J Korean Pediatr Soc.  2002 Dec;45(12):1528-1533.

Clinical Features of Isolated Noncompaction of the Ventricular Myocardium

• Moon EK
• Lee HY
• Chang MY
• Kil HR
• Chung YH

Abstract

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+/-53 months(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.
CONCLUSIONS
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.

Keyword

Isolated noncompaction of the ventricular myocardium

MeSH Terms

Adult
Arrhythmias, Cardiac
Atrioventricular Block
Cardiomyopathies
Child
Diagnosis
Ductus Arteriosus, Patent
Early Diagnosis
Electrocardiography
Embolism
Follow-Up Studies
Heart Septal Defects, Atrial
Heart Ventricles
Hemodynamics
Humans
Incidence
Isolated Noncompaction of the Ventricular Myocardium*
Medical Records
Mitral Valve
Mitral Valve Prolapse
Prognosis
Recurrence
Sinoatrial Node
Tachycardia, Supraventricular
Tachycardia, Ventricular
Wills
Wolff-Parkinson-White Syndrome