Advocacy at the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery.

The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery (WCPCCS) will be held in Washington DC, USA, from Saturday, 26 August, 2023 to Friday, 1 September, 2023, inclusive. The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery will be the largest and most comprehensive scientific meeting dedicated to paediatric and congenital cardiac care ever held. At the time of the writing of this manuscript, The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery has 5,037 registered attendees (and rising) from 117 countries, a truly diverse and international faculty of over 925 individuals from 89 countries, over 2,000 individual abstracts and poster presenters from 101 countries, and a Best Abstract Competition featuring 153 oral abstracts from 34 countries. For information about the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery, please visit the following website: [www.WCPCCS2023.org]. The purpose of this manuscript is to review the activities related to global health and advocacy that will occur at the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery.Acknowledging the need for urgent change, we wanted to take the opportunity to bring a common voice to the global community and issue the Washington DC WCPCCS Call to Action on Addressing the Global Burden of Pediatric and Congenital Heart Diseases. A copy of this Washington DC WCPCCS Call to Action is provided in the Appendix of this manuscript. This Washington DC WCPCCS Call to Action is an initiative aimed at increasing awareness of the global burden, promoting the development of sustainable care systems, and improving access to high quality and equitable healthcare for children with heart disease as well as adults with congenital heart disease worldwide.

Survey of multinational surgical management practices in tetralogy of Fallot.

BACKGROUND: A wide variety of surgical strategies are used in tetralogy of Fallot repair. We sought to describe the international contemporary practice patterns for surgical management of tetralogy of Fallot. METHODS: Surgeons from 18 international paediatric cardiac surgery centres (representing over 1800 tetralogy of Fallot cases/year) completed a Research Electronic Data Capture-based survey. Participating countries include: China (4), India (2), Nepal (1), Korea (1), Indonesia (1), Saudi Arabia (3), Japan (1), Turkey (1), Australia (1), United States of America (2), and Canada (1). Summary measures were reported as means and counts (percentages). Responses were weighted based on case volume/centre. RESULTS: Primary repair is the prevalent strategy (83%) with variation in age at elective repair (range). Approximately 47% of sites use patient age as a factor in determining the strategy, with age 90% of all trans-annular repairs. CONCLUSIONS: In this cohort representing 11 countries, there is variation in tetralogy of Fallot surgical management with no consensus on standard of practice. A large international prospective cohort study would allow analysis of impact of underlying anatomy and repair strategy on early and late outcomes.

Accidental finding of ALCAPA in a child with severe mitral regurgitation: A case study.

CASE REPORT: A six-year-old child who had undergone cardiac surgery five years ago presented to us with severe mitral valve regurgitation. During her current surgery, the coronary artery was injured during the attempt to release extensive epicardial adhesion, resulting in very poor contractility that prompted a delay in the intended valve repair. The injured coronary vessels were successfully repaired, yet low cardiac output syndrome persisted during perioperative care, necessitating further investigation of the coronary problem. It was later discovered that the patient had anomalous left coronary artery from pulmonary artery (ALCAPA) syndrome. DISCUSSION: The presence of severe mitral regurgitation, pulmonary hypertension, and anatomical factors may have contributed to the delayed presentation and diagnosis in this case. The severity of mitral regurgitation and the most likely underlying mechanism indicates a low possibility of recovery following coronary repair alone, warranting the need for concomitant mitral surgery. Coronary and mitral repair were performed in this patient, resulting in a favorable outcome. CONCLUSIONS: The management of ALCAPA presents unique challenges, especially in cases with delayed diagnosis. Proper diagnosis and tailored surgical approaches are crucial for achieving favorable outcomes in patients with ALCAPA.

Ductal stenting vs. surgical shunting in late presenting duct-dependent pulmonary circulation: a single-center experience.

