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Natural history and familial characteristics of isolated left ventricular non-compaction

Murphy, RT; Thaman, R; Blanes, JG; Ward, D; Sevdalis, E; Papra, E; Kiotsekolglou, A; ... Elliott, PM; + view all (2005) Natural history and familial characteristics of isolated left ventricular non-compaction. EUR HEART J , 26 (2) 187 - 192. 10.1093/eurheartj/ehi025.

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Abstract

Aims Non-compaction of the left ventricle (LVNC) is a disorder of endomyocardial morphogenesis that results in multiple trabeculations in the left ventricular myocardium. The current literature suggests that LVNC in adults is rare and associated with a poor prognosis. Given that the disorder is present at birth and that several studies have reported asymptomatic familial disease in some patients, we hypothesized that there is a long pre-clinical phase of the disease. The aim of this study was to define the prognosis and familial incidence of LVNC.Methods and results This study cohort comprised 45 patients (mean age at diagnosis 37 years) consecutively identified at a referral Centre for cardiomyopathy over a 10-year period. Twenty-eight patients (62 %) had dyspnoea at presentation; 41 (91 %) an abnormal ECG; and 30 (66 %) left ventricular dilatation and impaired systolic function. Nine patients (20 %) had non-sustained ventricular tachycardia on 24 h Hotter monitoring. Mean survival from death or transplantation was 97 % at 46 months. There were three thromboembolic events in two patients (4 %). On systematic family screening, 8 of 32 (25 %) asymptomatic relatives had a range of echocardiographic abnormalities, including LVNC, LVNC with impaired systolic function, and Left ventricular enlargement without LVNC.Conclusion This study demonstrates that LVNC is associated with a better prognosis than previously reported. In patients with familial disease, relatives may have features consistent with dilated cardiomyopathy rather than LVNC.

Type: Article
Title: Natural history and familial characteristics of isolated left ventricular non-compaction
DOI: 10.1093/eurheartj/ehi025
Keywords: left ventricular, non-compaction, cardiomyopathy, prognosis, DILATED CARDIOMYOPATHY, ISOLATED NONCOMPACTION, ASYMPTOMATIC RELATIVES, BARTH-SYNDROME, MYOCARDIUM, MUTATIONS, DISEASE, CLASSIFICATION, ADULTS
UCL classification: 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 Pop Health Sciences > Institute of Cardiovascular Science
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Pop Health Sciences > Institute of Cardiovascular Science > Clinical Science
URI: http://discovery.ucl.ac.uk/id/eprint/117983
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