Clinical features and prognosis of isolated noncompaction of ventricular myocardium in adults
Wang Xin
Yang Xihui
Liu Xiaowei
Hao Xiaoyan
Ruan Yanping
He Yihua
Abstract：Objective To summarize the clinical features and outcomes of adults with isolated noncompaction of ventricular myocardium (NVM).Methods Clinical data of 57 patients diagnosed of isolated NVM in Beijing Anzhen Hospital,Capital Medical University between December 2007 and December 2017 were retrospectively reviewed.Long-term adverse events(ADE) were observed and the patients were divided into ADE group and ADE-free group.Clinical records and echocardiographic data were analyzed.Results Forty-six males (80.7％) were involved and the mean age of 57 patients was (42 ± 16)years.Cardiac functional grading of New York Heart Association demonstrated grade Ⅲ/Ⅳ in 27 cases (47.7％).Thirty-one patients (54.4％) presented arrhythmia and ventricular arrhythmia was the most common type(19 cases,33.3％).Eight patients(14.0％) had thromboembolic events.Fifty-three patients (93.0％) presented enlarged left ventricular end-diastolic dimension and 39 patients (68.4％) presented left ventricular ejection fraction ＜ 50％ on admission.Moderate to severe mitral regurgitation occurred in 30 patients (52.6％).Noncompaction of papillary muscle was observed in 29 patients (50.9％).During 4(0,9)years of follow-up,5 cases(8.8％) were lost;11 patients(21.2％) had adverse events,including 5 cardiac death,1 cardiac transplantation,1 cardioverter defibrillator implantion and 4 pacemaker implantation.Rates of arrhythmia,left ventricular end-diastolic diameter ＞ 70 mm,right ventricular enlargement,right atrial enlargement and moderate to severe pericardial effusion in ADE group(11 cases) were significantly higher than those in ADE-free group(41 cases) (all P ＜0.05).Conclusions Prognosis of adult patients with isolated NVM is poor;men are the majority;arrhythmia is common and thromboembolism is rare.NVM patients usually present enlarged left ventricular end-diastolic dimension,decreased left ventricular ejection fraction,moderate to severe mitral regurgitation and non-compact papillary muscle.Arrhythmia,left ventricular end-diastolic diameter ＞ 70 mm,right heart enlargement and moderate to severe pericardial effusion are associated with poor prognosis.
Keywords：Noncompaction of ventricular myocardiumClinical featuresPrognosis
Publication Date：2019-01-01
Online Publishing Date：2026-09-14(First online date of this platform, not the publication date of the document)
Pages：4( 501-504 )