Femoral artery (FA) occlusion is a prevalent adverse event after catheter interventions, particularly in neonates and infants. Several treatments are available for FA occlusion, but the optimal approach remains unknown. Herein, we report three cases of infants with FA occlusion after catheter interventions, which were successfully treated by transcatheter revascularization. Case 1 is a 1-month-old boy with pulmonary valve stenosis who was treated with percutaneous transluminal pulmonary valvuloplasty. Case 2 is a 5-month-old boy with truncus arteriosus who underwent percutaneous transluminal angioplasty for pulmonary artery stenosis after intracardiac repair. Case 3 is a 3-month-old boy with pulmonary atresia and an intact ventricular septum treated with transluminal angioplasty for bilateral pulmonary artery banding site stenosis. All three cases were detected for FA occlusion within 1 day after the intervention and successfully recanalized by transcatheter angioplasty within four days after the intervention. Transcatheter revascularization can be used as potential treatment option for FA occlusion after the catheter procedure.

Coronary artery involvements occur rarely both during cardiac repair and in the late period after surgery, and it may result in myocardial ischaemia and infarction. We present six cases who underwent percutaneous transluminal coronary balloon angioplasty for coronary artery stenosis in the late period after surgery. The patients included four boys and two girls. Post-operative states involving anomalous origin of the left coronary artery from the pulmonary artery and d-transposition of the great arteries were observed in two patients each. Two patients with univentricular heart had coronary artery injuries during surgery. The age at the angioplasty ranged from 1 month to 14 years, with a median of 3 years. The interval from the operation to angioplasty ranged from 37 days to 14 years (median 8 months). The interval from the angioplasty to follow-up coronary angiography ranged from 2 months to 14 years (median 11 months). The follow-up period ranged from 2 months to 20 years (median 8 years). One patient underwent a stent implantation because of post-procedure recoil. Coronary artery stenosis improved immediately after procedure in the six patients without complication, and restenosis occurred post-procedure in one patient. Five patients had no cardiac events. Although the angioplasty's initial effect may not be dramatic, it can improve late after the procedure. It was considered that the optimal balloon-reference vessel ratio to obtain a minimal effective lumen diameter was about 1.0. Angioplasty post-surgery for CHD in children was feasible and without complications.

Background We evaluated the significance of perinatal plasma natriuretic peptide (NP) levels in neonates with congenital heart defects (CHDs) or arrhythmias and determined whether measurement of perinatal plasma NP levels and echocardiographic assessment in utero could predict heart failure after birth. Methods The study was conducted between 2012 and 2016 to evaluate the correlation of perinatal atrial NP (ANP) and brain NP (BNP) levels at birth with the modified Ross score after birth and the cardiovascular profile (CVP) score before birth. Results A total of 122 singletons with CHDs or arrhythmias and 27 controls were analyzed. Neonatal blood sampling was performed at a median of 0.7 h (range, 0.1-1.5) after birth. The neonatal plasma ANP and BNP levels shortly after birth were significantly higher than those in the umbilical artery (UA) plasma. The ANP and BNP levels in UA and neonatal blood were correlated with the modified Ross score. The neonatal plasma ANP and BNP levels and the modified Ross scores were inversely correlated with the CVP score in neonates with CHDs or arrhythmias. The area under the receiver operating characteristic curve of UA ANP levels for predicting neonatal heart failure was highest among those for the CVP score, perinatal plasma ANP and BNP levels, and their combinations. Conclusions The plasma ANP and BNP levels increased markedly shortly after birth. Assessment of the UA plasma ANP level at birth and the CVP score in utero may be utilized to predict neonatal heart failure.

Wire atrial septostomy, a new transcatheter technique, combines 2 atrial septal defects (ASDs) into a large one in a thickened atrial septum. Although the thin-wire-looped atrial septum between ASDs needs to be pulled deeply into a hard catheter to tear the septum, this action might harm the surrounding cardiac structures. We utilized radiofrequency wire energy to facilitate the tearing of the septum, making the procedure easier and safer. This technical article presents the modified procedure in detail; the procedure was performed in a 6-month-old boy with double outlet right ventricle and intact ventricular septum. Mini-abstract This technical article describes modified wire atrial septostomy for thickened atrial septum in detail. Radiofrequency energy facilitated a thin wire-loop in tearing the septum between 2 atrial septal defects to combine the defects into a large one. We believe that this transcatheter procedure is easier and safer than the original one not using a radiofrequency wire energy.

