RESUMEN
Aortopulmonary window (APW) is an abnormal congenital connection between the main pulmonary artery (MPA) and the ascending aorta, with intact aortic and pulmonary valves, leading to heart failure or, if not repaired early, to pulmonary vascular obstructive disease. We report the rare case of an asymptomatic adult with an unrestrictive APW, whose pulmonary arterial hypertension was remarkably still reversible, permitting successful repair.
Asunto(s)
Defecto del Tabique Aortopulmonar/diagnóstico , Hipertensión Arterial Pulmonar , Defecto del Tabique Aortopulmonar/diagnóstico por imagen , Defecto del Tabique Aortopulmonar/cirugía , Diagnóstico Diferencial , Ecocardiografía , Cardiopatías Congénitas/diagnóstico , Cardiopatías Congénitas/diagnóstico por imagen , Cardiopatías Congénitas/cirugía , Humanos , Imagen por Resonancia Magnética , Masculino , Tomografía Computarizada por Rayos X , Adulto JovenRESUMEN
Aorto-ventricular tunnel (AoVT), a rare congenital anomaly, is a channel originating in the ascending aorta just above the sinotubular junction and leading to the cavity of the left ventricle (AoLVT), or, rarely, the right (AoRVT). This study reviews our collective 30-year experience with the surgical treatment of AoVT. Data were submitted by 15 participating centers on 42 patients who underwent correction of AoVT between 1987 and 2018. Of these, 36 had AoLVT, and 6 AoRVT. The tunnel originated above the right coronary sinus in 28 (77.8%) patients. For AoLVT, most operations were performed early (median age 25 days, range: 1 day-25 years). In contrast, AoRVT was diagnosed and repaired later (median age 6 years, range: 1 month-12 years). Surgically important coronary ostial displacement was common. Patch closure of the aortic orifice only was the commonest surgical repair for AoLVT (23 patients), while in AoRVT, both orifices or only the ventricular one was closed. Aortic valvar insufficiency, severe or moderate, coexisted in 11 (30.5%) patients with AoLVT, and aortic valvuloplasty was performed in 8, mainly due to aortic valve stenosis. Aortic valvar insufficiency at discharge ranged from trivial to mild in almost all patients. Early mortality was 7.14%, with 3 patients with AoLVT succumbing to cardiac failure. There were 2 early reoperations and 1 late death. AoVT is a rare malformation. AoLVT usually necessitates surgery in early life. AoRVT is rarer, diagnosed and repaired later in life. Surgical repair by patch closure, with concomitant aortic valve repair as needed, is associated with good results.
Asunto(s)
Aorta/cirugía , Válvula Aórtica/cirugía , Procedimientos Quirúrgicos Cardíacos , Cardiopatías Congénitas/cirugía , Ventrículos Cardíacos/cirugía , Adolescente , Adulto , Aorta/anomalías , Aorta/diagnóstico por imagen , Aorta/fisiopatología , Válvula Aórtica/diagnóstico por imagen , Válvula Aórtica/fisiopatología , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Procedimientos Quirúrgicos Cardíacos/mortalidad , Niño , Preescolar , Europa (Continente) , Femenino , Cardiopatías Congénitas/diagnóstico por imagen , Cardiopatías Congénitas/mortalidad , Cardiopatías Congénitas/fisiopatología , Ventrículos Cardíacos/anomalías , Ventrículos Cardíacos/diagnóstico por imagen , Ventrículos Cardíacos/fisiopatología , Hemodinámica , Humanos , Lactante , Recién Nacido , Masculino , Complicaciones Posoperatorias/mortalidad , Complicaciones Posoperatorias/cirugía , Recuperación de la Función , Estudios Retrospectivos , Factores de Tiempo , Resultado del Tratamiento , Estados Unidos , Función Ventricular Izquierda , Adulto JovenRESUMEN
The coronavirus disease 2019 (COVID-19) pandemic currently gripping the globe is impacting the entire health care system with rapidly escalating morbidities and mortality. Although the infectious risk to the pediatric population appears low, the effects on children with congenital heart disease (CHD) remain poorly understood. The closure of congenital heart surgery programs worldwide to address the growing number of infected individuals could have an unintended impact on future health for COVID-19-negative patients with CHD. Pediatric and congenital heart surgeons, given their small numbers and close relationships, are uniquely positioned to collectively assess the impact of the pandemic on surgical practice and care of children with CHD. We present the results of an international survey sent to pediatric and congenital heart surgeons characterizing the early impact of COVID-19 on the care of patients with CHD.