Transcatheter retrieval of atrial septal defect and patent ductus arteriosus occluder: a guidance for device retrieval based on comprehensive bench tests.

OBJECTIVES: The aim of this study is to establish a guidance for device retrieval based on comprehensive bench tests. BACKGROUND: Device embolisation remains a major complication in transcatheter closure of atrial septal defect and patent ductus arteriosus. Although percutaneous retrieval is feasible in the majority of cases, surgical retrieval may be required in complicated circumstances. However, the methods of transcatheter device retrieval have not been completely established. METHODS: Bench tests of device retrieval were performed to verify the appropriate retrieval method according to device type/size. The devices used for testing were Amplatzer Septal Occluder (Abbott, Chicago, IL, United States of America), Figulla Flex II (Occlutech GmbH, Jena, Germany), Amplatzer Duct Occluder-I (Abbott), Amplatzer Duct Occluder-II (Abbott), and Amplatzer Vascular Plug-II (Abbott). The retrieval equipment constituted diagnostic catheters (multipurpose catheter and right Judkins catheter, 4-Fr or 5-Fr, Gadelius Medical, Tokyo, Japan), delivery sheath and cables for each device, Amplatz goose neck snares (Medtronic, Minneapolis, MN, United States of America), OSYPKA CATCHER (Osypka ag, Rheinfelden-Herten, Germany), and OSYPKA LASSOS (Osypka). We investigated the retrieval equipment and sheath sizes required for a successful retrieval procedure for variously sized devices. RESULTS: For patent ductus arteriosus devices, the type of snare and the snaring position are considered important. For atrial septal defect devices, simple snare capture or a double-snare technique with a sufficiently large sheath is effective. Special care should be taken when using the OSYPKA CATCHER for device retrieval. CONCLUSIONS: The results of this study may assist in the selection of both capture devices and a retrieval sheath or a catheter for complete retrieval.

Risk Factors and Predictors of Cardiac Erosion Discovered from 12 Japanese Patients Who Developed Erosion After Atrial Septal Defect Closure Using Amplatzer Septal Occluder.

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.

Efficacy and Safety of Low-Dose Amiodarone Therapy for Tachyarrhythmia in Congenital Heart Disease.

Amiodarone (AMD) is a class III anti-arrhythmic drug that is highly effective for tachyarrhythmia treatment. AMD is widely used in adults with congenital heart disease (CHD); however, higher doses of AMD (> 200 mg/day) can cause various non-cardiac side effects. The purpose of this study was to assess the efficacy, safety, and adverse events of low-dose AMD (≤ 200 mg/day) for tachyarrhythmia in patients with CHD. We retrospectively studied 80 patients with CHD and tachyarrhythmia who received oral low-dose AMD (≤ 200 mg/day) from January 2004 to March 2016. Low-dose AMD therapy was used to treat supraventricular tachycardia (SVT) in 51 patients and ventricular tachycardia (VT) in 29 patients. After a mean follow-up of 2.9 years for SVT and 3.2 years for VT, 36% and 65% of the patients with SVT and VT, respectively, were free from a first tachyarrhythmia recurrence for 3 years. The incidence of AMD-induced side effects was 23%, and all these cases consisted of thyroid dysfunction. Low-dose AMD was effective for the treatment of tachyarrhythmia in patients with CHD and had a relatively low incidence of side effects. These findings suggest that low-dose AMD is useful and effective for decreasing the frequency of tachyarrhythmia in patients with CHD and has a low incidence of side effects.

Ductal stenting using side-branch cell dilation for aortic coarctation in high-risk patients with hypoplastic left heart syndrome.

For high-risk neonates with hypoplastic left heart syndrome (HLHS) undergoing Norwood operation, the strategy of bilateral pulmonary artery banding and ductal stenting is risky in case of coarctation of the aorta (CoA), often resulting in death. Therefore, we devised a new method of ductal stenting with side-branch cell dilation, which could overcome the constriction of the ductal arch with CoA in two HLHS patients. This is the first report that presents this method and the results. © 2015 Wiley Periodicals, Inc.

Morphological and Hemodynamic Effectiveness of Stenting for Pulmonary Artery Stenosis　- Subanalysis of JPIC Stent Survey.

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).

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.