Background: Although transcatheter closure of atrial septal defect (ASD) is safe and effective for patients with sufficient rim, ASD patients with absent and/or malaligned aortic and/or superior rim have higher risks of device embolization and cardiac erosion. We have treated such high-risk patients using a Figulla Flex II (FFII) device shaped flared and straddling behind the aorta because this method would avoid such serious complications. However, its long-term efficacy and safety remain unclear. Therefore, the midterm efficacy and safety of this method were studied. Methods: We retrospectively evaluated the outcome of 47 consecutive patients with such rim (age 6-73 years, weight 17-75 kg, 31 females) treated with this method at our hospital between February 2016 and September 2019. To make the flared and straddling shape, we selected a FFII 4-6 mm larger than the balloon sizing diameter by stop-flow technique. We also studied the device shape, the disc pressure to the Valsalva wall and their changes over 6 months by transesophageal echocardiography. Results: All procedures were successful, and leakage disappeared within 1 year. During a mean follow up of 37 +/- 12 months, complications included a transient sinus node dysfunction and one I degrees atrioventricular block within 3 months. Whole device shape changed from bulky to thin: the device waist and thickness significantly decreased by around 1.5 mm and 3.5 mm, respectively (p < 0.05), but the two discs remained flared and straddling behind the aorta over 6 months; therefore, the disc edges seldom pressed the Valsalva wall perpendicularly, even though the inner plane of either disc often slightly pressed the wall. Conclusions: ASD closure using a FFII shaped flared and straddling behind the aorta is probably effective and safe for patients with absent and/or malaligned aortic and/or superior rim although requiring care for conduction disorders.

Heterograft and artificial materials have been used for extracardiac conduit implantation to create right ventricular (RV) to pulmonary artery (PA) continuity for biventricular repair in Japan because of the limited availability of homograft valves. However, few studies have examined morphological changes and number of candidates for transcatheter pulmonary valve implantation (TPVI) in which the conduit includes more than one type of material. Overall, 88 patients who underwent biventricular repair with an external conduit were included in this evaluation. Based on catheterization data and surgical records, we estimated morphological change in the RV outflow tract for each material and the number of candidates for Melody valve implantation based on premarket approval application criteria established by the U.S. Food and Drug Administration. There were 63 candidates for TPVI (72%, 63/88). Median anteroposterior and lateral diameter of the RV outflow tract was 20.4 mm (range 9.0–41.5) and 17.8 mm (range 9.5–34.9), respectively. Bovine pericardium tended to dilate by 11.2%. Polytetrafluoroethylene (ePTFE), homograft, and Dacron polyethylene terephthalate (PET) tended to become stenotic by 11.1%, 28.0%, and 13.4%, respectively. While ePTFE (27/33, 82%) and Dacron PET (2/2, 100%) were highly suitable for TPVI, bovine pericardium (32/48, 67%) was less suitable. In Japan, many patients with hemodynamic indications for TPVI following extracardiac conduit implantation to create RV to PA continuity may also meet the morphological indications.

Background: The supply of Rashkind balloon atrial septostomy (BAS) catheters (Medtronic, Minneapolis, MN, USA) has suddenly been discontinued in the world due to its recall. Consequently, Japan fell into a critical shortage of standard BAS catheters. Although the use of static BAS is off-label in Japan, its importance is increasing in such a situation. A nationwide survey of static BAS is needed in such a critical period. Methods: A questionnaire survey was conducted among centers in Japan regarding BAS performed between October 1, 2020, and December 15, 2020, while the supply of Rashkind BAS catheter was discontinued. Results: We received answers from 70 of the 90 centers, for a response rate of 78%. In this survey, 25 patients who underwent static BAS were enrolled, and a total of 47 BAS procedures were performed. Median age and weight at static BAS were 10 days of life and 3001 g, respectively. The most common diagnosis was transposition of the great arteries without pulmonary stenosis and hypoplastic left heart syndrome and its variants, with 8 cases each. The most frequently used balloon diameter was 10 mm (13 balloons), followed by 12 mm (10 balloons), and 3 cases required double-balloon techniques. The 3-point scale of the efficacy of static BAS rated by physicians were 10 excellent, 15 good, and 0 poor, respectively. Complications included cardiac tamponade during the procedure in 1 patient and the need for Rashkind BAS later in 2 patients. Comparing the share of static BAS in all transcatheter atrial septostomy, its share in the current survey (28/53) is significantly higher compared to the annual registry data in 2018 (86/304) ( p < 0.01). Conclusions: This survey shows that static BAS is widely performed in Japan and is effective and safe. Static BAS cases have increased significantly due to a shortage of standard BAS catheters . (c) 2021 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.

