Ann Thorac Surg 1998;66:1406-1409
© 1998 The Society of Thoracic Surgeons
Case Reports
Origin of the circumflex coronary artery from the pulmonary artery in infants
Vladimir Alexi-Meskishvili, MD, PhDa,
Ingo Dähnert, MDa,
Roland Hetzer, MD, PhDa,
Peter E. Lange, MD, PhDa,
Tom R. Karl, MDb
a Department of Cardiothoracic and Vascular Surgery, and Pediatric Cardiology, German Heart Institute Berlin, Berlin, Germany
b Cardiac Unit, Royal Children’s Hospital, Melbourne, Australia
Accepted for publication April 22, 1998.
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Abstract
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Two infants with anomolous origin of the left circumflex coronary artery from the pulmonary artery had to be operated on at ages 40 days and 30 days because of severe myocardial dysfunction. This illustrates that the clinical course of anomalous origin of the circumflex artery from the pulmonary artery may not always be as favorable as reported in the literature.
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Introduction
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Origin of the left circumflex coronary artery from the pulmonary artery is an extremly rare coronary anomaly. Only 9 cases have been reported [1–9]. In this article we describe such anomalies seen in infancy.
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Patient 1
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A 40-day-old male infant (weight, 3.5 kg) was transferred to the German Heart Institute, Berlin, Germany, intubated and ventilated. His condition had been normal until the 38th day of life, when acute and severe cardiopulmonary decompensation developed. Chest radiography revealed an extremely enlarged heart with a cardiothoracic ratio of 0.68. The electrocardiogram showed severe left ventricular strain pattern with Q waves in leads III and aVF and an elevated QT segment in leads V4 and V5. Echocardiography documented extreme left ventricular dilatation and depressed contractility (shortening fraction, 0.18; ejection fraction, 0.35). Cardiac catheterization and angiography revealed an elevated left ventricular end-diastolic pressure of 30 mm Hg, an extremely dilated left ventricle with poor contractility, moderate mitral valve incompetence, origin of the left anterior descending artery and right coronary artery from the right aortic sinus, and origin of the circumflex coronary artery from the left pulmonary artery (Fig 1 ), with insufficient collaterals.
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Fig 1. Pulmonary angiogram from patient 1 before operation. Note the origin of the circumflex coronary artery from the left pulmonary artery (arrow).
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The infant underwent an operation on the 41st day of life. On hypothermic (24°C) cardiopulmonary bypass and myocardial protection with crystalloid cardioplegia, reimplantation of the circumflex artery into the aorta was performed. An attempt to discontinue cardiopulmonary bypass resulted in an increase in the left atrial pressure to more than 25 mm Hg with systemic arterial hypotension. Despite reperfusion for 3 hours, further attempts to discontinue bypass were also unsuccessful. Therefore the patient was supported with extracorporeal membrane oxygenation for low cardiac output and depressed pulmonary function (arterial oxygen tension, 60 mm Hg; inspired oxygen fraction, 1.0). Extracorporeal membrane oxygenation was maintained for 63 hours at a rate of 2.6 L · min-1 · m-2. The infant was disconnected from the extracorporeal membrane oxygenation circuit on the third postoperative day and extubated on the 25th postoperative day.
Two weeks after the operation, his left ventricular shortening fraction was increased to 0.36 and the left ventricular ejection fraction to 0.70. After extubation, mild respiratory incompetence developed because of right diaphragm paralysis. On the 33rd postoperative day a right diaphragmatic plication was performed. Four hours afterward, while the infant was still intubated, acute hemodynamic instability developed. Transthoracic echocardiography revealed significant pericardial effusion and depressed biventricular function. During emergency thoracotomy, ventricular fibrillation developed and the infant could not be resuscitated. Autopsy permission was not obtained.
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Patient 2
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The second patient was a female twin born at 35 weeks’ gestation, with a birth weight of 1.9 kg. The other twin died of diaphragmatic hernia in utero. Antenatal ultrasound revealed some cardiac dilatation and a small pericardial effusion. At 1 month of age the patient was noted to have tachypnea and failure to thrive. The baby’s chest radiograph showed cardiomegaly, and her electrocardiogram showed Q waves in leads I, AvL, and V6, with ST elevation in lead AvL. Echocardiography showed a dilated left atrium and left ventricle with a thin, dyskinetic inferoposterior wall. There was moderate mitral insufficiency, and the papillary muscles and endocardium were abnormally echogenic. A cardiac catheterization study showed an anomalous circumflex coronary artery from the posterior aspect of the main pulmonary artery (Fig 2 ). The right coronary artery and left anterior descending artery arose from appropriate sinuses and communicated with the circumflex artery via collaterals. There was retrograde flow from the circumflex artery to the pulmonary artery. The pulmonary artery pressure was 45/17 mm Hg, versus 100/55 mm Hg in the ascending aorta.
When the patient was 6 weeks of age, ligation of the circumflex coronary artery via left thoracotomy was performed in the Cardiac Unit, Royal Children Hospital, Melbourne, Australia. The postoperative course was characterized by continued mild congestive heart failure and feeding difficulties. Twenty-four months postoperatively the child is asymptomatic, but remains in the third percentile for weight and takes angiotensin-converting enzyme inhibitors. Left ventricular contractility is good, but the left ventricle and left atrium are dilated as a result of moderate mitral regurgitation. Mitral valvuloplasty is planned in the near future.
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Comment
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Origin of the circumflex coronary artery from the pulmonary artery is an extremely rare congenital coronary anomaly. An extensive search of the medical literature revealed only 9 patients with this anomaly [1–9] (Table 1 ). Seven were children and 2 were adults. All children had additional congenital cardiac defects (see Table 1). Well-developed collaterals between circumflex and other coronary arteries were observed in all patients in whom angiography was performed. Four children had cardiac symptoms [2, 6, 7, 9], which could be attributed to coronary anomaly, but the other 5 patients were asymptomatic [1, 3, 4, 5, 8]. In 6 of the 9 reported cases, the anomalous circumflex coronary artery originated from the right pulmonary artery; in the other 3 it originated from the main pulmonary artery. In our patients, the origin was left pulmonary artery and main pulmonary artery, respectively.
In 3 patients the coronary anomaly was corrected by means of closure of anomalous coronary ostium [2], ligation of the circumflex coronary artery at its origin from the pulmonary artery combined with surgical bypass [7], or direct reimplantation into the aorta [4, 9]. Contrary to cases published earlier, our patients had development of severe myocardial dysfunction early in life. In patient 1, aortic reimplantation of the anomalous circumflex coronary artery was performed because of poorly developed collaterals. In patient 2, the copious retrograde flow into the pulmonary artery, a well-developed right coronary artery and left anterior descending coronary artery system, and the baby’s size led us to choose ligation.
In conclusion, our observations illustrate that the clinical course of anomalous origin of the cirumflex artery from the pulmonary artery may not always be as favorable as reported in the literature.
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References
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Abstract
Introduction
