Status of adult outpatients with congenital heart disease in Japan: The Japanese Network of Cardiovascular Departments for Adult Congenital Heart Disease Registry.

BACKGROUND: The Japanese Network of Cardiovascular Departments for Adult Congenital Heart Disease (JNCVD-ACHD) was founded in 2011 for the lifelong care of adult patients with congenital heart disease (ACHD patients). This network maintains the first Japanese ACHD registry. METHODS AND RESULTS: From 2011 to 2019, the JNCVD-ACHD registered 54 institutions providing specialized care for ACHD patients in 32 of the 47 prefectures in Japan. The registry collected data on the disease profile for 24,048 patients from 50 institutions and the patient characteristics for 9743 patients from 24 institutions. The most common ACHDs were atrial septal defect (20.5 %), ventricular septal defect (20.5 %), tetralogy of Fallot (12.9 %), and univentricular heart (UVH)/single ventricle (SV; 6.6 %). ACHD patients without biventricular repair accounted for 37.0 % of the population. Also examined were the serious anatomical and/or pathophysiological disorders such as pulmonary arterial hypertension (3.0 %) including Eisenmenger syndrome (1.2 %), systemic right ventricle under biventricular circulation (sRV-2VC; 2.8 %), and Fontan physiology (6.0 %). The sRV-2VC cases comprised congenitally corrected transposition of the great arteries without anatomical repair (61.9 %) and transposition of the great arteries with atrial switching surgery (38.1 %). The primary etiology (86.4 %) for Fontan physiology was UVH/SV. In addition, developmental/chromosomal/genetic disorders were heterotaxy syndromes (asplenia, 0.9 %; polysplenia, 0.7 %), trisomy 21 (4.0 %), 22q11.2 deletion (0.9 %), Turner syndrome (0.2 %), and Marfan syndrome (1.1 %). CONCLUSIONS: Although the specific management of ACHD has systematically progressed in Japan, this approach is still evolving. For ideal ACHD care, the prospective goals for the JNCVD-ACHD are to create local networks and provide a resource for multicenter clinical trials to support evidence-based practice.

Use of the novel curved GOKU balloon catheter for acute-angled lesions in palliative surgery for congenital heart disease: comparison with a conventional straight balloon.

Obstruction develops commonly at the acute-angled portion of the vessels following palliative surgery, such as systemic-pulmonary shunt (SP shunt), right ventricle-to-pulmonary artery shunt (RV-PA shunt) in the Norwood-Sano procedure for hypoplastic left heart syndrome, and cavopulmonary (Glenn) anastomosis. Although balloon angioplasty is a treatment option, dilation with existing straight balloons is sometimes ineffective and technically complicated because of balloon slippage and target vessel distortion. In this study, we investigated the effectiveness of a curved GOKU balloon catheter for balloon angioplasty in postoperative acute-angled lesions associated with palliative surgery for congenital heart disease. We reviewed patients who underwent balloon angioplasty for angled lesions complicated by SP shunt, RV-PA shunt, or Glenn anastomosis, using the novel curved GOKU or a conventional balloon catheter, such as a Sterling balloon catheter. We evaluated patients' backgrounds, balloon specifications, target lesion anatomical features and angles, and short-term outcomes. We evaluated 45 procedures in 18 patients. A curved GOKU was used in 20 procedures, and a Sterling balloon in 25 procedures. The angulation of the lesions at maximum balloon inflation was significantly smaller using a curved GOKU vs a Sterling balloon [70-120 (mean ± standard deviation, 97 ± 40) degrees vs 110-180 (149 ± 46) degrees, respectively; p < 0.001], while the original angle was similar between the groups. Patients' short-term outcomes with the curved GOKU were excellent, with a significantly better percent increase in minimum lumen diameter of 0-220% (92% ± 66%) vs 0-46% (18% ± 15%) with the Sterling balloon (p < 00.1) and with less frequent balloon slippage. The curved GOKU was more effective in balloon angioplasty for acute-angled lesions compared with a conventional straight balloon, likely because of better conformability to the lesion angle and slip resistance.

Percutaneous pulmonary artery debanding.

