Practice and Safety of Static Balloon Atrial Septostomy Based on a Nationwide Registry Data.

BACKGROUND: Balloon atrial septostomy (BAS) is an essential catheterization procedure for congenital heart lesions. Recently, a balloon catheter for static BAS was approved for the first time in Japan as an alternative to the conventional pull-through BAS. Despite the expected increase in the use of static BAS, reports on its safety are scarce worldwide.Methods and Results: Data on static and pull-through BAS registered in a national registry between 2016 and 2018 were collected. During the study period, 247 sessions of static BAS and 588 sessions of pull-through BAS were performed on a total of 674 patients. Patients who underwent static BAS were older (P<0.001). The incidence of serious adverse events (4.3% vs. 0.9%, P=0.03) and the overall incidence of adverse events (8.1% vs. 3.2%, P=0.03) were higher in static BAS than in pull-through BAS. Among patients who underwent static BAS, the risk factor for adverse events was a body weight <3 kg at the time of the procedure (odds ratio: 4.3 [confidence interval: 1.7-11], P=0.003). CONCLUSIONS: This nationwide study revealed differences in patient background between static and pull-through BAS, as well as a higher incidence of adverse events related to static BAS. Patients weighing <3 kg are at high risk for adverse events after static BAS and may require surgical and circulatory support backup.

Exacerbation of heart failure due to total pacemaker extrusion.

Total extrusion of a pacemaker is rare and may result from skin and/or pocket infection or skin erosion because of fragility caused by actions such as scratching an itch. Total extrusion of a pacemaker may cause fatal arrhythmias, exacerbation of heart failure, and infection. We report the case of a 37-year-old man with a pacemaker implanted for complete atrioventricular block who presented with exacerbation of advanced heart failure due to complete extrusion of the pacemaker from the left groin and pacing failure. No fever was observed during the clinical course, but exudate leaked from his abdominal pocket. Electrocardiography showed a complete atrioventricular block. He successfully underwent implantation of a new pacemaker. Learning objective: There are various complications related to permanent pacemaker implantation; however, total extrusion, which is extremely rare, can lead to pacemaker failure, sepsis, and life-threatening arrhythmias. It is essential not only to check the pacemaker pocket and generator but also to educate patients on the importance of pacemaker check-ups and problems.

Successful treatment of symptomatic first-degree atrioventricular block after Occlutech Figulla Flex II atrial septal defect occluder placement.

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. .

Patch testing with the Japanese baseline series 2015: A 4-year experience.

BACKGROUND: The Japanese baseline series (JBS), established in 1994, was updated in 2008 and 2015. The JBS 2015 is a modification of the thin-layer rapid-use epicutaneous (TRUE) test (SmartPractice Denmark, Hillerød, Denmark). No nationwide studies concerning the TRUE test have previously been reported. OBJECTIVES: To determine the prevalence of sensitizations to JBS 2015 allergens from 2015 to 2018. METHODS: We investigated JBS 2015 patch test results using the web-registered Skin Safety Care Information Network (SSCI-Net) from April 2015 to March 2019. RESULTS: Patch test results of 5865 patients were registered from 63 facilities. The five allergens with the highest positivity rates were gold sodium thiosulfate (GST; 25.7%), nickel sulfate (24.5%), urushiol (9.1%), p-phenylenediamine (PPD; 8.9%), and cobalt chloride (8.4%). The five allergens with the lowest positivity rates were mercaptobenzothiazole (0.8%), formaldehyde (0.9%), paraben mix (1.1%), mercapto mix (1.1%), and PPD black rubber mix (1.4%). CONCLUSIONS: Nickel sulfate and GST had the highest positivity rates. The JBS 2015, including a modified TRUE test, is suitable for baseline series patch testing.

Comparison of 5-year safety and efficacy of laser photocoagulation and intravitreal bevacizumab injection in retinopathy of prematurity.

PURPOSE: To compare laser photocoagulation and intravitreal injection of bevacizumab (IVB) treatment for retinopathy of prematurity (ROP). METHODS: The study included 52 eyes of 26 patients after ROP treatment who were observed up to 5 years of age. Twenty-eight eyes received laser photocoagulation as the initial treatment (laser group), and twenty-four eyes underwent IVB (IVB group). We collected data on gestational age, birth weight, 1- and 5-min Apgar scores, zone and stage at the time of treatment, recurrence of ROP and best-corrected visual acuity (BCVA) (logMAR), equivalent spherical value (SE), ocular complications, and developmental delay at the age of 5. RESULTS: More zone I low-stage eyes were treated with IVB than laser. There was no difference in BCVA (p = 0.836). Although the mean SE was not different between the groups (p = 0.280), the prevalence of myopia was significantly higher in the laser group (p = 0.020). Developmental delay was observed in 3 of 14 and 3 of 12 cases in the laser and IVB groups, respectively (p = 0.596). Retinal holes were observed in 2 eyes in the IVB group, with 1 developing localized retinal detachment. There were no significant differences between the groups in the other factors. CONCLUSIONS: Compared to laser for ROP, IVB was not inferior in neurodevelopment or visual outcome and was superior in refractive error. As cases in the IVB group showed retinal holes, long-term follow-up with fundus examination is recommended after IVB.

