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Objective: The aim of this study was to assess the short- and long-term outcomes of patients who underwent the arterial switch operation (ASO) at Siriraj Hospital in Thailand, and to identify postoperative complications and factors that significantly affect patient survival., Materials and Methods: We retrospectively studied all patients with dextro-transposition of the great arteries and anatomic variants who underwent the ASO from January 1995 to December 2020. Twenty-year overall survival and 15-year freedom from reoperation/reintervention were estimated using the Kaplan-Meier method. Univariate and multivariate Cox regression analyses were used to identify factors independently associated with 30-day mortality., Results: Of the 171 patients included, there were 116 males (67.84%). The median (minimum, maximum) age at operation was 33.5 (3-4,499) days, and the median (minimum, maximum) bodyweight was 3.4 (2.2-44.9) kg. Most patients (115/171, 67.25%) had an intact ventricular septum, whereas 48/171 (28.07%), had ventricular septal defects. The typical coronary anatomic pattern (1LCx-2R) was found in 120 patients (70.1%). Early mortality was 8.7% ± 4.4%, and there was no late death up to 20 years postoperatively with a median follow-up of five years. Fifteen-year freedom from reoperation or reintervention was 85.7%. Atypical coronary pattern was an independent factor for survival with an adjusted hazard ratio of 5.1 (95% confidence interval: 1.22-21.4; P  = .026)., Conclusions: The results of this study revealed excellent short- and long-term outcomes of the ASO at our center. Atypical coronary anatomy was found to be the only factor that independently predicted 30-day mortality., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Pulmonary arterial hypertension (PAH) associated with congenital heart disease (PAH-CHD) is a consequence of unrepaired large systemic-to-pulmonary shunts. The long-term data of adult patients who have PAH-CHD with elevated pulmonary vascular resistance (PVR) are limited. We aimed to investigate the survival of adults who had PAH-CHD with predominantly left-to-right (L-R) shunts with (1) borderline-to-high PVR and (2) treat-and-repair compared with those with Eisenmenger syndrome (ES). From 1995 to 2021, 99 adults with ES (age 34.1 ± 11.2 years) and 118 adults with PAH-CHD with predominantly L-R shunts (age 39.1 ± 13.7 years) were eligible. The PVR in the ES group was 21.0 ± 13.1 WU. Most ES patients (97%) received pulmonary vasodilator therapy. Among the 118 patients with PAH-CHD with predominantly L-R shunts, the baseline PVR was 7.6 ± 4.6 WU, and 78 patients (66.1%) had borderline to high PVR (≥ 5 WU). In the group, 105 patients (88.9%) underwent repair; 84 had defect closure, and 21 had fenestrated closure. Treat-and-repair was used to treat 53 patients with a preoperative final PVR of 3.58 ± 2.63 WU. No early postoperative deaths were reported. At a median follow-up time of 5.4 years (range 0.1-23.6 years), the 10- and 15-year survival rates of adults with borderline PVR were 82.3% and 82.3%, respectively, which were not inferior to the rates for patients with ES, which were 77.8% and 71.2%, respectively (p = 0.41). The survival rate of patients who underwent treat-and-repair was slightly better than that of patients who underwent ES, although the difference was not statistically significant (p = 0.19). Independent mortality risk factors were functional class III-IV at initial presentation (hazard ratio 5.7, 95% CI 1.2-26.6; p = 0.02) and oxygen saturation < 94% at the most recent visit (hazard ratio 9.4, 95% CI 2.1-42.9; p = 0.004).Trial registration: TCTR20200420004., Competing Interests: Declarations. Competing Interests: The authors declare that they have no competing interests., (© 2024. The Author(s).)

