Late hepatic dysfunction and cirrhotic change were often seen in Fontan patients. Moreover, hepatic complications were correlated with the duration of Fontan circulation. Therefore, after a Fontan operation, regular evaluation of the hepatic condition is required--for which some non-invasive hepatic fibrosis markers can be effectively used.

Midterm outcomes of PVR in patients with chronic PR were acceptable. PVR should be considered before RV EDVI exceeds 163 ml/m(2) or RV ESVI exceeds 80 ml/m(2), with more attention to RV ESVI.

Background: There have been no studies on the clinical characteristics and prognostic factors of pediatric ventricular tachycardia (VT). Methods and Results:Eighty-one patients with pediatric VT were studied retrospectively at a single center. The median follow-up period was 6.0 years (0.7-23.5 years). Patients were categorized into 6 groups: idiopathic VT (IVT, n=37), catecholaminergic polymorphic VT (CPVT, n=10), congenital heart disease-associated VT (n=15), myocarditis-associated VT (n=8), cardiomyopathy-associated VT (CMP-VT, n=5) and miscellaneous. The age distribution of VT had 2 peaks (infant and teenager). VT with left bundle branch block configuration was more frequently nonsustained than VT with right bundle branch block configuration (61% vs 8%). Although 22% were asymptomatic, 38% experienced syncope or seizure and 16% had cardiac arrest. The overall mortality rate was 7.4%. The expected life span without cardiac arrest was <4 years in the CMP-VT group and the 10-year survival rate in CPVT patients was approximately 55%. Onset at infancy, monomorphic type and transcatheter/surgical ablation were related to the successful resolution of VT. Logistic regression analysis revealed that CPVT, CMP-VT, polymorphic VT and sustained VT were significantly correlated with death or cardiac arrest. Conclusions:The clinical features and prognosis of pediatric VT differed with the VT type, clinical categories and onset age. Accurate diagnosis and proper treatment according to the clinical categories may improve the outcome. (Circ J 2010; 74: 1951 - 1958

To investigate intrinsic vascular abnormalities of the ascending aorta before adulthood in Turner syndrome patients (TS), we compared 25 adolescent TS patients (mean age 14.6 ± 3.4 years) and 16 healthy controls from a university hospital. Blood pressure and other vascular indices were evaluated using echocardiography such as aortic strain, stiffness index, distensibility, and pulse wave velocity. Aortic strain (0.1237 vs. 0.1865, respectively; P = 0.003) and aortic distensibility (0.0049 vs. 0.0081, P = 0.002) were significantly lower in the TS group than in the controls. The fractional area change on velocity vector imaging was also lower in TS patients (29.05 vs. 36.19, P = 0.002). These findings were still observed after adjustment for age, body mass index, and systolic blood pressure. The aortic stiffness index was greater in the TS patients than in the control group (6.79 vs. 3.34, P = 0.02). The pulse wave velocity and ascending aorta diameter did not significantly differ between the TS and control groups. Blood pressures were significantly higher in the TS patients than in the controls (systolic, 120.4 vs. 108.4 mmHg, P = 0.001; diastolic 71.5 vs. 61.7 mmHg, P < 0.001). Although the dimensional changes in the aorta were not clearly observed in adolescents with TS, the elastic properties of the aorta were significantly decreased in TS patients as compared to control subjects.

Detachment of the TV can be used safely for better exposure of the VSD without increased risk of tricuspid regurgitation in infants younger than 3 months. Preoperative echocardiography might be useful for predicting the possibility of detachment of the TV for enhancing exposure of the VSD.