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Los albinos tienen predisposición al padecimiento de determinados tipos de cáncer cutáneo, pero el melanoma no es común. Entre los pocos artículos que documentan su aparición en estos pacientes, la afección gastrointestinal por melanoma aparece con relativa frecuencia, ya sea en forma de tumor primario o metástasis. Se presenta el caso de una paciente mujer de 70 años con una lesión gástrica y otra intestinal, detectadas mediante tomografía computada. El estudio anatomopatológico fue compatible con el diagnóstico de melanoma amelanótico. Pese a su escasa incidencia en albinos, el melanoma es una opción que no puede descartarse. Es importante, además, revisar los diferentes componentes del tracto gastrointestinal ante la existencia (o no) de un melanoma primario conocido...

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Los albinos tienen predisposición al padecimiento de determinados tipos de cáncer cutáneo, pero el melanoma no es común. Entre los pocos artículos que documentan su aparición en estos pacientes, la afección gastrointestinal por melanoma aparece con relativa frecuencia, ya sea en forma de tumor primario o metástasis. Se presenta el caso de una paciente mujer de 70 años con una lesión gástrica y otra intestinal, detectadas mediante tomografía computada. El estudio anatomopatológico fue compatible con el diagnóstico de melanoma amelanótico. Pese a su escasa incidencia en albinos, el melanoma es una opción que no puede descartarse. Es importante, además, revisar los diferentes componentes del tracto gastrointestinal ante la existencia (o no) de un melanoma primario conocido.(AU)

Albinos are predisposed to suffer certain types of skin cancer, but not usually melanoma, with just a few reports documenting it .Gastrointestinal tract is a relatively frequent site of melanoma occurrence, weather as a primary tumour or metastasis. We report a case of a 70 year-old female patient who underwent a computed tomography where a gastric and a bowel tumor were found. The pathology study was compatible with the diagnosis of an amelanotic melanoma. Despite that the diagnosis of melanoma in albine patients is a rare finding, it must be considered. It is also important to search through the digestive system even if there is no primary melanoma known.(AU)

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BACKGROUND: Heart transplantation (HT) as a therapeutic option for end-stage chronic Chagas' heart disease (CCHD) is controversial. Reactivation of Trypanosoma cruzi infection and recurrence of the disease in the allograft are likely to occur. Furthermore, active myocarditis has been reported to predispose patients to an increased incidence and severity of rejection. METHODS AND RESULTS: We prospectively investigated the long-term follow-up of 10 patients with CCHD who underwent HT. Immunosuppression was based on cyclosporine A and azathioprine. T cruzi reactivation was prevented with benzonidazole. Besides allograft rejection surveillance, T cruzi infection was monitored through blood tests, myocardial biopsies, and serological tests. Over a mean follow-up period of 34 +/- 38 months (range, 73 to 124 months), 7 patients are alive and in NYHA functional class I. Life expectancy was 78% for the second year and 65% for 10 years. Rejection was less frequent in chagasic than in age- and sex-matched control patients (mean +/- SD, 1.60 +/- 1.26 versus 5.70 +/- 1.89 episodes per patient, respectively; P = .0001); decreased severity of rejection was also observed (P = .006). T cruzi parasitemias detected on three occasions were successfully treated with benzonidazole. There were no signs of recurrence of the disease in the allograft. CONCLUSIONS: These results suggest an important role of HT in the treatment of CCHD. There was a low frequency of T cruzi infection reactivation and no signs of recurrence of the disease in the allograft. The surprisingly decreased rejection incidence and severity require further studies for elucidation.

