Patrícia Marques-Alves1, Rui Baptista2, Catarina Canha3, Fátima Franco4, Lèlita Santos3, Mariano Pêgo4. 1. Department of Cardiology, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal. Electronic address: pat.marques.alves@gmail.com. 2. Department of Cardiology, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal; CNC.IBILI Research Consortium, University of Coimbra, Coimbra, Portugal. 3. Department of Internal Medicine, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal. 4. Department of Cardiology, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.
Abstract
INTRODUCTION: Systemic sclerosis (SSc) is a systemic autoimmune disease involving multiple organs. We present a rare case of SSc in which clinical manifestations of cardiac fibrosis occurred early in the disease course. CASE REPORT: We report the case of a 40-year-old Caucasian man, previously diagnosed with SSc, who presented with decompensated heart failure. Transthoracic echocardiography was remarkable for severe right ventricular systolic dysfunction, abnormal ventricular septal motion, severe functional tricuspid regurgitation and normal pulmonary artery systolic pressure. Left ventricular ejection fraction was 45%. Right heart catheterization revealed no signs of pulmonary hypertension. Cardiac magnetic resonance (CMR) showed diffuse myocardial infiltration, later confirmed as myocardial fibrosis by endomyocardial biopsy. CONCLUSIONS: Myocardial fibrosis is an important cause of early heart failure in SSc patients and is associated with poor prognosis. Echocardiography and CMR help establish the diagnosis and enable an appropriate therapeutic strategy to be developed in such cases.
INTRODUCTION:Systemic sclerosis (SSc) is a systemic autoimmune disease involving multiple organs. We present a rare case of SSc in which clinical manifestations of cardiac fibrosis occurred early in the disease course. CASE REPORT: We report the case of a 40-year-old Caucasian man, previously diagnosed with SSc, who presented with decompensated heart failure. Transthoracic echocardiography was remarkable for severe right ventricular systolic dysfunction, abnormal ventricular septal motion, severe functional tricuspid regurgitation and normal pulmonary artery systolic pressure. Left ventricular ejection fraction was 45%. Right heart catheterization revealed no signs of pulmonary hypertension. Cardiac magnetic resonance (CMR) showed diffuse myocardial infiltration, later confirmed as myocardial fibrosis by endomyocardial biopsy. CONCLUSIONS:Myocardial fibrosis is an important cause of early heart failure in SSc patients and is associated with poor prognosis. Echocardiography and CMR help establish the diagnosis and enable an appropriate therapeutic strategy to be developed in such cases.