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.
Subject(s)
Adult , Child , Humans , Arrhythmias, Cardiac , Atrioventricular Block , Cardiomyopathies , Diagnosis , Ductus Arteriosus, Patent , Early Diagnosis , Electrocardiography , Embolism , Follow-Up Studies , Heart Septal Defects, Atrial , Heart Ventricles , Hemodynamics , 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 SyndromeABSTRACT
PURPOSE: Valproic acid is effective and safe in adults and children for the treatment of a variety of seizure types. But, according to the recent reports, this drug induce hyperandrogenism, obesity, and polycystic ovaries, particularly when the medication is started before the age of 20. This research was designed to study the effect of valproic acid on ovary of pubertal child. METHODS: The sample group consisted of 23 girls who were diagnosed to epilepsy by our department of pediatrics, and were taking valproic acid from January 2000 to July 2000. They were evaluated reproductive endocrine function with the serum sex hormones and ultrasonographic study of ovary. RESULTS: 1) The mean serum sex hormones concentration of prepubertal, pubertal, and postpubertal girls taking valproic acid were belong to the normal range of reference values of textbook of Nelson and Park's report. 2) The obesity were seen in two of the 23 girls taking valproic acid. 3) The rate of polycystic ovaries in normal girls with regular menstruation has been reported in 9%, but, in our report, polycystic ovaries were seen in five of the 20 girls taking valproic acid(25%). CONCLUSION: Ultrasonographic study of ovaries is required for the girls taking valproic acid. If the problems from the polycystic ovary are developed on patients taking valproic acid, the substitution of other anticonvulsant drug for valproic acid may be required. However, further study was needed to clarify whether this ovarian morphologic changes were elicited by valproic acid or not.