Sindrome de corazón izquierdo hipoplásico: análisis de las características de una población en un hospital público / Hipoplastic left heart syndrome: analysis of the characteristics of a population in a public hospital

The authors present a study on prenatal diagnosis of hipoplastic left heart syndrome, and an analysis of the characteristics of a population in a public hospital. The hypoplastic left heart syndrome, and an analysis of the characteristics of a population in a public hospital. The hypoplastic left heart syndrome (HLHS) is a rare congenital heart deficit consisting in the hypodevelopment of the left ventricle, aortic valve, mitral valve and the ascendent aorta. Its incidence is 0,016% to 0.034% of the total of newborns alive. It represents between 1 - 3% of the whole congenital cardiopaties (cc). Its natural evolution is severe and it represents the greater cause of death by CC during the neonatal period. There is a predominance in males between the 55 to 67%. The etiology is multifactorial. Presents risk of recurrency between brothers, and because of that circumstance it could have genetical basis. The ais of this report were to analyze the clinical variables, electrocardiographis, echocardiographic and therapeutical, of a population with diagnosis fo HLHS. The incidence of HLHS in the population studied was 0.06% of life births. The results obtained are detailed (AU)

Dominância coronariana na síndrome da hipoplasia do coração esquerdo / Coronary dominance patterns in hypoplastic left heart syndrome

INTRODUÇÃO: Apesar de a síndrome do coração esquerdo hipoplásico (SCEH) ser extensamente estudada, esta doença ainda apresenta mortalidade elevada se comparada a outras doenças tratadas como fisiologia univentricular. Assim, diferenças morfológicas entre fenótipos dentro do espectro da SCEH podem ser fatores de risco e sua identificação pode auxiliar na escolha terapêutica entre os subgrupos anatômicos. OBJETIVOS: Determinar a forma mais frequente de dominância coronariana em corações com SCEH nos subgrupos com atresia (AM) e estenose mitral (EM). MÉTODOS: Análise da anatomia coronariana de acordo com a distribuição dos ramos epicárdicos e o padrão de dominância, classificadas em direita, esquerda ou balanceada. O grupo controle foi composto por nove peças de corações morfologicamente normais. O grupo SCEH constituiu-se de nove peças com AM e 24 peças com EM. Para análise estatística foi utilizado o teste do qui-quadrado. RESULTADOS: Houve diferença significativa entre os dois grupos em relação à dominância coronariana (x²= 9,298; P=0,01). A dominância esquerda esteve presente em 75 por cento dos casos de EM, e a balanceada só foi observada na EM. No grupo controle, observou-se dominância direita em todos os casos (P<0,01). CONCLUSÕES: A dominância esquerda é mais frequente na SCEH que no grupo controle de corações normais e, na SCEH, a dominância coronariana esquerda é mais frequente no subgrupo com EM.

INTRODUCTION: Although hypoplastic left heart syndrome (HLHS) be extensively studied, this disease still has a high mortality rate compared to other diseases treated as univentricular physiology. In this way, morphological differences between phenotypes within the spectrum of HLHS may be risk factors and their identification can assist in choosing treatment between subgroups. OBJECTIVE: To identify the most prevalent form of coronary artery dominance in hearts with HLHS groups with mitral atresia (MA) and mitral stenosis (MS). METHODS: Analysis of coronary anatomy according to the distribution of epicardial branches and the pattern of dominance, classified as right, left or balanced. The control group was composed of nine pieces of morphologically normal hearts, the HLHS group consisted of 9 pieces with MA and 24 pieces with MS. For statistical analysis we used the x² test. RESULTS: There were significant differences between the two groups in relation to coronary artery dominance (x² = 9.298, P = 0.01). Left dominance was present in 75 percent of cases of MS, and balanced one was only observed in pieces with MS. The control group had right dominance in all cases (P <0.01). CONCLUSIONS:Left dominance is more common in HLHS than in the control group of normal hearts and in HLHS. The left coronary dominance is more frequent in the subgroup with mitral stenosis.

Síndrome de corazón izquierdo hipoplásico: producción experimetal, anatomía quirúrgica y clasificación

Este trabajo monográico se inicío hace 7 años con la producción experimental del Síndrome de Corazón Izquierdo Hipoplásico, realizada en el Laboratorio de Embriología Cardiovascular del Centro Especial Ramón y Cajal de Madrid España, siendo Jefe de Laboratorio la Dra. María Victoria de la Cruz. El estudio morfológico fue elaborado con material de los Museos de piezas anatómicas del Instituto Materno Infantil de la Misericordia de Bogotá (31 corazones) y del Hospital Ciudad Sanitaria Virgen del Rosario de Sevilla España (17 corazones). Por ser colecciones de piezas anatómicas, no fue posible conocer datos de los pacientes, pero se deduce por el tamaño de los corazones, que pertenecían a recien nacidos