[Hypoplastic left heart syndrome. Clinical evaluation of 40 patients].
OBJECTIVE
To study the hypoplastic left heart syndrome (HLHS) emphasizing clinical aspects and therapeutic measures.


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.


CONCLUSIONS
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.
Bhering
M A
MA
Instituto do Coração do Hospital das Clínicas, FMUSP, São Paulo.
Atik
E
E
Iwahashi
E R
ER
Albuquerque
A M
AM
Aielo
V D
VD
Barbero-Marcial
M
M
Rati
M
M
Ribeiro
I G
IG
Ebaid
M
M
D001011:Aorta / Q000002:abnormalities* / Q000000981:diagnostic imaging; D015150:Echocardiography, Doppler; D004562:Electrocardiography; D005260:Female; D006346:Heart Septum / Q000601:surgery; D006352:Heart Ventricles / Q000002:abnormalities* / Q000000981:diagnostic imaging; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D010166:Palliative Care; D011379:Prognosis; D013577:Syndrome
Síndrome de hipoplasia do coração esquerdo, avaliação clínica de 40 casos.