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.

Comparison of risk stratification scoring system as a predictor of mortality and morbidity in congenital heart disease patients requiring surgery.

Backgrounds: Risk stratification systems have been important in reducing morbidity and mortality among congenital heart disease (CHD) patients requiring cardiac surgery. Multiple risk stratification scoring systems have been developed, including Aristotle Basic Complexity Score (ABC), Aristotle Comprehensive Complexity Score (ACC), Society of Thoracic Surgeons and European Association for Cardiothoracic Surgery (STS-EACTS), and Risk Adjustment in Congenital Heart Surgery (RACHS-1). This study aims to access the superior risk stratification scoring system model in predicting mortality and morbidity. Methods: The authors used Embase, PubMed, Scopus, and ProQuest as the primary databases for searching and included studies from hand searching. The area under the receiver operating characteristic curve was compared. Results: A total of 11 articles were included in this review. The AUC of ABC for predicting mortality ranges from 0.59 to 0.71, and morbidity ranges from 0.673 to 0.743. The AUC of ACC score for predicting mortality ranges from 0.704 to 0.87, and a study revealed the AUC of morbidity is 0.730. The AUC of RACHS-1 for predicting mortality ranges from 0.68 to 0.782. The AUC of STS-EACTS for predicting mortality ranges from 0.739 to 0.8 and 0.732 for predicting morbidity. Conclusion: ABC, ACC, RACHS-1, and STS-EACTS have acceptable to excellent discriminatory ability in predicting mortality and morbidity among CHD patients requiring cardiac surgery.

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.

Impact of older age at Fontan completion on mid-term survival.

BACKGROUND: The optimum age of Fontan completion remains unknown. Currently, the majority of centers worldwide are performing Fontan completion at 2-4 years of age. In Indonesia, lack of awareness and limited resources probably explain why patients seek treatment at advanced stage. This study aimed to evaluate the impact of older age at Fontan completion on mid-term survival. RESULTS: A single-center retrospective cohort study was performed on 261 patients who underwent Fontan completion between 2008 and 2019 and survived to discharge. The patients were followed up until April 2020, with a median follow-up period of 3 years (range 0-12 years). The median age was 5 years (range 2-24 years). The survival rates of patients with the age at operation ≤ 6 years and > 6 years were 92.1% and 82.8%, respectively. A subgroup analysis showed that the survival rates for age < 4 years, 4-6 years (reference age), 6-8 years, 8-10 years, 10-18 years, and > 18 years were 85.7%, 94.8%, 85.4%, 78.8%, 85.7%, and 66.7%, respectively. Age at Fontan completion of > 6 years (HR 3.84; p = 0.020) was associated with a lower 12-year survival rate. The age at operation of 8-10 years (HR 6.79; p = 0.022) and > 18 years (HR 15.30; p = 0.006) had the worst survival rates. CONCLUSIONS: An older age at Fontan completion (> 6 years) significantly reduced mid-term survival rate. The age at Fontan of 8-10 years and > 18 years had higher risk of mid-term death than age of 4-6 years.

Indonesian Study: Triiodothyronine for Infants Less than 5 Months Undergoing Cardiopulmonary Bypass.

This study evaluates the efficacy and safety of oral triiodothyronine on time to extubation for infants less than 5 months undergoing heart surgery in Indonesia, and primarily relates to patients in emerging programs with high malnutrition and mortality. In this randomized, double-blind, placebo-controlled trial, oral triiodothyronine (T3, Tetronine®) 1 µg/kg-body weight/dose or placebo (saccharum lactis) was administered via nasogastric tube every 6 h for 60 h to treatment group. A total of 120 patients were randomized into T3 (61 patients) and placebo (59 patients) groups. The majority of the patients had moderate to severe malnutrition (55.83%) with a high post-operative mortality rate of 23.3%. The T3 group showed significantly higher serum FT3 levels from 1 until 48 h post cross-clamp removal (p < 0.0001), lower incidence of low cardiac output syndrome at both 6 h (28 [45.9%] vs. 39 [66.1%] patients, p = 0.03, OR 2.3, 95% CI: 1.10-4.81) and 12 h after cross-clamp removal (25 [41.7%] vs. 36 [63.2%], p = 0.02, OR 2.40, 95% CI: 1.14-5.05). Although not statistically significant, the treatment group had shorter median (IQR) intubation time (2.59 [1.25-5.24] vs. 3.77 [1.28-6.64] days, p = 0.16, HR 1.36, 95% CI: 0.88-2.09)] and lower mortality (10 [16.4%] vs. 18 [30.5%], p = 0.07]. Patients with Aristotle score < 10.0 (low risk) receiving T3 had faster extubation than placebo patients (p = 0.021, HR of 1.90, 95% CI: 1.10-3.28) and were significantly less likely to require CPR or experience infection (p = 0.027, OR 8.56, 95% CI:0.99-73.9 and p = 0.022, OR 4.09 95% CI: 1.16-14.4, respectively). Oral T3 supplementation reduced overall incidence of low cardiac output syndrome and significantly reduced the time to extubation in low-risk patients. Therefore, prophylactic oral T3 administration may be beneficial in these patients.Trial Registration: ClinicalTrials.gov NCT02222532.

