Very late presentation of anomalous origin of the left coronary artery from the pulmonary artery: case report.

Background: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary anomaly. The enlarged right coronary artery provides retrograde collaterals to supply the left ventricle then preferentially directs into the lower pressure pulmonary artery system causing coronary steal phenomenon. Few patients who survive through adulthood without surgery must have abundant, well-formed functioning collaterals with adequate perfusion of the left ventricle. We present the oldest reported patient with ALCAPA to undergo corrective surgery.

Case Presentation: A 79-year-old woman presented with a 3-months history of worsening shortness of breath and orthopnea. Physical examination discovered a soft continuous murmur at the left upper chest. Transthoracic echocardiography demonstrated an unusual, tubular-like structure inside the interventricular septum with a turbulent flow from color Doppler. Moreover, there was a severe mitral regurgitation from posterior mitral leaflet restriction associated with ventricular remodeling in combination with mitral annular dilatation. Coronary angiography and coronary computed tomography angiography established the diagnostic hallmark of ALCAPA syndrome. Stress cardiovascular magnetic resonance perfusion imaging demonstrated no myocardial ischemia suggesting adequate collateral circulation. Remarkably, there was a left coronary ostial stenosis, which served as a protective mechanism against myocardia ischemia by limiting the steal effect. The patient successfully underwent the ligation of anomalous artery at its origin in combination with bioprosthetic mitral valve replacement. Her postoperative course was uneventful.

Conclusions: This case utilized multimodality imaging for delineating the course of abnormal vessels and helping to formulate therapeutic decision.