From asymptomatic adult patient to cardiopulmonary resuscitation – treatment of ALCAPA with total arterial myocardial revascularisation and mitral valve repair

Abstract

BackgroundAnomalous left coronary artery originating from the pulmonary artery (ALCAPA), is a unique congenital anomaly, comprising only 0.24–0.46% of all congenital cardiac anomalies. Late presentations, ranging from asymptomatic cases to sudden cardiac arrest, are exceptionally rare. This unique case highlights the complexity of managing ALCAPA in adulthood and underscores the necessity of a comprehensive surgical approach addressing both coronary and valvular issues.PresentationA 34-year-old female patient, who had been diagnosed with ALPACA in her early childhood, suffered sudden cardiac arrest at work, followed by out-of-hospital resuscitation. The patient had been followed-up regularly until adolescence, however, she had refused surgery. In the diagnostic work-up following successful resuscitation severe mitral valve regurgitation was additionally revealed. Subsequently, the patient underwent surgery, involving coronary artery bypass grafting (CABG), using bilateral internal mammary arteries, and mitral valve repair, with an excellent postsurgical result. At 16-month follow-up, the patient was asymptomatic and quality of life had significantly improved.ConclusionThis rare case initially presented as silent myocardial ischemia, resulting in reduced left ventricular function and secondary mitral incompetence. Surgical treatment of ALCAPA in adults poses greater challenges and a higher risk than in children. CABG procedure offers an excellent prognostic therapeutic strategy, since this procedure is a routine in adult cardiac surgery.