Introduction: PDA stenting is an option to mBTT shunt for younger patients; nevertheless, few reports of this palliative approach have been made for the late presenter population, especially for patients who are older than 30 days but under 5 years. This study aimed to evaluate the clinical result and intra-hospital costs of ductal stenting in late-presenting patients in comparison to surgical shunting. Methods: A single-center, retrospective cohort study was conducted from August 2016 to August 2022. This study included patients with pulmonary duct dependent CHD who were hospitalized for palliative therapy. The extracted data were baseline characteristics, clinical findings, supportive examination findings, complications, outcomes, and length of stay of the patients. Monitoring was carried out during treatment up to 30 days after the procedure. Results: A total of 143 patients were included in the analysis; 43 patients underwent PDA stent and 100 patients underwent mBTT shunt with median age of PDA stent group 110 (31-1,498) days and mBTT shunt group 174.5 (30-1,651) days. Primary outcome composite was not significant in both groups including 30 days mortality [6 (14%) vs. 14 (14%), p = 1.000], reintervention [1 (2.3%) vs. 7 (7%), p = 0.436], and 30 days rehospitalization [0 (0%) vs. 2 (2%), p = 0.319]. Secondary outcome analysis showed shorter ICU length of stay in the PDA stent group [2 (0-16) days vs. 4 (1-63) days, p = 0.002]. Conclusions: PDA stent has an outcome that is non inferior from the mBTT shunt procedure in the composite outcome including 30 days mortality, reintervention, and 30 days rehospitalization but significantly lower in ICU length of stay.

Posterior Annulus Elevation Technique in Reducing Residual Regurgitation During Mitral Valve Repair in Children: A Randomized Controlled Trial.

Background: The current technique used in severe mitral regurgitation in children can occasionally lead to residual regurgitation. To address this issue, the posterior annulus elevation technique was developed to enhance coaptation and reduce residual lesions. This study aims to evaluate the effectiveness of this technique in reducing residual regurgitation during mitral valve repair in children. Methods: A total of 64 patients aged <18 years old undergoing mitral valve repair were randomized into two groups: the intervention (with posterior annulus elevation) group and the control group, which underwent conventional repair techniques. Various parameters, including coaptation area, residual mitral regurgitation, clinical outcomes, metabolic, and hemolytic markers, were measured on days 0, 5, 2 weeks, and 3 months after surgery. Results: The intervention group (n = 32) showed a significant reduction in residual mitral regurgitation compared with the control group (n = 32) on each evaluation. At three months after surgery, we found that the posterior annulus elevation technique could be a protective factor that reduces the chance of residual regurgitation compared with the control group (RR = 0.31; confidence interval: 0.18-0.54; P ≤ .001). Coaptation length and index were also found to be significantly higher in the intervention group (P ≤ .001). Clinical outcomes, metabolic markers, and hemolysis marker did not show any significant differences between the two groups. Conclusions: The posterior annulus elevation technique demonstrated effectiveness in reducing residual mitral regurgitation and improving coaptation area in pediatric mitral valve repair. This technique showed better short-term surgical outcomes in children with mitral regurgitation compared with the conventional technique.

Retrospective study of preoperative sildenafil in prolonged pleural effusion after Fontan.

Background: By rerouting the systemic venous return into the pulmonary circulation, the Fontan operation is perhaps the most effective palliative procedure in patients with complex congenital heart disease. The Fontan, however, still comes with several significant complications and morbidities, one of them being postoperative prolonged pleural effusion. In our center from 2017 to 2021, 10% of all our patients who underwent Fontan operation developed prolonged pleural effusion. With this study, we aimed to analyze the preoperative use of sildenafil for 6 months and its role in prolonged pleural effusion after the Fontan operation. Materials and Methods: In total, 158 patients were included in the analysis. The cohort was divided into patients who developed prolonged pleural effusion after Fontan surgery (n = 17) and those who did not (n = 141). We analyzed several risk factors and the history of sildenafil use in each group in the cohort. We found that patients who used sildenafil for at least 6 months prior to Fontan surgery versus those who did not use (n = 112 vs. n = 46) were found to be at lower risk of developing prolonged pleural effusion (odds ratio [OR]: 0.32 [confidence interval 95%: 0.11-0.88], P = 0.027). Conclusion: Preoperative administration of sildenafil for at least 6 months before Fontan might reduce the risk of postoperative prolonged pleural effusion.

