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Introduction: It is reported that pressure wave reflection is enhanced by external compression of the femoral artery. Therefore, it is possible that cardiac catheterization itself can influence the aortic pressure waveform., Aim: The purpose of this study is to clarify the influence of sheath placement in a femoral artery on the pressure waveform., Methods: This study enrolled 21 pediatric patients (5.1±4.0years) who underwent cardiac catheterization. A sheath was placed in the femoral arteries of all patients. The change in the pressure waveform induced by the placement of the sheath was investigated using the b/a and d/a ratio of second derivative of a fingertip photoplethysmogram. A high b/a ratio means a stiff aorta and a low d/a ratio represents an enhancement of the aortic pressure wave reflection., Results: By the placement of the sheath in their femoral arteries, the b/a ratio was not influenced (sheath (-): -0.556±0.081 vs. sheath (+): -0.558±0.072; p=0.896). However, the d/a ratio was significantly decreased (-0.150±0.074 vs. -0.185±0.084; p=0.0003)., Conclusions: The placement of the femoral arterial sheath enhances the pressure wave reflection and would lead to a change in the central aortic pressure waveform., (Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.)

Background: Recent studies showed that children with univentricular heart have elevated plasma B-type natriuretic peptide (BNP) levels prior to bidirectional cavopulmonary anastomosis (BDCPA). However, it remains to be established whether BNP levels reflect the degree of hemodynamic overload and relate to long-term outcome in univentricular circulation., Methods: Fifty one consecutive children with functionally univentricular heart prior to BDCPA were studied. All patients underwent cardiac catheterization and BNP measurement. Ventricular end-systolic wall stress (ESWS) and end-diastolic wall stress (EDWS) were calculated from cardiac catheterization data., Results: Median age was 1.1 years and 34% were female. Median BNP concentration was 90.4 pg/ml. Patients with high BNP (≥ 100 pg/ml) had higher pulmonary to systemic flow ratio (p = 0.014), a greater end-diastolic volume (p=0.009), more severe atrioventricular valve regurgitation (p= 0.02) and lower ventricular mass to end-diastolic volume ratio (p=0.006). BNP levels strongly related to EDWS (r = 0.75, p< 0.0001) and ESWS (r = 0.63, p < 0.0001). During median follow-up period of 3.2 years, 15 patients died and one underwent heart transplantation for refractory heart failure. On multivariate Cox regression analysis, high BNP concentration was an independent predictor of death or transplantation (HR 3.05, CI: 1.06-8.83, p = 0.04)., Conclusions: High BNP concentration at the first palliative stage towards Fontan circulation reflects high wall stress due to high volume load and insufficient ventricular hypertrophy. Moreover, high BNP levels at this stage were independently related to long-term outcome. BNP may be used as a guide to identify patients with high workload due to inadequate adaptation to hemodynamic load, who are at high risk., (Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.)

Objective: The extracardiac conduit procedure is widely used for patients with heterotaxy syndrome with complex systemic and pulmonary venous anomalies; however, it lacks conduit-growth potential and requires long-term anticoagulation. We present the intracardiac Fontan procedure, which eliminates the above-mentioned disadvantages., Patients and Methods: Twenty-four patients (mean age, 4.1 years; weight, 13.0 kg) with heterotaxy syndrome underwent intracardiac Fontan operations between March 1995 and March 2008. In each patient, the anomalous systemic venous return with the isolated hepatic vein was redirected to the pulmonary artery using an intra-atrial baffle without obstructing the pulmonary venous pathway; this was accomplished by anterior (n=5), lateral (n=15) or posterior tunnel methods (n=4), depending on the anatomical relationship of the systemic and pulmonary venous pathways., Results: There was one (4%) in-hospital and two (8%) late deaths in total. Five (21%) patients underwent re-operation for either pulmonary venous obstruction or supraventricular tachycardia. The actuarial 12-year survival was 86% (the Kaplan-Meier survival plot). The freedom from re-operation at 10 years was 77%. Anticoagulation was not required except for two patients (8%) who had prosthetic valves and coagulation disorder. Obstruction of the systemic venous pathway was not observed in any patient; however, five (20%) patients had clinically significant postoperative arrhythmias. At the final follow-up, all survivors were categorised as the New York Heart Association class I., Conclusions: Aided by detailed preoperative anatomical and physiological diagnoses, intracardiac Fontan procedures were technically feasible in patients with complex systemic and pulmonary venous anomalies. The specific cardiac anatomy in these patients warranted this procedure; however, taking into consideration the improved outcomes of the modified Fontan procedure, this method should be performed with deliberation., (Copyright 2009 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.)