Pediatric Medical Devices　- Survey of Pediatric Cardiologists and Cardiovascular Surgeons in Japan.

Background: In Japan, the choice of pediatric medical devices is limited because of 2 "device lag" problems: Japan lags behind the USA and Europe in device development, and development of pediatric devices lags behind that of adult devices. We aimed to identify the problems with and impediments to pediatric medical device development as recognized by pediatric physicians in Japan. Methods and Results: A voluntary survey of pediatric medical devices for all council members of the Japanese Society of Pediatric Cardiology and Cardiac Surgery was conducted in 2019. The response rate was 47.1% (154/327). The respondents were 115 pediatric cardiologists (74.7%) and 39 cardiovascular surgeons (25.3%). Approximately 90% believed that difficulties in development existed. Approximately 70% were dissatisfied with the pediatric medical devices currently available in Japan, which was a result of the unavailability of medical devices approved overseas, few types and sizes, and off-label use. Factors that hindered the development of pediatric medical devices included anatomical issues specific to children with congenital heart disease, as well as system issues such as lack of corporate profitability, development cost, and amount of time for development. Conclusions: Pediatric cardiologists and cardiovascular surgeons regard "device lag" and "off-label use" in Japan as important hindrances to the delivery of better medical care for pediatric patients with congenital heart disease.

Bilateral coronary ostial atresia in a patient with pulmonary atresia and ventricular septal defect.

This is a case of a female infant with bilateral coronary ostial atresia associated with pulmonary atresia and ventricular septal defect. She developed coronary ischemia at 1-month of age, when she underwent an aortopulmonary shunt and an aorta-right ventricle shunt. The double-orifice tricuspid valve was separating the right ventricle from the left ventricle. She required extracorporeal cardiopulmonary support because of ventricular dysfunction and mitral regurgitation. Although she was temporarily weaned off the support after mitral valvuloplasty, she died from multiple organ failure. To the best of our knowledge, bilateral coronary ostial atresia associated with pulmonary atresia with ventricular septal defect has not been reported previously.

Stent Implantation for Congenital Heart Diseases in Japan　- Comprehensive Analysis From the Japanese Society of Congenital Interventional Cardiology Registry.

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.

The mid-term outcome of Fontan conversion compared with primary total cavopulmonary connection.

BACKGROUND: The indication of Fontan conversion (FC) from atriopulmonary connection (APC) to total cavopulmonary connection (TCPC) is unclear. We sought to analyze the mid-term outcome of prophylactic and therapeutic Fontan conversion compared with that of primary TCPC. METHODS: Patients with a univentricular heart who underwent cardiac catheterization at >18 years of age between July 2005 and July 2019 were included and divided into three groups: symptomatic APC patients who underwent therapeutic FC (t-FC, n = 13), asymptomatic APC patients after prophylactic FC (p-FC, n = 15), and patients who had primary TCPC procedure (pTCPC, n = 24). RESULTS: The mean last follow up was at the age of 32.0 ± 7.8, 26.8 ± 3.8, and 27.3 ± 7 years (p = 0.07) in t-FC, p-FC, and pTCPC, respectively. There was no late death. All of t-FC and 12 (80%) of p-FC cases underwent concomitant arrhythmic surgery. Consequently, five and four patients in t-FC and p-FC groups required pacemaker implantations mostly due to sinus node dysfunction. Thromboembolism was seen in 2 cases in both t-FC (15%) and p-FC (13%), and 1 case in pTCPC (4%) (p = 0.50). The last cardiac catheterization was performed at the age of 29.5 ± 8.5, 24.6 ± 3.8, and 26.3 ± 7.1 years (p = 0.11) in t-FC, p-FC, and pTCPC patients, respectively. There was no significant difference in central venous pressure, aortic pressure, and cardiac index among the three groups. There was no late supraventricular tachyarrhythmic event seen in t-FC and p-FC, whereas two patients in pTCPC had newly developed atrial flutter. CONCLUSIONS: FC is a safe and feasible procedure to bring APC patients back onto the same track of primary TCPC patients in terms of hemodynamics as well as arrhythmia. The antiarrhythmic procedure should be carefully chosen because sinus node dysfunction can frequently occur and FC itself would reduce the risk of arrhythmia.

Multicenter 1-month follow-up study of the patch-test reaction to the gold sodium thiosulfate of the TRUE Test and its association with piercings and dental metal history.

