Fístulas coronarias congénitas, un reto en el manejo terapéutico

Congenital coronary fistulas are uncommon vascular anomalies. They typically progress asymptomatically andare identified in the evaluation a heart murmur. Clinical follow-up and multimodal imaging are crucial for establishing appropriate management for each case. We present a patient who has been followed 25 years of followup for multiple coronary fistulas with significant left-to-left shunting, leading to left ventricular dilation that physiopathologically mimics severe aortic regurgitation. Therapeutic management will depend on clinical evolutionand the anatomical feasibility of closing the fistulous pathways.Las fístulas coronarias congénitas son anomalías vasculares poco comunes. Generalmente son asintomáticas y se diagnostican en el estudio de un soplo cardíaco. El seguimiento clínico y por imagen multimodal es esencial para establecer un manejo adecuado para cada paciente. Presentamos el caso de un varón que lleva 25 de años de seguimiento por múltiples fístulas coronarias con un importante cortocircuito izquierda-izquierda que ha causado una dilatación ventricular izquierda con una fisiopatología similar al de una insuficiencia aórtica severa. El manejo terapéutico dependerá de la evolución clínica y de la factibilidad anatómica para realizar el cierre de los trayectos fistulosos

A Rare Case of Left Ventricular Non-Compaction with Coronary Artery Anomaly Complicated by ST-Elevation Myocardial Infarction and Subcutaneous Defibrillator Implantation

Left ventricular non-compaction (LVNC) is a rare congenital cardiomyopathy caused by arrest of normal endomyocardial embryogenesis and characterized by the persistence of ventricular hypertrabeculation, isolated or associated to other congenital defects. A 33-year-old male, with family history of sudden cardiac death (SCD), presented to our ER with typical chest pain and was diagnosed with anterior STEMI. Coronary angiography showed an anomalous origin of the circumflex artery from the right coronary artery and a critical stenosis on the proximal left anterior descending artery, treated with primary percutaneous coronary intervention. The echocardiogram documented left ventricular severe dysfunction with lateral wall hypertrabeculation, strongly suggestive for non-compaction, confirmed by cardiac MRI. At 3 months follow up, for the persistence of the severely depressed EF (30%) and the family history for SCD, the patient underwent subcutaneous ICD (sICD) implantation for primary prevention. To the best of our knowledge, this is the first case of LVNC associated with anomalous coronary artery origin and STEMI reported in the literature. Arrhythmias are common in LVNC due to endocardial hypoperfusion and fibrosis. sICD overcomes the risks of transvenous ICD, and it is a valuable option when there is no need for pacing therapy for bradycardia, cardiac resynchronization therapy and anti-tachycardia pacing