Objectives: We sought to provide a new method for quantifying collateral aortopulmonary flow in patients subsequent to construction of a bidirectional cavopulmonary shunt, and to clarify the clinical advantages of the new method., Methods: We performed lung perfusion scintigraphy and cardiac catheterization in 10 patients subsequent to construction of a bidirectional cavopulmonary shunt. First, the ratio of collateral to systemic flow was determined by whole-body images of lung perfusion scintigraphy, dividing the total lung count by the total body count minus the total lung count. Second, we integrated lung perfusion scintigraphy and cardiac catheterization data using a formula derived from the Fick principle, taking the ratio of pulmonary to systemic flow to be 1 plus the ratio calculated above and multiplied by the systemic saturation minus the inferior caval venous saturation divided by the pulmonary venous saturation minus the inferior caval venous saturation. Finally, the amount of collateral flow was obtained from the ratio of pulmonary to systemic flow. We evaluated the impact of collateral flow on the calculation of pulmonary vascular resistance., Results: The median age at bidirectional cavopulmonary shunt was 1.41 years, and the median age at catheterization was 2.33 years. The mean amount of collateral flow was 1.75 +/- 0.46 litres/min/m(2). The pulmonary vascular resistance calculated without considering the collateral flow was overestimated by an average of 57 +/- 23%, compared to the resistance calculated with our new method., Conclusions: The use of scintigraphy combined with catheterization allows accurate determination of aortopulmonary collateral flow, and avoids overestimation of pulmonary vascular resistance in these candidates for the Fontan circulation.

Purpose: The purpose of this study was to identify the risk factor of interim failure and to consider the method for detecting high-risk patients after Blalock-Taussing shunt., Methods: Between 1990 and 2004, 64 patients with functional single ventricle and low pulmonary flow underwent Blalock-Taussing shunt. We evaluated risk factors of interim failure by Logistic regression. Furthermore, assessments by catheterization and echocardiography were compared between Group Completion (Group C; 36 patients who had completed the Fontan operation or were Fontan candidates) and Group Failure (Group F; 20 patients who died before the Fontan operation or dropped out from Fontan candidate)., Results: In Group C, the survival at 10 years was 93%. The risk factor of interim failure was a systemic ventricular end-diastolic volume (SVEDV) of more than 250% of normal after shunt. The ventricular volume by echocardiography in Group C had been maintained between 190% and 200% of normal. By contrast, the ventricle in Group F had been significantly dilated after postshunt short term., Conclusions: The risk factor of interim failure was postshunt ventricular dilation, and the dilation was identified after postshunt short term. We recommend volume study within two to three months after shunt, and bidirectional cavopulmonary anastomosis should be immediately indicated to patients with ventricular dilation.

Recently, GATA4 and NKX2.5 were reported as the disease genes of atrial septal defect (ASD) but the relationship between the locations of their mutations and phenotypes is not clear. We analyzed GATA4 and NKX2.5 mutations in 16 familial ASD cases, including four probands with atrioventricular conduction disturbance (AV block) and two with pulmonary stenosis (PS), by PCR and direct sequencing, and examined their phenotypes clinically. Five mutations, including two GATA4 and three NKX2.5 mutations, were identified in 31.3% of the probands with ASD, and three of them were novel. The two GATA4 mutations in the probands without AV block were S52F and E359Xfs (c.1075delG) that was reported previously, and three NKX2.5 mutations in the probands with AV block were A88Xfs (c.262delG), R190C, and T178M. Additionally, we observed some remarkable phenotypes, i.e., dextrocardia with E359Xfs (c.1075delG) and cribriform type ASD with R190C, both of which are expected to be clues for further investigations. Furthermore, progressive, most severe AV block was closely related with a missense mutation in a homeodomain or with a nonsense/frame-shift mutation of NKX2.5 for which classification has not been clearly proposed. This pinpoints essential sites of NKX2.5 in the development of the conduction system., ((c) 2005 Wiley-Liss, Inc.)

