A retrograde approach for transcatheter valvotomy procedure in infants with pulmonary atresia intact ventricular septum (PA-IVS): retrograde versus antegrade approach.

INTRODUCTION: This study evaluates the retrograde approach compared to the antegrade approach in infants with PA-IVS who underwent transcatheter pulmonary valvotomy procedure at National Cardiovascular Center Harapan Kita, Jakarta, Indonesia. MATERIAL AND METHOD: This is a single-centre retrospective study conducted from January 2017 to June 2019 consisting of infants undergoing transcatheter pulmonary valvotomy procedures from our centre. RESULTS: Among 3733 records of cardiac catheter procedure in paediatric patients during the last 3 years, there were 12 subjects with PA-IVS, where five subjects were done by antegrade approach and seven by retrograde approach. The retrograde approach is shown to excel the antegrade approach in terms of procedural time by 58.64 minutes (CI 95 % 32.97-84.29, p = 0.008) and PA-RV crossing time by 27 minutes (CI 95 % 14.01-39.99, p = 0.02). There was no significant difference in contrast used (120.23 ± 25.77 versus 150.27 ± 39.26 ml/BSA, p = 0.518), and right ventricle to pulmonary artery systolic pressure gradient after valvotomy (39.571 ± 5.814 versus 53.52 ± 29.15, p = 0.329) between the retrograde and the antegrade approach. CONCLUSION: The retrograde approach offered shorter procedural time and comparably satisfying results than the antegrade approach. The shorter procedural time was preferred due to the shorter duration of general anaesthesia, which may decrease the risk of neurodevelopmental deficits in the patient.

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.

Nitroglycerin inhalation for acute treatment of pulmonary arterial hypertension in children with congenital heart disease.

OBJECTIVES: Acute pulmonary hypertension and pulmonary hypertensive crisis may result in adverse clinical outcomes if unsuccessfully treated. Inhaled nitric oxide has long been considered as the standard pharmacotherapy for acute pulmonary hypertension, but lack of feasibility in some settings and evidences challenging its benefits lead to the use of alternative treatment, amongst which is nitroglycerin inhalation. The purpose of this review article is to discuss available data on the use of nitroglycerin inhalation for acute treatment of pulmonary hypertension in children with CHD and its potential benefit in post-operative setting. DATA SOURCES: Literatures included in this review were acquired by searching in PubMed online database. Keywords used were "Pulmonary Hypertension", "Congenital heart defects", "Pediatrics", "Inhaled nitroglycerin", and its synonyms. STUDY SELECTION: Title and abstract were screened to select relevant literatures including the three paediatric clinical trials on nitroglycerin inhalation. Critical appraisal of the clinical trials was then done using the University of Oxford Centre of Evidence-Based Medicine Critical Appraisal Tools. CONCLUSIONS: Paediatric studies showed the benefit of nitroglycerin inhalation in uncorrected cases of CHD during catheterisation procedures. Until recently, there have been no studies conducted in paediatric post-operative CHD cases. Further study is required to provide evidence for inhaled nitroglycerin use in this setting including the appropriate dosing and potential side effects with repeated administration.

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.

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.

Clinical Profile and Management of Rheumatic Heart Disease in Children and Young Adults at a Tertiary Cardiac Center in Indonesia.

Introduction: Rheumatic heart disease (RHD) remains a major public health issue affecting children and young adults in developing countries. This study aimed to evaluate the clinical characteristics, management, and reactivation of RHD among children and young adults. Patients and Methods: This was a hospital-based retrospective study conducted at the National Cardiovascular Center Harapan Kita, Indonesia; we retrieved relevant data from patients diagnosed with RHD between 2012 and 2018. Results: Two hundred and seventy-nine patients were diagnosed with rheumatic heart disease, of whom 108 were children (mean age of 12.02 ± 3.36 years) and 171 were young adults (mean age was 24.9 ± 3.84). RHD was more common in female than in male young adults (1.5:1). Hospitalization due to RHD complications such as congestive heart failure was seen in 11.11% of cases in children, while pulmonary hypertension was present in 19.95% young adult cases. Reactivation of RHD occurred in 17.2% (48/279) cases, significantly in children (p < 0.001). Overall, the mitral valve (either isolated or combined) was the organ most affected in children (39.13%) and young adults (44.81%). Isolated mitral regurgitation was more common in children (13/21, 61.9%), while isolated mitral stenosis was more common in young adults (19/47, 40.42%). There was a high rate of rheumatic tricuspid valve disease in all populations (193/279, 69.17%) and reported involvement of pulmonary regurgitation (46/279, 16.48%). Multivalve lesions were more common than single lesions in both groups, with a combination of mitral and tricuspid regurgitation the predominant type in children (32/43, 74.41%) and mixed mitral lesion and tricuspid regurgitation in young adults (22/72, 30.56%). We observed a significant occurrence of quadrivalve lesions in children (p = 0.039). Valve repair was more common in children (49.07%) and replacement in young adults (32.16%), with low in-hospital mortality. Compliance with secondary prophylaxis was a significant challenge. Conclusion: Chronic RHD often presented with complications of the disease or reactivation of rheumatic fever (RF). Inadequate treatment of RF/RHD leads to extensive valvular damage and consequent disabilities. Efforts toward active early diagnosis and prompt treatment of RF/RHD and effective preventive measures are essential.

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.

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.