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

Transcatheter closure of a fistula between the right pulmonary artery and left atrium using the Amplatzer septal occluder

A congenital fistula between the right pulmonary artery (RPA) and left atrium (LA) is a rare condition that results in central cyanosis. An 11-year-old boy was admitted with exertional dyspnea and easy fatigability. He had severe cyanosis of the lips and limbs with clubbing of the fingers. Systemic oxygen saturation was 70%. There was no abnormal finding on electrocardiography, chest radiography, and echocardiography. Agitated saline injection showed early appearance of contrast bubbles in the LA. A pulmonary arteriovenous fistula was suspected and diagnostic cardiac catheterization was performed. Angiography demonstrated a large fistula between the proximal RPA and LA. The narrowest part of the fistula was 13.8 mm in balloon sizing. A 14-mm Amplatzer septal occluder was deployed at the narrowest site; however, the device migrated to the LA and then to the aortic arch. The device was removed and was successfully reimplanted to the fistula. After the procedure, arterial oxygen saturation increased from 70% to 96% and control angiography demonstrated complete occlusion of the fistula. The patient was symptom-free on follow-up evaluations at 6, 12, and 18 months, with a mean oxygen saturation of 96%. This case represents the first pediatric patient in whom a septal occluder was used

Left-sided aortic arch, right-sided descending aorta, and right-sided arterial duct associated with interruption of the aortic arch and presence of Abbott's artery

We describe a male infant, seen at the age of 10 days, with a very rare form of vascular ring. The aortic arch was left-sided, but the aorta descended on the right, with a right-sided persistently patent arterial duct associated with interruption of the aortic arch and presence of Abbott's artery. We performed end-to-side anastomosis of the descending aorta to the ascending aorta, divided the Abbott's artery and the right-sided arterial duct, and banded the pulmonary trunk. Despite our best efforts, the patient died on the eighth day after the surgical intervention

Balloon angioplasty of severe aortic coarctation in a newborn, using axillary artery access: The first experience in Turkey

Axillary artery access for demanding interventions in newborns is rarely described, in spite of the many clear advantages. This access route in newborns with critical congenital heart disease is a real alternative to the more commonly used femoral or carotid artery routes. In brief, axillary access is an attractive alternative approach in newborns with complex heart diseases. It is advisable to stop an elective procedure if axillary access is not successful. Presently described is the use of axillary artery access for balloon angioplasty in a newborn with critical aortic coarctation. To the best of our knowledge, it was the first instance in Turkey

A rare cause of recurrent wheezing and respiratory distress: Scimitar syndrome

Scimitar syndrome is characterized by partial or total anomalous pulmonary venous return from the right lung. We present a 15-month-old boy who was diagnosed with Scimitar syndrome after examinations for recurrent wheezing and respiratory distress. The chest radiograph showed a scimitar sign in the right hemithorax, obscuring the contours of the right atrium. Echocardiography showed dilatation of the right heart cavities and increased flow in the inferior vena cava, without a cardiac abnormality. The patient underwent cardiac catheterization for radiographic and hemodynamic evaluations, during which a scimitar vein was detected, draining the right pulmonary veins to the inferior vena cava. Coil occlusion was performed on the abnormal artery arising from the infradiaphragmatic aorta. The patient was referred to surgery for repair of the anomalous pulmonary venous return and resection of the sequestered pulmonary segment

Stent implantation for postoperative conduit anastomosis stenosis

Anatomical complications may occur after surgical repair procedures for congenital heart diseases. A 13-year-old boy presented with progressively decreased effort capacity. He had a history of bilateral Blalock-Taussig pulmonary shunt operation shortly after birth with diagnoses of transposition of the great arteries, pulmonary atresia, ventricular septal defect, and patent ductus arteriosus. In addition, he underwent a Rastelli operation (a left ventricular to aorta and right ventricular to pulmonary artery conduits) and reconstruction of the right ventricle outflow tract at the age of seven years. Right ventriculography showed bilateral stenosis of the pulmonary artery conduit anastomoses. Balloon angioplasty with stent implantation was performed, after which his complaints disappeared and effort capacity increased

Balloon pulmonary valvuloplasty in a premature neonate

Interventional cardiac catheterization procedures in low-birth-weight neonates are associated with specific problems including hypothermia during the procedure, difficulty in vascular access, and problems related to the small size of cardiac structures. Physical examination of a premature neonate, delivered by cesarean section at the 34th week of gestation with birth weight of 1790 g revealed a systolic murmur of 3/6 intensity. The murmur continued on the following day and cardiomegaly was observed on a chest radiogram. Echocardiographic examination revealed a patent foramen ovale with right-to-left shunt, patent ductus arteriosus, right ventricular hypertrophy, and valvular pulmonary stenosis. Balloon pulmonary valvuloplasty was performed upon detection of increased right ventricular pressure and pulmonary artery pressure gradient and development of marked tachypnea and dyspnea. Following the procedure, right ventricular pressure and pulmonary gradient decreased and the infant was discharged eight days after the procedure. Pulmonary artery pressure gradient was 56 mmHg after a month

Electrocardiographic and echocardiographic findings in street children known to be substance abusers

Substance abuse is prevalent in adolescent street children, and death is reported as secondary to aspiration, accidental trauma, asphyxia, cardiac arrhythmia, anoxia, vagal inhibition and respiratory depression. In this study, we examined electrocardiographic and echocardiographic findings from 53 street male adolescents, comparing our findings to those obtained from 61 controls in the same age group. The street children smoked cigarettes (98.1%) and had used, or were using, thinner (73.6%), glue (75.5%), hashish (79.2%), morphine or its products (24.5%), ecstasy (37.7%), anti-emetics (13.2%) and alcohol (60.4%). On examination, their blood pressures were lower than the control group. Electrocardiographically, PR, QRS, QT were found to be longer (p less than 0.05) than the values for healthy controls. Although it was not statistically significant, QTc duration was also longer than the control group. Echocardiography revealed increased diameters of the left ventricle and atrium, the aorta, and the coronary arteries as compared to the healthy children (p less than 0.05)