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Even today, when the surgical outcome of congenital heart disease in the neonatal period has improved, the prognosis for heterotaxy syndrome and functional single ventricle complicated with total anomalous pulmonary venous connection (TAPVC), especially the infra-cardiac type, is catastrophic. We describe a strategy that combines percutaneous ductus venosus (DV) stent placement and occlusion after TAPVC repair to ensure survival from initial surgery to bidirectional cavopulmonary shunt (BCPS) procedure and facilitate subsequent treatment. Three consecutive patients with heterotaxy syndrome and functional single ventricle complicated by infra-cardiac TAPVC treated with our own strategy were retrospectively studied. In two infants, DV stent placement was performed on the day of birth. In one case at 11 days of age. The risk of pulmonary vein obstruction was reduced, and on-pump surgery, including TAPVC repair, was performed on a standby basis. Since the rapid increase in hepatic enzymes occurred on postoperative day 0 to 1 in all cases, percutaneous stent occlusion was performed until postoperative day 3. The procedure improved liver function. One patient died due to severe atrioventricular valve regurgitation, one case underwent BCPS, and one patient was waiting to undergo. DV stent placement can avoid TAPVC repair in the early neonatal period. After TAPVC repair, the portosystemic shunt remained, resulting in hepatic dysfunction, but this could be improved by stent and vertical vein occlusion. A series of stepwise treatments can be useful to help such critically ill infants survive the high-risk neonatal period and achieve good BCPS circulation.

Objectives The aim of this study was to investigate whether milrinone infusion improved one-year survival in patients who underwent the Norwood-Sano procedure. Design A retrospective observational study. Setting A single-institution university hospital. Participants Children who underwent the Norwood-Sano procedure from January 2008 to December 2014. Interventions Patients were categorized into two groups: group E+D, who received routine epinephrine and dopamine infusion, and group M, who received routine milrinone infusion for intra- and postoperative inotropic support. Measurements and Main Results The primary outcome of this study was one-year survival after the Norwood-Sano procedure. A total of 45 patients were included (group E+D, 22; group M, 23). One-year survival in group M was significantly higher than that in group E+D (95.7% [22/23] v 72.7% [16/22], p = 0.03). A Kaplan-Meier curve also showed that one-year survival in group M was significantly higher than that in group E+D (p = 0.04), from the result of the log-rank test. The number of patients who had any arrhythmias in the intensive care unit (ICU) was significantly lower in group M than in group E+D (21.7% [5/23] v 50% [11/22], p = 0.03). The duration of ICU stay did not have statistical difference between groups (group M 19; interquartile range [IQR], 15-28) v group E+D 19.5 (IQR, 16.3-35.5) days, p = 0.57). Conclusions Perioperative milrinone infusion improved the mortality after the Norwood-Sano procedure. Potential advantages of milrinone compared with epinephrine are fewer arrhythmias and better systemic perfusion, which could decrease lethal cardiac events in the ICU.

We present our experience with transcatheter vascular occlusion using 0.035-inch hydrogel expandable coils, which has been reported only in a few cases in the pediatric cardiology fields.This study is a retrospective analysis of all patients who underwent transcatheter embolization with 0.035-inch hydrogel coils at the Department of Pediatrics, Okayama University Hospital, between October 2018 and September 2020.Twenty patients with a median age of 5.1 years (0.05-26.0 years) and a median weight of 13.8 kg (3.0-56.8 kg) were included. A total of fifty-four 0.035-inch hydrogel coils, including 35 Azur 35 and nineteen Azur CX 35 coils (Terumo, Tokyo, Japan), were successfully deployed in 22 target vessels. The target vessels consisted of 10 aortopulmonary collaterals, 8 veno-venous collaterals, and 4 pulmonary arteriovenous malformations. We achieved technical success in all the target vessels. In total, the mean target vessel diameter was 4.4 mm, the mean number of 0.035-inch hydrogel coils was 2.5 per vessel. The mean device to vessel ratio was 1.6 for the anchor coil and 1.2 for the additional coil. Post-implantation angiograms revealed that the primary occlusion rate was 18/22 (82%). There were no periprocedural complications.The 0.035-inch hydrogel expandable coils are effective and safe in patients with congenital heart disease and vascular anomalies. These occlusion devices could be valuable options for interventional pediatric cardiologists.