Thiamine levels in Indonesian children with congenital heart diseases undergoing surgery using cardiopulmonary bypass machine.

BACKGROUND: This study evaluated thiamine levels in Indonesian children with congenital heart diseases before and after cardiopulmonary bypass and their relationship with clinical and surgical outcomes. METHOD: A prospective, single center cross-sectional study was conducted to evaluate thiamine levels in 25 children undergoing congenital heart diseases surgery with cardiopulmonary bypass procedure. Thiamine levels were quantified using a high-performance liquid chromatography method. RESULT: Preoperative thiamine deficiency was observed in one subject. Thiamine levels did not differ statistically between nutritional status and clinical outcomes categories. There were no significant changes in thiamine levels before and after cardiopulmonary bypass (median pre versus post (P25-75): 50 ng/mL (59.00-116.00) and 83.00 ng/mL (70.00-101.00), p = 0.84), although a significant reduction in thiamine levels were observed with longer cardiopulmonary bypass duration (p = 0.017, R = -0.472). CONCLUSION: Thiamine levels were not significantly impacted by cardiac surgery except in patients undergoing extremely long cardiopulmonary bypass duration. However, clinical outcome was not affected by thiamine levels.

Berry syndrome; a successful one-stage repair in neonate periods, evaluation result after 9 years, a case report.

BACKGROUD: IAA with an intact ventricular septum is distinctly unusual. Combination with an Aortopulmonary Window (APW), ascending aortic origin of the right pulmonary artery and PDA may be present which is called as Berry syndrome, a rare combination of cardiac anomalies, reported to be 0.046%, lethal combination and die shortly after birth. CASE REPORT: We report a 9 days-old male neonates weighing 3.85 kg was referred by local hospital to our center and was ventilated with history of respiratory distress and severe infection since he was born. Admitted to our PCICU, 2D echo showed an IAA type A associated with a huge APW type II and restrictif PDA. A PGE1 infusion was started, during the following days the baby experienced several epileptic episodes. After improvement of the clinical condition, surgery was performed on the 20th days of life on year 2011. A successful one-stage repair of such anomalies in which cutting of PDA that arised from PA trunk and distally becoming into descending aorta, extended end to end anastomosis to conduct the ascending aortic blood flow into the descending aorta and intra arterial baffle was used. A 4-0 Gore-Tex baffle was used both to close the APW and separated the RPA from aortic origin with a good result, as his recently grown up as a cheerful 9 year old child who is growing actively and has entered elementary school in grade 2. CONCLUSION: Berry syndrome is a rare but well-identified and surgically correctable anomaly. Early diagnosis and surgical treatment to avoid irreversible pulmonary hypertension is mandatory.

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.

The effect of oral triiodothyronine supplementation on lactate and pyruvate after paediatric cardiac surgery.

OBJECTIVE: To determine if triiodothyronine alters lactate, glucose, and pyruvate metabolism, and if serum pyruvate concentration could serve as a predictor of low cardiac output syndrome in children after cardiopulmonary bypass procedures. METHODS: This study was ancillary to the Oral Triiodothyronine for Infants and Children undergoing Cardiopulmonary bypass (OTICC) trial. Serum pyruvate was measured in the first 48 patients and lactate and glucose were measured in all 208 patients enrolled in the OTICC study on the induction of anaesthesia, 1 and 24 hours post-aortic cross-clamp removal. Patients were also defined as having low cardiac output syndrome according to the OTICC trial protocol. RESULT: Amongst the designated patient population for pyruvate analysis, 22 received placebo, and 26 received triiodothyronine (T3). Lactate concentrations were nearly 20 times greater than pyruvate. Lactate and pyruvate levels were not significantly different between T3 and placebo group. Glucose levels were significantly higher in the placebo group mainly at 24-hour post-cross-clamp removal. Additionally, lactate and glucose levels peaked at 1-hour post-cross-clamp removal in low cardiac output syndrome and non-low cardiac output syndrome patients, but subsequently decreased at a slower rate in low cardiac output syndrome. Lactate and pyruvate concentrations correlated with glucose only prior to surgery. CONCLUSION: Thyroid supplementation does not alter systemic lactate/pyruvate metabolism after cardiopulmonary bypass and reperfusion. Pyruvate levels are not useful for predicting low cardiac output syndrome. Increased blood glucose may be regarded as a response to hypermetabolic stress, seen mostly in patients with low cardiac output syndrome.

