Is the QRS duration useful for determining the optimal timing of pulmonary valve replacement after tetralogy of Fallot repair?

OBJECTIVES: In pulmonary valve replacement (PVR) after tetralogy of Fallot (TOF) repair, the right ventricular end-diastolic and end-systolic volume index (RVEDVI and RVESVI) of cardiac magnetic resonance imaging (cMRI) are often used as indicators of the RV volume. We examined the utility of QRS duration, cardiothoracic ratio (CTR), and plasma brain natriuretic peptide (BNP) as indicators of the appropriate timing of cMRI to assess the RV volume and function before PVR. METHODS: We assessed the correlation of QRS duration, CTR, and BNP with RVEDVI and RVESVI on cMRI in 26 patients after TOF repair. Fifteen underwent PVR (age, 45.2 ± 11.4 years). Twelve underwent post-PVR cMRI. The RV volume change from before to after PVR was investigated. RESULTS: QRS duration, BNP, and CTR were positively correlated with RVEDVI and RVESVI after TOF repair. The post-PVR QRS duration was also positively correlated with post-PVR RVEDVI (p = 0.017) and RVESVI (p = 0.001). From before to after PVR, in 5 cases with QRS duration ≤ 160 ms, the QRS duration decreased from 110.4 ± 28.9 to 101.8 ± 30.5 ms (p = 0.063). Both RVEDVI and RVESVI decreased to the normal range in 4 of 5 cases. In contrast, in 7 cases with QRS duration > 160 ms, the QRS duration decreased from 183.0 ± 17.4 to 160.3 ± 23.8 ms (p = 0.013); however, RVESVI did not normalize in 6 of 7 cases. CONCLUSIONS: A prolonged QRS duration is a useful marker of RVEDVI and RVESVI enlargement after TOF repair. We recommend performing cMRI before the QRS duration reaches 160 ms due to normalization of the RV volume after PVR.

Hospital Support for Siblings of Children With Illness in Japan.

Recent years have seen increased attention to the needs and support of siblings of children with chronic illness, and reports of intervention studies on siblings are gradually increasing worldwide. In Japan, the basic policy approved by the Cabinet in 2021 of The Basic Law for Child and Maternal Health and Development stipulates promoting support for the siblings of children with chronic illness, medical care, and disabilities. Simultaneously, practical reports are emerging. However, reports on the actual state of sibling support at medical institutions in Japan are limited. This study aimed to describe the actual state of support for siblings of children with illness in Japanese medical institutions using a cross-sectional design. Responses were obtained from 207 of 484 registered training facilities for Board-Certified Pediatricians of the Japan Pediatric Society through anonymous questionnaires investigating the actual state of siblings' support. Descriptive statistics were calculated, and the state of siblings' support was described. Fifty-two participants (25.1%) answered that the entire ward, including two outpatient departments, provided siblings' support, while 37 (17.9%) answered some staff made an effort, and 117 (56.5%) did not. Support mentioned included conversing with siblings, actively speaking to siblings, calling siblings' names, and counseling care through the parents. Of the 45 cases (21.7%) where siblings were invited to events and gatherings, 10 (22.2%) were siblings-centered events. Some cases involved collaboration with local sibling support groups such as non-profit organizations. This study clarified the actual state of siblings' support, and further expansion of this support is required.

Installation of multiple automated external defibrillators to prevent sudden death in school-aged children.

BACKGROUND: Recently, a student died of idiopathic ventricular fibrillation in a school where an automated external defibrillator (AED) had been installed. The tragedy could not be prevented because the only AED in the school was installed in the teachers' office, far from the school ground where the accident took place. This prompted establishment of a multiple AED system in schools. The aim of this study was to analyze the efficacy of the multiple AED system to prevent sudden death in school-aged children. METHODS: Assumed accident sites consisted of the school ground, gymnasium, Judo and Kendo hall, swimming pool, and classrooms on the first and the fourth floor. Multiple AED were installed in the teachers' office, gymnasium, some classrooms, and also provided as a portable AED in a rucksack. The time from the accident site to the teachers' office for single AED, and from the accident site to the nearest AED for multiple AED, was calculated. RESULTS: The AED retrieval time was significantly shorter in 55 elementary schools and in 29 junior high schools when multiple AED were installed compared with single AED. Except for the classroom on the fourth floor, the number of people who took >120 s to bring the AED to the accident site was lower when multiple AED were installed compared with the single AED. CONCLUSION: Multiple AED provided in appropriate sites can reduce the time to reach the casualty and hence prevent sudden death in school-aged children.

DGCR6 at the proximal part of the DiGeorge critical region is involved in conotruncal heart defects.

Cardiac anomaly is one of the hallmarks of DiGeorge syndrome (DGS), observed in approximately 80% of patients. It often shows a characteristic morphology, termed as conotruncal heart defects. In many cases showing only the conotruncal heart defect, deletion of 22q11.2 region cannot be detected by fluorescence in situ hybridization (FISH), which is used to detect deletion in DGS. We investigated the presence of genomic aberrations in six patients with congenital conotruncal heart defects, who show no deletion at 22q11.2 in an initial screening by FISH. In these patients, no abnormalities were identified in the coding region of the TBX1 gene, one of the key genes responsible for the phenotype of DGS. However, when copy number alteration was analyzed by high-resolution array analysis, a small deletion or duplication in the proximal end of DiGeorge critical region was detected in two patients. The affected region contains the DGCR6 and PRODH genes. DGCR6 has been reported to affect the expression of the TBX1 gene. Our results suggest that altered dosage of gene(s) other than TBX1, possibly DGCR6, may also be responsible for the development of conotruncal heart defects observed in patients with DGS and, in particular, in those with stand-alone conotruncal heart defects.

Pulmonary artery banding for neonates and early infants with low body weight.

