Suvipaporn Siripornpitak

Cardiac CT angiography in children with congenital heart disease

Cardiac imaging plays an important role in both congenital and acquired heart diseases. Cardiac computed tomography (angiography) cCT(A) is a non-invasive, increasingly popular, complementary modality to echocardiography in evaluation of congenital heart diseases (CHD) in children. Despite radiation exposure, cCT(A) is now commonly used for evaluation of the complex CHD, giving information of both intra-cardiac and extra-cardiac anatomy, coronary arteries, and vascular structures. This review article will focus on the fundamentals and essentials for performing cCT(A) in children, including radiation dose awareness, basic techniques, and strengths and weaknesses of cCT(A) compared with cardiac magnetic resonance imaging (cMRI), and applications. The limitations of this modality will also be discussed, including the CHD for which cMRI may be substituted.

N-Terminal Pro-Brain Natriuretic Peptide as a Marker in Follow-Up Patients with Tetralogy of Fallot After Total Correction

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.

Transcoronary Bone Marrow-Derived Progenitor Cells in a Child With Myocardial Infarction: First Pediatric Experience

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.

Hypoplastic Right Lung Associated with Right Pulmonary Vein Stenosis and Systemic Collateral

We present a case of a 6-month-old girl diagnosed as unilateral pulmonary vein stenosis (right inferior pulmonary vein) with dextroposition of the heart, hypoplastic right lung, hypoplastic right pulmonary artery, collateral from the descending aorta supplied right lower lung, connection between collateral and right pulmonary artery with retrograde flow from right pulmonary artery into main pulmonary artery, and right superior and middle pulmonary vein agenesis. A combined modality of imaging including chest film, echocardiography, multidetector computerized tomography, and the cardiac catheterization nicely demonstrated all of the unique and rare congenital abnormalities.

Prevalence and predictors of cardiac and liver iron overload in patients with thalassemia: A multicenter study based on real-world data

Prevalence of cardiac and liver iron overload in patients with thalassemia in real-world practice may vary among different regions especially in the era of widely-used iron chelation therapy. The aim of this study was to determine the prevalence of cardiac and liver iron overload in and the management patterns of patients with thalassemia in real-world practice in Thailand. We established a multicenter registry for patients with thalassemia who underwent magnetic resonance imaging (MRI) as part of their clinical evaluation. All enrolled patients underwent cardiac and liver MRI for assessment of iron overload. There were a total of 405 patients enrolled in this study. The mean age of patients was 18.8±12.5years and 46.7% were male. Two hundred ninety-six (73.1%) of patients received regular blood transfusion. Prevalence of cardiac iron overload (CIO) and liver iron overload (LIO) was 5.2% and 56.8%, respectively. Independent predictors for iron overload from laboratory information were serum ferritin and transaminase for both CIO and LIO. Serum ferritin can be used as a screening tool to rule-out CIO and to diagnose LIO. Iron chelation therapy was given in 74.6%; 15.3% as a combination therapy.

Pulmonary atresia and ventricular septal defect with collaterals to right lung associated with anomalous left pulmonary artery from the ascending aorta

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.