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.

Oral Triiodothyronine Supplementation Decreases Low Cardiac Output Syndrome After Pediatric Cardiac Surgery.

The oral triiodothyronine for infants and children undergoing cardiopulmonary bypass (OTICC) trial showed that Triiodothyronine (T3) supplementation improved hemodynamic and clinical outcome parameters. We tested the validity of low cardiac output syndrome (LCOS), derived using clinical parameters and laboratory data, by comparing the LCOS diagnosis with objective parameters commonly measured in a cardiac intensive care unit (CCU) setting. OTICC, a randomized, placebo-controlled trial included children younger than 3 years with an Aristotle score between 6 and 9. We used the existing trial data set to compare the LCOS diagnosis with echocardiographic hemodynamic parameters. Additionally, we determined if LCOS, prospectively assigned during a clinical trial, served as an early predictor of clinical outcomes. All LCOS subjects at 6 and 12 h after cross-clamp release later showed significantly lower pulse pressure, stroke volume and cardiac output, and higher systemic vascular resistance. These LCOS patients also had significantly longer time to extubation (TTE) and higher mortality rate. LCOS incidence was significantly lower in the T3 treatment group [n = 86 vs. 66, respectively, p < 0.001; OR (95% CI) 0.43 (0.36-0.52)] particularly at 6 h. Also, LCOS patients in the placebo group had significantly lower FT3 serum levels over time. These analyses confirm that early clinically defined LCOS successfully predicts cardiac dysfunction determined later by objective hemodynamic echocardiographic parameters. Furthermore, early LCOS significantly impacts TTE and mortality. Finally, the data support prior clinical trial data, showing that oral T3 supplementation decreases early LCOS in concordance with reducing TTE.

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.

Oral Triiodothyronine for Infants and Children Undergoing Cardiopulmonary Bypass.

BACKGROUND: This study evaluated the efficacy and safety of oral triiodothyronine (T3; Tetronine, Dalim BioTech, Korea) for infants and children undergoing cardiopulmonary bypass in an Indonesian population. METHODS: We performed a single-center, randomized, double-blind, and placebo-controlled trial in children aged 3 years and younger undergoing congenital heart disease operations with cardiopulmonary bypass. We administered oral T3 (1 µg/kg per body weight/dose) or placebo (saccharum lactis) by nasogastric tube every 6 hours for 60 hours after induction of anesthesia. The primary end point, time to extubation, was compared with Cox regression. RESULTS: The modified intention-to-treat group included 101 placebo and 104 treated patients. The stratified log-rank test did not show a significant treatment difference (p = 0.061) for time to extubation, but after adjustment for age, the nutritional Z score, and Aristotle surgical complexity, the hazard ratio was 1.33 (95% confidence interval, 1.00 to 1.76; p = 0.049). The effect of T3 was stronger in the strata aged 5 months and younger (hazard ratio, 1.86; 95% confidence interval 1.02 to 3.39; p = 0.043). Median intubation time was 47.3 hours for the placebo and 32.1 hours for the T3 group in aged 5 months and younger. Adverse events rates, including arrhythmia, were similar between groups, although sepsis was more frequent with placebo. CONCLUSIONS: Oral T3 supplementation may shorten time to extubation in children undergoing congenital heart disease operations, particularly infants aged 5 months or younger. Administration is relatively safe, simple and inexpensive.

Genetic polymorphism in postoperative sepsis after open heart surgery in infants.

BACKGROUND: Sepsis is one of the complications following open heart surgery. Toll-like receptor 2 and toll-interacting protein polymorphism influence the immune response after open heart surgery. This study aimed to assess the genetic distribution of toll-like receptor 2 N199N and toll-interacting protein rs5743867 polymorphism in the development of postoperative sepsis. METHODS: A prospective cohort study was conducted in 108 children <1-year old who underwent open heart surgery with a Basic Aristotle score ≥6. Patients with an accompanying congenital anomaly, human immunodeficiency virus infection, or history of previous open heart surgery were excluded. The patients' nutritional status and genetic polymorphism were assessed prior to surgery. The results of genetic polymorphism were obtained through genotyping. Patients' ages on the day of surgery and cardiopulmonary bypass times were recorded. The diagnosis of sepsis was established according to Surviving Sepsis Campaign criteria. RESULTS: Postoperative sepsis was observed in 21% of patients. There were 92.6% patients with toll-like receptor 2 N199N polymorphism and 52.8% with toll-interacting protein rs5743867 polymorphism. CONCLUSIONS: Toll-like receptor 2 N199N polymorphism tends to increase the risk of sepsis (odds ratio = 1.974; 95% confidence interval: 0.23-16.92; p = 0.504), while toll-interacting protein rs5743867 polymorphism tends to decrease the risk of sepsis (odds ratio = 0.496; 95% confidence interval: 0.19-1.27; p = 0.139) in infants <1-year old undergoing complex open heart surgery.