The relationship between regional cerebral oxygen saturation (rSO2) and the amount of left-to-right shunt in ventricular septal defect (VSD) patients has not yet been investigated. The purpose of this study was to identify the association of preoperative pulmonary to systemic blood flow (Qp/Qs) ratio and preoperative rSO2 in patients with VSD. We retrospectively evaluated 49 VSD surgical closure candidates at a single institution. Preoperative Qp/Qs ratio was compared with rSO2 measurements at the time of VSD closure surgery. Forty-nine were eligible for the final analysis. The median age at surgery was 6 (interquartile range [IQR]: 3, 12) months, and 36.7% were male. Atrial septal defects coexisted in 51.0%. There were no genetic abnormalities except trisomy 21 in 32.6% of the patients. Pulmonary hypertension was found in 42.8%. The median Qp/Qs ratio, calculated based on catheter testing results before the surgery, was 2.7 (IQR: 2.1, 3.7). Postoperative rSO2 was significantly higher than preoperative values (52.2 ± 12.9, 63.5 ± 13.1%, p

BACKGROUND Stenting for aortic coarctation (CoA) has been accepted as an alternative to surgery for adolescents and adults, but only a few case have been reported in Japan. The purpose of this study was to provide a detailed review of Japanese national data on stenting of CoA. METHODS In a subanalysis of the data of the Japanese Society of Pediatric Interventional Cardiology (JPIC), we identified 35 patients with CoA who underwent stenting. We analyzed procedural characteristics including factors that may have contributed to hemodynamic effectiveness, and we compared these parameters between the patients under and over 15 years of age. RESULTS The mean ratio of balloon diameter/minimum lumen diameter (MLD) before stenting was 1.7 (range, 1.2-4.0), and the mean difference between the balloon diameter and the reference vessel diameter was -0.7 mm (range, -5.0 to +3.0 mm). %MLD/balloon diameter, which was defined as [(balloon diameter - MLD after dilation)/balloon diameter] × 100 predicted achievement of

Background and purpose Stent implantation is an important treatment option for pulmonary artery stenosis (PS), even if complicated by univentricular physiology (UVP). However, there is paucity of evidence concerning not only its hemodynamic and morphologic indications but also on markers for its optimal target attainment in UVP. The purpose of this study was to evaluate the acute outcome and factors associated with efficacy of stenting for PS complicating UVP. Methods and subjects A subanalysis was performed using the data of the Japanese Society of Pediatric Interventional Cardiology (JPIC) stent survey. We analyzed the morphologic and hemodynamic data of 11 patients with UVP who underwent stenting for PS. We defined “a 50% increase in the minimum lumen diameter (MLD)” as “morphologically effective,” and “an achievement of 0 mmHg pressure gradient” as “hemodynamically effective.” We analyzed the success rate for each criterion and determined factors which may have contributed to hemodynamic effectiveness. Results Stenting was morphologically effective in all patients, while it was hemodynamically effective in 6/11 (55%). The percent diameter stenosis after stenting was significantly lower in the “hemodynamically effective” group (2.5 ± 5.5% vs 19.6 ± 13.1%, p = 0.017). The cutoff value of percent diameter stenosis after stenting to “hemodynamically effective” was 14.6%; the sensitivity was 80% and the specificity was 100% (area under the curve 0.825, p = 0.021). Conclusions The percent diameter stenosis after stenting significantly contributed to achieving a “0 mmHg” pressure gradient, while in order to achieve a “0 mmHg” pressure gradient, the residual percent diameter stenosis should be less than around 15%.

