Transplante cardíaco em portadora de endomiocardiofibrose

Abstract

a therapeutic option, although little used, with only one report of heterotopic transplantation reported in the literature. Case ReportThe patient is a 50-year-old white female, housewife, born in the town of São Sebastião, in the state of Alagoas, who was referred to InCor in 1996, complaining of enlargement of the abdominal volume associated with lower limb edema for 3 years. The patient reported dyspnea associated with huge ascites, which improved with relief punctures performed every 20-30 days, even though she was using diuretics. The patient underwent investigation for hepatopathy in the gastroenterology unit of the Hospital das Clínicas. Once the viral, alcoholic and schistosomal etiologies were excluded, the patient was referred to InCor with the diagnostic hypothesis of chronic hepatopathy secondary to right heart failure. On admission, the physical examination showed significant jugular venous distension, irregular cardiac rhythm with a blood pressure of 140/80 mmHg, and heart rate of 80 bpm. Her pulmonary auscultation was normal. The patient had voluminous ascites with significant lower limb edema. The electrocardiogram showed atrial fibrillation and right bundle-branch block. The laboratory tests were normal.The echocardiogram showed a dilated and hypokinetic right ventricle, with a diastolic diameter of 3.8 cm, and left ventricular diastolic diameter and ejection fraction of 4.5 cm and 73%, respectively. The right atrium was enlarged. The pericardium was thickened with a mild effusion. The atrioventricular valvular filling was of the protodiastolic type with the E wave greater than the A wave. The clinical diagnostic hypothesis of severe restrictive cardiomyopathy with right predominance and significant tricuspid insufficiency was established.The patient underwent cardiac catheterization, which showed coronary circulation without obstructive lesions, a normal left ventricle, and loss of the right ventricular intracavitary geography and amputation of the right ventricular trabecular region, suggesting the diagnosis of right ventricular endomyocardial fibrosis.As the patient remained symptomatic with refractory ascites, requiring relief punctures every 15 days, surgical treatment was chosen, with resection of the right ventricular endomyocardial fibrosis and DeVega tricuspid valve annuloplasty.The anatomicopathological examination of the surgical specimen confirmed the clinical and surgical impression, revealing "dense fibrosis of the endocardium, with a band containing neoformed vessels and calcified areas, an aspect compatible with endomyocardial fibrosis".

Heart Failure in South America

“…Surgical excision of endocardial fibrous tissue has been performed early in the clinical course of endomyocardial fibrosis, thus allowing the preservation of the AV valves [59]. Successful heart transplantation was reported for treatment of end-stage HF due to endomyocardial fibrosis [60] (Table 2 ).…”

Reporte de un caso de endomiocardiofibrosis en Colombia

“…Después de la aparición de los síntomas, el tratamiento es eminentemente quirúrgico. Los pacientes tratados clínicamente tienen una sobrevida media del 37% a diez años después del inicio de los síntomas 7,8 , mientras que los pacientes operados presentan una sobrevida del 55% en este mismo periodo 9 .…”

“…Por esta razón, el seguimiento ecocardiográfico de pacientes operados es de extrema importancia para el diagnóstico precoz de recidivas. El trasplante cardiaco en el paciente con endomiocardiofibrosis es una alternativa promisora en casos refractarios al tratamiento convencional 8 .…”

Tropical endomyocardial fibrosis: an overview

“…75 In a second study, 44% of patients died within one year after the onset of symptoms, and another 40% of patients died 1-3 years after onset of symptoms. Gupta and colleagues 76 defined the natural history of the disease in Kerala in the late 1980s. Follow up of the initial 200 patients showed a 10 year survival of only 37 per cent.…”