RESUMO
Cardiac sarcoidosis is a rare but important differential diagnosis in patients who present with progressive heart failure and arrhythmia. It may be diagnosed on endomyocardial biopsy. An excellent response can be achieved with steroid therapy in the early acute inflammatory stage. Progression of the disease may lead to end-stage heart failure that requires implantation of a permanent pacemaker, implantable cardioverter-defibrillator, or mechanical circulatory support as a bridge to heart transplantation. We present three Hong Kong Chinese patients with cardiac sarcoidosis.
Assuntos
Cardiomiopatias/terapia , Sarcoidose/terapia , Adulto , Arritmias Cardíacas/etiologia , Arritmias Cardíacas/terapia , Biópsia , Cardiomiopatias/diagnóstico , Desfibriladores Implantáveis , Feminino , Glucocorticoides/uso terapêutico , Granuloma/patologia , Insuficiência Cardíaca/etiologia , Insuficiência Cardíaca/terapia , Transplante de Coração , Humanos , Imunossupressores/uso terapêutico , Masculino , Miocárdio/patologia , Sarcoidose/diagnóstico , Disfunção Ventricular Esquerda/etiologia , Disfunção Ventricular Esquerda/terapiaRESUMO
Management of primary pulmonary hypertension is usually difficult because the disease is uncommon and the aetiology of the disease is not well understood. The disease is potentially lethal because it can lead to failure of the right ventricle, low cardiac output, and ensuing multiple organ failure. We report the successful treatment of a case of low-output syndrome due to primary pulmonary hypertension using combined drug therapy and atrial septostomy. Latest developments in the treatment of this disease are also discussed.
Assuntos
Baixo Débito Cardíaco/etiologia , Septos Cardíacos/cirurgia , Hipertensão Pulmonar/etiologia , Piperazinas/uso terapêutico , Vasodilatadores/uso terapêutico , Disfunção Ventricular Direita/complicações , Adulto , Baixo Débito Cardíaco/terapia , Feminino , Átrios do Coração/cirurgia , Humanos , Hipertensão Pulmonar/terapia , Purinas , Citrato de Sildenafila , Sulfonas , Disfunção Ventricular Direita/terapiaRESUMO
Post-transplantation lymphoproliferative disease (PTLD) is an unique iatrogenic complication after bone marrow transplantation (BMT) and solid organ transplantation (SOTx). The pattern of EBV related lymphoma in Chinese is different from Caucasians. We surveyed the incidence, clinical and pathological spectrum of PTLD among 541 cases of allogeneic BMT, 145 cases of renal transplant, 35 cases of heart/lung transplantation and 146 cases of orthotopic liver transplantation (OLT). From 1994 to 2001, 13 consecutive cases of PTLD were diagnosed, ranging from disseminated NK cell lymphoma to localized plasmacytoma. Both donor and recipient derived PTLD was documented. Disease was often heralded by cytomegaloviral disease and antithymocyte globulin (ATG) usage. Two cases were diagnosed post-mortem, and six patients died of PTLD at a median of 3 months. Complete and partial remission was only achieved in 3 and 2 cases, respectively, despite a range of treatment (reduced immunosuppression, explantation, radiotherapy, combination chemotherapy, donor lymphocytes, autologous marrow infusion and rituximab). Most responding patients died subsequently of rejection, infection and graft versus host disease (GVHD). The incidence of PTLD is not increased in Chinese patients. However, some patients may be at increased risk, especially mismatched allogeneic BMT, parental OLT (especially involving young infants) and heavy ATG exposure.
