[Follow-up of longstanding ventricular septal defects]. / Seguimiento de las comunicaciones interventriculares de larga evolución.

OBJECTIVE: Ventricular septal defect is the most frequently diagnosed congenital heart defect. The prognosis is usually good. The aim of this study was to describe this idea to general pediatricians. MATERIAL AND METHODS: We review the follow-up of 81 patients with ventricular septal defect. Defects that spontaneously closed in the first 12 months of life and those that formed part of a malformative syndrome or a complex congenital heart defect were excluded. RESULTS: Localization was perimembranous, including all defects affecting mainly the septal membrane independent of whether the surrounding tissues were involved, in 66.7 %, muscular in 29.6 % and mixed in 3.7 %. Perimembranous position was more frequent among large and medium-sized defects. Large and perimembranous defects were characterized by holosystolic murmur; in small, muscular defects, murmur was cut off in mid-systole. In 45.8 % of large defects, weight development was delayed, but there was no appreciable effect on height. Generally we observed a tendency to partial closure and to improvement. Surgical closure was required in 9.8 %. CONCLUSIONS: Because of the trend to partial or complete spontaneous closure, the prognosis of ventricular septal defect is generally good.

[Primary pancreatic hydatid cyst and primordial familial dwarfism]. / Quiste hidatídico pancreático primitivo y enanismo primordial familiar.

A 10 years and 7 months old male, was studied for a pathologic, primordial hipogrowing disease, probably due to a recessive and autosomic transmission, because he has got a sister with the same illness, and he was diagnostified of a pancreatic cyst, whind was found in an echographical exploration and established by scanner as intraglandular cyst. There are no others cysts found detected in a complete body-check. The chirurgic findings was a solitary hydatidic intrapancreatic cyst whid was extirpated. The patient's and sister's facial characteristics (big nose, long and thin neck, large forehead), the normal hormonal study in this case, the observation of small bone defects (first finger of the foot thicker than usual, cone epifisis in hands), a normal intelligence and the not accordance between them and the usual in literature described kind, suggest to be a different form the congenital autosomic, recessive dwarfism, not according to the usual one.

Seguimiento de las comunicaciones interventriculares de larga evolución / Follow-up of longstanding ventricular septal defects

Objetivo: La comunicación interventricular (CIV) es la cardiopatía congénita diagnosticada más frecuentemente. El pronóstico, en general, es bueno. El objetivo de este trabajo es llevar esa idea al pediatra general. Material y métodos: Se revisa la evolución de 81 casos de comunicación interventricular. Se excluyeron las que cerraron de forma espontánea en los primeros 12 meses de vida y las que formaban parte de un síndrome polimalformativo o de una cardiopatía compleja. Resultados: La localización perimembranosa, en la cual se englobaron todas las que afectaban principalmente al septo membranoso, independientemente de que tuviesen alguna extensión hacia tejidos vecinos, se encontró en el 66,7 por ciento de casos, la muscular en el 29,6 por ciento y mixta en el 3,7 por ciento. Predominó la primera en las de tamaño grande y mediano. En las grandes y perimembranosas ha sido característico el soplo holosistólico; en las pequeñas musculares, el soplo solamente se oía en la primera mitad de la sístole. Se encontró retraso en el desarrollo ponderal en el 45,8 por ciento de los defectos grandes, sin que fuera apreciable en la talla. En general observamos tendencia al cierre parcial y a la mejoría. Precisaron cierre quirúrgico el 9,8 por ciento. Conclusiones: La habitual tendencia al cierre parcial o total de la mayoría de las CIV hace que el pronóstico de este defecto sea, generalmente, bueno (AU)