Experiência com transplante cardíaco heterotópico em pacientes com resistência pulmonar elevada: seguimento tardio / Experiencia con trasplante cardíaco heterotópico en pacientes con resistencia pulmonar elevada: seguimiento tardío / Experience with heterotopic heart transplantation in patients with elevated pulmonary vascular resistance: late follow-up

FUNDAMENTO: Nos últimos anos o numero de artigos sobre transplante cardíaco heterotópico tem sido escasso na literatura, inclusive internacional, e em particular do seguimento de longo prazo destes pacientes, o que levou ao presente relato. OBJETIVO: Relatar a experiência clínica inicial e evolução tardia de quatro pacientes submetidos a transplante cardíaco heterotópico, sua indicação e principais complicações. MÉTODOS: As cirurgias ocorreram entre 1992 e 2001, sendo que a indicação de transplante heterotópico, em todas, foi pela RVP, variável de 4,8UW a 6.5UW, com gradiente transpulmonar acima de 15mmHg. No 3º paciente, foi realizada uma anastomose direta entre as artérias pulmonares sem emprego de tubo protético e, no coração nativo, foi realizada uma valvoplastia mitral e aneurismectomia de ventrículo esquerdo (VE). O esquema imunossupressor imediato foi duplo com ciclosporina e azatioprina nos três primeiros pacientes e ciclosporina e micofenolato mofetil no 4º paciente. RESULTADOS: Um óbito imediato por falência do enxerto, um óbito após dois anos e meio por endocardite em trombo intraventricular no coração nativo, e um terceiro óbito seis anos após o transplante, por complicações pós-operatórias de cirurgia na valva aórtica do coração nativo. O remanescente, 15 anos após o transplante, encontra-se bem, em classe funcional II (NYHA), seis anos após a oclusão cirúrgica da valva aórtica do coração nativo. CONCLUSÃO: O transplante cardíaco heterotópico é um procedimento com resultado inferior ao transplante cardíaco ortotópico, por apresentarem maior RVP. Os trombos intraventriculares no coração nativo, que exigem anticoagulação prolongada, bem como as complicações de válvula aórtica, também no coração nativo, podem exigir tratamento cirúrgico. Entretanto, em um paciente, a sobrevida de 15 anos mostrou a eficácia de longo prazo desse tipo de alternativa, para pacientes selecionados.

BACKGROUND: Along the past few years the number of papers on heterotopic cardiac transplant has been very scarce in the medical literature, including at the international level; this is particularly true in reference to the long term follow-up of these patients and the reason which led to the presentation of our report. OBJECTIVE: To report the initial clinical experience and late evolution of 4 patients undergoing heterotopic heart transplantation, indications for this procedure and its major complications. METHODS: The surgeries were performed between 1992 and 2001, and all had as indication for heterotopic transplantation the PVR, which ranged from 4.8 WU to 6.5WU, with a transpulmonary gradient above 15mmHg. In the 3rd patient, a direct anastomosis between the pulmonary arteries was performed without the use of a prostetic tube, and a mitral valvuloplasty and a LV aneurysmectomy were performed in the native heart. The immediate immunosuppressive regimens were double, with cyclosporine and azathioprine in the first 3 patients, and cyclosporine and mycophenolate mofetil in the 4th patient. RESULTS: One immediate death occurred from graft failure, one death occurred after 2 ½ years, from endocarditis in an intraventricular thrombus in the native heart, and a third death occurred 6 years after transplantation, from post-operative complications of the aortic valve surgery in the native heart. The remaining patient is well, 15 years after the transplantation. This patient is in functional class II (NYHA), 6 years after a surgical occlusion of the native heart aortic valve. CONCLUSION: Heterotopic heart transplantation results are inferior to those of orthotopic heart transplantation because they present higher RVP. The intraventricular thrombi, in the native heart, which require prolonged anticoagulation, and aortic valve complications, also in the native heart, may require surgical treatment. However, a patient's 15-year survival has demonstrated ...

