Search

Transcatheter closure is an established method to treat coronary artery fistula (CAF). We present transcatheter closure in a 6-year-old girl with CAF and anomalous aortic origin of the left main coronary artery from the right aortic sinus. The CAF originated from the left coronary artery (LCA), coursed through the interventricular septum (intraseptal course) with prominent dilation, and drained into the right ventricular outflow tract. She underwent transcatheter closure and was in a stable condition at the 3-year follow-up with regression of the dilated portion of the intraseptal-type LCA. Hence, transcatheter closure of CAF is feasible in patients with anomalous origins of coronary arteries.

An extremely low birth weight infant (810 g) was born with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries accompanied by pulmonary overcirculation, which eventually resulted in intestinal hypoperfusion and focal intestinal perforation from the very early stage of life. Based on the echographic findings, we performed banding operations twice to regulate the pulmonary blood flow on day 2 and day 9. At 6 months of age, a definitive repair simultaneous with unifocalization of major aortopulmonary collateral arteries was performed. At 1 year of age, the right ventricle/left ventricle pressure ratio was 0.44 after balloon angioplasty was performed for the right-sided pulmonary artery stenosis. The patient is in a stable condition and was followed-up for more than 2 years after definitive repair. This is the first known successful repair of pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries in an extremely low birth weight infant.

Background Stent implantation for vascular stenosis associated with congenital heart diseases is commonly performed as an off-label procedure in Japan because there is no officially approved stent for any congenital heart disease.Methods and Results:We analyzed data from the Japanese Society of Congenital Interventional Cardiology Registry collected from January 2016 to December 2018. Patients who underwent stent implantation were enrolled in the present analysis. During the study period, there were 470 procedures, 443 sessions, and 391 cases. Of 443 sessions, 427 (96.4%) succeeded procedurally. There were no differences in the procedural success rates among age groups. In all, 416 sessions (367 patients; 94%) resulted in survival to 30 days after catheter intervention. Of 392 admissions, 357 patients (91%) survived to discharge. Only 4 deaths were directly related to stent implantation. Some in-hospital complications were observed during 55 of 443 sessions. Both hospital deaths and serious complications were significantly more frequent in the group with various preoperative risk factors. Conclusions Although not officially approved for congenital heart diseases in Japan, stent implantation in congenital heart diseases has been widely and routinely performed for many years with safety and efficacy. The aim of stenting was variable and broad because of many different applications and morphological variations. These data may facilitate approval of such an important device in Japan.

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).

Background: The atrioventricular valve function is one of the most important determinants of mortality in patients with heterotaxy syndrome (HS). Though there are many reports to show the impact of atrioventricular valve regurgitation (AVVR) on the outcome of patients with HS, there are paucity of

Intact atrial septum (IAS) and left ventriculocoronary arterial fistula (LVCAF) are the significant risk factors of hypoplastic left heart syndrome (HLHS), and when they are present, prognosis is grim. We report a case of prenatally diagnosed HLHS with IAS and LVCAF that was treated with surgical atrial-septostomy and bilateral pulmonary arterial banding (BPAB) immediately after birth. The Doppler flow pattern of left anterior descending coronary artery (LAD) with dominant retrograde flow was normalized after atrial-septostomy with decompression of left atrial (LA) hypertension. This is the first report showing the influence of atrial-septostomy on coronary flow in HLHS patient with LVCAF.

In some congenital heart disease, AV node and its pathway are unusual in morphology and property. We present two AVNRT cases which were difficult to diagnose and ablation. Case 1 is 12 yr old male who has had atrioventricular septal defect surgically corrected 5 yrs ago. He had PSVT initiated during exercise, EKG showed early retrograde P′. Electrophysiological study proved that it was slow/fast AVNRT but electroanatomical mapping revealed that fast and slow pathway exits were both near coronary sinus. After careful ablation to slow pathway, AVNRT couldn’t be induced. Case 2 is 2 yr old male who had right isomerism, single ventricle, and finally Total Cavo Pulmonary shunt with intracardiac conduit. He had PSVT after final operation, and EKG showed early retrograde P′. Transconduit puncture was done, but EPS couldn’t induce AVNRT. Fast pathway was mapped and ablation was done near the AVN. After ablation no clinical PSVT has been observed. In complex congenital heart disease, circulation is fragile and maynot tolerate tachycardia. Radiofrequency ablation has higher risk of AV block. Cryo ablation has been shown to be much safer, but not introduced in Japan. Early introduction is strongly needed.

SummaryA Genesis stent was implanted in two children, one with superior vena caval (SVC) stenosis and one with pulmonary artery branch stenosis. Case 1 was a 2-month-old baby with SVC stenosis following intracardiac repair for total anomalous pulmonary venous connection (TAPVC) and case 2 was a 2-year-old child with left lower pulmonary artery stenosis following one-stage unifocalization for dextrocardia, double outlet right ventricle, ventricular septal defect, pulmonary atresia and major aortopulmonary collateral arteries. Both procedures resulted in immediate clinical and hemodynamic improvement. The Genesis stent has a closed-cell design with sigma hinges interpositioned between each cell. With improved deliverability and expandability of the stent, we can easily deliver it through smaller sheaths, which will facilitate its use in infants and smaller children with vascular stenosis.