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Relationship between aetiology and left ventricular systolic dysfunction in hypertrophic cardiomyopathy

Rosmini, S; Biagini, E; O'Mahony, C; Bulluck, H; Ruozi, N; Lopes, LR; Guttmann, O; ... Elliott, PM; + view all (2017) Relationship between aetiology and left ventricular systolic dysfunction in hypertrophic cardiomyopathy. Heart , 103 (4) pp. 300-306. 10.1136/heartjnl-2016-310138. Green open access

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Abstract

BACKGROUND: Severe left ventricular (LV) systolic dysfunction is an uncommon complication of hypertrophic cardiomyopathy (HCM) that is associated with poor prognosis. Small observational series suggest that patients with rare causes of HCM are more likely to develop systolic impairment than those with idiopathic disease or mutations in cardiac sarcomeric protein genes. The aim of this study was to test this hypothesis by comparing the prevalence of systolic dysfunction and its impact on prognosis in patients with different causes of HCM. METHODS AND RESULTS: 1697 patients (52 (40-63) years, 1160 (68%) males) with HCM followed at two European referral centres were studied. Diagnosis of specific aetiologies was made on the basis of clinical examination, cardiac imaging and targeted genetic and biochemical testing. The primary survival outcome was all-cause mortality or heart transplantation (HTx) for end-stage heart failure (HF). Secondary outcomes were HF-related death, sudden cardiac death, stroke-related death and non-cardiovascular death. Systolic dysfunction (LV ejection fraction <50% by two-dimensional (2D) echocardiography) at first evaluation was more frequent in rare phenocopies than in idiopathic or sarcomeric HCM (105/409 (26%) vs 40/1288 (3%), respectively (p<0.0001)). All-cause death/HTx and HF-related death were more frequent in rare phenocopies compared with idiopathic or sarcomeric HCM (p<0.0001). All-cause mortality and HF-related death were highest in patients with cardiac amyloidosis (p<0.0001). CONCLUSIONS: In adults with HCM, LV systolic dysfunction is more frequent in those with rare phenocopies. When combined with age at presentation, it is a marker for specific aetiologies and is associated with poorer long-term survival.

Type: Article
Title: Relationship between aetiology and left ventricular systolic dysfunction in hypertrophic cardiomyopathy
Location: England
Open access status: An open access version is available from UCL Discovery
DOI: 10.1136/heartjnl-2016-310138
Publisher version: http://dx.doi.org/10.1136/heartjnl-2016-310138
Language: English
Additional information: Copyright © Article author (or their employer) 2016. Produced by BMJ Publishing Group Ltd (& BCS) under licence. This article has been accepted for publication in Heart following peer review. The definitive copyedited, typeset version [Rosmini, S; Biagini, E; O'Mahony, C; Bulluck, H; Ruozi, N; Lopes, LR; Guttmann, O; (2017) Relationship between aetiology and left ventricular systolic dysfunction in hypertrophic cardiomyopathy. Heart , 103 (4) pp. 300-306. 10.1136/heartjnl-2016-310138] is available online at: http://dx.doi.org/10.1136/heartjnl-2016-310138
UCL classification: UCL
UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Cardiovascular Science
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Cardiovascular Science > Clinical Science
URI: https://discovery.ucl.ac.uk/id/eprint/1528555
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