BACKGROUND: There is a paucity of data on palliative or total percutaneous pulmonary artery debanding (p-debanding), particularly with use of a stent. METHODS: Twelve p-debandings in eight patients were included in this study. Age at pulmonary artery banding (PAB) ranged from 3 days to 1 year (median, 13 days), while p-debanding was performed at 2-157 (7) months. The body weight at the p-debanding ranged from 3.2 to 22.2 (7.3) kg. We chose the balloon diameter of 30-50% to the circumference of the band for palliative, and larger than 50% for total p-debanding, respectively. In either way, the balloon diameter did not exceed 1.5 times the reference vessel diameter. Stent was implanted for palliative p-debanding in 2 patients. RESULTS: 1. The circumference of the band ranged from 16 to 23 (20) mm, while the balloon diameter ranged from 20-60 (40)% to that, where larger than 50% was used for 2 procedures intended total p-debanding. 2. PAB diameter increased from 2.5-4.7 (3.0) mm to 2.8-9.5 (4.5) mm (p<0.01), however, there was no significant change in the diameter in 2 procedures. In one patient, p-debanding was the definitive treatment associated with spontaneous near closure of muscular ventricular septal defect, in another patient of congenitally corrected transposition of the great arteries, severely depressed left ventricular ejection fraction was recovered following p-debanding. 3. Arterial oxygen saturation (SaO2) increased from 64-97 (80)% to 66-95 (90)% (p<0.01), while in 10 procedures of 6 patients where the indication of p-debanding was hypoxia, SaO2 increased in 8 procedures. There was no significant pulmonary hypertension following p-debanding. CONCLUSION: Palliative or total p-debanding using balloon and/or stenting is generally feasible and effective. A balloon diameter 35-50% to the band circumference in palliative, and more than 50% in total p-debanding, while in either way less than 1.5 times the reference vessel diameter, is safe.

Clinical trial of the CP stent for pulmonary artery stenosis: the first investigator-initiated clinical trial for pediatric interventional cardiology in Japan.

Stenting is an important treatment option for pulmonary artery stenosis (PS) associated with congenital heart disease (CHD). However, no stent has been approved for this indication in Japan, despite negotiation between academia and the regulatory bodies for longer than 20 years. To evaluate efficacy and safety of the CP stent, we performed the first investigator-initiated clinical trial for pediatric interventional cardiology in Japan. This trial was designed as a single-arm, prospective, clinical trial. Patients who had postoperative PS associated with CHD were included. Stenting was attempted in 24 cases and succeeded in 22 cases. The median age of the patients was 11 years (3-36 years) and weight was 38 kg (12-69 kg), while follow-up for 12 months was completed. In all 22 cases, stenting was successful, with a 50% increase in the minimum lumen diameter (MLD) in 86.4% of patients (90% confidence interval, 68.4-96.2%). The mean percent change in MLD was 119.3 ± 52.5%. In two-ventricle repair, the mean percent change in systolic right ventricular/aortic pressure was - 8.5 ± 16.1%, while that of pressure gradient was - 55.9 ± 41.7%. In single-ventricle repair, the percent change in the mean pressure gradient was - 100.0 ± 0%, while that of SaO2 was 1.4 ± 1.7%. No serious adverse events or significant restenosis was reported. The CP stent is highly effective and safe for PS associated with CHD. This study has significant importance in not only scientific but also social considerations.

Successful everolimus-eluting stent implantation into the left main trunk stenosis in the anomalous coronary artery after neo-aortic valve replacement in a 6-year-old boy.

CHDs occasionally have coronary complications; however, stent implantation is technically difficult in small children. We reported a successful drug-eluting stent implantation into the congenital anomalous coronary artery in a 6-year-old boy. This treatment is useful for rescuing coronary stenosis, and dual antiplatelet therapy is important to prevent stent restenosis.

Percutaneous pulmonary debanding for an infant complicated by spontaneously closing muscular ventricular septal defect: A case report and in vitro study.

Pulmonary artery banding (PAB) is a standard operation for various congenital heart defects complicated by pulmonary hypertension (PH) and judged unsuitable for primary intracardiac repair. We report successful percutaneous pulmonary artery debanding in a baby complicated by muscular ventricular septal defect (VSD), that was initially large and multiple, but closed spontaneously later. The 5-month-old boy was referred to our hospital on day 3, diagnosed as having aortic coarctation (CoA), with multiple muscular VSDs and severe PH. On day 6, he underwent CoA repair and PAB using expanded polytetrafluoroethylene (ePTFE), while the muscular VSDs were left open. We planned percutaneous pulmonary debanding at the age of 5 months, as the muscular VSDs had become small. After dilation with a Mustang® (Boston Scientific, Marlborough, Massachusetts, United State) balloon (12 mm diameter) there was a persistent waist indicating a residual narrowing. Use of an extra-high pressure balloon, Conquest® (Medicon, Osaka, Japan) balloon of the same size, completely eliminated the waist. In in vitro experiments, the Mustang® partially tore the ePTFE, while a Conquest® of the same diameter completely opened the band. The mechanism of debanding was tearing of the ePTFE by the knot of the suture thread. Percutaneous pulmonary debanding to avoid unnecessary surgery is feasible in such a patient if the VSD becomes small. .

Static balloon atrial septostomy for the purpose of left heart intervention in postoperative adult CHD.

