Follow-up of Cardiac Fabry Disease Treated by Cardiac Resynchronization Therapy

摘要

class="kwd-title">Keywords: Cardiac resynchronization therapy, Echocardiography, Fabry disease class="head no_bottom_margin" id="sec1title">IntroductionThe cardiac variant of Fabry disease is associated with significant morbidity and early death. We report the case of a 60-year-old man who was admitted with severe dyspnea at rest. He developed heart failure with severe left ventricular (LV) systolic dysfunction, sustained atrial flutter, and frequent nonsustained ventricular tachycardia. He was diagnosed with the cardiac variant of Fabry disease and was treated with flutter ablation, optimal medical therapy, and cardiac resynchronization therapy (CRT) with a defibrillator. Three years after CRT, he is totally asymptomatic and is able to perform activities of daily living and work with no limitations. This report highlights the role of advanced echocardiography to evaluate LV function in patients with the cardiac variant of Fabry disease.We previously reported a case of cardiac variant of Fabry disease treated with CRT. Three years of follow-up on this rare case will be detailed, as excellent images were successfully recorded along with the clinical course. This report highlights the role of advanced echocardiography to evaluate LV function in patients with cardiac variant of Fabry disease.