Background Percutaneous stenting for branch pulmonary artery stenosis is an established interventional choice in congenital heart disease. The apparent morphologic change in the vessel diameter often differs from the hemodynamic result. Methods and results We performed a subanalysis of the data from the Japanese Society of Pediatric Interventional Cardiology (JPIC) stent survey. The factors that may have contributed to morphologic effectiveness included reference vessel diameter (RVD), minimum lumen diameter (MLD) and percent diameter stenosis (%DS) and the relation between morphologic and hemodynamic effectiveness was evaluated in 206 lesions treated with stenting. We defined a "50% increase in MLD" as "morphologically effective", while "achievement of either a reduced pressure gradient greater than 50% or an increase of perfusion ratio to the affected side to the contralateral side greater than 20%" as "hemodynamically effective". Morphologic effectiveness was achieved in 84% of patients. Before stenting, %DS was significantly larger, while RVD was smaller in the "effective" group than in the "non-effective" group. The cutoff value for effective stenting was 51% for %DS and 14.7 mm for RVD before stenting. Hemodynamic effectiveness was obtained more often in the "morphologic effective" group. Conclusions RVD and %DS were the 2 main contributors to acute morphologic effectiveness. There was a significant relationship between "morphologic effectiveness" and "hemodynamic effectiveness", judging from increased perfusion of the affected lung and/or decreased pressure gradient. (Circ J 2016; 80: 1852-1856).

We diagnosed hypoplastic left heart syndrome in a 26-week-old fetus using fetal echocardiography. Color Doppler ultrasonography was helpful for evaluating the structural abnormalities. The diameters of the aorta and the pulmonary artery were measured periodically from 26 to 38 weeks of gestation. Aortic diameter was below the normal range throughout gestation. The diameter of the pulmonary artery was normal at 26 weeks of gestation but gradually dilated and was abnormally dilated after the 36th week of gestation. Here we discuss the cause of enlarged pulmonary artery in fetal hypoplastic left heart syndrome.

Objectives: To evaluate acute cardiac remodeling after transcatheter closure of atrial septal defect (ASD) in adult patients. Background: In adult patients with ASD, longer periods of cardiac adaptation should be expected after the procedure due to long-standing RV volume overload and subsequent changes in the pulmonary vasculature. There are limited reports about this remodeling in adult patients. Methods: We prospectively enrolled 17 adults (mean age 58.4 ± 17.3 years) who underwent successful transcatheter closure of their ASDs from August 2005 to July 2006. We performed routine transthoracic echocardiographic studies, including LV and RV myocardial performance indices, or Tei indices, and plasma brain natriuretic peptide (BNP) sampling before closure of the ASD, and 1 day, 1 month, and 3 months after closure. Results: We found (1) LV end diastolic diameter increased, and RVEDD decreased markedly after the closure; (2) differences existed in LV and RV adaptation. While LV Tei index improved soon after the procedure, RV Tei index worsened until 1 month after the procedure, then recovered by the 3 month follow-up visit; and (3) BNP elevated 1 day after closure of the ASD and declined by the 1-month follow-up visit. Conclusion: “Shrinkage” of the RV and “expansion” of the LV occurred soon after the procedure, even in elderly patients. Device closure of ASDs caused rapid improvement of LV function, but RV function underwent transient deterioration, probably due to delayed changes in RV ventricular mass in the face of acute volume reduction in this aged cohort. © 2007 Wiley-Liss, Inc.

Results: Significant (P< 0.05) improvement of EF (from 27% to 36% in group A, from 23% to 36% in group B) and LVESVI (from 83ml/m2 to 63ml/m2 and from 112ml/m2 to 60ml/m2, respectively) was observed at 6 months. Hospital death due to ventricular tachycardia occurred in one patient in group A, and late cardiac death due to heart failure occurred in two patients in group B. Of 38 survivors at 6 months, all patients in group A and 95% of those in group B showed NYHA functional class I or II, whereas preoperatively 73% and 76%, respectively, showed NYHA functional class III or IV. Conclusions: LEMRI is a promising tool for selecting patients and areas for SVR, providing excellent clinical outcomes.