ABC | Volume 113, Nº2, August 2019

Anatomopathological Correlation Case 4/2019 - 26-Year-Old Man with Congenital Chagas Disease and Heart Transplantation Henrique Trombini Pines i, T ânia Mara Varejão Strabelli , Vera Demarchi Aiell o Instituto do Coração (InCor) – Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (HC-FMUSP), São Paulo, SP – Brazil Mailing Address: Vera Demarchi Aiello • Avenida Dr. Enéas de Carvalho Aguiar, 44, subsolo, bloco I, Cerqueira César. Postal Code 05403-000, São Paulo, SP – Brazil E-mail: demarchi@cardiol.br , anpvera@incor.usp.br Keywords Heart Defects, Congenital; Chagas Cardiomyopathy; Heart Transplantation; Heart Failure; Diagnosis Imaging. Section Editor: Alfredo José Mansur (ajmansur@incor.usp.br ) Associated Editors: Desidério Favarato (dclfavarato@incor.usp.br ) Vera Demarchi Aiello (anpvera@incor.usp.br ) DOI: 10.5935/abc.20190162 A 26-year-old man with cardiopathy due to congenital Chagas’ disease was submitted to heart transplantation for heart failure; amastigote forms of Trypanosoma cruzi were found in an endomyocardial biopsy in the third month after transplantation. The patient had been diagnosed with Chagas’ disease due to transplacental transmission and was followed at Hospital das Clínicas of Faculdade de Medicina of Universidade de São Paulo (FMUSP) until the age of 2 years, when he was discharged from the follow-up. He was diagnosed with cardiopathy due to Chagas’ disease at age 20. He sought emergency medical care on July 23, 2016, at age 25, for dyspnea on moderate exertion and paroxysmal nocturnal dyspnea and edema for three weeks. The physical examination disclosed blood pressure of 118/98 mmHg, heart rate of 87 bpm, respiratory rate of 28 breaths/min, oxygen saturation of 99% and pulmonary auscultation showed decreased vesicular murmur at the bases. Cardiovascular examination disclosed increased jugular venous pressure, thin pulses, cardiac stroke deviated 2 cm beyond the nipple line, 2 digital pulps, arrhythmic heart sounds, normal heart sounds with the presence of a third heart sound and mitral systolic murmur. Abdominal examination revealed painful hepatomegaly, with the liver palpated 6 cm from the right costal border and ++ /4 edema in the lower limbs. The electrocardiogram showed sinus rhythm, heart rate of 107 bpm, PR interval of 187 ms, QRS duration of 146 ms, left atrial overload, right bundle-branch block and anterosuperior left bundle-branch block and probable left ventricular overload (Figure 1). The posteroanterior chest x-ray disclosed veil-like opacification of the both hemithoraces, compatible with pleural effusion, increased pulmonary hila with signs of pulmonary congestion and cephalization of the pulmonary vasculature network and ++++ / 4 global cardiomegaly (Figure 2). The patient was re-hospitalized on July 29, after two episodes of syncope that occurred on the day before hospitalization. The physical examination disclosed a patient in regular overall status, hydrated, eupneic, acyanotic, conscious and oriented. Blood pressure was 90x60 mmHg, heart rate was 88 bpm, oxygen saturation was 97%; pulmonary auscultation was normal; cardiac auscultation disclosed an irregular heart rhythm, with no heart murmurs or accessory sounds; the abdominal examination showed no visceromegaly, no lower- limb edema, and no calf stiffness. Laboratory tests (July 29, 2016) showed: red blood cells: 5200000/mm³, hemoglobin 15.6g/dL, hematocrit of 47%, leukocytes 14,570/mm³ (74% of neutrophils), platelets 157,000/mm³, potassium 3.8 mEq/L, sodium 137 mEq/L, BNP 1,128 pg/mL, ALT 54 U/L, AST 42 U/L, gamma-GT 99 U/L. Urinalysis was normal. The chest x-ray (July 29, 2016) disclosed clear pulmonary fields and cardiomegaly at the expense of the right ventricle (Figures 3A and B). The transthoracic echocardiogram (July 29, 2016) showed: left atrium, 52 mm; right ventricle, 43x32 mm; septum, 8 mm; posterior wall, 8 mm; left ventricle 66x60 mm; left ventricular ejection fraction, 20%; pulmonary artery systolic pressure, 39 mmHg. There was a marked increase in left atrium (volume measured by Simpson's rule was estimated at 61 mL/m 2 , normal value < 34 mL/m 2 ); moderate enlargement of the left ventricle and right atrium; slight enlargement of the right ventricle. Systolic function was decreased due to diffuse hypokinesia. The right ventricle showed mild hypokinesia. There was also mild to moderate mitral regurgitation, as well as mild to moderate tricuspid regurgitation. The heart MRI (August 1, 2016) showed marked right ventricular dilatation with an ejection fraction of 17%, left ventricle with diffuse hypokinesia, with late mesocardial enhancement in the basal segment of the septum and transmural enhancement in the basal, middle and apical segments of the anterior, lateral and inferior walls, and less than 50% in the basal inferosseptal segment. The ejection fraction of this ventricle was also 17% (Figure 4). In the electrophysiological study (August 4, 2016) there was no onset of atrial or ventricular arrhythmias after the extra stimuli. Right catheterization (August 23, 2016) showed mean right atrial pressure of 11 mmHg, right ventricular end-systolic and diastolic pressures of 45/29 mmHg, and pulmonary capillary pressure of 30 mmHg. The pulmonary vascular resistance was 3.6 Wood Units (normal: 0.25 to 1.6 Wood U) and the cardiac index was 2.1L / min / m². After the use of 10 μg/kg/min of dobutamine, pulmonary vascular resistance decreased to 1.6 Wood U. The patient was placed on a transplant waiting list with a priority status, as vasoactive drug weaning was not achieved 286