Bidirectional Glenn procedure improves the mechanical efficiency of a total cavopulmonary connection in high-risk fontan candidates.

Background: A total cavopulmonary connection (TCPC) is a widely performed surgical procedure for Fontan candidates. High-risk candidates who have undergone the bidirectional Glenn procedure (BDG) before TCPC have shown good results. The exact mechanism of this procedure, however, is still poorly understood.

High-intensity basal ganglia lesions on T1-weighted images in two toddlers with elevated blood manganese with portosystemic shunts.

We report two toddlers with portosystemic shunts who had symmetrical high-signal globus pallidus lesions on T1- but not T2-weighted MRI, and measurement of whole blood manganese at 2 years of age. These cases suggest that portosystemic shunts can cause elevation of blood manganese and result in manganese accumulation in the globus pallidus, causing high signal on T1-weighted images even in asymptomatic toddlers.

Perinatal management of congenital complete atrioventricular block: report of nine cases.

Background: The addition of fetal ultrasonography has allowed the prenatal diagnosis and observation of congenital complete atrioventricular block (CCAVB). Thus, the management of the affected fetuses should be modified accordingly.

Methods: The medical records were reviewed to identify patients with CCAVB, and clinical and laboratory data were collected.

Diagnosis and management of partial anomalous pulmonary venous connection. A review of 28 pediatric cases.

Medical records, angiograms and operative records of 28 patients with partial anomalous pulmonary venous connection (PAPVC) were reviewed. Twenty patients had one anomalous pulmonary vein (APV), and 8 had more than two APVs. Twenty-five patients (89%) had APVs originating from the right lung, 2 (7%) from the left lung and 1 (4%) from both lungs.

Life expectancy and social adaptation in individuals with Down syndrome with and without surgery for congenital heart disease.

Life expectancy and social adaptation in 373 children with Down syndrome with and without congenital heart disease (CHD) were assessed retrospectively. Survival at age 24 years was 92.2% for patients without CHD (n=200), and 74.