| Clinical Features of Isolated Noncompaction of the
Ventricular Myocardium |
|
Eun-Kyoung Moon, Hoon-Young Lee, Mea-Young Chang, Hong-Ryang Kil, Yong-Hun Chung |
|
Department of Pediatrics, College of Medicine, Chungnam National University, Taejeon, Korea |
| 심근의 단독 비경화증(Isolated Noncompaction of Ventricular Myocardium)의 임상 양상 |
|
문은경, 이훈영, 장미영, 길홍량, 정용헌 |
|
충남대학교 의과대학 소아과학교실 |
Correspondence:
Hong-Ryang Kil, Email: gilhong@medimail.co.kr
|
|
|
|
|
| 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?3 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.
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. |
| Key Words:
Isolated noncompaction of the ventricular myocardium |
|
PDF Links
Download Citation