FUNDAMENTO: En los últimos años el número de artículos sobre trasplante cardíaco heterotópico y, en particular, del seguimiento a largo plazo de estos pacientes, ha sido escaso en la literatura, inclusive internacional, lo que llevó al presente relato. OBJETIVO: Relatar la experiencia clínica inicial y la evolución tardía de cuatro pacientes sometidos a trasplante cardíaco heterotópico, su indicación y principales complicaciones. MÉTODOS: Las cirugías se realizaron entre 1992 y 2001, y la indicación de trasplante heterotópico, en todas, fue mediante RVP, variable de 4,8 UW; a 6.5 UW, con gradiente transpulmonar superior a 15 mmHg. En el tercer paciente, se realizó una anastomosis directa entre las arterias pulmonares sin empleo de tubo prostético, y, en el corazón nativo, se realizó una valvuloplastia mitral y aneurismectomía de VI. El esquema inmunosupresor inmediato fue doble, con ciclosporina y azatioprina en los tres primeros pacientes y ciclosporina y micofenolato mofetil en el cuarto paciente. RESULTADOS: Un óbito inmediato por falla del injerto, un óbito luego de dos años y medio por endocarditis en trombo intraventricular en el corazón nativo, y un tercer óbito seis años después del trasplante, por complicaciones postoperatorias de una cirugía en la válvula aórtica del corazón nativo. El restante, 15 años después del trasplante, se encuentra bien, en clase funcional II (NYHA), seis años después de una oclusión quirúrgica de la válvula aórtica del corazón nativo. CONCLUSIÓN: El trasplante cardíaco heterotópico es un procedimiento con resultado inferior al trasplante cardíaco ortotópico, por presentar mayor RVP. Los trombos intraventriculares en el corazón nativo, que exige anticoagulación prolongada, así como las complicaciones de válvula aórtica, también en el corazón nativo, pueden exigir tratamiento quirúrgico. Sin embargo, en un paciente, la sobrevida de 15 años mostró la eficacia a largo plazo de este tipo de alternativa, ...

Heart transplantation in primary amyloidosis.

Cardiac amyloidosis is a disease with a gloom life expectancy after the beginning of the symptomatic phase, usually with sudden death as the final event. The aggression to other organs, although, can make heart transplantation a disputable form of treatment taking into consideration the shortage of donor organs. The aim is to report the evolution with a survival of seven years after heart transplantation and in very fair condition of a patient with amyloidosis. One year after the heart transplantation, there was indication of renal transplantation also from the aggression from the disease. This patient compares' favorable with three other patients also from our service, who died early after de diagnosis. Even considering the multi systemic nature of amyloidosis, we can accept that in peculiar patients justified the heart transplantation, taking in the consideration the very bad prognosis of the disease.

Transplante cardíaco em amiloidose primária / Heart transplantation in primary amyloidosis

A amiloidose cardíaca é doença altamente limitante da sobrevida, por morte súbita na maioria dos pacientes. Pela agressão contra outros órgãos, particularmente rins e sistema nervoso central, o transplante cardíaco tem sido opção questionável, face à escassez de órgãos. O objetivo é relatar a evolução, com sobrevivência de 7 anos, da paciente após transplante cardíaco por amiloidose, em boas condições. Um ano após o transplante cardíaco, houve indicação de transplante renal, também pela agressão da doença. Esta paciente contrasta com outros três pacientes de nosso serviço que foram a óbito, ainda na fase de avaliação. Apesar de sua natureza multisistêmica, a amiloidose cardíaca pode, em pacientes selecionados, justificar o transplante cardíaco, pela gravidade do seu potencial evolutivo após o início dos sintomas

Cardiac amyloidosis is a disease with a gloom life expectance after the beginning of the symptomatic phase, usually with sudden death as the final event. The aggression to other organs, although, can make heart transplantation a disputable form of treatment taking into consideration the shortage of donor organs. The aim is to report the evolution with a survival of seven years after heart transplantation and in very fair condition of a patient with amyloidosis. One year after the heart transplantation, there was indication of renal transplantation also from the aggression from the disease. This patient compares' favorable with three other patients also from our service, who died early after de diagnosis. Even considering the multi systemic nature of amyloidosis, we can accept that in peculiar patients justified the heart transplantation, taking in the consideration the very bad prognosis of the disease

Transplante cardíaco heterotópico. Experiência clínica inicial / Heterotopic heart transplantation. Initial clinical experience

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.

El trasplante cardiopulmonar / Cardiopulmonary transplantation

Se presentan los primeros cinco casos de trasplante de corazón-pulmón en el Hospital de la Beneficiencia Portuguesa de Sao Paulo, Brasil. El programa se inicio en diciembre de 1988; se analizan los resultados de esta experiencia preliminar. Uno de los pacientes sufría cardiomiopatía con elevada resistencia pulmonar, dos tenían hipertensión pulmonar primaria y el cuarto silicosis pulmonar. El bloque cardiopulmonar donante fue rescatado utilizando circulación extracorpórea e hipotermina profunda, y fue trasplantado al receptor anastomosando la tráquea, la aurícula derecha o las venas cavas y la aorta ascedente.

Transplante cardiopulmonar: experiência clínica inicial / Heart-lung transplantation: initial clinical experience

Purpose To assess this initial clinical experience with heart-lung transplantation in Brazil. Methods Four patients underwent heartlung transplantation from December 1988 to March 1990, one patient with cardiomyopathy and high pulmonary resistance, two patiens with primary pulmonary hypertension and another with pulmonry silicosis. The heart lung blocks were harvestedfrom the donor using cardiopulmonary bypass and deep hypotermia. 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 insuffciency with consequent brain lesions. The first and third patients had survived nine manths and 42 days respectively. The 4th patients is assymptomatic 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.