Correlation Between Adolescent Mental Health and Sleep Quality: A Study in Indonesian Rural Areas During the COVID-19 Pandemic.

Objective: The study aims to analyze the correlation between sleep quality and adolescent mental health in rural areas during the Coronavirus Disease 2019 (COVID-19) pandemic. Material and Methods: A cross-sectional study was conducted in Bandung rural area in February 2023. The subjects were adolescents aged 12-15 years who attended high school in Sagaracipta Village. To assess mental health, the Strength and Difficulties Questionnaire (SDQ) was used. Meanwhile, sleep quality was assessed using the Pittsburgh Sleep Quality Index (PSQI) questionnaire. The questionnaires were distributed and then filled in directly by the participants under close supervision. The relationship between mental problems and sleep quality was analyzed with the Spearman Rank correlation. The confidence range employed was 95%, while p< 0.05 was considered statistically significant. Results: The results showed that at two junior high schools in Sagaracipta Village, 70.6% of the 109 subjects had poor sleep quality. Among the subjects, a significant number exhibited abnormal scores on various subscales. Specifically, 24 subjects (22%) showed abnormalities in the emotional subscales, 18 (16.5%) in conduct, 5 (4.6%) in hyperactivity, 15 (13.8%) in problems with peers, 26 (23.9%) in total difficulty, but there were no abnormal subjects on the pro-social sunscale. Based on the Rank Spearman correlation test, there was a statistically significant correlation between sleep quality and the emotional, behavioral, hyperactivity, and overall difficulties subscales, with coefficients of 0.247; 0.258; 0.22; and 0.310 as well as p-values of 0.010; 0.007; 0.021; and 0.001 respectively. Conclusion: During the COVID-19 pandemic, there was a correlation between mental health and sleep quality among adolescents in rural area.

Operability of atrial septal defect with borderline pulmonary vascular resistance index: A study in developing country.

Background: Pulmonary arterial hypertension secondary to atrial septal defect (ASD) is an important determinant of morbidity and mortality in defect closure. We aimed to compare perioperative outcome between preoperative borderline and low pulmonary vascular resistance index (≥4 WU.m2 and <4 WU.m2, respectively) in surgical closure of secundum atrial septal defect with concomitant pulmonary arterial hypertension. Methods and results: This was a single-center retrospective cohort study between January 2015 and January 2020. We classified patients with low and borderline PVRI who underwent ASD closure and recorded the perioperative outcomes. Results: We analyzed a total of 183 patients with atrial septal defect and pulmonary arterial hypertension; 92 patients with borderline PVRI and 91 patients with low PVRI. Borderline pulmonary vascular resistance index was not associated with increased risk of postoperative mortality (p = 0.621; OR0.48, 95% CI 0.04-5.48), but associated with higher risk of overall morbidity in bivariate analysis (p = 0.002; OR3.28, 95% CI 1.5-6.72). Multivariate analysis showed positive association of borderline pulmonary vascular resistance index (p = 0.045; OR2.63, 95% CI 1.02-6.77) and preoperative tricuspid valve gradient ≥64 mmHg (p = 0.034; OR2.77, 95% CI 1.08-7.13) with overall morbidity. Conclusion: There is no difference in incidence of in-hospital mortality between preoperative borderline and low pulmonary vascular resistance index patients. However, preoperative borderline pulmonary vascular resistance index and tricuspid valve gradient ≥64 mmHg are associated with increased overall morbidity after surgical closure in secundum atrial septal defect patients with pulmonary arterial hypertension.

