• Clinical and Experimental Pediatrics
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• v.45 no.12
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• pp.1528-1533
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• 2002

Purpose : Isolated noncompaction of the ventricular myocardium(INVM) is one of the unclassified cardiomyopathies that is characterized by numerous, excessively prominent trabeculations, and deep intertrabecular recesses. We performed this study to evaluate the clinical features of INVM in children. Methods : The medical records of 10 patients with INVM were reviewed. We analyzed the clinical manifestations, hemodynamics, pattern of inheritance, and long-term prognosis of INVM in children. Results : Age at diagnosis was $45{\pm}53months$(1 day-14 years) with follow-up lasting as long as 78 months. Most INVM was asymptomatic on diagnosis. Associated cardiac anomalies were noted in six patients(ventricualr or atrial septal defect, patent ductus arteriosus with mitral valve prolapse, or mitral valve cleft). Depressed or flat changes of T wave in lead II, III and aVF were observed on electrocardiography. Various arrhythmia including WPW syndrome with paroxysmal supraventricular tachycardia, third-degree atrioventricular block, and familial sick sinus node dysfuction were observed. The degree of trabeculation in INVM was significantly prominent from level of mitral valve to apex compared to age-matched control. Familial recurrences were noted in two patients. The systolic function of the left ventricle was decreased in 20% of patients during the follow-up period, but systemic embolism or ventricular tachycardia was not observed. Conclusion : INVM is not a rare disorder. The cardiac function may be deteriorated in children as well as adults during long-term follow up. Thus early diagnosis and long-term follow-up must be done. So, the nation-wide multicenter clinical study would be mandatory to evaluate the incidence, long-term prognosis, and establishment of objective diagnostic criteria of INVM.

• Clinical and Experimental Pediatrics
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• v.52 no.1
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• pp.129-132
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• 2009

We describe here our experience with a neonate presenting with cyanosis, grunting, and cardiomegaly, who was diagnosed with isolated left ventricular noncompaction (IVNC) by echocardiography. The patient had high levels of N-terminal pro-B-type natriuretic peptide (NT pro-BNP) and symptoms of heart failure including poor feeding and tachypnea. During the period in which NT pro-BNP levels steadily increased, the patient suffered sudden cardiac arrest despite heart failure management. Following cardiopulmonary resuscitation, cardiac arrest was resolved, NT pro-BNP levels decreased, and all symptoms showed improvement. We consider that assessment of NT pro-BNP with cardiac functional analysis using echocardiography could help in the prediction of disease progress in IVNC.