No abstract

Patients with tetralogy of Fallot (TOF) after total correction usually have residual pulmonary regurgitation resulting in right ventricular (RV) dilatation and dysfunction. This study was performed to evaluate N-terminal pro-brain natriuretic peptide (NT-proBNP) in predicting RV dilatation and RV dysfunction in TOF after total correction. Twenty-one patients with TOF after total correction (12 males and 9 females, 12.06 +/- 2.54 years old) underwent echocardiography, cardiac magnetic resonance imaging (MRI), and blood sampling for NT-proBNP. Mean time after total correction was 7.59 +/- 2.30 years. From cardiac MRI study, mean right ventricular end diastolic volume index (RVEDVi) was 148.36 +/- 64.50 ml/m2 and mean right ventricular ejection fraction (RVEF) was 35.50 +/- 10.50%. Right ventricular dilatation was considered if RVEDVi was >108 ml/m2 and RV dysfunction was considered if RVEF was <40%. A plasma NT-proBNP level of 115 pg/ml was identified by receiver operating characteristic analysis in predicting RV dilatation and/or dysfunction. At this value, the sensitivity and specificity for predicting RV dilatation, RV dysfunction, and both RV dilatation and dysfunction were 71 and 100%, 71 and 71%, and 83 and 78%, respectively. In conclusion, plasma NT-proBNP level may be helpful in follow-up patients. Plasma NT-proBNP levels >115 pg/ml can be used as a marker in the detection of RV dilatation and dysfunction.

Background: Recent advances in stem cell therapy to restore cardiac function have great promise for patients with congestive heart failure after myocardial infarction in an adult population. Objective: We examined the benefits of bone marrow-derived progenitor cells treatment modality for the pediatric patient. Methods and Results:We present our first case of transcoronary autologous stem cell transplantation in a 9-year-old girl with refractory congestive heart failure secondary to myocardial infarction 1 year after transcatheterrevascularization. The child received daily injections of granulocyte colony-stimulating factor for 3 days prior to the bone marrow aspiration.The bone marrow cells were isolated to constituteCD133+/CD34+ more than 90% of the total number. Subsequently, the progenitor cell suspension was injected via a transcoronary catheter without any complication. Three months after stem cell therapy, her cardiac function, assessed by both cardiac magnetic resonance and echocardiogram, has been improved with the left ventricular ejection fraction at 47% compared to the baseline of 30%. Conclusion: This is the first reported pediatric case of successful transcoronary injection of bone marrowderived progenitor cells for end-stage heart disease. The procedure is considered safe and feasible for the pediatric population. IntroductionMyocardial infarction (MI) occurs rarely in children. Given the infrequency of direct experience with MI in pediatric patients, the management of these children is an extrapolation of the experience in adult patients. Young patients are at a further disadvantage due to the lack of treatment options usually available to adult patients, particularly cardiac transplantation for end-stage heart disease due to the shortage of organ donors. With the recent advances in stem cell therapy, the use of this technique to restore cardiac function holds great promise for patients with postinfarction congestive heart failure (CHF) refractory to medical therapy. 1,2 To our knowledge, there have been no previously reported cases on the use of stem cell transplantation for the treatment of postinfarction heart failure in children. We present our first experience in transcoronary autologous stem cell transplantation for a 9-year-old girl with CHF secondary to MI.

We recommended the appropriate CVC length via RIJV approach should be between these two calculated lengths and the CVC length in each age according to the BSA.

Giant coronary artery aneurysms caused by Kawasaki disease are not common; however, they are one of the most serious complications and can be fatal. Here, we describe a 5-year-old girl with Kawasaki disease who initially had normal coronary arteries. Despite intravenous immunoglobulin, she developed progressive giant coronary artery aneurysms. Echocardiography is a non-invasive tool for imaging the condition but it does have some limitations, whereas selective coronary angiography is the gold standard. However, multidetector CT may be a better non-invasive tool, alternate to invasive catheterized selective coronary angiography, in the long-term follow-up of patients with a giant coronary aneurysm.

We present a 10-month-old boy with cyanosis. This is a rare case of pulmonary atresia, ventricular septal defect (VSD), major aorto-pulmonary collateral arteries (MAPCAs) to the right lung with absent native right pulmonary artery (RPA) in association with anomalous left pulmonary artery (LPA) from the ascending aorta (AAo). Echocardiography was unable to identify all of the cardiovascular abnormalities. Multidetector CT demonstrated all of these abnormalities and is the investigation of choice instead of cardiac catheterization.

We report a boy with constitutional deletion 13q chromosome associated with dysmorphic features and bilateral retinoblastoma. The patient developed secondary Burkitt lymphoma 5 years after the diagnosis of retinoblastoma at the age of 8 months. He has completed treatment for both malignancies. At present, he is 7 years old and still in remission.

We present a case of an 8-year-old boy diagnosed as a tetralogy of Fallot with anatomically corrected malposition of the great arteries by echocardiography and cardiac catheterization. Multidetector CT nicely elucidated the course of the single coronary artery from the right coronary cusp, which traverses across the right ventricular outflow tract.