Given the rarity of significant first-degree atrioventricular block (AVB) after Occlutech Figulla Flex II atrial septal defect occluder (OFF II, Occlutech International AB, Helsingborg, Sweden) placement, its management and predictors of recovery have not been established. We report a 9-year-old boy who developed significant first-degree AVB a day after OFF II deployment. Thereafter, oral prednisolone was initiated at 1.5 mg/kg/day. On day 2 of steroid treatment, normal sinus rhythm without conduction delays was occasionally observed, while the prolonged PR interval on the exercise stress test was ameliorated. After 5 days of steroid administration, normal sinus rhythm without conduction delays was completely achieved. An increase in the frequency of normal sinus rhythm and the absence of conduction delays on electrocardiogram monitoring and exercise stress test play an important role in predicting recovery from prolonged PR interval. Thus, steroid treatment can be effective and should be considered for patients who develop first-degree AVB after undergoing OFF II placement for transcatheter device closure of atrial septal defect. .

Background: Congenital heart disease (CHD) is often diagnosed prenatally using fetal echocardiography, but few studies have evaluated the accuracy of these fetal cardiac diagnoses in detail. We investigated the discrepancy between pre- and postnatal diagnoses of CHD and the impact of discrepant diagnoses. Methods and Results: This retrospective study at a tertiary institution included data from the medical records of 207 neonates with prenatally diagnosed CHD admitted to the cardiac neonatal intensive care unit between January 2011 and December 2016. Pre- and postnatal diagnoses of CHD differed in 12% of neonates. Coarctation of the aorta and ventricular septa! defects were the most frequent causes of discrepant diagnosis. Unexpected treatments were added to 38% of discrepant diagnostic cases. However, discrepant diagnoses did not adversely affect the clinical course. The 9% of the 207 neonates who required invasive intervention within 24h of delivery were accurately diagnosed prenatally. Conclusions: Pre- and postnatal diagnoses differed in only a few neonates, with differences not adversely affecting the clinical course. Neonates who required invasive intervention immediately after delivery were accurately diagnosed prenatally. Prenatal diagnosis thus seems to contribute to improved prognosis in neonates with CHD.

Cardiac erosion is a rare serious complication following Amplatzer septal occluder (ASO) placement for atrial septal defect. Although multiple risk factors have been found, a useful predictor to prevent it has not been established yet. In 12 patients who developed erosion between 2005 and 2016 in Japan, we retrospectively observed patients’ characteristics and transesophageal echocardiography findings immediately before and after ASO placement and at erosion onset. We compared risk factors of erosion, including absent aortic rim, device size/body weight ratio, device shape, or Valsalva sinus wall deformation pressed by either disk-edge, the maximum depth of which from the standard curve of the Valsalva wall was defined as Dent, between the 12 patients and 95 patients with Valsalva sinus wall deformation immediately after placement who did not develop erosion for 5 ± 3 years. Of the 12 patients, nine developed pericardial effusion with eight cardiac tamponade and three aorta-atrium fistula; all were surgically rescued. Surgical findings revealed that erosion in all patients occurred at the right and/or left atrial roof beside the Valsalva in the non-coronary cusp on which the disk-edge seemed to be pressing. The mean Dent immediately after the placement in patients with erosion was significantly deeper than without (2.48 ± 0.32 vs. 1.28 ± 0.38; p < 0.001). There were no differences in the other risk factors between the two groups. Dent is believed to be a useful indicator of erosion development after ASO placement. If Dent is > 2.0 mm, it is desirable to change the size or to replace the device.

