Isolated Noncompaction of the Ventricular Myocardium : Clinical features and Outcomes.
- Author:
Soo Jung KANG
;
Chung Il NOH
;
Young Mee SEO
;
Hyuk Joo KWON
;
Ki Bum KIM
;
Young Hwan SONG
;
Eun Jung BAE
;
Jung Yun CHOI
;
Yong Soo YUN
- Publication Type:Original Article
- Keywords:
Isolated noncompaction of the ventricular myocardium;
Child
- MeSH:
Arrhythmias, Cardiac;
Cardiomegaly;
Child;
Cyanosis;
Diagnosis;
Echocardiography;
Electrocardiography;
Follow-Up Studies;
Heart;
Heart Failure;
Heart Murmurs;
Humans;
Isolated Noncompaction of the Ventricular Myocardium*;
Mass Screening;
Pulmonary Atresia;
Tachycardia, Supraventricular;
Tachycardia, Ventricular;
Trabecular Meshwork;
Ventricular Septum;
Wolff-Parkinson-White Syndrome
- From:Journal of the Korean Pediatric Cardiology Society
2001;5(1):42-48
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
PURPOSE: Isolated noncompaction of the ventricular myocardium(INVM) can present as heart failure or arrhythmias in a child. It is a rare disorder, characterized by prominent trabecular meshwork and deep intertrabecular recesses. We still know little about the diagnosis, symptoms, and clinical outcomes of INVM. METHODS: We included in our study 6 patients who showed ventricular noncompaction on echocardiography. Patients were diagnosed as INVM were excessively prominent trabeculations with deep intertrabecular recesses were found on echocardiography. Patients who had other complex heart lesions such as pulmonary atresia with intact ventricular septum in addition to ventricular noncompaction, were excluded. RESULTS: Age at presentation ranged from 1 day 7 years, with follow up being as long as 6 years. Symptoms at initial presentation were heart murmur, paroxysmal supraventricular tachycardia, cyanosis, feeding intolerance, ventricular tachycardia, and cardiomegaly at fetal screening. Prominent trabeculations and intertrabecular recesses were observed at left ventricular apex in all six patients. All patients were alive at last follow-up. One patient showed WPW syndrome on electrocardiography. Echocardiography revealed decreased systolic function in 4 patients, and decreased systolic and diastolic function in 1 patient. One patient is currently asymptomatic. CONCLUSION: Six patients were diagnosed with INVM with various symptoms at initial presentation. Echocardiography is the most important tool in the diagnosis of INVM due to its morphological characteristics. INVM can rarely be the cause of long term systolic dysfunction, and early detection by echocardiographic screening may be beneficial.