Objectives: To determine the prevalence of sensorineural hearing loss (SNHL) in children who underwent congenital cardiac surgery (CCS) by using a pre- and postoperative hearing test, a conventional audiometry, an extended high-frequency audiometry (HFA) or auditory steady-state response (ASSR), and distortion-product otoacoustic emissions (DPOAE)., Study Design: This prospective study enrolled children with CCS in Siriraj Hospital, Thailand, between 2019 and 2023. Conventional audiometry including HFA or ASSR and DPOAE were performed pre- and postoperatively. The patients with bilateral abnormal hearing loss or an incomplete examination were excluded. Collected data included: demographics, cardiac surgery and ototoxic medication. Prevalence of SNHL by conventional audiometry and subclinical hearing impairment by HFA or DPOAE were ascertained, and risks were analysed., Results: Ninety-eight patients were eligible for the study. The median age (IQR) was 5.3 (1.5-9.6) years. Fifteen patients (15.3%) had univentricular hearts. The preoperative audiologic test was performed 1 day prior to the CCS. The postoperative test was performed at a median of 4.4 (IQR: 2.7-8.0) months postoperatively. Preoperative unilateral hearing impairments were reported in 17 patients (17.3%). Postoperatively, four patients (4.1%) showed significantly abnormal audiogram (> 25 dB) or 15 dB shift at 250-8000 Hz consistent to a new SNHL. Subclinical hearing impairment by HFA was affected in 10 patients (10.2%). Thirty-three patients (33.6%) had abnormal DPOAE exclusively. Therefore, new SNHL, including subclinical hearing loss, revealed a prevalence of ototoxicity up to 47.9%. Age <1 year at surgery was the independent risk of postoperative SNHL (adjusted OR 18.5, 95% CI 1.2 to 293.8, p=0.04)., Conclusion: Routine post-CCS audiological surveillance especially CCS in infancy is recommended for early recognition and timely management based on the 43.8% subclinical and the 4.1% SNHL that was found in this study., Trial Registration Number: TCTR20200421001., Competing Interests: Competing interests: No, there are no competing interests., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: The management of atrial isomerism across various countries may impact survival outcomes. Methods: This retrospective study involved patients diagnosed with atrial isomerism undergoing single ventricular palliation between 2000 and 2021. The objective was to evaluate survival outcomes within the right atrial isomerism (RAI) and left atrial isomerism (LAI) groups, as well as overall survival. Results: Of the 125 patients diagnosed with atrial isomerism, 105 (84%) had RAI, and 20 (16%) had LAI. The median age at presentation was 3 days (range: birth to 7.1 years), with median follow-up of 6.6 years (range: 0.59 months to 30.8 years). In the overall cohort, survival rates at one, five, and ten years were 85.6%, 72.3%, and 66.8%, respectively, with no statistically significant difference between RAI and LAI groups (log rank P value = .293). Specifically, survival rates in the RAI group at one, five, and ten years were 83.8%, 69.0%, and 62.6%, respectively, while in the LAI group, there were 95.0%, 89.7%, and 81.6%, respectively. Following the third-stage operation, overall survival rates at one and five years were 76.4% and 76.4%, respectively. Among patients in the RAI group, survival rates at one and five years post-third stage operation were 80.2% and 80.2%, respectively. Conclusions: Despite resource limitations, the survival outcomes of patients with atrial isomerism were found to be similar with those observed in high-income countries. Although an increased mortality rate was noted within the first year of life, focusing efforts on neonatal and infant care holds potential for improving overall outcomes., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Pulmonary arterial hypertension (PAH) associated with congenital heart disease (PAH-CHD) is a complication that occurs after unrepaired significant systemic-to-pulmonary shunt. Treatment options for PAH-CHD-predominantly left-to-right (L-R) shunt in children with borderline-high pulmonary vascular resistant index (PVRi) have been debated. We aimed to assess the treatment and survival of children with PAH-CHD-predominantly L-R shunt with borderline to high PVRi, using Eisenmenger syndrome (ES) for comparison. In 1995-2021, a total of 142 patients with ES and 192 children with PAH-CHD-predominantly L-R shunt were eligible for our analysis. The PVRi in ES patients was 26.7 ± 16.8 WU m 2 . Most patients (91%) received PAH-targeted therapy. Of the 192 children with PAH-CHD-predominantly L-R shunt, the baseline PVRi was 9.2 ± 5.8 WU m 2 . A total of 64 patients (33.3%) had borderline PVRi (4-8 WU m 2 ) and 98 patients (51%) had high PVRi (> 8 WU m 2 ). Most patients (88.5%) responded to acute pulmonary vasodilatory testing and underwent repair, with 158 undergoing defect closure and 12 having fenestrated closure. A treat-and-repair strategy was used in 33 children (17.1%). The 10- and 15-year survival rates for patients with ES were 79.3% and 72.4%, respectively, which was significantly inferior to children with borderline PVRi [97.3% and 87.8% (p = 0.02)]; and high PVRi [91.6% and 89.5% (p = 0.06)], respectively. The survival rate of children receiving treat-and-repair was slightly higher than that of ES (p = 0.16). The independent mortality risk in children with PAH-CHD-predominantly L-R shunt was persistent PAH following the defect correction (adjusted hazard ratio 5.8, 95% CI 1.7-19.9, p = 0.005).Trial registration: TCTR20200420004., (© 2024. The Author(s).)

