Abstract 13879: Clinical Features and Prognosis of Patients With Left Ventricular Noncompaction Cardiomyopathy: A Comparison Between Infantile and Juvenile Types
Introduction: Left ventricular noncompaction cardiomyopathy (LVNC) is characterized by a left ventricle with a prominent trabecular meshwork. Long-term prognosis of LVNC has not been elucidated yet.
Purpose: The aim of this study is to clarify the difference between infantile and juvenile cases and identify the risk factor of poor prognosis of LVNC in the largest series to date.
Methods: Based on nationwide surveys of LVNC in Japanese children, we compared the clinical features and anatomical properties of infantile cases of LVNC (< 2 years: 85 cases) with that of juvenile cases (2-15 years: 73 cases). In addition to the standard noncompacted to compacted layer (N/C) ratio, we developed an echocardiographic criteria which represents an average of N/C ratios in five wall segments to estimate the severity of LVNC.
Results: The duration of follow-up ranged from 15 days to 22 years (median 5 years). Although most patients in the infantile group had clinical signs of heart failure at initial presentation (70.1%), the majority of juvenile cases were asymptomatic and identified when screened for cardiac abnormalities, such as ECG screening (55.8%). WPW syndrome was higher in both groups (infantile group: 9.7%, juvenile group: 10.0%), the incidence of LBBB and VT was lower than those reported in adults. On echocardiography, the maximum N/C ratio was observed at the apex in both groups. Neither noncompaction score nor N/C score was significantly different between groups. Left ventricular ejection fraction (LVEF) at initial presentation was significantly lower in the infantile group than in the juvenile group. Although survival analysis showed poor prognosis in the infantile group, the significant risk factor was LVEF below 50% (p = 0.0004, hazard ratio = 9.0), rather than age of onset.
Conclusions: LVNC in both infantile and juvenile groups showed poor prognosis when correlated with depressed LVEF at initial presentation.
Author Disclosures: A. Takasaki: None. S. Ozawa: None. N. Miyao: None. H. Nakaoka: None. K. Ibuki: None. K. Hirono: None. N. Yoshimura: None. F. Ichida: None.
- © 2015 by American Heart Association, Inc.