RESUMO
The genetics of human congenital coronary vascular anomalies (hCCVA) remains largely underresearched. This is surprising, because although coronary vascular defects represent a relatively small proportion of human congenital heart disease (CHD), hCCVAs are clinically significant conditions. Indeed, hCCVA frequently associate to other congenital cardiac structural defects and may even result in sudden cardiac death in the adult. In this brief chapter, we will attempt to summarize our current knowledge on the topic, also proposing a rationale for the development of novel approaches to the genetics of hCCVA.
Assuntos
Anomalias dos Vasos Coronários , Humanos , Anomalias dos Vasos Coronários/genética , Predisposição Genética para Doença/genética , Cardiopatias Congênitas/genéticaAssuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Transposição dos Grandes Vasos/cirurgia , Ponte Cardiopulmonar , Cianose/etiologia , Oxigenação por Membrana Extracorpórea , Humanos , Recém-Nascido , Masculino , Complicações Pós-Operatórias/terapia , Choque/etiologia , Choque/terapia , Transposição dos Grandes Vasos/diagnóstico por imagem , UltrassonografiaRESUMO
INTRODUCTION AND OBJECTIVES: Minimally invasive approaches in less-complex cardiac procedures can avoid unpleasant cosmetic results. Moreover, surgery can be scheduled in younger patients. In previous papers, we compared submammary and midline sternotomy. We present our initial experience with an axillary, compared to submammary, approach to repair atrial septal defects under extracorporeal circulation. METHODS: 20 patients are included: 10 in the submammary group (7 ostium secundum, 2 sinus venosus, 1 ostium primum) and 10 in the axillary group (7 ostium secundum, 3 sinus venosus). Mean age and weight are 6.4±3.62 years (range 3-13) and 23.5±8.74 Kg (range 12-38) in the submammary group, and 5.5±2.04 years (range 3-9) and 19.7±5.88 Kg (range 14-29) in the axillary one, respectively. Muscles are spared (pectoralis in submammary and latissimus in axillary). The whole procedure (cannulation and correction) is performed through a single incision, with no side ports. RESULTS: No residual defects were found at discharge. Surgical approach maneuvers are more cumbersome through the axillary than the submammary approach. In a peer comparison, extracorporeal circulation and cross-clamp time were similar in both groups (P>.05). CONCLUSIONS: 1. Axillary approach is as safe as submammary access in selected patients and for defects approached through the atrium. 2. Cosmetic result is excellent.