Contributions of Respiration and Heartbeat to the Pulmonary Blood Flow in the Fontan Circulation.

Background: In the Fontan circulation, driving forces with respiration, heartbeat, and lower limb muscle pump are relevant. However, the mechanics of these forces has not been proven, and their effects on the Fontan circulation remain unclear.

Methods: We performed catheter examinations and measured pressure and flow velocity simultaneously in the bilateral pulmonary arteries of 12 Fontan patients 1 year after the operation.

Timing of Haemophilus influenzae type b vaccination after cardiac surgery.

Background: The best time for vaccination in infants with congenital heart disease (CHD) after cardiopulmonary bypass (CPB) surgery is unclear, but it is important to prevent Haemophilus influenzae type b (Hib) infection in infants with CHD after CPB surgery. To identify the best time for Hib vaccination in infants with CHD after CPB surgery, we investigated the immunological status, and the efficacy and safety of Hib vaccination after CPB surgery.

Methods: Sixteen subjects who underwent surgical correction of ventricular septal defect with CPB were investigated.

Quantitative evaluation of hemodynamics in the Fontan circulation: a cross-sectional study measuring energy loss in vivo.

Flow energy loss (EL) at the Fontan anastomosis has been thought to reflect flow efficiencies and to influence on hemodynamics in the Fontan circulation and has been often discussed in numerical studies. However, in vivo EL measurements have to date not been reported. We directly measured EL in the Fontan circulation and examined the relationship between the structural configuration and EL, as well as the influence of EL, on the hemodynamics in the Fontan circulation.

Noninvasive assessment of pulmonary vascular resistance and pressure in patients with congenital heart disease: a new method using M-mode echocardiography.

Background: The accurate evaluation of pulmonary vascular resistance (PVR) and mean pulmonary artery pressure is important to determine the optimal management and therapeutic strategy for patients with congenital heart disease (CHD). We evaluated the PVR and mean pulmonary artery pressure in 46 patients with several CHD types using the interventricular septum (IVS) motion determined by M-mode echocardiography.

Methods: We divided the patients into 2 groups according to the different IVS motions.

The lower limit of the pulmonary artery index for the extracardiac Fontan circulation.

Objective: In the era of the staged Fontan operation, small pulmonary artery index (<250 mm(2)/m(2)) has not affected the early or midterm results. The lower limit of pulmonary artery index, however, has not yet been determined. We created numeric models of the Fontan circulation to investigate the lower limit of the pulmonary artery size.

Clinical score and transcript abundance patterns identify Kawasaki disease patients who may benefit from addition of methylprednisolone.

Intravenous immunoglobulin (IVIG) treatment-resistant patients are high risk of developing coronary artery lesions with Kawasaki disease. The IVIG-responsive (Group A; n = 6) and IVIG-resistant patients (Group B) were predicted before starting the initial treatment using the Egami scoring system and randomly allocated as a single-IVIG treatment group (group B1; n = 6) or as a IVIG-plus-methylprednisolone (IVMP) combined therapy group (group B2; n = 5). We investigated the transcript abundance in the leukocytes of those patients using a microarray analysis.

Optimal conduit size of the extracardiac Fontan operation based on energy loss and flow stagnation.

Background: In the extracardiac Fontan operation, larger conduits are used when considering the patients' growth rate. However, larger conduits may cause inefficient flow due to turbulence or stagnation, resulting in late problems such as thrombosis or stenosis. Our objective was to reveal the physiologic effects of respiration and exercise using numerical models, based on the energy loss and flow stagnation, and to determine optimal conduit size.

The strategy of immune globulin resistant Kawasaki disease: a comparative study of additional immune globulin and steroid pulse therapy.

Background: We compared the clinical utility of additional intravenous immune globulin (IVIG) therapy with the clinical utility of steroid pulse therapy in patients with IVIG-resistant Kawasaki disease.

Methods: We enrolled 164 patients with Kawasaki disease who were treated with a single dose of IVIG (2 g/kg) and aspirin (30 mg/kg per day). Twenty-seven of these patients (16%) were resistant to the initial IVIG treatment.

Visualization of interscapular brown adipose tissue using (99m)Tc-tetrofosmin in pediatric patients.

Unlabelled: Brown adipose tissue (BAT) is a site of nonshivering thermogenesis in mammals. The mitochondria of BAT operate in an uncoupled mode and increase fatty acid oxidation to produce heat at birth. Thus, the BAT of human infants and children contains more active mitochondria than that of adults.

Transthoracic Doppler assessment of coronary flow velocity reserve in children with Kawasaki disease: comparison with coronary angiography and thallium-201 imaging.

Objectives: The purpose of this study was to determine the feasibility of coronary flow velocity reserve (CFVR) measurement by transthoracic Doppler echocardiography (TTDE) in children with Kawasaki disease (KD).

Background: Doppler-derived CFVR is a reliable marker predicting the presence of myocardial ischemia.

Methods: We studied 49 patients (median age 11 years) with KD.