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A resistência vascular pulmonar elevada, que ocorre na fase final das miocardiopatias, tem sido um dos maiores obstáculos para a realizaçäo do transplante cardíaco ortotópico pelo risco de falência precoce do ventrículo direito do órgäo transplantado. A aplicaçäo clínica do transplantado cardíaco heterotópico, com início na era pré-ciclosporina, ampliou a possibilidade dos transplantes cardíacos nesse grupo de pacientes. Dentre outras indicaçöes desse procedimentos, podemos citar sua utilizaçäo nasisfunçöes ventriculares potencialmente reversíveis e em casos de desproporçäo de tamanho entre o doador e o receptor. Nossa experiência clínica com o transplante cardíaco heterotópico teve início em Outubro de 1992, e consiste de três pacientes com miocardiopatia em fase final e com resistência vascular pulmonar fixa elevada. O primeiro paciente teve indicaçao de transplante heterotópico reforçada pelo encontro de um doador de tamanho inferior. Utilizamos uma inovaçäo tácnica original em um dos paciets, permitindo a anastomose entre as artérias pulmonares sem emprego de tubo protético. Houve um óbito precoce e um óbito tardio após um retransplante heterotópico. Um deles está em perfeitas condiçöes clínicas, praticando esportes regularmente. O transplante cardíaco heterotópico pode ser considerado alternativa terapêutica em portadores de cardiopatia em fase terminal com resistência vascular pulmonar elevada, especialmente naqueles com boa funçäo ventricular direita.

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O transplante cardiopulmonar é modalidade terapêutica aceita internacionalmente para o tratamento de pacientes com doença que comprometem irreversivelmente os pulmöes e o coraçäo. As cardiopatias congênitas complexas, as cardiomiopatias primárias e as secundárias à doença valvar associadas a hiper-resistência vascular pulmonar irreversíveis säo indicaçöes precisas desse procedimento. Já no grupo de portadores de funçäo cardíaca preservada, como na doença pulmonar parenquimatosa primária e na hipernsäo pulmonar primária ou secundária à cardiopatia congênita de fácil correçäo, os transplantes pulmonares unilaterais ou bilaterais têm sido preferidos como forma de tratamento cirúrgico. Nossa experiência pessoal consiste de seis pacientes, três com diagnóstico de hipertensäo pulmonar primária, dois com miocrdiopatia e hipertensäo pulmonar e um com doença pulmonar isolada, todos em fase terminal da donça. Houve um óbito precoce e quatro óbitos tardios. A sobrevivência variou de 5 dias a 40 meses e todsapresentaram rejeiçäo pulmonar durante sua evoluçäo. O último paciente, atualmente no 26o. mês de evoluçäo, foi submetido no oitavo mês de pós-operatório a transplante pulmonar bilateral, devido a insuficiência respiratória progressiva por rejeiçäo pulmonar crônica. Os resultados do transplante cardiopulmonar, a longo prazo, säo ruins, tanto no tempo de sobrevivência quanto na qualidade de vida. Isso se deve, principalmente, à rejeiçäo pulmonar crônica, expressa pelo quadro clínico de insuficiência resirtória progressiva e histologicamente definida como bronquiolite obliterante. Podemos concluir que o transplante cardiopulmonar é uma forma de tratamento a ser considerada em pacientes terminais, porém limitada ao controle da rejeiçäo pulmonar a longo prazo.

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PURPOSE: To report our initial clinical experience with heterotopic heart transplantation, stressing its indications and a new modification in surgical technique. METHODS: We studied three patients underwent heterotopic heart transplantation, using an original technique in one of them. This new technique allows to connect both pulmonary arteries without any prosthetic tube. RESULTS: One early death and two late survivors, with 17 and 20 months of follow-up. One of them is clinically well and practices sports, regularly. CONCLUSION: Heterotopic heart transplantation could be considered as a good therapeutic option for end-stage patients with cardiomyopathy and right pulmonary vascular resistance, specially those with right ventricle. It can improve general results of heart transplantation because it reduces the early risks related to hemodynamic problems in a selected group of patients.

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At the present time there is great interest in seeking non invasive methods for reduction and precise orientation of the endomyocardial biopsy after heart transplant. Two cases of orthotopic heart transplant followed by spectro temporal mapping of signal averaging electrocardiography and integral calculation are reported. The results that identified the rejection process are presented and several aspects of this method are discussed.

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PURPOSE: To assess this initial clinical experience with heart-lung transplantation in Brazil. METHODS: Four patients underwent heart-lung transplantation from December 1988 to March 1990, one patient with cardiomyopathy and high pulmonary resistance, two patients with primary pulmonary hypertension and another with pulmonary silicosis. The heart lung blocks were harvested from the donor using cardiopulmonary bypass and deep hypothermia. They were transplanted to the recipient by anastomosing the trachea, right atrium or venae cavae, and the ascending aorta. RESULTS: The second patient died on the 5th postoperative day due to respiratory insufficiency with consequent brain lesions. The first and third patients had survived nine months and 42 days respectively. The 4th patients is asymptomatic eight months after the operation. The main complications were bleeding in two patients, acute pulmonary rejection in three of them and obliterans bronchiolitis in one patients. CONCLUSION: The heart lung transplantation is a challenging procedure which results are progressively improving with the development of better surgical technique and clinical concepts.

