[Hypoplastic left heart syndrome. Clinical evaluation of 40 patients]. / Síndrome de hipoplasia do coração esquerdo, avaliação clínica de 40 casos.

PURPOSE: To study the hypoplastic left heart syndrome (HLHS) emphasizing clinical aspects and therapeutic measures. PATIENTS AND METHODS: Forty patients, 24 male, ages ranging between one to 120 days (mean six days). Diagnosis was established by clinical elements, eletrocardiogram, chest X-ray and Doppler echocardiogram. Eight patients underwent cardiac catheterization. In 21 patients anatomical diagnosis was confirmed by necropsy. RESULTS: Twenty-two patients (55%) died, in spite of all possible clinical measures. In 16 (40%) palliative surgery was performed, Norwood technique in 14 and pulmonary artery banding with Dacron graft interposition between pulmonary trunk and brachiocephalic trunk in two. Immediate postoperative results were favorable in three patients (Norwood technique). Both patients underwent the other described technique died at 12th and 30th postoperative day due to infection. CONCLUSION: HLHS is a severe cardiac anomaly with a fast downhill evolution mostly at first week of life. The good results depend basically of early diagnosis as well as of an adequate clinical management for an appropriate time in indication of the palliative surgical technique.

[Infusion of prostaglandin E1 in ductus-dependent congenital heart diseases. Analysis of 47 cases]. / Infusão de prostaglandina E1 em cardiopatias congênitas canal-dependentes. Análise de 47 casos.

Prostaglandin E1, used since 1975, has changed favorably the clinical and surgical prognosis of neonates with congenital heart defects, mainly those with a variety of ductus-dependent defects. Due to recent modifications that have been observed with this drug as far as dosage, side effects, duration of venous infusion and the response of the different cardiac anomalies, this study was undertaken on 47 neonates evaluated with drug infusion between December 1985 and April 1988. The ages of the patients varied from 12 hours to 70 days (median age of 10.3 days), body weight ranged from 1990 to 4430 g (median of 3005 g). The average dose corresponded to 0.021 mcg/kg/min, varying between 0.013 to 0.0089 mcg/kg/min. The therapy was considered effective in 36 (76.5%) patients, evaluated by clinical improvement, increase of arterial oxygen saturation greater than 15 vol. O2% and increased ductus diameter measured by echocardiographic study. In the correlation between the therapeutic result and the patient age, the greatest elevation of arterial oxygen saturation occurred until 21 days of age, especially up to 7 days of age where the elevation in this period was of 24.5 vol. O2%. The cardiac defects that best responded to PGE1 were pulmonary atresia with or without ventricular septal defect, Ebstein's anomaly, tricuspid atresia, pulmonic stenosis, double outlet right ventricle, and those that the arterial oxygen saturation increase was less than 10 vol. O2% such as the hypoplastic left heart syndrome, tetralogy of Fallot and transposition of the great arteries. (ABSTRACT TRUNCATED AT 250 WORDS)

An infant with two "half-hearts" who survived for five days: a clinical and pathological report.

The case of a five-day-old boy with two "half-hearts," diagnosed at necropsy but not clinically, is presented. Each "half-heart" was totally separated from the other and each had a single atrium and ventricle. The two "half-hearts" were enveloped in a common pericardium. The left-sided atrium and the right-sided atrium had the morphologic characteristics of left and right atrium, respectively, but the morphology of the two ventricles was less characteristic. There were double truncuses and double superior and inferior venae cavae, too. Pulmonary venous drainage was totally anomalous; splenic abnormalities were not found. An embryologic lack of fusion of the primitive cardiac tubes is a possible starting point for these malformations.