Kawasaki disease (KD) is an acute systemic vasculitis syndrome of infants and young children. Although its etiology is largely unknown, epidemiological findings suggest that genetic factors play a role in the pathogenesis of KD. To identify genetic factors, affected sib-pair analysis has been performed. One of the identified peaks was located on the Xq26 region. A recent report of elevated expression of CD40 ligand (CD40L), which maps to Xq26, during the acute-phase KD, and its relationship to the development of coronary artery lesions (CAL) prompted us to screen for polymorphism of CD40L and to study the association of the gene to KD. A newly identified SNP in intron 4 (IVS4+121 A>G) is marginally over-represented in KD patients as compared to controls (109/602, 18.1 vs 111/737, 15.1%). When male KD patients with CAL were analyzed as a patient group, the SNP was significantly more frequent than in controls (15/58, 25.9%, vs 111/737, 15.1%, OR=2.0, 95% CI=1.07-3.66; P=0.030). Interestingly, this variation was extremely rare in a control Caucasian population (1/145, 0.7%). Our results suggest a role of CD40L in the pathogenesis of CAL and might explain the excess of males affected with KD.

It is rare to find multiple fistulas arising from all three coronary arteries and draining into the left ventricle. Coronary angiography revealed this anomaly in a one-year-old girl with tricuspid atresia after conversion to the Fontan circulation. To the best of our knowledge, this is the first report of such multiple fistulas in the setting of tricuspid atresia, and also the first report in childhood.

This study assessed the validity of cine magnetic resonance imaging (MRI) for measuring right and left ventricular volumes by using Simpson's rule in children with complex congenital heart disease. Forty-five patients with complex congenital heart disease (average age 2.6 years) and 10 controls (average age 2.3 years) were evaluated. The whole heart was encompassed by contiguous transverse sections. Ventricular volumes were calculated by adding luminal areas determined in each section at end diastole and end systole. End-diastolic and end-systolic volumes by MRI in both groups correlated well with those by ventriculography (r>0.89). Comparison of the ejection fraction in both ventricles in both groups yielded a good correlation between MRI and ventriculography (r>0.67). MRI technique in both groups had low intraobserver and interobserver variation (<6%). Cine MRI provides a suitable noninvasive means of quantifying ventricular volume in children with complex congenital heart disease.

Background: We report on 10 patients with Kawasaki disease and thrombocytopenia who were found to have a high incidence of coronary artery aneurysm and acute myocardial infarction. The clinical features of these patients, the cause of their thrombocytopenia and the relationship between cardiovascular pathology and thrombocytopenia were analyzed., Methods: The clinical features of 10 patients with Kawasaki disease found to have thrombocytopenia (group A: mean age 8.0 +/- 7.0 months) and those of 293 patients with Kawasaki disease (group B, controls: mean age 13 +/- 20 months) were analyzed. Coagulation studies and bone marrow aspiration were performed to ascertain the cause of the thrombocytopenia in nine out of 10 subjects in group A., Results: The minimum platelet count was 4-12 x 10(4)/mm3 (average day of illness, 10.3) and platelet counts were elevated to the baseline value within 1-2 weeks of onset of the illness. Low fibrinogen concentrations, high levels of fibrin degradation products, and low erythrocyte sedimentation rates with high C-reactive protein levels were observed in seven patients. In two other patients, immature megakaryocytes with normal coagulation values were observed. The differences in the incidence of coronary artery aneurysm and acute myocardial infarction between groups A and B were highly significant (coronary artery aneurysm: 60% in group A, 8.9% in group B; acute myocardial infarction: 40% in group A, 0.3% in group B)., Conclusions: In many patients with Kawasaki disease and thrombocytopenia, the thrombocytopenia appears to be a result of intravascular coagulation, and to be one of the risk factors for acute myocardial infarction.

