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In this study, we tested our hypothesis that thyroid function is impaired and contributes to perturbed hemodynamics in patients after Fontan operation. Cardiac catheterization and blood tests for thyroid function were performed in 37 patients who underwent a Fontan operation. Among them, 12 patients (33%) had subclinical thyroid dysfunction with an elevated thyroid-stimulating hormone level despite normal thyroxine levels. Thyroid-stimulating hormone levels were significantly correlated with central venous pressure (p0.01, R

Introduction: While improvement of perioperative care markedly expanded candidacy of Fontan surgery, increasing number of Fontan patients is currently subjected to advanced heart failure therapies. Low pulmonary flow before Fontan procedure has been considered to be acceptable as it keeps pulmonary resistance low, however, compromised growth of pulmonary vascular bed after Fontan completion may deteriorate potential to preserve better Fontan circulation later. We tested our hypothesis that sufficient growth of pulmonary vascular bed before Fontan surgery chronically preserves favorable Fontan hemodynamics and prevents patients from cardiovascular remodeling. Methods and Results: Consecutive 33 patients who had undergone Fontan operation (median year after the surgery: 5.3years) were enrolled in this study. During cardiac catheterization, pulmonary arterial index (PAI) as the representative of vascular bed both before and after Fontan procedure was measured and hemodynamics as well as blood samples as a marker for end-organ dysfunction were analyzed. Before Fontan procedure, PAI ranged 74-426 and resistance of pulmonary artery (PAR) ranged 0.4-3.1. After median of 5.3 years of Fontan procedure, CVP exhibited modest decline with preserving cardiac index (CI). Interestingly, central venous pressure (CVP) with chronic Fontan circulation was negatively correlated with PAI of pre-Fontan procedure (P=0.02) whereas it was independent of PAR. Consistent with this, higher PAI before Fontan procedure was also significantly associated with higher CI (P Conclusion: Sufficient pulmonary vascular bed before Fontan procedure independently lowers CVP, may prevent cardiovascular remodeling by RAAS suppression as well as end organ dysfunction in chronic Fontan patients. Our result suggested importance of strategies to accelerate pulmonary vascular growth before Fontan procedure.

Uhl’s anomaly is a rare cardiac disease of unknown etiology characterized by a markedly distended right ventricle because of partial or complete absence of right ventricular myocardium, which consequently develops a parchment-like appearance. Patients usually present with symptoms in infancy and rarely survive to adulthood. We report the case of an 18-year-old patient with Uhl’s anomaly who was diagnosed in infancy and survived to adulthood without symptoms of heart failure. Angiocardiography at the age of 6-years showed a dilated right ventricle. Magnetic resonance imaging (MRI) at the age of 12-years showed an extremely dilated thin-walled right ventricle with almost complete absence of right ventricular myocardium. At the age of 15-years, his MRI images showed further dilatation of the right ventricle. Follow-up examinations revealed to manifestation of right heart failure, despite persistent right ventricular dilatation and impaired function. This case may be useful for understanding the natural history of Uhl’s anomaly.

We examined the adaptive mechanism of the pulmonary ventricle (PV) in response to increased afterload secondary to pulmonary stenosis in tetralogy of Fallot (TOF, n = 47) and congenitally corrected transposition of the great arteries (cCTGA, n = 18), where the PV is morphologically different. We also elucidated the effects of such adaptation on systemic ventricular (SV) function. PV contractility, assessed by dp/dtmax, showed significant positive correlations with PV pressure (r = 0.82, p

Flow profiles in the pulmonary veins (PVs) and arteries (PAs), Fontan conduit (conduit), and superior vena cava (SVC) were examined using phase-contrast magnetic resonance imaging. The relationship of the ratio of systolic forward flow volume and diastolic forward flow volume (FV-S/D) with ventricular relaxation time constant (tau) was then investigated. According to the flow profiles in the PVs, the forward flow volumes in diastole were dominant over those in systole. The FV-S/D was significantly negatively correlated with tau, not only in the PVs but also in the PAs and conduit. Moreover, tau was positively correlated with pulmonary wedge pressures, while pulmonary wedge pressures were significantly negatively correlated with FV-S/D in the PVs. In contrast, the FV-S/D in the SVC was also negatively correlated with tau, but the correlation coefficient of this relationship was lower than that of the other relationships. Lastly, the FV-S/D in the SVC correlated more significantly with the ejection fraction of the ventricle rather than with tau. On the basis of our findings, we believe that the flow profiles in the PVs are influenced by the ventricular relaxation performance. Moreover, the ventricular relaxation performance influences the flow profiles in the PAs and conduit, both of which are located upstream of pulmonary circulation. On the contrary, the flow profiles in the SVC seem to be influenced by ventricular contraction rather than ventricular relaxation.

