Left Ventricular Noncompaction Complicated with Myocardial Infarction with Barth Syndrome in a Newborn.
- Author:
Gyeong Hee YOO
1
Author Information
1. Department of Pediatrics, Soonchunhyang University Cheonan Hospital, Soonchunhyang University College of Medicine, Cheonan, Korea. yoogh@schmc.ac.kr
- Publication Type:Case Report
- Keywords:
Isolated noncompaction of the ventricular myocardium;
Barth syndrome;
Infant, newborn;
Myocardial infarction
- MeSH:
Arrhythmias, Cardiac;
Aspirin;
Barth Syndrome*;
Cardiomegaly;
Cardiomyopathies;
Chungcheongnam-do;
Cyanosis;
Depression;
Dyspnea;
Echocardiography;
Heart Defects, Congenital;
Heart Failure;
Heart Ventricles;
Heparin;
Hospitalization;
Humans;
Infant, Newborn*;
Isolated Noncompaction of the Ventricular Myocardium;
Male;
Milrinone;
Mothers;
Muscular Diseases;
Myocardial Infarction*;
Neutropenia;
Siblings;
Thromboembolism
- From:Soonchunhyang Medical Science
2016;22(1):38-41
- CountryRepublic of Korea
- Language:English
-
Abstract:
Left ventricular noncompaction (LVNC) is a rare cardiomyopathy characterized by a hypertrabeculation of the left ventricle. Patients may present with heart failure, arrhythmia, and thromboembolism. LVNC may be isolated or associated with congenital heart defects. The first discovered genetic cause of isolated LVNC was Barth syndrome (BTHS), an X-linked disorder caused by taffazin (TAZ) gene mutation. BTHS is characterized by cardiomyopathy, neutropenia, skeletal myopathy, and growth delay. A newborn male baby was referred to Soonchunhyang University Cheonan Hospital due to cyanosis and dyspnea. Based on findings of cardiomegaly, ST depression, and elevated cardiac enzyme, echocardiography was done, which revealed a hypocontractile, enlarged left ventricle with distinctive trabeculation in the apex. Heparinization for the treatment of myocardial infarction and continuous infusion of milrinone was started. During hospitalization, the TAZ gene mutation was detected in the patient, his mother, and elder sister. After 3 months, the patient was discharged with heart failure medication and aspirin.
