Permanent cardiac pacing in a 2.5 month-old infant with severe cyanotic breath-holding spells and prolonged asystole

We report the case of a 2.5 month-old infant with cyanotic breath-holding spells, loss of consciousness and seizures. Prolonged asystole up to 70 s despite cardiopulmonary resuscitation was documented by 24 hour Holter monitoring. An epicardial pacemaker was implanted followed by no further loss of consciousness and seizures during spells in a six month follow-up period. (Cardiol J 2011; 18, 6: 704–706

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

Evaluation of coronary artery abnormalities in Williams syndrome patients using myocardial perfusion scintigraphy and CT angiography

Background: Sudden death risk in Williams syndrome (WS) patients has been shown to be 25–100 times higher than in the general population. This study aims to detect coronary artery anomalies and myocardial perfusion defects in WS patients using noninvasive diagnostic methods. Methods: This study features 38 patients diagnosed with WS. In addition to physical examination, electrocardiography, and echocardiography, computed tomography (CT) angiography and rest/dipyridamole stress technetium-99m sestamibi (99mTc-sestamibi) single photon emission computed tomography (SPECT) myocardial perfusion scintigraphy (MPS) were performed. Results: Twenty-one (55%) patients were male; 17 (45%) were female. The average patient age was 12 ± 5 years (2.5–26 years); the average follow-up period was 7.2 ± 4.2 years (6 months–18 years). Cardiovascular abnormalities were found in 89% of patients, the most common one being supravalvar aortic stenosis (SVAS). CT angiography revealed coronary anomalies in 10 (26%) patients, the most common ones being ectasia of the left main coronary artery and proximal right coronary artery as well as myocardial bridging. SVAS was present in 80% of patients with coronary artery anomalies. 99mTc-sestamibi SPECT MPS revealed findings possibly consistent with myocardial ischemia in 29% of patients, and ischemia in 7 out of 10 patients (70%) with coronary anomalies shown on CT angiography (p = 0.03). Conclusions: Coronary artery abnormalities are relatively common in WS patients and are often accompanied by SVAS. CT angiography and dipyridamole 99mTc-sestamibi SPECT MPS seem to be less invasive methods of detecting coronary artery anomalies and myocardial perfusion defects in WS patients

Pulmonary arteriovenous malformation associated with Osler-Weber-Rendu syndrome

Osler-Weber-Rendu syndrome is a relatively common vascular displasia of children presented with telengiectasias of skin, mucosa, and visceral organs and arteriovenous malformations. We reported a 9-year-old boy diagnosed as having Osler-Weber Rendu syndrome with his central cyanosis, clubbing, facial and nasal mucosal telengiectasias and right pulmonary arteriovenous malformation during an attack of acute rheumatic carditis. After relief of the acute rheumatic carditis attack, his arteriovenous malformation was treated with coil embolisation by cardiac catheterization. (Turk Arch Ped 2011; 46:264-6

The effect of DALI lipid apheresis in the prognosis of homozygous familial hypercholesterolemia: Seven patients' experience at a DALI apheresis center

Introduction : Familial hypercholesterolemia (FH) is characterized by severe hypercholesterolemia that can result in coronary artery disease occurring at an early age. If patients are not cured with lipid-lowering drugs and diets, lipid apheresis may be an effective treatment option in these cases. Here, we evaluate the efficacy, selectivity and safety of the DALI apheresis technique

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