Transcatheter Closure of Perimembranous Ventricular Septal Defect Using the Lifetech Konar-Multi Functional Occluder: Early to Midterm Results of the Indonesian Multicenter Study.

Background: The alternative device to close perimembranous ventricular septal defect (pmVSD) has been searched for better result, less complications and applicable for infants. However, the ideal device is still unavailable. We aimed to evaluate the effectiveness and outcome of transcatheter pmVSD closure using the KONAR-multi functional occluder (MFO). Methods: Clinical, procedural, follow-up data of pmVSD patients with symptom of heart failure or evidence of significant left to right shunt, growth failure, recurrent respiratory tract infection, and history of endocarditis who underwent transcatheter closure using the MFO were prospectively evaluated. Results: Between January 2016 and December 2017, there were complete records of 132 pmVSD children closed using MFO from eleven centers in Indonesia. The median of age was 4.5 (0.3-17.4) years; weight 14.8 (3.5-57) kg, defect size at the smallest part 3.4 (1.0-8.1) mm, flow ratio 1.6 (1.3-4.9), mean pulmonary artery pressure 18 (7-79) mmHg, fluoroscopy time 18 (3.8-91) and procedural time 75 (26-290) minutes. A retrograde approach was done in 41 (31%) patients. Procedures succeeded in first attempt in 126 (95.4%), failed in three and migration in three patients. Six of eight infants with congestive heart failure were closed successfully. Of 126 patients with successful VSD closure, 12 months follow-up were completed in all patients. The rate of complete occlusion at 1 month, 3 months, 6 months and 12 months after intervention were 95.2%, 97.6%, 99.2%, and 99.2%, respectively. New-onset aortic regurgitation and moderate tricuspid regurgitation developed only in five and three patients. Neither complete atrioventricular block, nor other complications occurred. Conclusion: Transcatheter closure of pmVSD using the MFO is safe, effective, and feasible in infants and children.

Preoperative Left Ventricle End Diastolic Volume Index as a Predictor for Low Cardiac Output Syndrome After Surgical Closure of Secundum Atrial Septal Defect With Small-Sized Left Ventricle.

Introduction: Low cardiac output syndrome is one of the postoperative complications that are associated with significant morbidity and mortality after surgical closure of atrial septal defect (ASD) with small-sized left ventricle (LV). This study investigated whether preoperative left ventricular end-diastolic volume index (LVEDVi) could accurately predict low cardiac output syndrome (LCOS) after surgical closure of ASD with small-sized LV. Method: This retrospective cohort study involved adult ASD patients with small-sized LV from January 2018 to December 2019 in National Cardiovascular Center Harapan Kita. Preoperative MRI data to assess the left and right ventricle volume were collected. A bivariate analysis using independent Student's t-test was done. Diagnostic test using receiver operating characteristic (ROC) curve was also done to obtain the area under the curve (AUC) value. The best cutoff point was determined by Youden's index. Result: Fifty-seven subjects were involved in this study [age (mean ± SD) 32.56 ± 13.15 years; weight (mean ± SD) 48.82 ± 12.15 kg]. Subjects who had post-operative LCOS (n = 30) have significantly lower LVEDVi (45.0 ± 7.42 ml/m2 vs. 64.15 ± 13.37 ml/m2; p < 0.001), LVEDV (64.6 ± 16.0 ml vs. 85.9 ± 20.7 ml; p < 0.001), LVSV (38.97 ± 11.5 ml vs. 53.13 ± 7.5 ml; p < 0.001), and LVSVi (27.28 ± 8.55 ml/m2 vs. 37.42 ± 5.35 ml/m2; p < 0.001) compared to subjects who did not have post-operative LCOS (n = 27). ROC analysis showed that the best AUC was found on LVEDVi (AUC 95.3%; 95% confidence interval: 90.6-100%). The best cutoff value for LVEDVi to predict the occurrence of LCOS after surgical closure of ASD was 53.3 ml/m2 with a sensitivity of 86.7% and a specificity of 85.2%. Conclusion: This study showed that preoperative LVEDVi could predict LCOS after surgical closure of ASD with small-sized LV with a well-defined cutoff. The best cutoff value of LVEDVi to predict the occurrence of LCOS after surgical ASD closure was 53.5 ml/m2.

