Left ventricular non-compaction in children and adolescents: Clinical features, treatment and follow-up

Background: Left ventricular non-compaction (LVNC) is a specific cardiomyopathy that occurs following a disruption of endomyocardial morphogenesis. This study presents clinical findings, diagnostic features, treatment and follow-up of pediatric patients diagnosed with LVNC. Methods: Patients with LVNC who were followed from January 2006 to March 2010 were included in this study. Diagnosis was made with the use of characteristic findings of magnetic resonance imaging and echocardiography. Holter electrocardiography and metabolic screening tests were also performed in all patients. Results: A total of 24 patients were studied (18 male, six female). Patient age at diagnosis was 50 ± 60 months (eight days to 15 years). Average follow-up period was 22 ± 12 months (four months to four years). Findings at diagnosis were as follows: eight (33%) patients had heart failure, five (20%) had rhythm abnormalities, five (20%) had cardiomegaly, two had murmurs, two had cyanosis, and two presented with fatigue. Ten (41%) patients had been followed previously with other diagnoses. In 21 (87.5%) patients, electrocardiographic abnormalities were noted, especially left ventricular hypertrophy and ST-T changes. Patients had an average ejection fraction of 46% (18-73%) and three of them had additional congenital heart disease (patent ductus arteriosus, aortopulmonary window and complex cyanotic heart disease). Scanning for metabolic diseases revealed fatty acid oxidation disorder in one patient, and mitochondrial disease in another. During follow-up, a permanent pacemaker was implanted in a patient with severe bradycardia and ventricular dysfunction, and three patients died. Conclusion: LVNC can be diagnosed at any age from newborn to adolescent and has a variable clinical course. Closer study of patients with cardiomegaly and heart failure can reduce delays in diagnosis of LVNC. (Cardiol J 2011; 18, 2: 176-184

Signal-averaged electrocardiogram may be a beneficial prognostic procedure in the postoperative follow-up tetralogy of fallot patients to determine the risk of ventricular arrhythmias

Early detection of arrhythmias after congenital heart disease surgery is important because it can help decrease morbidity and mortality. Standard electrocardiograms (ECGs) contain frequencies between 0.05 and 100 Hz, but higher frequencies are also present. Using high-resolution technology, the highest amplitudes of these high-frequency components within the QRS complex can be recorded and analyzed. We studied the relationship between ventricular late potentials, ventricular arrhythmias and right ventricular systolic pressure in 22 patients who underwent tetralogy of Fallot repair (mean follow-up, 40.1 +/- 33.5 months). Holter ECG monitoring and signal-averaged electrocardiograms (SAECGs) were performed. SAECG parameters studied included the duration of the filtered QRS, the duration of terminal QRS below 40 mu V, and the root mean square amplitude of the terminal 40 msec. Cardiac catheterization was performed on 19 patients. Eighteen healthy volunteers were studied as a control. Ventricular arrhythmias were found in 13 patients; right ventricular systolic hypertension was found in 1 patient. No significant residual ventricular septal defects were detected. Eight patients had ventricular late potentials. Right ventricular systolic pressure did not differ significantly between patients with or without late potentials. There were significant differences between patients with ventricular arrhythmias and healthy volunteers; filtered QRS duration was significantly longer in patients with ventricular arrhythmias. SAECG may be beneficial in determining ventricular arrhythmia risk in tetralogy of Fallot patients postoperatively

Dilated cardiomyopathy associated with dystrophic epidermolysis bullosa: role of micronutrient deficiency?

Autosomal recessive dystrophic epidermolysis bullosa (DEB) is a chronic skin disorder characterized by widespread bullous formation, erosions, and scar formation. There have been reports of dilated cardiomyopathy and death in patients with DEB. The pathogenesis of cardiomyopathy in DEB remains uncertain, but some drugs, viral infections, iron loading, micronutrient deficiencies such as selenium and carnitine have been implicated. A 16-year-old boy who was followed-up from birth with the diagnosis of DEB presented with respiratory distress and heart failure symptoms of two-week history and early fatigue within the past year. Etiological evaluation showed a low plasma selenium level. Echocardiographic examination yielded the diagnosis of dilated cardiomyopathy. Findings of viral serology tests and metabolic screening were normal. Selenium replacement and anticongestive treatment were initiated, which led to partial improvement in cardiac functions. The authors draw attention to the possible role of micronutrient deficiency in the development of cardiomyopathy in patients with DEB

Recurrent hemiparesis due to anterior mitral leaflet myxomas

Strokes, cardiac or noncardiac, generally affect the elderly and only occasionally occur in children. A tendency to stroke occurs in cyanotic congenital heart disease; however, this report describes two female patients, aged 6 years and 4 years, who had no cyanotic disease. The first patient was admitted to the hospital after the first stroke but suffered a second one after admission. Her initial cardiac examination was normal. The other patient was admitted after a second right-sided hemiparesis. A mild pansystolic ejection murmur was observed at the lower left sternal border on auscultation; slight late diastolic murmurs with opening snap were found in the mitral valve area, and the second heart sound was increased. Transthoracic echocardiography revealed a mass in the anterior mitral valve in both patients. These masses were completely removed surgically and pathology revealed myxomas. Hemiparesis completely resolved after surgical and medical treatment. The clinical manifestations of cardiac tumors primarily depend on their number, size, location, and histology. Strokes and anterior mitral valve masses are extremely rare in children. In the two cases reported here, anterior mitral valve myxomas caused systemic embolization as a result of their location. Echocardiography should be performed early in patients presenting with recurrent strokes to exclude such pathology. (c) 2006 by Elsevier Inc. All rights reserved

Pandemic Influenza A (H1N1) virus-associated acute myopericarditis

Although pandemic influenza A (H1N1) virus may cause an upper respiratory tract infection similar to that caused by seasonal influenza, it can lead to serious complications such as pneumonia, encephalitis, and myocarditis. We describe a 2-year-old girl admitted with fever, cough, and vomiting followed by acute respiratory distress and diagnosed as H1N1-related myopericarditis. (Turk Arch Ped 2011; 46: 337-9

Pandemik İnfluenza A (H1N1) virüsü ile ilişkili akut miyoperikardit

Although pandemic influenza A (H1N1) virus may cause an upper respiratory tract infection similar to that caused by seasonal influenza, it can lead to serious complications such as pneumonia, encephalitis, and myocarditis. We describe a 2-year-old girl admitted with fever, cough, and vomiting followed by acute respiratory distress and diagnosed as H1N1-related myopericarditis. (Turk Arch Ped 2011; 46: 337-9

The comparison a 16-year follow-up results of balloon angioplasty for aortic coarctation in children of different age groups: a single-center experience

Objective: Pediatric patients with different age groups who underwent balloon angioplasty for aortic coarctation were evaluated for recoarctation, aneurysm, peripheral arterial injuries and concomitant diseases