Efficacy and safety of percutaneous transluminal balloon dilation to prevent progression of banding site stenosis after bilateral pulmonary artery banding.

OBJECTIVES: To investigate the efficacy and safety of percutaneous transluminal balloon dilation (PTBD) for the treatment of bilateral pulmonary artery banding (bil-PAB) site stenosis. BACKGROUND: Although bil-PAB is an alternative initial treatment for high-risk neonates with hypoplastic left heart syndrome (HLHS) or critical aortic stenosis (cAS), those patients often suffer from desaturation because of progressive stenosis of the bil-PAB sites during the interstage period. METHODS: We retrospectively evaluated the efficacy and safety of 11 consecutive PTBD procedures performed between 2006 and 2012 to treat bil-PAB site stenosis in four high-risk infants (three females) with HLHS or cAS. RESULTS: PTBD was repeated twice in two patients and three times in one patient over intervals. The mean balloon diameter (BD) and BD-to-band circumference (BC) ratio were 3.1 ± 0.5 mm and 0.31 ± 0.06, respectively. After the procedures, the mean minimum lumen diameter was dilated significantly from 1.1 ± 0.1 mm to 1.7 ± 0.3 mm (P < 0.01), and the mean peripheral oxygen saturation increased significantly from 75 ± 8% to 85 ± 4% (P < 0.01). All patients reached the next stage operation involving the Norwood & bidirectional Glenn or Ross procedure, after growth. No complications such as band rupture occurred. CONCLUSIONS: For progressive stenosis of bil-PAB sites, PTBD using a balloon size that did not exceed the BC (BD around 30% of the BC) was an effective and safe procedure.

The clinical characteristics of sudden cardiac arrest in asymptomatic patients with congenital heart disease.

Sudden cardiac arrest (SCA) is a major cause of death in patients with congenital heart disease (CHD). Systemic ventricular dysfunction is a reported risk factor for SCA. We retrospectively analyzed the medical records of 46 patients (age >6 years) who experienced SCA. The following underlying cardiac defects were observed: biventricular repair with affected subpulmonary right ventricle (n = 18, 39 %), biventricular repair with systemic right ventricle and Eisenmenger syndrome (n = 6 each, 13 %), Fontan circulation and unrepaired CHD (n = 5 each, 11 %), and others (n = 6, 13 %). Twenty-one patients (46 %) had no history of arrhythmias, and 21 of 43 (49 %) showed systemic ventricular ejection fraction >55 %. According to the New York Heart Association classification, 18 patients (39 %) were class I and 28 (61 %) were class II/III. SCA occurred at a younger age in class I (16 ± 5 years) than in the other classes (23 ± 10 years; P = 0.004). QRS duration was similar between the groups (136 ± 38 vs. 141 ± 50 ms; P not significant). Seven patients in class I (15 % of all SCAs) had no history of arrhythmias or features of hemodynamic abnormalities. The proportion of patients with biventricular repair and affected subpulmonary right ventricle was higher than that of patients with other defects, and the majority of SCA patients had more complicated defects than a simple repaired ventricular septal defect or an atrial septal defect. No symptoms of heart failure, history of arrhythmias, or features of hemodynamic abnormalities were observed in 15 % of the patients who experienced SCA. Prolonged QRS duration might be a predictor of SCA even in asymptomatic CHD patients. Prevention of SCA in CHD patients may require more detailed evaluation than is typically considered necessary.

The importance of hybrid stage I palliation for neonates with critical aortic stenosis and reduced left ventricular function.

The optimal management strategy for neonates with congenital aortic stenosis, two balanced ventricles, and duct-dependent systemic circulation (critical aortic stenosis) is still controversial. Thirteen patients with critical aortic stenosis underwent balloon aortic valvotomy (BAV) between 1996 and 2013, at the median age of 1 day old (range 0-28). Since 2010, bilateral pulmonary artery banding with ductal stenting following BAV was conducted for patients with reduced left ventricular (LV) function as a hybrid stage I palliation for the bridge to decision for further treatment. A follow-up was completed on all patients and the median follow-up period was 3.3 years (max 16.0). The overall survival rate at 15 years was 67.1 %. Six of the seven patients with maintained LV function could go on to the definitive Ross or Konno-aortic valve replacement at the median duration of 311 days after initial BAV, without any mortality. Three of four patients with reduced LV function died before 2010 with conventional treatment. With use of a hybrid stage I palliation, one of two patients ultimately underwent Fontan completion at 38 months of age and the other successfully underwent the definitive Ross-Konno operation at 9 months of age after recovery of the LV function. Although a statistically significant improvement has not been observed yet, the application of hybrid stage I palliation following BAV would be a favorable alternative for patients with reduced LV function to avoid a high-risk neonatal Ross or Norwood-type operation, and also to determine further treatment carefully.