OBJECTIVES:Our goal was to assess the efficacy of managing pulmonary blood flow from the Norwood procedure with a right ventricle-to-pulmonary artery (RV-PA) conduit until stage 2 palliation (S2P).METHODS:Among 48 consecutive patients undergoing the Norwood procedure between 2008 and 2018, 40 (83.3%) patients who survived to discharge were included in this study. The primary diagnosis was hypoplastic left heart syndrome in 28 (70%) patients and hypoplastic left heart syndrome variant in 12 (30%) patients. All patients received bilateral pulmonary artery banding. The median age and weight at the time of the Norwood procedure were 41 (25th-75th percentiles: 27-89) days and 3.2 (2.7-3.9) kg, respectively. In keeping with institutional strategy, S2P was undertaken when body weight exceeded 5.0 kg, and normal gross motor development was confirmed.RESULTS:The RV-PA conduit was clipped in 28 (70%) patients during the perioperative period of the Norwood procedure, then partial unclipping was performed in 8 (20%) patients and full unclipping was performed in 20 (50%) patients. Before S2P, the median pulmonary-to-systemic blood flow ratio was 1.0 (0.7-1.3). The median age and weight at the time of S2P were 10.7 (9.0-12.9) months and 6.3 (5.5-7.1) kg, respectively. The survival rate 5 years after Norwood discharge was 85.3%. Pre-S2P pulmonary-to-systemic blood flow ratio was linearly correlated with greater interstage changes in systemic atrioventricular valve regurgitation (R2 = 0.223, P = 0.004).CONCLUSIONS:Interstage management of pulmonary blood flow by RV-PA conduit clipping and gradual unclipping provided good interstage outcomes. The median pulmonary-to-systemic blood flow ratio could be controlled to 1.0 at pre-S2P catheter examination.

Background: The treatment of heart failure has changed with the use of angiotensin-converting enzyme inhibitors (ACEIs) and beta-blockers since the middle of the 1990s. However, the outcome in infantile dilated cardiomyopathy (DCM) when treated with them remains poorly understood. Methods: We reviewed the medical records of infants with DCM within 24 months old in our hospital between 1979 and 2012, and compared the outcome in the later group (1997-2012) with that in the early group (1979-1996). The survival and cardiac event (CE)-free survival rates were calculated by the Kaplan-Meier method. Results: There were 20 patients in the early group and 24 patients in the later group. The median left ventricular fractional shortening at the onset of disease in the early and later groups were 11% (range 4-17%) and 12% (range 4-25%), respectively. In the later group, ACEIs and beta-blockers were administered in 22 and 21 patients, respectively. An usual low-dose induction of carvedilol therapy (0.01-0.02 mg/kg/day) sometimes worsened the heart failure in 9 patients (43%) after the successful initial conventional treatment for acute heart failure. Nineteen patients died and 25 survived. The CEs were as follows: heart transplantation 4. mitral valvuloplasty 1, Batista operation with mitral valve replacement 1, and cardiac resynchronization therapy in the late period 1. The 20-year survival rate in the early and later groups were 5% (95% Cl 0.7-28) and 100%, respectively (p < 0.001). The 2-year CE-free survival rate in the early and later groups were 5% (95% CI 0.7-28) and 83% (95% CI 59-91), respectively (p < 0.001). Conclusions: The outcome in patients with infantile DCM has significantly improved with careful acute and chronic treatments using ACEIs and beta-blockers since the 2000s. Adopting a long-term supportive treatment during a period of low ventricular function and the use of beta-blockers corresponding to each patient's condition were key to survival. (C) 2019 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved,

Objective: Surgical outcomes of biventricular repair for hearts with hypoplastic left ventricle with congenital mitral valve stenosis are described. Serial changes of left ventricular geometry and clinical features after biventricular repair were reviewed. Methods: Eight patients with hypoplastic left ventricle and congenital mitral valve stenosis who underwent first surgical intervention for biventricular circulation in neonatal or infantile period between 2001 and 2014 comprise the study population. Serial change in left ventricular end-diastolic diameter, left ventricular mass index, and relative wall thickness after biventricular repair were evaluated by two-dimensional echocardiography. Results: The median Z-scores of left ventricular end-diastolic diameter and mitral valve diameter before the first surgical intervention were -3.0 (range, -4.8 to -2.0) and -1.0 (-2.9 to 2.1), respectively. Mitral valves were surgically treated in five patients; they were replaced in two and repaired in three patients. Left ventricular end-diastolic diameter Z-score at five years after biventricular repair was 0.1 (-3.0 to 1.0), which was significantly larger than before first surgical intervention (P = .005). Left ventricular mass index, on the other hand, did not change, but relative wall thickness significantly decreased (P = .009). Postoperative catheter study showed pulmonary hypertension with high left ventricular end-diastolic pressure in more than half of survivors. Conclusions: Left ventricle increased in size after the biventricular repair with appropriate mitral valve procedures before progression of pulmonary hypertension. Left ventricular mass, however, did not accompany the increase. Some patients may have suffered from mild, but certain restrictive left ventricular physiology and subsequent pulmonary hypertension as the result of abnormal remodeling process of the myocardium.

