Cleft mitral valve in association with anomalous left coronary artery arising from pulmonary artery

An anomalous origin of the left coronary artery from the pulmonary artery is commonly associated with mitral valve insufficiency. Usually this is secondary to left ventricular dysfunction or papillary muscle ischemia. We describe the association of an anomalous left coronary artery from the pulmonary artery with structural abnormalities of the mitral valve (cleft mitral leaflet) in 2 children, both of whom were being investigated for primary mitral valve disease. Both underwent successful operations for coronary transfer and mitral valve repair.     

Emergency ligation of anomalous left coronary artery arising from the pulmonary artery

We report two cases of successful emergency ligation of anomalous left coronary artery arising from the pulmonary artery (ALCAPA) in patients with previous cardiac arrest. Both patients had regained marginal cardiac output after cardiopulmonary resuscitation and had maximal doses of inotropic support. The ALCAPA ligation was then performed as a life-saving procedure in the absence of any kind of mechanical circulatory support.     

Repair of anomalous origin of the left coronary artery arising from right pulmonary artery with rolled-conduit-extended reimplantation in an adult

The left coronary artery arising from the pulmonary artery (ALCAPA) is a rare congenital anomaly. It causes high mortality if untreated in childhood. Only 10% to 15% of patients reach adulthood. We report a successful repair of ALCAPA in an adult with a rolled-conduit-extended-reimplantation technique. The procedure is easily applicable and has technical advantages for creating a longer and wider conduit and may decrease bleeding.     

Mitral valve arcade with concomitant anomalous left coronary artery from the pulmonary artery

The presence of congenital mitral valve arcade with concomitant anomalous coronary artery arising from the pulmonary artery (ALCAPA) is exceedingly rare. We describe a case of a 5-month-old female patient with both ALCAPA and severe mitral regurgitation secondary to mitral valve arcade.     

Successful mitral valve repair and myocardial revascularization in an infant with anomalous origin of the left coronary artery from the pulmonary artery

An 8-month-old girl of anomalous origin of the left coronary artery from the pulmonary artery associated with severe mitral regurgitation underwent radical operation. She had been in serious cardiac and respiratory failure. Aorto-left coronary arterial continuity was established by intrapulmonary arterial tunnel using Takeuchi's technique. Mitral annulus was plicated by modified Kay's technique. Post-operative course was uneventful.     

Surgical treatment of anomalous coronary artery arising from the pulmonary artery

Anomalous coronary arteries arising from the pulmonary trunk is a rare but potentially fatal condition. We report the clinical presentation, surgical treatment and long-term follow-up of seven surgical cases of anomalous left coronary and one case of anomalous right coronary artery arising from the pulmonary artery. Age ranged from 7 months to 13 years (average: 5.09+/-3.7 years) and weight ranged from 7 to 50 kg (average: 19.9+/-8.8 kg). Follow-up was 100% complete: average 78 months (S.D.: 52.7 months). Direct reimplantation was the surgical technique in six cases, Takeuchi procedure in one case and subclavian artery interposition in one case. Concomitant mitral valve repair was undertaken in two cases. In two children the coronary artery anomaly was diagnosed and treated only after a first surgery for other congenital heart anomaly. Left ventricle ejection fraction was restored in those cases of pre-operative dysfunction. Mortality was not observed and all children are asymtomatic and free of reoperation.     

Early recovery of left ventricle following coronary artery bypass grafting and mitral valve repair in a case of anomalous origin of left coronary artery from pulmonary artery

Anomalous origin of Left Coronary Artery from Pulmonary Artery (ALCAPA) is a rare congenital malformation, which can result in myocardial infarction, congestive heart failure and sudden death if left untreated. Reports of myocardial revascularization and Mitral Valve (MV) repair following ALCAPA repair in infants are uncommon. We report a critically ill infant with ALCAPA needing myocardial revascularization and MV repair after which there was early recovery of Left Ventricular (LV) function. At two years follow up, the child is clinically well; the postoperative cardiac catheterization shows good flow in the Left Internal Mammary Artery (LIMA) and Left Anterior Descending artery (LAD), good LV function and competent mitral valve.     