Background and purposeStatic balloon atrial septostomy is a widely accepted intervention for children with CHD. Successful surgical palliation is creating increasing numbers of adult CHD patients who need subsequent left heart intervention requiring transseptal access. In these patients, the interatrial septum is usually thick and fibrotic because of a previous open heart surgery or catheter intervention, and conventional transseptal puncture may be unsuccessful. Static balloon atrial septostomy to access the left atrium may facilitate intervention via the interatrial septum in such situations. The purpose of this study was to investigate the usefulness and the safety of static balloon atrial septostomy, and the evolution of an iatrogenic atrial septal defect post procedure in adult CHD. METHODS: We retrospectively reviewed six procedures in five adults with CHD and collected demographic characteristics, details of the procedures, clinical outcome, and size changes of the iatrogenic atrial septal defect. RESULTS: The mean age at the time of the procedure was 35 years. The intended primary interventions were pulmonary vein isolation, stenting for pulmonary vein obstruction, and catheter ablation for focal atrial tachycardia. All static balloon atrial septostomies were effective, and the left heart interventions were successfully achieved via transseptal sheaths. There were no major complications associated with the static balloon atrial septostomy. There were no adverse clinical outcomes related to iatrogenic atrial septal defect, and the size of the defects regressed over time in all cases. CONCLUSIONS: Static balloon atrial septostomy can be a safe and useful technique in adult CHD patients needing left heart procedures. The thick interatrial septum found in postoperative patients may reduce the risk of persistent iatrogenic atrial septal defect.

Current trends in stenting for aortic coarctation in Japan: Subanalysis of Japanese Society of Pediatric Interventional Cardiology (JPIC) stent survey.

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 <10 mmHg pressure gradient after stenting. The sensitivity and the specificity of its cut-off of 7% were 93% and 47% (AUC, 0.7), respectively. There was no statistical difference between the two age groups under and over 15 years of age, in terms of selection criteria of stent size, balloon type used for deployment and immediate angiographic and hemodynamic result. CONCLUSIONS: Stenting for CoA was clinically effective with few complications in Japan, even in patients not fully grown.

Restored left ventricular function following transcatheter closure of a persistent ductus arteriosus in an adult.

Recently, transcatheter device occlusion has become the first choice treatment for adult persistent ductus arteriosus (PDA). However, various complications such as atrial fibrillation requiring anticoagulation, pulmonary hypertension, and ventricular dysfunction may challenge the interventionist. We report a 61-year-old patient with a large PDA complicated by left ventricular dysfunction, atrial fibrillation, and left atrial thrombus. Computed tomography documented the PDA of Krichenko type A with the narrowest diameter of 8 mm. We successfully closed the PDA using an Amplatzer duct occluder under anticoagulation with wafarin. His post-operative course was complicated by ventricular tachycardia and deteriorating left ventricular pump function. Although endomyocardial biopsy from the left ventricle showed myocardial hypertrophy and interstitial fibrosis, possibly caused by chronic volume overload, left ventricular pump function improved dramatically with restoration of sinus rhythm during follow-up. Left ventricular dysfunction, even when associated with histological changes, may be nearly normalized by volume unloading in an adult with a large PDA. .

Elevated vascular endothelial growth factor levels are associated with aortopulmonary collateral vessels in patients before and after the Fontan procedure.

BACKGROUND: Aortopulmonary collaterals (APCs) are frequently observed before and after the Fontan procedure. However, the mechanism of the development of APCs is unknown. We hypothesized that one or several antiangiogenic and/or angiogenic growth factors might play a role in the development of APCs. METHODS: Eighty-five patients were enrolled and divided into 3 groups (Fontan group: 30 patients after the Fontan procedure, cyanotic group: 29 patients with cyanotic heart disease, and control group: 26 patients with cyanotic heart disease after biventricular repair). We measured basic fibroblast growth factor, vascular endothelial growth factor (VEGF), hepatocyte growth factor, and endostatin at catheterization. Angiographic evaluation for the presence of APCs using a 4-point scale (grade 1: absent APCs, > or = grade 2: significantly present APCs) was performed, and the relation of the serum levels of these factors to the presence of APCs was assessed. RESULTS: The grade of APCs significantly increased in the Fontan group, but it decreased in the control group. The serum VEGF levels were higher in the Fontan group (280 +/- 174 pg/mL) and the cyanotic group (302 +/- 245 pg/mL) than in the control group (111 +/- 91 pg/mL) (P = .0004), and they were higher in patients with APCs (383 +/- 204 pg/mL) than in those without APCs (115 +/- 65 pg/mL) (P < .0001). There was no significant difference in the serum basic fibroblast growth factor, hepatocyte growth factor, and endostatin levels between the 3 groups. CONCLUSIONS: Aortopulmonary collaterals increase after the Fontan procedure. Serum VEGF levels are associated with the presence of APCs. Vascular endothelial growth factor may play a role in the development of APCs in patients with cyanotic heart disease and after the Fontan procedure.