Use of triple ultra-high-pressure balloons for obstructed right ventricular outflow conduits in adults can be safe and effective.

To date, no transcatheter valve has been approved for placement in the pulmonary position in Japan. Consequently, percutaneous balloon dilatation may be advised for stenotic right ventricular outflow lesions; however, technical difficulties persist, particularly in adults. We describe the acute haemodynamic changes and outcome of balloon dilatation of right ventricular outflow obstruction using triple ultra-high pressure balloons. This is the first report of such a technical development, which seems to be safe and effective. A total of three adult patients, aged 25, 29, and 37 years, with severe conduit obstruction were referred for balloon dilatation. A triple ultra-high-pressure balloon technique was used in the three patients after unsuccessful double-balloon dilatation, or for highly calcified lesions, which were expected to require ultra-high pressure for effective relief. Following balloon dilatation, the pressure gradient decreased from 24, 30, 65 to 3, 25, 30 mmHg, respectively. There were no procedural complications except slightly increased pulmonary regurgitation. Balloon dilatation using a triple ultra-high pressure balloon technique can be a safe and effective palliative procedure for conduit obstruction in adult patients.

Evaluation of Valsalva sinus wall deformation due to compression by the Amplatzer septal occluder and the potential for erosion development.

OBJECTIVES: We studied whether the pressure exerted by the Amplatzer septal occluder (ASO) disk on the Valsalva sinus wall (VW) is a risk factor for erosion and attempted to determine the pressure that may trigger erosion. BACKGROUND: The causes and mechanisms of erosion after ASO placement remain unclear. METHODS: We reviewed 665 consecutive patients with atrial septal defects who underwent ASO closure at our hospital from 2005 to 2012. We used transesophageal echocardiography and other tests to evaluate disk-related VW deformation as a risk factor for erosion. RESULTS: Immediately after ASO placement, intermittent VW deformation from either disk was determined in 54 of the 665 patients (8%). Of these 54 patients, only 1 exhibited late erosion (0.15%) during a follow-up of more than 4 years (0.035% a year). The maximum VW deformation depth relative to the standard curve, or dent, was 2.6 mm in this patient, whereas that in the remaining patients ranged from 0.5 to 2.0 mm. The dent values were significantly deeper in patients with contact between the left atrial disk and posterior atrial wall than in those without contact (P = 0.03). There was a significant negative correlation between dent and the aortic side-maximum device thickness (DT)/middle part-DT ratio (P = 0.04). CONCLUSION: Although VW deformation, particularly deep VW deformation caused by pressure from either disk after ASO placement must be a recognized risk for erosion, which deformation level carries a stronger risk for erosion could not be concluded.

Association between parental decisions regarding abortion and severity of fetal heart disease.

The prenatal diagnosis of fetal heart disease potentially influences parental decision-making regarding pregnancy termination. Existing literature indicates that the severity, whether in complexity or lethality, significantly influences parental decisions concerning abortion. However, questions remain as to how fetal heart disease severity impacts parental decisions, given recent advancements in postsurgical outcomes. Therefore, we investigated risk factors associated with parents' decision-making regarding abortion following a prenatal diagnosis of fetal heart disease. Our analysis included 73 (terminated: n = 37; continued: n = 36) pregnancies with a fetal heart disease diagnosed before 22 weeks of gestation. Increased gestational age at diagnosis reduced the likelihood of parents' decision on termination (Model 1: adjusted odds ratio, 0.94; 95% confidence interval 0.89-0.99; Model 2: 0.95 0.90-0.997). Critical disease (5.25; 1.09-25.19) and concurrent extracardiac or genetic abnormalities (Model 1: 4.19, 1.21-14.53; Model 2: 5.47, 1.50-19.96) increased the likelihood of choosing abortion. Notably, complex disease did not significantly influence parental decisions (0.56; 0.14-2.20). These results suggest that parental decision-making regarding abortion may be influenced by earlier gestational age at diagnosis, the lethality of heart disease, and extracardiac or genetic abnormalities, but not its complexity if prenatal diagnosis and parental counseling are provided at a cardiovascular-specialized facility.