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Heart and left lung (en bloc) transplantation was used as an approach to end-stage cardiomyopathy with very high pulmonary vascular resistance. This surgical method was applied to a 13-year-old girl who did very well initially, but died 5 months later of severe pulmonary and cardiac rejection. The pulmonary perfusion scintigraphy showed equalization of the blood flow in both lungs after 42 days, and complete reversal of the pulmonary vascular changes in the opposite lung was observed at autopsy.

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PURPOSE: To assess this initial clinical experience with heart-lung transplantation in Brazil. METHODS: Four patients underwent heart-lung transplantation from December 1988 to March 1990, one patient with cardiomyopathy and high pulmonary resistance, two patients with primary pulmonary hypertension and another with pulmonary silicosis. The heart lung blocks were harvested from the donor using cardiopulmonary bypass and deep hypothermia. They were transplanted to the recipient by anastomosing the tracheae, right atrium or venae cavae, and the ascending aorta. RESULTS: The second patient died on the 5th postoperative day due to respiratory insufficiency with consequent brain lesions. The first and third patients had survived nine months and 42 days respectively. The 4th patients is asymptomatic eight months after the operation. The main complications were bleeding in two patients, acute pulmonary rejection in three of them and obliterans bronchiolitis in one patients. CONCLUSION: The heart lung transplantation is a challenging procedure which results are progressively improving with the development of better surgical technique and clinical concepts.

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The authors report the case of a 31 year-old male with right atrial myxoma whose clinical manifestations included: malaise, dizziness and dyspnea on great exertion. At physical examination, a tricuspid systolic and diastolic murmur of mean intensity was found. The chest radiography and the electrocardiogram signs were of right atrial and ventricular enlargement, and the diagnosis was made with the B-mode echocardiogram. The patient underwent surgical removal of the tumoral mass and diagnostic confirmation was made with the histopathologic study of the 75 x 50 x 40 mm piece. The patient obtained clinical, electrocardiographic and radiologic remission.

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Between December 1982 and June 1987, seven consecutive patients (52 to 77 years old) underwent early surgical repair of postinfarction ventricular septal defect. The defect was diagnosed 3 to 10 days after the myocardial infarction. A new repair technique was used which stresses that no part of the infarcted septum be resected. This technique consists of a transinfarction incision in the left ventricle, placement of a fine Dacron fabric patch that covers all the infarcted septum and closes the ventricular septal defect, and placement of a second Dacron fabric patch that reinforces the infarcted anterior wall of the heart and supports the buttressed double suture closure of the left ventriculotomy. One very ill patient of this series died during the operation (mortality rate 14.3%). Three patients required the help of intraaortic balloon counterpulsation postoperatively, and five needed inotropic drug support. None of the patients had excessive bleeding. Two initial patients had a small left-to-right interventricular shunt. Postoperative angiographic studies and Doppler echocardiography confirmed the existence of a nonsignificant residual ventricular septal defect in these two patients and showed good geometry of the left ventricle with no aneurysm formation in all six survivors. This technique seems to be efficacious. It can be expeditiously performed, and the risks of postoperative complications related to the technique appear to be minimal.

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E relatado o caso de paciente portadora de tetralogia de Fallot, submetida a correcao parcial da anomalia. Durante o ato cirurgico foi seccionada a arteria coronaria direita originaria de arteria coronaria esquerda unica. A interposicao de enxerto de safena homologa conservada em glicerina permitiu a sobrevida da paciente e posterior correcao de Fallot

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Os autores apresentam um caso de choque cardiogenico secundario a infarto predominante de ventriculo direito com pobre resposta as medidas clinicas convencionais. A recanalizacao e angioplastia da arteria coronaria direita, durante direita, durante o estudo hemodinamico levou a rapida melhora clinica, encontrando-se o doente bem, apos dois meses do procedimento. Discutem-se aspectos relacionados ao infarto de ventriculo direito e a recanalizacao e angiopatia

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