Methylenedioxymethamphetamine-induced toxic valvulopathy

A 33-year-old Caucasian woman was referred to our emergency department for progressive, severe dyspnoea (New York Heart Association Class IV) for the last 12 months. Electrocardiogram showed sinus rhythm with signs suggestive of left atrial enlargement and ventricular hypertrophy. Thoracic X-ray confirmed marked cardiomegaly with a cardiothoracic index of 0.58 and signs of interstitial lung oedema (Appendix  Figure 1). Transthoracic echocardiography revealed a dilated left ventricle with mildly reduced function. Severe mitral regurgitation and moderate stenosis (Video 1, Panels A and B) were evident with thickening and restrictive mitral valve leaflet motion (hockey stick mitral valve) (Video 2). There was also severe aortic regurgitation (Panel C) with doming of aortic valve cusps (Panel B). Transoesophageal echocardiography showed no vegetations. Coronary angiography was normal. Blood analysis revealed negative antinuclear antibody’s and no antiphospholipid antibody’s. C-reactive protein was low (31.1 mg/dL) indicating no active infection. Blood cultures remained negative, and urinary 5-hydroxyindoleacetic acid was not elevated (4.1 mg/g creatinine). There was no history of rheumatic fever or carcinoid symptoms. Other clinical risk factors to suspect rheumatic heart disease (RHD) were absent. The patient had no history of intravenous drug use, but longstanding methylenedioxymethamphetamine (MDMA) abuse was acknowledged. Aortic and mitral valve replacements were performed. The perioperative inspection confirmed the presence of thickened fibrotic valves (Panel D) and subvalvular apparatus (Panel E). Microscopic examination of the valves showed a preserved valvular architecture with increased extracellular matrix (Panel F) and randomly distributed, patchy fibroblastic proliferation with smooth muscle actin expression on immunohistochemistry (Panel G). Inflammation or overt neovascularization, as seen in RHD, was absent. The presence of severe restrictive aortic and mitral valve regurgitation in a patient with a potent 5HT_2B agonist abuse, together with the histopathological findings, suggested the diagnosis of MDMA-induced toxic valvulopathy. The patient currently consults our outpatient heart failure clinic with a left ventricular ejection fraction of 25% under optimal medical therapy.

Figure 1.

Echocardiogram with parasternal long axis view. Parasternal long axis view shows restrictive motion of both mitral valve leaflets (hockey stick pattern) with reduced opening and diffuse thickening.

Figure 2.