Extremely late presentation of children with transposition the great arteries.

We present three cases of primary arterial switch operation for extremely late presenting transposition the great arteries with intact ventricular septum: a 7-year-old female, 3-year-old male, and 6-year-old female. Two patients were discharged on postoperative day 9 and 11, the other developed hemodynamic instability 12 h after surgery and died due to left ventricular failure and pulmonary hypertension. Left ventricular mass index >35 g·m-2 and left ventricular posterior wall >4 mm are criteria for a primary arterial switch operation. Circulatory support post- or intraoperatively might provide better results. Two patients survived without extracorporeal membrane oxygenation or nitric oxide.

Current Status of Training and Certification for Congenital Heart Surgery Around the World: Proceedings of the Meetings of the Global Council on Education for Congenital Heart Surgery of the World Society for Pediatric and Congenital Heart Surgery.

The optimal training of the highly specialized congenital heart surgeon is a long and complex process, which is a significant challenge in most parts of the world. The World Society for Pediatric and Congenital Heart Surgery (WSPCHS) has established the Global Council on Education for Congenital Heart Surgery as a nonprofit organization with the goal of assessing current training and certification and ultimately establishing standardized criteria for the training, evaluation, and certification of congenital heart surgeons around the world. The Global Council and the WSPCHS have reviewed the present status of training and certification for congenital cardiac surgery around the world. There is currently lack of consensus and standardized criteria for training in congenital heart surgery, with significant disparity between continents and countries. This represents significant obstacles to international job mobility of competent congenital heart surgeons and to the efforts to improve the quality of care for patients with Congenital Heart Disease worldwide. The purpose of this article is to summarize and document the present state of training and certification in congenital heart surgery around the world.

Prevalence and predictors of postoperative peritoneal dialysis in infants.

BACKGROUND: Data regarding predictors of the eventual need for postoperative peritoneal dialysis in infants undergoing open heart surgery is still limited. We aimed to determine whether prolonged cardiopulmonary bypass time, surgical complexity classified according to Risk Adjustment for Congenital Heart Surgery category, younger age, and lower body weight increase the probability of requiring postoperative peritoneal dialysis. METHODS: We retrospectively analyzed data of 181 infants who underwent open heart surgery at our institution from January 1 to December 31, 2018. Cardiopulmonary bypass time, Risk Adjustment for Congenital Heart Surgery category, age, body weight, and the need for postoperative peritoneal dialysis were recorded and analyzed. RESULTS: Thirteen (7.2%) of the 181 patients required postoperative peritoneal dialysis. This group was found to have a longer cardiopulmonary bypass time, younger age, and lower body weight. Longer cardiopulmonary bypass time (p = 0.001), higher Risk Adjustment for Congenital Heart Surgery category (p = 0.018), younger age (p < 0.001), and lower body weight (p < 0.001) significantly increased the risk of postoperative peritoneal dialysis. CONCLUSION: Longer cardiopulmonary bypass time, more complex surgery, younger age, and lower body weight increase the probability of requiring postoperative peritoneal dialysis in infants undergoing open heart surgery.

Correlation between CD4 count and glomerular filtration rate or urine protein:creatinine ratio in human immunodeficiency virus-infected children.