Twin-twin transfusion syndrome or related conditions affect fetal loading. We report monochorionic-diamniotic twins. Twin 1 had Ebstein anomaly with mild tricuspid regurgitation (TR) and slightly thickened tricuspid valve leaflets with plastering. Twin 2 had tricuspid valve dysplasia (with abnormal thickening but without plastering) with moderate TR and mild right atrial dilatation. After birth, the severity of TR was greatly reduced in the recipient but increased in the donor. Therefore, intravascular volume change which was due to twin-twin transfusion syndrome seemed to affect the severity of the valvar disease in fetuses. This case suggests that the intrinsic severity of fetal tricuspid valvular disease may be overestimated in the recipient and underestimated in the donor twin. These factors need to be taken into consideration in clinical decision-making.

Questionnaires were sent to 14 maternity hospital staff members for qualitative assessment at the start of fetal telediagnosis and at the end of the study using a five-point Likert scale: 5, I strongly think so; 4, I think so; 3, I can't decide; 2, I don't think so; 1, I never think so. Ten questionnaires were returned to us (71%). The results showed that the staff reported a significant increase in confidence in performing fetal cardiac screening (score 2.3 at start, 3.4 at study completion; P = 0.034), the rate of score increase rose with the number of telediagnoses (r = 0.72, P < 0.05), feedback from a specialist was very useful (4.4 and 4.9, respectively), and real-time image transmission was preferred over recorded images (score 3.7 vs 2.4, respectively; P = 0.042). The excellent educational effect of telemedicine is useful for staff members to improve their skills while nurturing their motivation, leading to the promotion of fetal cardiac screening in regional areas.

We verified the feasibility of telediagnosis of fetal disease by (i) grading telediagnosis by a pediatric cardiologist into five confidence levels; and (ii) comparison of fetal telediagnosis with hands-on fetal diagnosis or postnatal diagnosis. In 114 patients suspected of having heart disease (real time, n = 15; recorded image transmission, n = 99), 79 patients were in level 5 (excellent), 17 in level 4 (good), eight in level 3 (fair), 10 in level 2 (poor), and no patients in level 1 (bad). The average was 4.5, and in 96 patients (84% of all) telediagnosis was accurate (above 4), whereas in 18 patients it was inaccurate (level 2 or 3). In re-examination of 25 patients, telediagnosis was confirmed in patients in level 4 and 5, whereas heart disease was missed in patients in levels 2 or 3. The correct diagnosis matched the high confidence level of a specialist based on recognizable transmitted images.

Children with congenital portosystemic venous shunt (PSVS) are at risk for developing pulmonary hypertension, irrespective of the severity of portal hypertension or liver damage. Altered metabolisms of nitric oxide (NO) and endothelin-1 (ET-1), which are linked with oxidative stress and control vascular tone, might contribute to the vascular disturbance. This study examined 14 children (aged 1-5 years) with congenital PSVS lacking major liver damage and portal hypertension. Serum levels of nitrite/nitrate (NOx) as stable metabolites of NO, and of asymmetric dimethylarginine (ADMA) as an endogenous NO synthase inhibitor were determined, along with the plasma level of ET-1. Oxidative stress, which might affect the production of such mediators, was also examined using specific urinary and blood markers. The NOx levels were significantly lower in affected children than in the age-matched control group, although ET-1 levels were significantly higher than the control levels. In the affected children, the ADMA levels and ADMA/NOx ratios were higher, respectively, by 30% and 130% and showed significant positive correlations with the shunt ratios. Oxidative stress markers, including plasma thiobarbiturate reactive substances and urinary acrolein-lysine and 8-hydroxy-2'-deoxyguanosine, were significantly higher in affected children than in the control group, consistent with them being subjected to enhanced oxidative stress. These results suggest the presence of altered metabolisms of vascular mediators and enhanced oxidative stress in asymptomatic preschool children with congenital PSVS.

