Clinical characteristics and prognosis of children with ventricular noncompaction / 中国小儿急救医学

ABSTRACT

Objective To investigate the clinical features and prognosis in ventricular noncompaction children. Methods Thirty-four cases who diagnosed with ventricular noncompaction were included in this study in Shengjing Hospital of China Medical University from January 2012 to May 2018. According to age, the children were divided into infantile type( age <1 year old) and juvenile type( age≥1 year old) . We ana-lyzed the clinical features,laboratory tests and prognosis. Results The average age at diagnosis was 3 years and 2 months. The sex ratio was 2. 41. Of these,32 cases were left ventricular noncompaction,1 was right ventricular noncompaction, and 1 was biventricular noncompaction. There were no statistically significant differences in family history,arrhythmia,and thrombotic events between infantile type and juvenile type. Heart failure was the first reason for most children,while older children often presented with fatigue when at diag-nosis. More than half of them showed significant left ventricular ejection fraction(LVEF) decreasing,and non-compacted layer to compacted layer(N/C) ratio showed negative correlation with LVEF(r= -0. 74, P<0. 001). Sixty-five percent(22/34) of the patients presented abnormal electrocardiogram. During the follow-up,one patient died of pulmonary embolism. No significant LVEF improvement was found in 35％(12/34) of the patient. The COX proportional hazards model showed that N/C ratio was an independent risk factor for poor prognosis of ventricular noncompaction(OR=14. 46,95％CI 1. 712 -120. 234,P<0. 05). Conclusion Children with ventricular noncompaction showed different clinical features and prognosis. Early diagnosis,treatment and long term follow up are key issues for the prognosis.