BACKGROUND: Studies on kidney complications in human immunodeficiency virus (HIV)-infected children are lacking. CD4 T lymphocytes are an important immune functions regulator and used as a basis for initiating antiretroviral therapy (ART) and monitoring disease progression. This study aims to determine the correlation between CD4 and estimated glomerular filtration rate (eGFR) or urine protein:creatinine ratio (uPCR) as markers of kidney complications. METHODS: This cross sectional study was conducted on HIV-infected children aged 5 to 18 years who visited the Teratai HIV Clinic at Hasan Sadikin Hospital for monthly monitoring in June 2019. CD4 count, eGFR based on the Schwartz formula, and uPCR were obtained. Correlation analysis was performed with the Pearson test. RESULTS: Subjects were 42 HIV-infected children, consisting of 23 males (54.8%) and 19 females (45.2%). Most children (65.0%) were in an advanced clinical stage and had been diagnosed with HIV for an average of 8 ± 3 years. All subjects had received ART, and six received tenofovir. Compliance to medications were good, and most subjects (79.0%) had normal nutritional status and CD4 count. All subjects had eGFR > 90 mL/min/1.73 m2, of which 21 (50.0%) were above normal value. Proteinuria was found in 12 patients (28.6%), and it was not significantly associated with clinical stages of HIV infection. CD4 count was correlated positively with eGFR (r = 0.473, P = 0.001) and negatively with uPCR (r = -0.284, P = 0.034). CONCLUSION: The degree of immunodeficiency appears to correlate with severity of renal injury. Screening at diagnosis and periodic monitoring of kidney functions are crucial in all childhood HIV patients.

Comparison between blood and non-blood cardioplegia in tetralogy of Fallot.

BACKGROUND: Cardioplegia is an integral part of myocardial protection. The superiority of blood cardioplegia in adult patients has been reported. However, this is yet to be studied in cyanotic pediatric patients. METHODS: A randomized open-label trial was conducted in 70 patients with tetralogy of Fallot. They were divided into two groups: 35 patients had crystalloid cardioplegia (controls), and 35 had blood cardioplegia. Lactate and coronary oxygen extraction in arterial blood and the coronary sinus were measured immediately after cessation of cardiopulmonary bypass, 15 and 30 min later. Postoperative mortality, major adverse cardiac events, mechanical ventilation time, inotrope administration, arrhythmias, right ventricular function, intensive care unit and hospital length of stay were observed. RESULTS: There were no significant differences in clinical outcomes or lactate levels. There was a significant difference in coronary oxygen extraction immediately and 15 min after cessation of cardiopulmonary bypass ( p = 0.038, p = 0.015). CONCLUSION: Blood cardioplegia gave a better postoperative oxygen extraction value but there were no differences in myocardial damage or clinical outcome between the two groups.

Systemic ventricle morphology impact on ten-year survival after Fontan surgery.

BACKGROUND: The Fontan procedure has been applied in many patients with single-ventricle physiology, with quite low mortality rates all over the world, and a 8.6%-9.1% rate in Indonesia. Structural and characteristic differences in the morphologically left and right ventricles influence the role of the systemic ventricle in the functionally univentricular heart and impact on postoperative outcomes. Mid- and long-term survival based on systemic ventricle morphology remains controversial. This study aimed to investigate the impact of systemic ventricle morphology on ten-year survival after the Fontan procedure. METHODS: The 162 patients who underwent a Fontan operation at our institution between 2008 and February 2018 and survived to discharge were reviewed and followed up until March 2018. Data were extracted from the registry and pediatric surgical conferences, medical records, surgery reports, echocardiography and catheterization reports, and follow-up to the end of the study period. Median follow-up was 26.5 months (range 10.75-54 months). RESULTS: The patients were divided into 2 groups based on systemic ventricle morphology. Seventy-four patients were included in the left morphology group and 88 in the right morphology group. There was no difference in 10-year survival rates between the two type of systemic ventricle morphology. Postoperative thromboembolic events influenced the 10-year survival rate after the Fontan procedure (hazard ratio 4.84, 95% confidence interval: 1.26-18.55, p = 0.021). CONCLUSION: Systemic ventricle morphology was not associated with the 10-year survival rate after the Fontan procedure. Postoperative thromboembolic events accounted for a 4.84-times higher mortality risk after the Fontan procedure.