Relationship between aetiology and left ventricular systolic dysfunction in hypertrophic cardiomyopathy
作者： Stefania Rosmini , Elena Biagini , Costantinos O'Mahony , Heerajnarain Bulluck , Niccolo’ Ruozi
DOI: 10.1136/HEARTJNL-2016-310138
关键词:
摘要: Background Severe left ventricular (LV) systolic dysfunction is an uncommon complication of hypertrophic cardiomyopathy (HCM) that associated with poor prognosis. Small observational series suggest patients rare causes HCM are more likely to develop impairment than those idiopathic disease or mutations in cardiac sarcomeric protein genes. The aim this study was test hypothesis by comparing the prevalence and its impact on prognosis different HCM. Methods results 1697 (52 (40–63) years, 1160 (68%) males) followed at two European referral centres were studied. Diagnosis specific aetiologies made basis clinical examination, imaging targeted genetic biochemical testing. primary survival outcome all-cause mortality heart transplantation (HTx) for end-stage failure (HF). Secondary outcomes HF-related death, sudden stroke-related death non-cardiovascular death. Systolic (LV ejection fraction Conclusions In adults HCM, LV frequent phenocopies. When combined age presentation, it a marker poorer long-term survival.
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