Usefulness of a Pulse Oximeter and Multimodality Imaging for Diagnosing Platypnea-orthodeoxia Syndrome.

Platypnea-orthodeoxia syndrome (POS) is a rare disease characterized by dyspnea and hypoxemia in orthostatism that improves in the recumbent position. We herein report an 81-year-old woman with dyspnea in the upright position following thoracic vertebral compression fractures. After the patient's daughter brought a recording showing decreasing SpO2 (peripheral capillary oxygen saturation) in the upright position as measured by a portable pulse oximeter outside the hospital, a small atrial septal defect (ASD) was detected. A contrast echocardiogram and four-dimensional flow magnetic resonance imaging demonstrated a right-to-left shunt. The patient's symptoms dramatically improved after percutaneous ASD closure. In conclusion, such new technologies are useful for diagnosing POS.

Successful transcatheter mitral valve repair with the MitraClip system in a patient with Duchenne muscular dystrophy.

Duchenne muscular dystrophy (DMD) is a congenital X-linked muscular dystrophy, and cardiomyopathy typically develops in the second decade of life. In those patients with severe heart failure, the use of mechanical circulatory support is considered one of the treatment options, however, the decision to implant the assist device should be made after gauging the inherent risks and potential benefits. Transcatheter mitral valve repair (TMVr) is performed in adults with heart failure due to severe mitral regurgitation (MR) refractory to guideline-directed medical therapies. Data on MitraClip-based treatment (Abbott Vascular, Menlo Park, CA, USA) of young patients with cardiomyopathy-associated severe heart failure remain limited. We present the first report on the safety and effectiveness of TMVr with the MitraClip in an 18-year-old man with DMD and severe MR who was at a prohibitive risk for mitral valve surgery or left ventricular assist device therapy due to comorbidities. He was discharged without complications and, is now asymptomatic at one year after TMVr. Learning objective: Readers will be able to:Understand the mechanism of mitral regurgitationUnderstand the less invasiveness and usefulness of this procedure.Discuss the indication of transcatheter mitral valve repair in young patients with severe heart failure.

Single-stage arterial switch operation conversion with dextro-transposition of the great arteries and pulmonary arterial hypertension due to baffle leak after a Senning procedure.

Patients who have undergone an atrial switch operation for dextro-transposition of the great arteries (dTGA) sometimes suffer from right ventricular dysfunction, tricuspid regurgitation, arrhythmias, or baffle leaks. We report the first case of single-stage arterial switch operation conversion in an adult patient with dTGA and pulmonary arterial hypertension (PAH) due to a baffle leak after a Senning procedure. Perioperative mechanical circulatory support was required for 3 weeks. Although chronic kidney disease persisted, her left ventricular function and pulmonary hypertension improved over the first postoperative year. This is a rare case, and we believe that the patient's PAH might have helped the left ventricle withstand systemic pumping for over two decades. Learning objective: Patients with dextro-transposition of the great arteries may face problems after an atrial switch operation because their right ventricle is responsible for systemic pumping. Staged arterial switch operation conversion is a strategy that can combat these problems but increases the risk of perioperative mortality. Pulmonary arterial hypertension allows for single-stage arterial switch operation, but the operative risk may be higher.

Long-term outcome of coil occlusion in patients with patent ductus arteriosus.

