Cláudio A Gomes

Surgery for endomyocardial fibrosis revisited

OBJECTIVE To identify life expectancy after surgery for endomyocardial fibrosis (EMF) and the events that influence it. METHODS Eighty-three patients with EMF underwent endocardial decortication and atrioventricular valve replacement or repair, between December 1977 and December 1997. There were 66 (79.6%) female and 17 (20.4%) male patients, ranging in age from 4 to 59 years (mean, 31). Thirty-seven (44.5%) had biventricular disease, 34 (41.0%) had disease of the right ventricle alone and 12 (14.5%) had EMF confined to the left ventricle. All were in functional class III or IV (New York Heart Association classification). RESULTS Sixty-eight (81.9%) patients survived the operation and were followed up for periods ranging from 2 months to 17 years. The total follow-up time was 6290 patient/months (mean, 92 months). There were 15 late deaths, but in six, the cause was not related to the underlying disease. Four (5.8%) patients presented recurrence of the fibrosis and were reoperated on and in six (8.8%), EMF appeared in the other ventricle. Five (7.3%) patients were reoperated on to replace either a valve prosthesis or a native valve which had been preserved during the first procedure. Only 24 (45%) of the 53 surviving patients are in functional class I or II. The actuarial probability of survival at 17 years, including operative mortality, was 55%. CONCLUSION 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.

Transplante cardíaco: a experiência do Instituto do Coraçäo de Pernambuco com 35 casos

Material and Methods: From August/ 1991 to February/ 2000, 35 orthotopic heart transplants were performed at the Heart Institute of Pernambuco. There were 29 male and 6 female patients ranging in age from 15 to 69 years (mean: 46.9 yr.). Recipient diagnoses included ischemic cardiomyopathy in 18, idiopatic dilated cardiomyopathy in 13, mixomatous disease in 2, rheumatic valvar disease in 1 and Chagas` disease in 1. All were in the final stage of heart failure (functional class III or IV of the New York Heart Association) and the ejection fraction ranged from 16 to 27% (mean: 20.9 ± 2.9). Seventeen (48.5%) patients had had previous cardiac operations. The classical surgical technique described by Lower and Shumway, in 1960, was used in all cases. Mean graft ischemic time was 91 ± 21 minutes (ranged from 60 to 180 minutes). Results: There were 7 (20%) deaths in the early postoperative period. Non-fatal postoperative complications occurred in other 13 patients including 5 episodes of rejection. Mean follow-up period of the 28 survivors was 31.2 months (ranged from 1 to 68 mo.) There were 14 late deaths owing to rejection (4), infection (4), graft coronary artery disease (2), chronic renal failure (2) and sudden death (2). The actuarial survival estimated is 70% at 1 year and 30% at 5 years. Conclusion: It is concluded that cardiac transplantation program is feasible in our community but poorer results in comparison to international experience should be expected owing to social problems of the recipient population.

Supravalvular congenital mitral stenosis.

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.

Profilaxia da fibrilação atrial no pós-operatório imediato de cirurgia coronária: comparação entre propranolol e sotalol utilizados em baixas doses

OBJETIVO: Comparar a eficacia de dois protocolos terapeuticos usando baixas doses de betabloqueadores para prevencao de fibrilacao atrial no pos-operatorio imediato de cirurgia coronaria. METODOS: 154 pacientes, submetidos a cirurgia coronaria, foram randomizados em dois grupos: no grupo I (n = 72), os doentes receberam Sotalol (80 mg/dia) e, no grupo II, (n = 82) usaram Propranolol (40 mg/dia), ambos iniciados no primeiro dia de pos-operatorio. Na unidade de terapia intensiva, o eletrocardiograma foi continuamente observado por monitorizacao a beira do leito. Adicionalmente, eletrocardiograma nas 12 derivacoes foi realizado no pre-operatorio, nos 1o, 3o e 6o dias de pos-operatorio e sempre que os pacientes apresentaram queixas de palpitacoes ou surgiram sinais de arritmias (pulso irregular ou frequencia cardiaca acima de 100 bpm). RESULTADOS: Fibrilacao atrial foi documentada em 3 (4,2%) pacientes do grupo I (Sotalol) e em 8 (9,8%) do grupo II (Propranolol). CONCLUSAO: A incidencia geral de fibrilacao atrial em pacientes que receberam baixas doses de Sotalol e Propranolol apos cirurgia coronaria foi baixa (7,1%), tendo alcancado o menor valor (4,2%) no grupo de pacientes que receberam Sotalol, embora nao tenha sido observada uma diferenca estatisticamente significante entre os dois grupos (p= 0,221).

Correção total da tétrade de Fallot no primeiro ano de vida

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.