Open heart surgery for infants with low body weight (BW) remains still a challenge. Pulmonary artery banding (PAB) is a useful surgical palliation for small neonates and early infants with excessive pulmonary blood flow who are unable to withstand a heart surgery. This study retrospectively reviewed neonates and infants who underwent PAB to assess the surgical results and the validity of our PAB. We selected 38 acyanotic infants and neonates and divided them into 2 groups: low BW (< 2.5 kg, n = 15, group L) and normal or high BW (≥ 2.5 kg, n = 23, group NH). The average BW at the time of PAB was 2.8 ± 1.1 kg (range, 1.2-5.8 kg), and the average age at the time of PAB was 41.8 ± 44.8 days (range, 2-151 days). Using a 3-mm-wide polyester tape, we tightened the main pulmonary artery to obtain the circumference of (19 mm + 1 mm for each kg of BW). There was no early death but one late death in each group. Postoperative BW continuously increased 1 month after PAB in both groups, although BW was significantly lower in group L than in group NH. Intracardiac repair (ICR) was accomplished in 31 patients (13 in group L and 18 in group NH) at average ages of 1.5 years, while the remaining 5 patients are awaiting ICR. In conclusion, PAB using our formula for the infants even weighing < 2.5 kg has low mortality and is effective as a bridge to ICR.

Total anomalous pulmonary venous drainage complicated by tracheoesophageal fistula.

We provided emergency treatment to a 1-day-old neonate (1600 g) with tracheoesophageal fistula (gross classification, type C) and total anomalous pulmonary venous drainage (infracardiac type) complicated by pulmonary venous obstruction. Emergency surgery was required because the tracheoesophageal fistula would have caused respiratory failure. Here we report the perioperative management techniques we used, including the surgical strategy.

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.

Use of a hand-made balloon-expandable covered stent for native coarctation of the aorta in an adult patient: a report of a first case in Japan.

In western countries, the use of a balloon-expandable covered stent is recommended for the treatment of native coarctation of the aorta (CoA) in adult patients because endovascular bare stents cannot completely prevent complications such as aneurysms or aortic rupture. However, such a product that is appropriate and officially approved is not available in Japan. We developed and used a handmade balloon-expandable covered stent in a 32-year-old patient with native CoA and achieved a good outcome. A Palmaz-Schatz stent (XL 10-series 4010; Johnson & Johnson, Warren, NJ, USA) was covered with an Ube woven-graft (WST series; 18 mm across; Ube Junken Medical, Tokyo, Japan). Because the stent shortens when dilated, one end of the graft was firmly sutured to one end of the stent, whereas the other end of the graft was stitched loosely to the other end of the stent so that it could slide along the struts of the stent to accommodate foreshortening. After meticulous in vitro simulations, the covered stent was implanted with right ventricular overdrive pacing. No complications were observed, and the pressure gradient disappeared. These results indicate that angioplasty using a balloon-expandable covered stent is highly safe and effective for correcting native CoA in adult patients and hopefully in children.

Successful stenting of the ductus venosus in 2 neonates with asplenia syndrome complicated by infracardiac type total anomalous pulmonary venous connection.

In the neonatal period, the surgical mortality of palliation is extremely high for asplenia syndrome complicated by single ventricle combined with total anomalous pulmonary venous connection (TAPVC). Recently, stent implantation for the pulmonary venous drainage route soon after birth has been used instead of surgery to prevent pulmonary venous occlusion and to maintain stable hemodynamics in the neonatal period or in early infancy. Here, we successfully implanted stents in the ductus venosus (DV) in 2 neonates with asplenia syndrome complicated by infracardiac type TAPVC. The first patient was a 3-day-old male neonate with severe cyanosis. Immediately after TAPVC was diagnosed, we implanted a stent in the DV. The second patient was a 0-day-old female neonate. She was diagnosed as TAPVC by fetal echocardiogram. After the scheduled delivery, a stent was successfully implanted. We believe that stent implantation in the DV in the neonatal period is effective and less invasive than surgery in patients with infracardiac type TAPVC.

High prevalence of abnormal glucose metabolism in young adult patients with complex congenital heart disease.

BACKGROUND: Abnormal glucose metabolism (AGM) adversely impacts morbidity and mortality in patients with chronic heart failure. No data on AGM in adult patients with congenital heart disease (ACHD) are available. METHODS: To assess the AGM in ACHD and compare the results with their clinical characteristics, we performed a 75-g oral glucose tolerance test to detect AGM, that is, insulin resistance, impaired glucose tolerance, and diabetes mellitus, in 205 consecutive ACHD (24 +/- 8 years), including 16 unrepaired patients, 67 Fontan patients, 122 postbiventricular (BV) patients, and 27 healthy controls (27 +/- 5 years). RESULTS: All ACHD groups had a high prevalence of AGM (unrepaired, 43.8%; Fontan, 43.3%; BV, 46.7%; control, 3.7%; P < .001). In the 2 postoperative groups, the Matsuda index was decreased (P < .0001), and greater waist circumference, liver dysfunction, higher plasma renin activity, and diuretic use were associated with AGM. Although male gender was associated with AGM (P < .01), baseline glucose and lipid metabolic variables did not correlate with the 75-g oral glucose tolerance test-induced hyperglycemia (area under the plasma glucose curve [AUC-PG]) in the Fontan patients but did correlate in the BV patients. The AUC-PG correlated inversely with exercise capacity (P < .05) in the 2 postoperative ACHD groups, and the AGM ACHD had a high incidence of future cardiac events (P < .05), especially the Fontan patients with diabetes mellitus (P < .01). CONCLUSIONS: Complex ACHD have a high prevalence of AGM, and this newly recognized pathophysiology should be considered in managing long-term survivors of complex ACHD.