released. Reconstruction of the MPA was performed with autologous pericardium, and the valve commissure reattached to the patch. Despite an adequate period of reperfusion, cardiac function after weaning from bypass on a moderate dose of dopamine was poor and the left atrial pressure increased rapidly. Full bypass was recommenced, and the infant was converted to LVAD using a Biomedicus centrifugal pump, with the left atrial cannula placed behind the interatrial groove. During the next 3 days the LV function improved, and LVAD decannulation was performed successfully. She recovered steadily and was discharged home 11 days later on digoxin, frusemide, and enalapril. One month after operation, she was clinically well, and echocardiography revealed a nearly normal sized LV, fractional shortening of 27%, echogenicity of both papillary muscles, and moderate mitral regurgitation. At 13 months of age, she was asymptomatic and growing well at 9.62 kg. On echocardiography the left ventricular end-diastolic dimension had decreased markedly to 3.0 cm, with satisfactory global function, fractional shortening of 27%, and moderate mitral regurgitation. A gated nuclear angiogram at 1 year after operation demonstrated a left ventricular ejection fraction of 64% with reduced anteroseptal wall motion. At 2 years after operation she remained asymptomatic, with no cardiomegaly but a murmur of mitral incompetence, and the electrocardiogram demonstrated sinus rhythm with residual T-wave inversion in the lateral chest leads. Pathologic examination of the resected LV muscle confirmed the presence of extensive fibrosis with foci of calcification, organizing granulation tissue and isolated bands of remaining myocytes.
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Fig 3. The appearance of the heart at operation, with pallor and infarction of the anterior and lateral walls of the left ventricle, and subepicardial hemorrhage at the site of incipient left ventricular rupture. The head of the patient and the ascending aorta are to the left side.
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Comment
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Anomalous origin of the left coronary artery from the pulmonary artery is an uncommon congenital anomaly. Wesselhoeft and colleagues [1] reported the largest review and described four common clinical patterns of presentation, the most common time of presentation being in infancy, with up to 90% mortality in untreated cases presenting in early infancy.
This infant presented with characteristic clinical and diagnostic findings except for the presence of a pericardial effusion. There are few other causes of myocardial infarction in infancy with such a presentation. Identification of the origin of the coronary arteries is essential, and in most cases can be accomplished by echocardiography, including color Doppler and contrast echocardiography [3]. However, in our total experience of 22 patients, we have seen delayed or missed diagnosis in 8 patients based on echocardiography, and have a low threshold for angiography to exclude this lesion in the infant with unexplained heart failure. Several surgical series have been reported [4], and the current mortality for operation is low. However, with a preoperative shortening fraction of 11%, this infant was in the high-risk group for perioperative mortality [4].
Hemopericardium with LV rupture is a well-recognized complication of transmural infarction in the adult population, occurring in 2% to 4% of all infarctions [5]. However, it has only rarely been reported in infants with this defect, probably because the process of ischemia occurs over a longer period, and there is time for development of fibrous tissue and a variable degree of collateral blood supply. This may explain the rarity of LV rupture and the development of LV aneurysm in ALCAPA patients. McKinley and colleagues [2] reported an infant with a hemopericardium due to myocardial rupture, which was rapidly fatal. Kaminer and Truemper [6] reported an infant with hemopericardium and tamponade attributable to ALCAPA, although there was no evidence of rupture. The infant was considered to have pericarditis with a hemorrhagic effusion analogous to that seen after myocardial infarction in adults. We believe that this patient may be the second reported case of LV rupture due to ALCAPA, although the occurrence of LV rupture is possibly underreported. The management of mitral regurgitation is contentious. We have followed a policy in infants of not attempting mitral valve repair, as the degree of incompetence decreases and is often mild in degree at late follow-up, in which case the procedure would be unnecessary, and because a mitral valve procedure would prolong the cardiac ischemic period in a damaged heart. The most important component of the operation is revascularization, and significant recovery and favorable remodeling of the infant myocardium can occur, as suggested by other investigators [4].
Left ventriculotomy is considered undesirable in infants, with adverse effects on long-term LV function in up to 50% of cases [7]. In this patient, there was clearly no choice and the resection involved diseased muscle rather than incision in healthy myocardium. In general, despite severe myocardial injury in ALCAPA, we agree with other researchers that there is little place for LV resection [4].
Support of the circulation with various LV assist devices has been described by our group and other researchers [8], and the ALCAPA patients provide one of the most favorable groups in our experience. This report clearly demonstrates the value of LVAD in a sick infant who otherwise would probably not have survived.
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References
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Wesselhoeft H., Fawcett J.S., Johnson A.L. Anomalous origin of the left coronary artery from the pulmonary trunk. Its clinical spectrum, pathology and pathophysiology, based on a review of 140 cases with seven further cases. Circulation 1968;38:403-425.[Abstract/Free Full Text]
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McKinley H.I., Andrews J., Neill C.A. Left coronary artery from the pulmonary artery. Three cases, one with cardiac tamponade. Pediatrics 1951;8:828-840.[Abstract/Free Full Text]
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Schmidt K.G., Cooper M.J., Silverman N.H., Stanger P. Pulmonary artery origin of the left coronary artery: diagnosis by two-dimensional echocardiography, pulsed Doppler ultrasound and color flow mapping. J Am Coll Cardiol 1988;11:396-402.[Abstract]
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Vouhé P.R., Tamisier D., Sidi D., et al. Anomalous left coronary artery from the pulmonary artery: results of isolated aortic reimplantation. Ann Thorac Surg 1992;54:621-627.[Abstract]
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Sutherland F.W.H., Guell F.J., Pathi V.L., Naik S.K. Postinfarction ventricular free wall rupture: strategies for diagnosis and treatment. Ann Thorac Surg 1996;61:1281-1285.[Abstract/Free Full Text]
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Kaminer S., Truemper E. Hemopericardium and cardiac tamponade as presenting findings of anomalous left coronary artery syndrome complicated by transmural myocardial infarction. Pediatr Cardiol 1996;17:198-200.[Medline]
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Hanna B., Colan S.D., Bridges N.D., Mayer J.E. Clinical and myocardial status after left ventriculotomy for ventricular septal defect closure. J Am Coll Cardiol 1991;17(Suppl):110A.
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Del Nido P.J., Mayer J.E., Wessel D.L., LaPierre R., Jonas R.A. Left ventricular assist device significantly improves survival in children with anomalous left coronary artery and left ventricular dysfunction. Ann Thorac Surg 1999;67:169-172.[Abstract/Free Full Text]
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Abstract
Introduction