Questionnaires were sent to 14 maternity hospital staff members for qualitative assessment at the start of fetal telediagnosis and at the end of the study using a five-point Likert scale: 5, I strongly think so; 4, I think so; 3, I can't decide; 2, I don't think so; 1, I never think so. Ten questionnaires were returned to us (71%). The results showed that the staff reported a significant increase in confidence in performing fetal cardiac screening (score 2.3 at start, 3.4 at study completion; P = 0.034), the rate of score increase rose with the number of telediagnoses (r = 0.72, P0.05), feedback from a specialist was very useful (4.4 and 4.9, respectively), and real-time image transmission was preferred over recorded images (score 3.7 vs 2.4, respectively; P = 0.042). The excellent educational effect of telemedicine is useful for staff members to improve their skills while nurturing their motivation, leading to the promotion of fetal cardiac screening in regional areas.

We verified the feasibility of telediagnosis of fetal disease by (i) grading telediagnosis by a pediatric cardiologist into five confidence levels; and (ii) comparison of fetal telediagnosis with hands-on fetal diagnosis or postnatal diagnosis. In 114 patients suspected of having heart disease (real time, n = 15; recorded image transmission, n = 99), 79 patients were in level 5 (excellent), 17 in level 4 (good), eight in level 3 (fair), 10 in level 2 (poor), and no patients in level 1 (bad). The average was 4.5, and in 96 patients (84% of all) telediagnosis was accurate (above 4), whereas in 18 patients it was inaccurate (level 2 or 3). In re-examination of 25 patients, telediagnosis was confirmed in patients in level 4 and 5, whereas heart disease was missed in patients in levels 2 or 3. The correct diagnosis matched the high confidence level of a specialist based on recognizable transmitted images.

Information and communication technology has been widely applied to various fields, including clinical medicine. We report here a telediagnosis system using ultrasound image transmission. The effect of telediagnosis, using a medical link between local maternity hospitals and our children's medical center, was verified. The number of fetal telediagnosis for cardiac disease, and cases referred to a perinatal care center and emergent transportation of neonates with congenital heart disease from maternity hospitals, were calculated based on the hospital records. The percentage of patients found to have heart disease was compared between out-patient clinic and telediagnosis cases. Telediagnosis increased, allowing maternity hospital staff to obtain support easily from a specialist when making a diagnosis. Many severe cases were transferred to tertiary centers with the correct diagnosis; consequently, the number of emergent transportations of neonates with severe cardiac anomalies continued to below. Telediagnosis was also useful as an educational tool for maternity hospital staff, who improved their skills during conversations with a specialist. Unlike in the outpatient clinic, consultation by telediagnosis was requested even for cases of mild abnormalities, and the number of false-positives increased, while many cardiac anomalies were found in the early stage. Furthermore, telediagnosis was helpful for pregnant women requiring bed rest, and also had the advantage of allowing a doctor to be able to talk with parents. Establishing a fetal telediagnosis system is a useful strategy to improve neonatal care through a medical link between local maternity hospitals and a tertiary center.

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.

We report a case of a 31-year-old woman with common variable immunodeficiency (CVID) complicated with intractable diarrhea and the functional disorder of renal tubules. The patient became hypogammaglobulinemic after she suffered from measles at 6 years of age. She also suffered from lupus-like syndrome at 7 years of age. The complete remission was obtained by glucocorticosteroid treatment. An intravenous immunoglobulin replacement therapy was introduced at 11 years of age, since then her general condition was stable for more than 20 years. When she was 29 years old, she suffered from generalized malaise, anorexia with body weight loss, and numbness of face. The intractable diarrhea as protein loosing syndrome, and the severe abnormality of electrolyte balance with metabolic acidosis as the functional disorder of renal tubules were found. Her condition was not improved by the electrolytes or alkali replacement therapy. She was admitted for further evaluation and treatment. The intractable diarrhea and the functional disorder of renal tubules were dramatically improved after absolute restriction of food intake under hyperalimentation. When she began to take food, the symptom and sign became worse again. The interstitial nephritis and nonspecific inflammation of intestine were found by the tissue biopsy. The most characteristic finding was the infiltration of lymphocytes (predominantly CD 8 + T lymphocytes) in both intestinal mucosa and renal interstitium. The introduction of glucocorticosteroids improved her general condition and biochemical findings. This CVID case is complicated with intractable diarrhea and the functional disorder of renal tubules which is associated with the infiltration of CD 8 + lymphocytes in intestine and kidney. We consider that such case is very rare and valuable to report.