Background: Use of the expanded polytetrafluoroethylene (ePTFE) valved conduit in the pulmonary position during the correction of congenital heart defects has significantly increased in popularity over the last decade due to its promising conduit longevity. We describe the standardized process to create and implant a trileaflet ePTFE pulmonary conduit along with the early outcomes of such procedures at our institute., Methods: Records of 100 consecutive patients who underwent ePTFE valved conduit placement using our technique from April 2018 through February 2022 were retrospectively analyzed. The function of the conduit was evaluated by transthoracic echocardiography., Results: The mean age of the patients was 28 ± 13.2 years old at the time of the operation. The conduit diameters ranged from 16 to 24 mm (mean 23.0 ± 1.9 mm). Conduit placement was utilized for pulmonary valve replacement in 68 patients, conduit change in 25 patients, and as a part of total repair in 7 patients. There were 2 in-hospital conduit-unrelated deaths from multi-organ dysfunction and pulmonary hypertensive crisis. From the postoperative echocardiography, the average peak pressure gradient across the conduit was 18.6 ± 9.0 mm Hg, and the conduit regurgitation was graded as none or trace in 81 patients, mild in 17 patients, and moderate in 2 patients. At 589 days of median follow-up, there was no conduit reoperation. Follow-up imaging of 60 available patients at a median time of 511 days did not show a significant change in conduit function., Conclusions: Our standardized ePTFE valved conduit creation and placement demonstrated satisfactory clinical and echocardiographic outcomes.

Background: Postoperative infection contributes to the worsening of congenital cardiac surgery (CCS) outcomes. Surgical site infection (SSI), bloodstream infection (BSI) and ventilator associated pneumonia (VAP) are common. An additional bundle of preventive measures against central-line associated bloodstream infection (CLABSI) bundle was implemented in April 2019., Objectives: To compare the incidence of major infections after pediatric CCS before and after the implementation of the CLABSI bundle and to identify risk factors for major infections., Methods: We conducted a single-center, retrospective study to assess the incidence of major infections including bloodstream infection (BSI), surgical site infection (SSI), and ventilator-associated pneumonia (VAP) after pediatric CCS one year before and after implementation of the CLABSI bundle during April 2018-March 2020. The demographics and outcomes of the patients were explored, and risk factors for major infections were identified using multivariate analysis., Results: A total of 548 children (53% male) underwent CCS with a median age of 1.9 years (range 0.01-17.5 years). The median Aristotle Basic Complexity score was 7.1 (range 3-14.5). The CLABSI bundle was applied in 262 patients. Overall mortality was 5.5%. 126 patients (23%) experienced major postoperative infections. During the year after the implementation of the CLABSI bundle, BSI was reduced from 8.4% to 3.1% ( p  = 0.01), with a smaller reduction in VAP (21% to 17.6%; p  = 0.33). The incidence of SSI was unchanged (1.7% to 1.9%; p  = 0.77). The independent risk factors for major infections were age at surgery <6 months ( p  = 0.04), postoperative ventilator usage >2 days ( p  < 0.01), central line usage >4 days ( p  = 0.04), and surgery during the pre-CLABSI bundle period ( p  = 0.01)., Conclusion: Following the implementation of the CLABSI prevention package in our pediatric CCS unit, the incidence of BSI was significantly reduced. The incidence of VAP tended to decrease, while the SSI was unchanged. Sustainability of the prevention package through nurse empowerment and compliance audits is an ongoing challenge., Competing Interests: The authors declare there are no competing interests., (©2022 Vachirapuranon et al.)

Background: Transcatheter pulmonary valve replacement (TPVR) has become an alternative to surgical pulmonary valve placement (SPVR) for patients after tetralogy of Fallot repair. This study compared the outcomes of TPVR with those of SPVR., Methods: We reviewed data from patients who underwent pulmonary valve replacement with a median of 2 years of follow-up., Results: Between 2010 and 2021, 215 patients underwent pulmonary valve replacement (72 TPVR and 143 SPVR). The median size of the right ventricular end-diastolic volume index in the TPVR group was 165 mL/m 2 (IQR, 136-190) and 184 mL/m 2 (IQR, 163-230) in the SPVR group ( P = .001). The median value of the maximum landing zone at the right ventricular outflow tract (RVOT) in patients with native RVOT was 26 mm (IQR, 24-28) in the 43 patients in the TPVR group and 31 mm (IQR, 28-34) in the 101 patients in the SPVR group ( P < .001). The median size of the pulmonary valve implant for the native RVOT in the TPVR group was 29.0 mm (IQR, 26.0-29.0) and 24.0 mm (IQR, 24.0-24.0) in the SPVR group ( P < .001). There were no deaths in the TPVR group and 8 deaths in the SPVR group ( P = .041). Major complications and the length of hospitalization were lower in the TPVR group ( P = .001). After 2 years, the mean decrease in QRS duration was 5 milliseconds (IQR, 1-14) in the TPVR group and 1 millisecond (IQR, -4 to 10) in the SPVR group ( P = .006)., Conclusions: TPVR allows for larger implants, resulting in lower mortality, shorter hospital stays, and fewer major cardiac events. SPVR may be preferable in patients with larger (>30 mm) native RVOT and in those who require concomitant surgical procedures., (© 2022 The Author(s).)

PHACE syndrome is a rare neurocutaneous syndrome that describes the association of large segmental infantile haemangioma involving the head and neck, along with other systemic anomalies. Complex congenital heart disease has rarely been reported in this syndrome. We present a report of a patient with PHACE syndrome and truncus arteriosus., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.)