Síndrome de hipoplasia do coraçäo esquerdo: avaliaçäo clínica de 40 casos / Hypoplastic left heart syndrome. Clinical evaluation of 40 patients

RESUMO

Estudar a síndrome de hipoplasia do coraçäo esquerdo (SHCE), enfatizando aspectos clínicos e conduta terapêutica. Quarenta pacientes, 24 do sexo masculino, com idades entre um e 120 (média seis) dias. O diagnóstico foi baseado no quadro clínico, eletrocardiograma. Oito pacientes foram submetidos a caterismo cardíaco. Em 21 casos houve confirmaçäo por estudo necroscópico. Vinte e dois (55%) pacientes evoluiram para o óbito apesar das medidas clínicas instituídas. Em 16 (40%) realizou-se cirurgia paliativa, técnica de Norwood em 14 e bandagem do tronco pulmonar com interposiçäo de tubo de dacron entre este e o tronco braquiocefálico em dois. Dos pacientes submetidos a cirurgia de Norwood, três tiveram resultados imediatos favoráveis. Os pacientes submetidos ao outro procedimento citado, faleceram nos 12§ e 30§ dias de pós-operatório, em conseqüência de infecçäo. A SHCE é cardiopatia grave, com insuficiência cardíaca de manifestaçäo precoce (geralmente na primeira semana de vida) e de evoluçäo inexorável para o óbito quando se utilizam apenas as medidas clínicas habituais. O sucesso das técnicas cirúrgicas paliativas depende basicamente do diagnóstico precoce e do manuseio clínico adequado

ABSTRACT

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.