CONGRESS ABSTRACT

Patient Overview: A 50-year-old female with no family history of cardiac disease, no cardiovascular risk factors, and no significant comorbidities. Since the age of 13, she has been under cardiological follow-up for frequent ventricular extrasystoles. At the age of 36, she was hospitalized for heart failure (HF) and diagnosed with a DCM with LVEF 30%. HF treatment was initiated, with follow-up showing stable LVEF around 40%. At the age of 49 she was referred to our center, asymptomatic for angina, reporting mild exertional dyspnea (NYHA I-II). ECG showed sinus rhythm, 61 bpm, non-specific repolarization abnormalities. NT-pro-BNP was 279 pg/ml. Echocardiography showed dilated left ventricle, LVEF 48%, moderate-to-severe mitral regurgitation (MR) due to tethering of the posterior leaflet, and regional wall motion abnormalities in the anterior and lateral segments. Cardiac MRI showed absence of LGE. Therefore, a Coronary CT Angiography was performed, revealing an anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). Elective surgical correction was advised. Emergency Event: After five months from the diagnosis, the patient experienced a cardiac arrest and electrical storm (VF), requiring intubation, inotropic and vasopressor support. Post-ROSC ECG showed no ST-segment elevation. However, urgent coronary angiography was performed, which confirmed ALCAPA and did not show obstructive coronary artery disease. Emergency CABG with great saphenous vein to the left anterior descending artery and ligation of the native coronary artery was performed. The postoperative course was uneventful, with gradual recovery of cardiac function. At discharge LVEF was 50%, with mild MR. Discussion: ALCAPA is a rare congenital coronary anomaly. It manifests in two forms: the infantile type, associated with high early mortality due to inadequate collateral circulation, and the adult type, where collateral development allows survival but with higher risk of ischemia due to coronary steal phenomenon, leading to LV dysfunction or SCD. Prompt diagnosis and surgical correction are critical to improving outcomes. This case highlights the critical importance of considering ischemic heart disease, even in young patients and individuals with low cardiovascular risk profiles, when evaluating left ventricular dysfunction. Early identification and timely surgical intervention are essential to prevent life-threatening complications and improve outcomes in these patients.