Catheter ablation of drug resistant supraventricular tachycardia in neonates and infants

Background: The aim of this study was to evaluate the indications, results and complicationsof radiofrequency ablation (RFA) and transcatheter cryoablation (TCA) in neonates andinfants with incessant drug-resistant supraventricular tachycardia (SVT).Methods: Out of 225 patients who underwent RFA and TCA at our center between January2010 and February 2012, 5 patients under the age of 1 (4 male, 1 female) were evaluated. Theindication for RFA/TCA was recurrent hemodynamically compromising drug-resistant SVT.Results: Over a 2-year period, 6 ablation procedures were performed in 5 patients. Averagepatient age was 3.3 ± 3.9 months (12 days – 9.5 months); average patient weight was 5.4 ± 2.2 kg (3.5–9 kg). One patient had ventricular septal defect, 1 had corrected transposition of great arteries, ventricular septal defect, right ventricular hypoplasia and pulmonary hypertension, while 3 had only patent foramen ovale. Electrophysiology study showed 1 accessory pathway in each patient (right posteroseptal in 2, left posteroseptal in 2 and left lateral in 1). The pathway was manifest in 1 patient with Wolff-Parkinson-White syndrome (WPW) andconcealed in the rest. Two of the concealed pathways had slow conduction time and decremental properties (the permanent form of junctional reciprocating tachycardia). Two patients underwentTCA and 3 — RFA, with an acute success rate of 100%. In the first week after the procedure, the patient with the complex cardiac anomaly and WPW developed recurrence and under went ablation again. Four of the procedures were carried out using an electroanatomic mapping system besides fluoroscopy. Average procedure time was 167 min (100–234); fluoroscopy time was 8.2 min (0.7–19.7). None of the patients developed major complications. After the average follow-upperiod of 6.5 months (3–18), all patients were symptom-free without medication.Conclusions: RFA and TCA can be performed successfully in neonates and infants within cessant medically refractory SVT

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

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

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

Osler-Weber-Rendu sendromu ile ilişkili pulmoner arteriyovenöz malformasyon

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

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

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

Acute pericarditis and transient erythroblastopenia associated with human parvovirus B19 infection

We report on an eight-year-old girl with acute pericarditis and transient erythroblastopenia associated with human parvovirus B19 (PVB19) infection. The patient presented with complaints of fever, chest pain, fatigue, and shortness of breath. On physical examination, she had tachycardia, hepatomegaly, and muffled heart sounds. Teleradiography exhibited cardiomegaly and echocardiography showed a pericardial effusion of 25 mm. Serum anti-PVB19 IgM and PVB19 DNA were positive. The patient developed anemia and reticulocytopenia in the second week, both of which persisted for two weeks then resolved spontaneously. At the end of three months, pericardial effusion resolved, hemoglobin and hematocrit levels were normal, and serum anti-PVB19 IgM was negative. This case represents the first report of acute pericarditis associated with PVB19 infection in a pediatric patient