Clinical Features of Isolated Noncompaction of the Ventricular Myocardium Long-term Clinical Course, Hemodynamic Properties, and Genetic Background

摘要: Abstract OBJECTIVES A nationwide survey was conducted to clarify the clinical features of isolated noncompaction ventricular myocardium (INVM) in Japanese children comparison with previously described patients INVM. BACKGROUND Isolated is a rare disorder characterized by an excessively prominent trabecular meshwork. It accompanied depressed function, systemic embolism and arrhythmia. METHODS questionnaire specifically designed for this study sent 150 hospitals Japan where pediatric cardiology division exists. RESULTS Twenty-seven were diagnosed two-dimensional echocardiography, their ages ranging from one week 15 years at presentation, follow-up lasting as long 17 years. The gross anatomical appearance extension noncompacted predominantly apex observed on echocardiograms similar observations reported previously. Dissimilarities included greater number asymptomatic initial longer course gradually left no embolism, tachycardia children. Cardiac catheterization disclosed normal end-diastolic volume increased pressure most cases, consistent restrictive hemodynamics. higher incidence Wolff-Parkinson-White syndrome found children, whereas bundle branch block rarer than adults. Familial recurrence high (44%) many women. CONCLUSIONS In INVM can be screening examinations stage, it might have function pattern familial we implies that distinctive entity heterogeneous genetic background.