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J Thorac Cardiovasc Surg 2007;134:518-520
© 2007 The American Association for Thoracic Surgery
Brief Communication |
a Cardiothoracic Department, Azienda Ospedaliera Universitaria Pisana, Pisa, Italy
b Department of Radiology, University Hospital of Pavia, Pavia, Italy.
Received for publication March 26, 2007; accepted for publication April 20, 2007. * Address for reprints: Pietro Bajona, MD, Cardiothoracic Department, Azienda Ospedaliera Universitaria Pisana, Via Paradisa 2, 56124 Pisa, Italy. (Email: pietro.bajona{at}libero.it).
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Anomalous origin of the left coronary artery (LCA) from the pulmonary artery (ALCAPA) in adulthood is rare, and clinical presentation depends on collateral circulation between and to the coronary arteries. We present an adult patient with ALCAPA with systemic collateral supply to the LCA and a giant right coronary artery (RCA) aneurysm who was successfully treated by means of closure of the anomalous origin of the left main artery and repair of the aneurysm.
A 44-year-old male physician was referred to our hospital because of a 6–month history of dizziness and increasing dyspnea on exertion. He had been a rugby player since childhood and had played semiprofessionally until a year before. He smoked 10 cigarettes per day, and he had no other risk factors for coronary artery disease. The results of physical examination were normal. Blood pressure was within normal limits. Laboratory test results were normal. Chest radiography revealed the presence of a moderate cardiomegaly and opacity overlapping the right heart border and the right pulmonary hilum. Electrocardiography revealed sinus rhythm and left ventricular hypertrophy with normal patterns of PR, QT, and ST segment.
Transthoracic echocardiography showed a 7.0 x 9.0–cm aneurysm involving the proximal RCA and wide ectasia of the coronary arteries. At Doppler scanning, a fistula between the left main artery and the main pulmonary artery (MPA) was observed.
Coronary angiography showed that the LCA was supplied through aneurysmal collateral branches from the RCA and from both anomalous branches of the right internal thoracic artery (RITA) and bronchial artery (BA; Figure 1). Retrograde filling of the LCA and MPA was observed by injecting the distal RCA. Chest computed tomographic scanning with contrast demonstrated a large aneurysm of the proximal portion of the RCA. The distal RCA was dilated and tortuous (Figure 2). At the time of the operation, a giant RCA aneurysm and a dilated and tortuous anterior interventricular artery were found. The aneurysm was located laterally to the ascending aorta and anterior to the superior vena cava. Both abnormal branches from the RITA and BA were not visible. Hypothermic (26°C) right femorofemoral cardiopulmonary bypass was established. The ascending aorta was clamped, and antegrade, cold, intermittent blood cardioplegia was delivered into the aortic root.
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The RCA aneurysm was transected and replaced with a 3-cm-long segment of an 8-mm-diameter Dacron graft. After patient rewarming, deairing was performed, the aortic crossclamp was released, and the operation was completed in a routine fashion.
Intraoperative transesophageal echocardiography showed a normal left ventricular volume with a normal ejection fraction. The postoperative course was uneventful. Pathologic examination of the resected RCA aneurysm revealed atherosclerotic change and chronic inflammatory tissue. Chest computed tomography was performed 10 months postoperatively and showed optimal reconstruction of the RCA.
At the 1-year follow-up, the patient was found to be symptom-free without medication.
ALCAPA is a rare congenital anomaly that is often referred to as Bland–White–Garland syndrome.1
It occurs in approximately 1 in 300,000 children. Most infants die within the first year of life without treatment. Survival beyond infancy depends on the development of adequate collateral circulation from the RCA or from another source to the LCA. However, even in the patients who survive to adulthood, sudden death frequently occurs.2 Surgical intervention is recommended in all patients, even in asymptomatic adults with no objective evidence of ischemia. Most patients will have significant RCA dilatation.3 This is likely related to the long-standing increased flow related to the left anomalous coronary artery, which, perhaps, increases the diameter over the course of time. However, a huge RCA associated with ALCAPA is an unusual presentation.4 The asymptomatic course of our case seems to be related to an optimal balance between collateral flow from the RCA and systemic blood supply from the RITA and BA branches. There are 4 similar cases describing ALCAPA with possible systemic collateral supply, but only in one of them was supply from the BA confirmed.5 To the best of our knowledge, there are no described cases with blood supply from RITA branches. In this case, a simple ligation was performed to avoid coronary flow imbalance and thrombosis in the dilated coronary arteries and because of the patient’s well-developed systemic collateral arteries. The present observation is unique in that it describes a case of ALCAPA with LCA circulation supplied by two abnormal systemic branches and a huge RCA aneurysm in adulthood.
References
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