BACKGROUND: There is controversy over late and long-lasting reactions to gold sodium thiosulfate (GST). OBJECTIVES: To study the GST patch-test reaction by observing the application site after 1 month, and to clarify the relevance of GST sensitization by piercings and dental metals. PATIENTS: A retrospective analysis was performed on 746 patients (143 male; 603 female) who were patch tested using GST of the TRUE Test. We conducted a questionnaire on the presence of piercings or dental metals in these patients. RESULTS: The GST positive rate was 27.9% at day (D)3 and/or D7 and 40.3% up to the 1-month reading. The positive rate was significantly higher in female patients and increased with age. Sixty-two percent of cases with a positive reaction at D7 continued to show a positive reaction after 1 month. Eleven percent of cases with a negative reaction at D3 and D7 showed a late reaction. Both piercings and dental metals were related to gold sensitization. CONCLUSIONS: The GST of the TRUE Test had a high positive and low false-negative rate. The 1-month reading after the patch test was important for identifying late reactions. Piercing history and dental metal were associated with gold sensitization.

A retrospective review of 10-year trends in general anesthesia for cesarean delivery at a university hospital: the impact of a newly launched team on obstetric anesthesia practice.

BACKGROUND: The indications for general anesthesia (GA) in obstetric settings, which are determined in consideration of maternal and fetal outcome, could be affected by local patterns of clinical practice grounded in unique situations and circumstances that vary among medical institutions. Although the use of GA for cesarean delivery has become less common with more frequent adoption of neuraxial anesthesia, GA was previously chosen for pregnancy with placenta previa at our institution in case of unexpected massive hemorrhage. However, the situation has been gradually changing since formation of a team dedicated to obstetric anesthesia practice. Here, we report the results of a review of all cesarean deliveries performed under GA, and assess the impact of our newly launched team on trends in clinical obstetric anesthesia practice at our institution. METHODS: Our original database for obstetric GA during the period of 2010 to 2019 was analyzed. The medical records of all parturients who received GA for cesarean delivery were reviewed to collect detailed information. Interrupted time series analysis was used to evaluate the impact of the launch of our obstetric anesthesia team. RESULTS: As recently as 2014, more than 10% of cesarean deliveries were performed under GA, with placenta previa accounting for the main indication in elective and emergent cases. Our obstetric anesthesia team was formed in 2015 to serve as a communication bridge between the department of anesthesiology and the department of obstetrics. Since then, there has been a steady decline in the percentage of cesarean deliveries performed under GA, decreasing to a low of less than 5% in the latest 2 years. Interrupted time series analysis revealed a significant reduction in obstetric GA after 2015 (P = 0.04), which was associated with decreased use of GA for pregnancy with placenta previa. On the other hand, every year has seen a number of urgent cesarean deliveries requiring GA. CONCLUSIONS: There has been a trend towards fewer obstetric GA since 2015. The optimized use of GA for cesarean delivery was made possible mainly through strengthened partnerships between anesthesiologists and obstetricians with the support of our obstetric anesthesia team.

A case report: stent implantation to treat coarctation of persistent 5th aortic arch associated with interrupted 4th aortic arch.

BACKGROUND: Persistent 5th aortic arch is a rare cardiac anomaly that is usually surgically corrected during infancy or early childhood if it is associated with coarctation of the aorta. Here, we report an adult with coarctation of the 5th aortic arch who was successfully treated by stent implantation. CASE SUMMARY: An asymptomatic 32-year-old woman presented with hypertension and a significant arm-leg difference in pressure. On suspicion of coarctation of the aorta, a chest computed tomography was performed, leading to a diagnosis of an interrupted 4th aortic arch with coarctation of a persistent 5th aortic arch. Percutaneous catheter intervention using a PALMAZ large stent dilated to 12 mm resulted in a minimal peak-to-peak pressure gradient. The patient was discharged home after a 2-day monitoring without hypertension and arm-leg blood pressure difference. She remained normotensive with a patent aortic arch on echocardiography performed 10 months after treatment. DISCUSSION: As for simple coarctation of the aorta, stent implantation was feasible and effective in an adult patient with coarctation of the 5th aortic arch.

HLA-DQ and RBFOX1 as susceptibility genes for an outbreak of hydrolyzed wheat allergy.