Oral triiodothyronine normalizes triiodothyronine levels after surgery for pediatric congenital heart disease*.

OBJECTIVES: This study was conducted to determine if oral triiodothyronine supplementation could prevent the decrease of serum triiodothyronine levels that commonly occurs after cardiopulmonary bypass for pediatric congenital heart surgery. Secondary objectives included identifying any significant adverse effects of oral triiodothyronine supplementation, including any effects on the thyroid/pituitary axis. DESIGN: Randomized, placebo-controlled, doubleblind clinical trial SETTING: Operating room and ICU. SUBJECTS: Infants and children younger than 2 years of age undergoing congenital heart surgery using cardiopulmonary bypass (n = 43). INTERVENTIONS: Subjects were assigned to placebo (n = 15, group A) or one of two treatment groups: a low-dose group (group B, n = 14, 0.5 mcg/kg triiodothyronine orally every 24 hr for 3 d) or a high-dose group (group C, n = 14, 0.5 mcg/kg triiodothyronine orally every 12 hr for 3 d). MEASUREMENTS AND MAIN RESULTS: Thyroid hormone, including total and free triiodothyronine levels at predetermined time points, potential side effects indicating hyperthyroidism, indicators of the thyroid-pituitary axis, and clinical endpoints. Oral triiodothyronine supplementation twice-daily maintained serum triiodothyronine levels within normal limits in group C, whereas serum levels progressively declined in groups A and B. A statistically significant difference in triiodothyronine levels between the treatment groups occurred between 18 and 36 hours post cross-clamp release, with the largest difference in serum levels between group C and group A noted at 36 hours post cross-clamp release (total triiodothyronine, 0.71 ± 0.15 [0.34-1.08] ng/mL [p < 0.01]; free triiodothyronine, 2.56 ± 0.49 [1.33-3.79] pg/mL [p < 0.01]). There was no evidence of hyperthyroidism or suppression of the pituitary-thyroid axis in either treatment group CONCLUSIONS: Oral triiodothyronine supplementation at a dose of 0.5 mcg/kg every 12 hours for 3 days can maintain total and free triiodothyronine levels within normal limits after open-heart surgery using cardiopulmonary bypass for congenital heart disease.

Adult congenital cardiac surgery in Indonesia.

BACKGROUND: Successful paediatric cardiac surgery and cardiology treatment has resulted in an increase in the use of surgery as a method of treatment of congenital cardiac disease in adult population. However, late detection and lower socio-economic condition in developing countries might change patients' characteristics by the time they come for treatment. This study aimed to elaborate the long-term surgical results of adult congenital cardiac disease in Indonesia as a developing country. METHODS AND RESULTS: We reviewed retrospectively all adult congenital cardiac disease patients with a mean age of 28 years plus or minus 9.5 years, who underwent surgery at National Cardiovascular Center. The types of procedures used were corrective in 338 patients (89.2%), palliative in 10 patients (2.6%), and re-operations in 31 patients (8.2%). The overall hospital mortality rate was 2.6% but as high as 20% with palliative surgery. Post-operative New York Heart Association class III-IV is the only independent predictor of death at 60 months (hazard ratio 61.48, 95% confidence interval 9.41-401.69, p<0.001). The survival rates were 96.3% and 95% for overall and non-atrial septal defect in patients at 60 months, which was highest in corrective procedures (97.6%). The percentage of patients free of re-operation at 5 years' follow-up was 85.4% and 42.7% at 10 years. CONCLUSION: In developing countries, surgical treatment of adult congenital cardiac disease is effective and safe, with an overall survival rate of 96.3% at 60 months. Due to high mortality rate, palliative surgery of a non-atrial septal defect patient is recommended to be discontinued. The independent predictor of mortality was post-operative New York Heart Association functional class III-IV.

Pulmonary artery sling associated with tetralogy of fallot.

We describe a rare case of pulmonary artery sling occurring simultaneously with tetralogy of Fallot. This report describes the successful concomitant repair of both intracardiac anomalies.