Surgical treatment of EMF should be considered a palliative procedure because surgery does not alter the progressive nature of the disease. However, surgical therapy is recommended for patients with EMF and heart failure as it is their only hope of survival.

Congenital supravalvular mitral stenosis is a rare malformation characterized by the presence of a shelf-like fibrous membrane, with 1 or 2 small orifices, covering and obstructing the mitral valve. The membrane is positioned closely to the mitral valve (and sometimes it is attached to it); therefore, a preoperative diagnosis is inevitably difficult, even with the use of biplane echocardiography. Two patients with supravalvular mitral stenosis aged 3 years and 3 months are described. In 1 patient, a preoperative diagnosis was made, and both successfully underwent correction.

De janeiro de 1996 a novembro de 1997, 15 crianças com idade variando de 3 a 11 meses (média: 6 meses) e pesando entre 5 kg a 9 kg (média: 7,2 kg) foram eletivamente submetidas à correção total de tétrade de Fallot. Treze tinham sintomas de hipoxemia, e 2 eram acianóticos. O diagnóstico definitivo foi obtido em todos os casos por ecocardiografia bidimensional. Utilizou-se circulação extracorpórea convencional e hipotermia moderada. Obteve-se proteção miocárdica com infusão na aorta de solução cardioplégica cristalóide gelada e hipotermia tópica do coração. O tempo de CEC variou de 50 min a 125 min (média: 56 min) e o de pinçamento aórtico de 32 min a 86 min (média: 56 min). A correção foi realizada por via transventricular em 14 e por via transatrial em 1. Em 11 casos, utilizou-se enxerto de pericárdio bovino para ampliar a via de saída do ventrículo direito, sendo que em 4 a ampliação foi transanular. Após a correção, o gradiente entre o ventrículo direito e a artéria pulmonar variou de 2 a 25 mmHg (média: 12 mmHg). Não ocorreram óbitos ou complicações significativas nesta série. Conclui-se que a correção total da tétrade de Fallot no primeiro ano de vida pode ser realizada com baixa mortalidade, podendo essa conduta ter vantagens sobre a correção em dois tempos.
From January 1996, to November 1997, 15 consecutive infants ranging in age from 3 to 11 months (mean: 6 months) and weigh ing from 5 to 9 kilograms (mean: 7.2 kilograms) underwent elective total repair of tetralogy of Fallot. Thirteen had symptoms of hypoxemia and two were acyanotic. Definitive diagnosis was established in all cases by two-dimensional echocardiography. Intracardiac correction was accomplished with conventional cardiopulmonary bypass and moderate hypothermia. Myocardial protection was obtained by cold cristaloide cardioplegia infused into the aorta and topical hypothermia of the heart. Bypass time ranged from 50 to 125 minutes (mean: 84 minutes) and aorta cross clamp time ranged from 32 to 86 minutes (mean: 56 minutes). Intracardiac repair was accomplished through right ventriculotomy in 14 cases and by a transatrial approach in one. A right ventricular outflow patch was used in 11 cases but was extended across the pulmonary valve ring only in four. Transannular pulmonary gradient after correction ranged from 2 to 25 mmHg (mean: 12 mmHg). There were no deaths or major complications in this series. It is concluded that intracardiac correction of the tetralogy of Fallot in the first year of life may be accomplished with low mortality and may have advantages over the two-stage approach