Prader-Willi syndrome: a nest for premature coronary artery disease?. (7th February 2018)

• Record Type: Journal Article
• Authors: Brás, Diogo Rodrigues; Semedo, Pedro; Piçarra, Bruno Cordeiro; Fernandes, Renato
• Is Part Of: BMJ case reports. Volume 2018
• Publication Date: 2018-02-07
• Subjects: ischaemic heart disease -- genetics -- endocrinology -- interventional cardiology -- metabolic disorders; Medicine -- Case studies -- Periodicals; 610.5
• DOI: 10.1136/bcr-2017-222828
• Languages: English
• ISSNs: 1757-790X

Abstract

Abstract : Individuals affected by Prader-Willi syndrome (PWS) may show increased risk for coronary artery disease (CAD), which probably relates, at least, with high burden of cardiovascular risk factors. A 27-year-old man with PWS, obesity, hypertension, diabetes mellitus and dyslipidaemia attended the emergency department with complaints of flu-like condition and chest pain. The ECG revealed a mild ST-segment elevation in inferior leads, followed by positive myocardial necrosis biomarkers. Attending to the high cardiovascular risk profile, ST-segment elevation in inferior territory and wall motion abnormalities, a coronary angiogram was performed. The latter showed a three-vessel CAD, 60% stenosis in midanterior descending artery, total occlusion (100%) of the obtuse marginal artery and 99% stenosis with high thrombi burden in the proximal right coronary artery. The present case report emphasises the plausibility of premature CAD in patients with PWS, a possible underdiagnosed feature of this condition.