Diagnóstico e tratamento cirúrgico do teratoma intrapericárdico

We report two cases of intrapericardial teratoma, a rare cardiac tumor, usually found in neonates and young infants, which may cause respiratory distress, large pericardial effusions and cardiac compression leading to intra-uterine or neonatal death. Both cases were diagnosed noninvasively by echocardiogram, in a 3 month-old girl with signs of cardiac tamponade, and by fetal echocardiography, in a 38 week pregnant woman. Successful surgical removal of the tumor was undertaken in both patients at the ages of 3 months and 3 days of life, respectively. Histology confirmed the diagnosis of teratoma. It is emphasized the diagnostic accuracy of achocardiography and the importance of early surgical treatment in the management of intrapericardial teratoma.

A cirurgia da endomiocardiofibrose revisitada

Eighty-three patients with endomyocardial fibrosis (EMF) underwent endocardial decortication and atrioventricular valve replacement or repair between December, 1977, and December, 1997. There were 66 (79.6%) female and 17 (20.4%) male patients, ranging in age from 4 to 59 years (mean, 31). Thirty-seven (44.5%) had biventricular disease 134 (41.0%) had disease of the right ventricle alone and 12 (14.5%) had EMF confined to the left ventricle. All were in functional class III or IV (New York Heart Association classification). Sixty-eight (81.9%) patients survived the operation and were followed-up for periods of time ranging from 1 month to 17 years. The total follow-up time was 6,290 patient/months (mean, 92 months). There were 15 late deaths but in 6 the cause was not related to the underlying disease. Four (5.8%) patients presented recurrence of the fibrosis and were reoperated and in 6 (8.8%) EMF appeared in the other ventricle. Five (7.3%) patients were reoperated to replace either a valve prosthesis or a native valve which had been preserved during the first procedure. Only 24 (45%) of the 53 living patients are in functional class I or II. The actuarial probability of survival at 17 years, including operative mortality, was 55%. In conclusion, surgical treatment of EMF should be considered a palliative procedure because surgery does not stop the progressive nature of the disease. However, surgical therapy is recommended for patients with EMF and heart failure as it is the only hope for them.

Operação de Rastelli utilizando-se conduto valvulado de pericárdio bovino: experiência inicial

O presente trabalho descreve a experiencia inicial com a operacao de Rastelli, utilizando-se um conduto valvulado de pericardio bovino preservado em glutaraldeido. O enxerto consta de um conduto, que serve para reconstruir a via de saida do ventriculo direito e o tronco pulmonar, com uma valvula tricuspide suturada em seu interior sem anel de suporte, permitindo, assim, um mecanismo de fluxo unidirecional. De maio de 1986 a outubro de 1987, 5 criancas foram submetidas a operacao de Rastelli empregando-se este tipo de conduto valvulado. Tres eram do sexo feminino e 2, do masculino, variando a idade de 1 a 8 anos x 5). Tres pacientes tinham transposicao das grandes arterias, comunicacao interventricular (CIV) e estenose subpulmonar. Uma crianca era portadora de atresia pulmonar com CIV e de um shunt previo tipo Waterston e, finalmente, a outra tinha um tronco arterial comum tipo II. Dois doentes faleceram de causas nao relacionadas ao tipo de conduto valvulado utilizado. O conduto valvulado de pericardio bovino preservado em glutaraldeido mostrou excelente flexibilidade, facilitando o manuseio cirurgico e permitindo perfeita adaptabilidade dentro do saco pericardico. A ausencia de anel de suporte tem a vantagem de eliminar qualquer gradiente e de abolir o turbilhonamento do sangue - causas reconhecidas de calcificacao.

Dez anos de cirurgia da endomiocardiofibrose: o que aprendemos?

In the last 10 years(1977-1987), 53 patients with endomyocardial fibrosis were submitted to endocardial decortication and atrioventricular valve replacement. There were 42 female and 11 male patients, ranging in age from 11 to 59 years (mean 31). The patients were divided into three groups: Group I included 25 patients with bilateral disease; Group II consisted of 23 patients with endomyocardial fibrosis of the right side; and Group III included 5 patients with the disease confined to the left ventricle. All were in Functional Class III or IV (New York Heart Association classification). The 30-day mortality was 20.7% (11 cases). The remainder 42 patients survived the early postoperative period which was quite stormy in 21 (39.6%). There were 10 (18.8%) late deaths. One patient was reoperated two years later to replace the mitral valve which had been preserved during the first intervention. Among the 32 survivors (mean follow-up 4.1 patients/year), 22 (41,5%) are in Functional Class I or II. The actuarial curve showed that the 5-year probability of survival is 75%. Despite the high early and late mortality, and the fact that only 41.5% of the operated patients had good clinical improvement in the late postoperative period, surgical treatment is, at present, the only hope for patients with endomyocardial fibrosis. Technical aspects of the operation are outlined.

Pentalogia de Cantrell: relato de caso

The authors describe the surgical cure of a case of Pentalogy of Cantrell, a rare anomaly characterized by congenital lesions involving the abdominal wall, the inferior third of the sternum, the ventral diaphragm, the pericardium and the heart.