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

Balloon valvuloplasty for aortic stenosis using umbilical vein access in a newborn: First experience in Turkey

Despite risk of development of postprocedural aortic insufficiency, and restenosis at midterm, balloon valvuloplasty is an effective therapy for severe congenital aortic valve stenosis No consensus currently exists regarding optimal vascular approach for balloon dilatation in newborns with critical or severe aortic valve stenosis. Critical aortic valve stenosis in newborns must be treated promptly and effectively. Surgical therapy has been associated with significant rates of morbidity and mortality. Transcatheter therapy has significant advantages in this group of patients when compared with, surgical treatment. Percutaneous balloon dilatation is frequently performed as emergent therapy of valve ste-nosis, with various options for vascular approach. While umbilical artery and vein access has been tried in very few number of patients in the treat-ment of critical aortic valve stenosis and aortic coarctation. Umbilical artery, and vein access should be thought as an alternative to balloon dilatation of critical aortic stenosis. With available catheters this is a safe, simple, and effective procedure even in newborns weighing under 2.5 kg

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

Saccular aneurysm formation of the descending aorta associated with aortic coarctation in an infant

Aneurysm of the descending aorta associated with CoA is an extremely rare congenital abnormality. In this report, we present a 16 months old female patient in whom cardiac catheterization had been performed which had revealed a segment of coarctation and saccular aneurysm in the descending aorta. The patient was operated and a 3x2 centimeters aneurysm which embraces the coarcted segment in descending aorta was resected. In summary, we present a case of saccular aortic aneurysm distal to aortic coarctation in an infant without any history of intervention or vascular inflammatory disease. Our case report seems to be the youngest patient in literature with this pathology

Saccular Aneurysm Formation of the Descending Aorta Associated with Aortic Coarctation in an Infant

Aneurysm of the descending aorta associated with CoA is an extremely rare congenital abnormality. In this report, we present a 16 months old female patient in whom cardiac catheterization had been performed which had revealed a segment of coarctation and saccular aneurysm in the descending aorta. The patient was operated and a 3x2 centimeters aneurysm which embraces the coarcted segment in descending aorta was resected. In summary, we present a case of saccular aortic aneurysm distal to aortic coarctation in an infant without any history of intervention or vascular inflammatory disease. Our case report seems to be the youngest patient in literature with this pathology

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

Large pleural effusion necessitates chest tube drainage in a patient with Kawasaki disease

An 11-month-old Turkish boy was hospitalised with clinical and roentgen graphic evidence of large pleural effusion on the third day of fever and misdiagnosed as parapneumonic effusion. Due to worsening respiratory distress chest tube drainage was performed. Four days later the classic signs of Kawasaki disease appeared. His clinical condition improved gradually and fever subsided after intravenous gammaglobulin and aspirin treatment. A mild transient dilatation of the right coronary artery was seen and returned to the normal diameter within a few weeks. To our knowledge, large pleural effusion in a case of Kawasaki disease, in which chest tube drainage was needed, has not been reported. We describe here a patient with complete Kawasaki disease whose initial presentation mimicked a parapneumonic effusion