Successful mitral valve replacement and myocardial revascularization in an adult with anomalous origin of the left coronary artery from the pulmonary artery

A 35-year-old white woman with anomalous origin of the left coronary artery from the pulmonary artery had infective endocarditis and severe mitral regurgitation. She survived mitral valve replacement and occlusion of the anomalous left main stem, but subsequently deterioration of her condition necessitated urgent saphenous vein bypass grafts to the left anterior descending and circumflex coronary arteries. Pulmonary hypertension with raised right ventricular end-diastolic pressure probably compromised collateral flow from the right to the left coronary systems.     

Repair of an anomalous left coronary artery

We describe a patient with an anomalous single coronary artery who presented with a syndrome of atypical chest pain. Coronary angiography revealed a single right coronary ostium, with a narrowed left coronary artery originating at the right coronary ostium. The proximal portion of the left coronary artery that was narrowed was noted to run in the aortic wall. We describe the operative management of this patient using ostial remodeling.     

The need for follow-up after surgical correction of anomalous left coronary artery arising from the pulmonary artery

Four patients are described with an anomalous origin of the left coronary artery from the pulmonary artery. Three were treated by ligation of the abnormal left coronary artery at its anomalous origin; one of them died during surgery. One patient was treated with reimplantation of the left coronary artery into the aorta. Of the survivors 2 are in normal health, but 1 developed aortic valve incompetence after reimplantation of the left coronary artery into the aorta and 1 had persistent left ventricular wall motion abnormalities and developed mitral valve prolapse. The third survivor has impaired health with diffuse left ventricular wall motion abnormality. Careful follow-up after surgery for an anomalous left coronary artery arising from the pulmonary artery is necessary for secondary prevention of problems due to incomplete recovery of myocardium or problems as a consequence of surgical therapy.     

Anomalous left main coronary artery arising from the pulmonary artery in an adult: Treatment by direct reimplantation

We herein report the case of a 37-year-old woman in whom an anomalous origin of the left coronary artery from the pulmonary artery was surgically corrected. A magnetic resonance angiogram showed the left main coronary artery connecting to the right posterior portion of the pulmonary trunk, and exercise-stressed thallium-201 perfusion scintigrams demonstrated a large reversible anterior defect. She was successfully treated by direct aortic reimplantation of the abnormal left coronary artery. We were able to obtain a sufficient length of the left main trunk by excising the large cuff of pulmonary artery wall surrounding the ostium of the anomalous left coronary artery while transecting the pulmonary artery. Postoperative angiograms demonstrated a widely patent left coronary artery, a decrease in the size of the right coronary artery, and no collaterals, and exercise-stressed thallium-201 perfusion scintigrams demonstrated no remaining ischemic defect at all. Direct aortic reimplantation is an ideal operation but is still limited by the anatomical position of the left coronary artery. In this case, magnetic resonance angiography was an excellent method for deciding the optimum operative procedure for the anomalous left coronary artery. In addition, exercise thallium-201 scintigraphy was found to be useful in recognizing the revascularized effect of the left ventricle.     

Isolated anomalous origin of right coronary artery from the main pulmonary artery

The anomalous origin of the right coronary artery (ARCA) from the main pulmonary artery (MPA) is a rare congenital cardiac malformation and usually associated with other cardiac anomalies. Most patients with isolated ARCA from MPA remain asymptomatic, but they may develop myocardial ischemia and even sudden death. We reported an asymptomatic 7-year-old boy referred for evaluation of a heart murmur. Isolation of ARCA from MPA was diagnosed by echocardiography and then confirmed by cardiac catheterization and angiography. The right coronary artery was re-implanted into the ascending aorta. A preoperative thallium-201 myocardial perfusion showed a myocardial ischemia pattern in the anterolateral septal area after a dipyridamole stress test; the ischemia was completely resolved after surgery.     

Anomalous left coronary artery from pulmonary artery with mitral stenosis

The usual presentation of anomalous left coronary artery from pulmonary artery is severe left-sided heart failure and mitral valve insufficiency presenting during the first months of life. The manifestations of left heart failure may be masked if pulmonary artery pressure remains high. We believe this is a rarest of rare case of anomalous left coronary artery from pulmonary artery with severe mitral stenosis and pulmonary hypertension in which pulmonary hypertension, along with good collateral circulation helped to preserve left ventricular function.