BACKGROUND: Coil occlusion has been widely indicated for the closure of patent ductus arteriosus (PDA). Although many reports have shown the efficacy and safety of coil occlusion, the long-term outcome in patients remains controversial. Here, we analyzed the long-term outcome of coil occlusion in patients with PDA in Japan. METHODS AND RESULTS: We collected the longitudinal data of patients who underwent coil occlusion between 1995 and 2009. A total of 310 coil occlusions were performed in 298 patients with PDA. The median minimum duct diameter was 1.4mm. Successful coil occlusion was achieved in 286 patients (96.0%), and total adverse events were seen in only 28 cases (9.0%). The median follow-up period was 50 months. The occlusion rates at 1 month, 6 months, 1 year, 2 years and 5 years were 90.1%, 94.4%, 97.4%, 97.8% and 97.8%, respectively. Patients with a large PDA (≥4mm) showed a higher rate of residual leakage than those with a small (<2mm) or moderate (2-4mm) PDA (P=0.004). Patients who underwent this procedure in the early study period also showed a higher rate of residual leakage than those in the late study period. CONCLUSIONS: Coil occlusion is an effective procedure for patients with PDA. Our data indicate that the long-term outcome is promising without any adverse events.

The clinical course and incidence of supraventricular tachyarrhythmias after extra-cardiac conduit Fontan procedures in relation to an atrial situs.

BACKGROUND: The extra-cardiac conduit Fontan (EC) has a lower incidence of tachyarrhythmias than other types of Fontan. However, some intrinsic arrhythmogenic conditions, such as atrial isomerism, have been associated with a high incidence of arrhythmias. METHODS AND RESULTS: We retrospectively reviewed the clinical course of the supraventricular tachyarrhythmias (SVTs) in 212 patients after an EC, compared the results according to the atrial situs and investigated the substrate of the SVTs. The atrial situs was a solitus or inversus (SS/SI) in 152 patients, right isomerism (RI) in 45 and left isomerism (LI) in 15. Twenty-four SVTs occurred in 22 (11%) patients ≥3 months after the EC. The freedom from SVT after the EC was 95%, 76% and 77% in the SS/SI, RI and LI patients at 5 years, respectively (P<0.0001). Among the 16 SVTs associated with atrial isomerism, 10 were not related to the atrioventricular (AV) node. For the atrial isomerism, the predictors of SVT ≥3 months after the EC were a history of an AV valve repair (risk ratio (RR) 1.9; P=0.02) and complications associated with sinus node dysfunction (RR 1.9; P=0.03). Death related to SVT occurred in 3 patients. CONCLUSIONS: Postoperative SVTs after the EC with an atrial isomerism were not uncommon, mostly did not involve the AV node and possibly were caused by atrial tissue damage. A comprehensive therapeutic strategy should be considered.

The influence of morphological changes in amplatzer device on the atrial and aortic walls following transcatheter closure of atrial septal defects.

OBJECTIVE: This study prospectively investigated morphological changes in Amplatzer Septal Occluder (ASO) over time and the influences of these changes on the atrial and aortic walls after atrial septal defect (ASD) closure. METHODS: Between August 2005 and December 2007, 78 patients with ASD were treated with ASO devices and changes in the device shape, the device thickness, and relations of the discs to the atrial and aortic walls over time were evaluated by transesophageal echocardiography immediately and 3-12 months after deployment. RESULTS: The maximum unstretched ASD diameter was 16.2 +/- 4.8 mm and the device diameter selected was 20.6 +/- 5.5 mm. At the time of last follow-up, the device thickness decreased by 17-33%, 6 of 26 devices with a flare shape on the aortic side developed a closed shape, and the relations of the discs to the anterior atrial and aortic walls changed from touching to intermittent compression in 14 of the 78 cases. In these 14 cases, the aortic rim was significantly smaller, the number of flared device shapes on the aortic side/the number of closed shapes immediately after deployment was significantly larger, and the maximum device thickness at the middle part was significantly more decreased than those in other cases. CONCLUSION: As the device becomes thinner, loses its flexibility, and often changes from a flare-to-closed shape on the aortic side over time, the edges of ASO can start to compress the atrial and aortic walls. However, erosion was not recognized in these cases.

Primary palliative stenting against obstructive mixed-type total anomalous pulmonary venous connection associated with right atrial isomerism.

This case report presents a low-birth-weight neonate who received primary stent implantation as a long-term palliative intervention for obstructive mixed-type total anomalous pulmonary venous connection (TAPVC) without common pulmonary venous chamber associated with right atrial isomerism, which was considered difficult to surgically repair in the neonatal period. Stent redilation with balloon catheters was repeated for in-stent stenosis from neointimal proliferation, resulting in successful TAPVC repair with cavopulmonary connection at 17 months of age.