Background: Although ductal stenting (DS) has been performed in patients with severe congenital heart disease in Japan, the frequency of DS performed in Japan remains unclear; moreover, no stent has been approved for arterial duct (AD) under Japanese health insurance (JHI). Methods: The Health Insurance Committee of the Japanese Society of Pediatric Interventional Cardiology conducted a questionnaire-based survey to investigate the current status of DS performed and its necessity in Japan and evaluated the efficacy and safety of the procedure to seek approval for its use under JHI. Results: DS was performed in 64 cases at 12 institutions between April 2013 and March 2016. A total of 62 DS were performed for restoring the systemic circulation in patients with hypoplastic left heart syndrome, subaortic stenosis with interrupted aortic arch, and others. The three major indications for performing DS were high risk of alternative surgery such as the Norwood procedure, urgent procedures owing to the closure of AD, and based on own institutional principle. Complete success was achieved in all the 62 procedures, and prostaglandin E1 was withdrawn in 60 cases (97%). Major complications included stent migration/embolization in 6 cases (9.7%), 4 of which were replaced using a balloon, and dissection in 3 cases (4.8%). Mortality related to the procedures or complications was absent. Majority of the patients reached the next stage surgery. Conclusions: The present study strongly recommends that both balloon-expandable and self-expandable stents must be approved for the safe and effective ductal intervention under JHI as soon as possible.

The authors have retracted this article [1] because, contrary to the statement in the article, this research did not obtain ethics approval from the National Cerebral and Cardiovascular Center Institutional Review Board prior to submission of the manuscript to Pediatric Cardiology. All authors agree to this retraction.

receptor antagonist.This strategy consequently improved mPAP and

Background. Although adverse effects of phrenic nerve palsy (PNP) on early Fontan circulation have been reported, detailed late impact remains unclear. Methods. Of 218 patients undergoing extracardiac total cavopulmonary connection between 1995 and 2008, 160 who all underwent cardiac catheter examination, spirometry, and exercise capacity testing 10 years after the operation were enrolled. The cohort was divided into 2 groups: with (N = 21) or without PNP (control group, N = 139). The patients with PNP were further divided into the recovered PNP group (n = 10) and the persistent PNP group (n = 11). All but 2 patients who developed PNP (90.9%) underwent diaphragmatic plication. There was no difference in hemodynamic indices at pre-Fontan evaluation among the three groups. Results. Ten years after the Fontan procedure, the averaged forced vital capacity was 81% +/- 18% of predicted in the control group, 86% +/- 17% in the recovered PNP group, and 56% +/- 12% in the persistent PNP group (P < .001). Peak oxygen consumption was linearly correlated to the forced vital capacity (r = 0.222, P = .009). There was no significant difference in the peak oxygen consumption between groups. Significant veno-venous collaterals into the diaphragm from lower body to pulmonary vein(s) or atria more frequently developed in patients who underwent diaphragmatic plication compared with those who did not (P < .001). Conclusions. Persistent PNP resulted in reduced forced vital capacity; however, its influence on exercise intolerance could not be identified. Diaphragmatic plication should be reserved for patients who experience clinically significant respiratory or hemodynamic sequelae of PNP. (C) 2020 by The Society of Thoracic Surgeons

The purpose of the study is to analyze the impact of hybrid stage 1 palliation on right ventricular myocardial pathology in hypoplastic left heart syndrome. Sufficient amount of right ventricular biopsies could be obtained from 16 of 32 patients who underwent Norwood operation between 2007 and 2013. Histopathological findings of right ventricle in patients who underwent primary Norwood operation (primary group, n = 5), patients with aortic atresia (HS1P AA group, n = 6) or aortic stenosis (HS1P AS group, n = 5) who underwent staged Norwood palliation following hybrid stage 1 palliation were compared. To eliminate the influence of right ventricular pressure afterload, right ventricular biopsies were obtained from patients with truncus arteriosus communis (TAC group, n = 6) at total correction. The percentage of myocardial fibrosis was significantly higher in both HS1P groups than in TAC group; moreover, it was significantly higher in HS1P AA group than in primary group. Capillary vascular density was significantly lower in all hypoplastic left heart syndrome groups than in TAC group. At the sub-endocardial layer, collagen type I/III ratios were higher in HS1P AA group than in other hypoplastic left heart syndrome groups. The proportions of N-cadherin immunolocalized to myocyte termini were lower in all hypoplastic left heart syndrome groups than in TAC group. Right ventricle in hypoplastic left heart syndrome showed more significant ischemic change and myocardial immaturity than that in truncus arteriosus communis. Hybrid stage 1 palliation for aortic atresia would be a risk factor for further right ventricular myocardial ischemia.