Caso 2 - homem de 20 anos com insuficiência cardíaca por Síndrome Restritiva

Abstract

A 20-year-old man (born 10/16/1985), from the town of Dona Ines (state of Paraiba, northeastern Brazil), was admitted at the hospital due to mild effort dyspnea (2006).The patient had presented shortness of breath triggered by mild effort since the age of 13 (1998); at this time, he also presented an episode of syncope while running. Since then, there was a slow progression of the dyspnea. He felt tired and dizzy when walking around 100 meters on level surfaces and he started to present lower-limb edema.He also complained of diffuse joint pain. A heart murmur was diagnosed. He had received the diagnosis of rheumatic fever at 16 years of age (2001).At the age of 18 (September 2004) he presented a picture of coughing and yellow expectoration, which was treated as pneumonia; subsequently, he sought hospital treatment.The physical examination (2004) showed: weight = 60 kg; height = 1.76 m, pulse = 102 bpm, blood pressure (BP) = 96 / 74 mmHg. Pulmonary semiology was normal. The assessment of the precordium showed shock palpated on the 5 th intercostal space, on the left hemiclavicular line. The heart sounds were normal. There was a ++ systolic murmur in the mitral area. At the abdominal assessment, the liver was palpated at 9 cm from the right costal border. He presented lower-limb edema.The medications were maintained -25 mg of hydrochlorothiazide, 40 mg of furosemide, 50 mg of captopril, 25 mg of atenolol daily and a monthly administration of benzathine penicillin IM .The Electrocardiogram (ECG) (Sept 23, 2004) disclosed sinus tachycardia (107 bpm), left atrial overload and QRS axis at 90° parallel to the frontal plane, little prominent and nonprogressive R waves in V 5 and V 6 , suggesting right ventricular overload (Figure 1). The echocardiograms (2004, 2005 and 2006) showed biatrial dilatation and mitral and tricuspid valve prolapse. The Doppler examination showed moderate mitral and tricuspid failure. The last echocardiographic assessment (2006) showed right ventricular dilatation and hypokinesis ( Table 1).The chest x-ray (June 2005) showed bilateral pleural thickening with sparse calcification. The pleural thickening was more marked on the left, where the heterogeneous and loculated content could be observed, with areas of atelectasis in the adjacent pulmonary parenchyma. An increased number of mediastinal and axillary lymph nodes were identified to the left. The heart presented normal morphology and dimensions. The aorta had a normal caliber and regular contours, with no signs of dissection. Hepatomegaly and bilateral gynecomastia were also diagnosed.After two years, the fatigue persisted and the patient presented worsening of the lower-limb edema that reached the thigh root as well as scrotal edema. The patient sought emergency care and a diagnosis of atrial fibrillation was made. The dose of diuretics was increased and warfarin was associated to the therapy. Two weeks later, during an ambulatory visit, edema persistence and INR increase to 11.1 were observed. The patient was then admitted to the hospital...