Objectives: This study assessed the usefulness and safety of magnetic resonance imaging (MRI) for systematically diagnosing heterotaxia in infants., Background: Although it is important to diagnose and treat infants with heterotaxia, which is associated with viscerobronchial cardiovascular anomalies, systematic diagnosis of these anomalies by a single imaging technique is difficult., Methods: Twenty patients with heterotaxia were evaluated. The infants ranged in age from 21 days to 12 months (average 5.2 months, average body weight 4.3 kg). Electrocardiographically gated MRI was performed by spin echo imaging techniques operating at 0.5 tesla., Results: In all 20 patients, MRI results were sufficient to evaluate these anomalies without serious complications. In 17 patients, neither a spleen nor splenules were detected, but in 3 patients, a polymorphous spleen was visualized. In all 20 patients, bronchial anatomies were clearly visualized (bilateral eparterial bronchi in 14 patients, bilateral hyparterial bronchi in 2 and normal bronchial patterns in 4). Additionally, in a comparison of 149 observations of cardiovascular anatomy by MRI with those by angiography, discrepancies were found in only 10 observations (6.7%)., Conclusions: Magnetic resonance imaging was found to be safe and very useful for the systematic diagnosis of heterotaxia in infants.

A 3-month-old boy, mildly cyanosed and tachypneic, was found by cineangiography to have a nonbranching main pulmonary artery arising from the right ventricle and connecting to the descending aorta via a large persistent ductus arteriosus. The left and right pulmonary arteries arose as a common "trunk," before branching, from the ascending aorta. Complete surgical correction was performed at 7-months of age. Subsequent cardiac catheterization has demonstrated normal pressures and blood oxygen saturation in all right-heart chambers and the great arteries.

Septation is one of the surgical choices for double-inlet left ventricle, yet postoperative hemodynamics have not been well defined. We studied ventricular volume characteristics in 10 patients with double-inlet left ventricle before and after septation. Preoperative end-diastolic volume (EDV) of the ventricle was 291 +/- 111% (+/- SD) of normal and ejection fraction (EF) was 0.59 +/- 0.07. Postoperatively, EDV of the right-sided ventricle (RV) was 82 +/- 24%, and EDV of the left-sided ventricle (LV) was 153 +/- 41%. Ejection fraction of the RV was 0.77 +/- 0.10, and LVEF was 0.49 +/- 0.13. On the short-axis view of two-dimensional echocardiography, fractional change of the cross-sectional area was 0.65 +/- 0.16 for the RV and 0.23 +/- 0.11 for the LV. Fractional shortening of the septum-to-ventricular free wall axis was 0.51 +/- 0.17 in the RV and -0.05 +/- 0.09 in the LV. Analysis of the curvature of the new septum during cardiac cycle on two-dimensional echocardiography revealed that the septum shifted to the right side during systole in all patients in whom the systolic LV/RV pressure ratio was larger than 1.0. The septum shifted toward the LV during diastole in eight patients in whom end-diastolic pressure in the RV was higher than or equal to that in the LV, whereas it remained in the right side in two patients with higher left-side pressure. The cardiac index of these two patients was 2.4 and 2.6 l/min/m2, respectively, whereas it averaged 4.4 +/- 1.0 l/min/m2 in the other eight patients.(ABSTRACT TRUNCATED AT 250 WORDS)

We analyzed hemodynamic variables in patients with transposition of the great arteries with intact ventricular septum (simple transposition) after pulmonary artery banding and Blalock-Taussig anastomosis to establish the criteria for two-staged arterial switch operation. The study included 35 patients who underwent the switch operation after banding and anastomosis; two died shortly after surgery, and one died of tachycardia 31 days after the operation. The left-to-right ventricular pressure ratio was above 0.83 in all surviving patients, 0.68 and 0.63 in the two operative deaths, and 0.84 in the other death. In the 32 surviving patients, angiographically determined left ventricular (LV) end-diastolic volume and ejection fraction averaged 147 +/- 46(SD)% of normal and 0.64 +/- 0.12, respectively. We also measured LV mass using angiographic LV semiaxes and end-diastolic LV posterior wall thickness obtained from an echocardiogram. These variables of the patients who died were not different from those of the surviving patients. The wall thickness was greater than 4 mm in the surviving patients, while it was 3.5 mm in the two patients who died early and 5 mm in the patient who died later. From the semiaxes, the wall thickness, and aortic diastolic pressure, all of which were obtained before the anatomic repair, we calculated the predictive LV wall stress. This wall stress would indicate the LV wall stress at the time of aortic valve opening immediately after the arterial switch operation. Predictive wall stress was less than 120 x 10(3) dynes/cm2 in all but one of the surviving patients, whereas it was 151, 153, and 186 x 10(3) dynes/cm2 in the three patients who died.(ABSTRACT TRUNCATED AT 250 WORDS)