BACKGROUND: Food allergy is a growing health problem worldwide because of its increasing prevalence, life-threatening potential, and shortage of effective preventive treatments. In an outbreak of wheat allergy in Japan, thousands of patients had allergic reactions to wheat after using soap containing hydrolyzed wheat protein (HWP). OBJECTIVES: The aim of the present study was to investigate genetic variation that can contribute to susceptibility to HWP allergy. METHODS: We conducted a genome-wide association study of HWP allergy in 452 cases and 2700 control subjects using 6.6 million genotyped or imputed single nucleotide polymorphisms. Replication was assessed by genotyping single nucleotide polymorphisms in independent samples comprising 45 patients with HWP allergy and 326 control subjects. RESULTS: Through the genome-wide association study, we identified significant associations with the class II HLA region on 6p21 (P = 2.16 × 10-24 for rs9271588 and P = 2.96 × 10-24 for HLA-DQα1 amino acid position 34) and with the RBFOX1 locus at 16p13 (rs74575857, P = 8.4 × 10-9). The associations were also confirmed in the replication data set. Both amino acid polymorphisms (HLA-DQß1 amino acid positions 13 and 26) located in the P4 binding pockets on the HLA-DQ molecule achieved the genome-wide significance level (P < 5.0 × 10-8). CONCLUSIONS: Our data provide the first demonstration of genetic risk for HWP allergy and show that this genetic risk is mainly represented by multiple combinations of HLA variants.

Atropine-induced monomorphic ventricular tachycardia in an adolescent: A rare case.

A 13-year-old girl had a history of episodic palpitations lasting for approximately 5 min since the first grade of junior high school. She was noticed to have tachycardia during auscultation at a school-based health screening program. Since non-sustained ventricular tachycardia of the left bundle branch block type was induced by a triple master exercise load at a local doctor's clinic, she was referred to our pediatric cardiology department for further management. Tachycardia could not be induced by programmed stimulation in an electrophysiological study, although ventricular tachycardia was induced by atrial high frequency pacing with intravenous injection of atropine under continuous isoproterenol infusion. .

Incomplete isolation of the left innominate artery from the right aortic arch: a rare case of double-outlet right ventricle with pulmonary stenosis.

Isolation of the left innominate artery from the right aortic arch is a rare anomaly. Herein, we present an even rarer case of incomplete isolation of the proximal left innominate artery with the right aortic arch in a 3-month-old female infant with a double-outlet right ventricle and pulmonary stenosis. Surgical repair at 6 months of age was successful, leading to the restoration of adequate flow in the left arm.

Short-term intraocular pressure changes after intravitreal injection of bevacizumab for retinopathy of prematurity.

PURPOSE: To evaluate the short-term changes in intraocular pressure (IOP) after intravitreal injection of bevacizumab (IVB) in premature infants with retinopathy of prematurity (ROP). STUDY DESIGN: Prospective cohort study. METHODS: Twenty-two eyes of 11 premature infants with ROP were evaluated. The control eyes were from adults who received an intravitreal injection of antivascular endothelial growth factor agent. The patients received a 0.025 mL (0.625 mg) IVB for the ROP. The IOP was measured with a rebound tonometer at baseline, immediately after the injection, and at 5, 10, 15, 20, and 30 minutes after the injection. The clinical data were collected. The main outcome measures were the IOP values at baseline, immediately after the injection, and at 5, 10, 15, 20, and 30 minutes after the injection. RESULTS: The mean IOP values at baseline, immediately after the injection, and at 5, 10, 15, 20, and 30 minutes after the injection were 12.3 ± 3.0, 40.1 ± 10.7, 20.8 ± 5.1, 15.1 ± 4.8, 11.9 ± 3.2, 9.9 ± 2.6, and 8.8 ± 2.1 mmHg, respectively. The IOP was significantly higher at 5 minutes after the injection (P < .01); however, the pressure in all the eyes normalized (< 21 mmHg) within 15 minutes. None of the clinical parameters, including axial length, was correlated with a high IOP after IVB. CONCLUSION: In premature infants with ROP, IVB is tolerable in terms of the short-term elevation in IOP, which returned to a safe range (< 21 mmHg) within 15 minutes of the injection in all the patients. Consecutive IOP measurement might not be necessary after IVB in premature infants with ROP.

Case report: Atrioventricular block after transcatheter atrial septal closure using the Figulla® Flex II ASD occluder.

We report a 7-year-old male patient who developed severe atrioventricular block after transcatheter closure of the atrial septal defect with an Occlutech Figulla® Flex II ASD occluder (FSO). He had a small aortic rim and the defect measuring 22.3 mm by balloon sizing. When a 24 mm FSO was deployed, he developed Wenckebach second-degree heart block; however, it recovered to sinus rhythm. Hence, the device was implanted. The rhythm deteriorated to a fixed 2:1 heart block within 7 hr. He underwent surgical retrieval of the device and closure of the defect. Intraoperative findings demonstrated the right atrium disk compressing the triangle of Koch, resulting in a small hematoma. The rhythm recovered completely by 7 days after the surgery. Care must be taken when a relatively large device is deployed in a patient with small rims as even "soft and flexible" device like the FSO can injure the endocardium.