An electrocardiographic score to predict pulmonary hypertension in children with atrial septal defect.

BACKGROUND: In limited resource settings, identification of factors that predict the occurrence of pulmonary hypertension(PH) in children with atrial septal defect(ASD) is important to decide which patients should be prioritized for defect closure to prevent complication. Echocardiography and cardiac catheterization are not widely available in such settings. No scoring system has been proposed to predict PH among children with ASD. We aimed to develop a PH prediction score using electrocardiography parameters for children with ASD in Indonesia. METHODS: A cross-sectional study reviewing medical record including ECG record was conducted among all children with newly diagnosed isolated ASD admitted to Dr Sardjito Hospital in Yogyakarta, Indonesia during 2016-2018. Diagnosis of ASD and PH was confirmed through echocardiography and/or cardiac catheterization. Spiegelhalter Knill-Jones approach was used to develop PH prediction score. Accuracy of prediction score was performed using a receiver operating characteristic (ROC) curve. RESULTS: Of 144 children, 50(34.7%) had PH. Predictors of pulmonary hypertension were QRS axis ≥120°, P wave ≥ 3 mm at lead II, R without S at V1, Q wave at V1, right bundle branch block (RBBB), R wave at V1, V2 or aVR > normal limit and S wave at V6 or lead I > normal limit. ROC curve from prediction scores yielded an area under the curve (AUC) 0.908(95% CI 0.85-0.96). Using the cut-off value 3.5, this PH prediction score had sensitivity of 76%(61.8-86.9), specificity 96.8%(91.0-99.3), positive predictive value 92.7%(80.5-97.5), negative predictive value 88.4%(82.2-92.6), and positive likelihood ratio 23.8(7.7-73.3). CONCLUSIONS: A presence of PH in children with ASD can be predicted by the simple electrocardiographic score including QRS axis ≥120°, P wave ≥3 mm at lead II, R without S at V1, Q wave at V1, RBBB, R wave at V1, V2 or aVR > normal limit and S wave at V6 or lead I > normal limit. A total score ≥ 3.5 shows a moderate sensitivity and high specificity to predict PH among children with ASD.

Outcome and factors associated with undernutrition among children with congenital heart disease.

BACKGROUND: Congenital heart disease (CHD) is associated with high morbidity and mortality, especially among those with undernutrition. Evaluating risk factors associated with undernutrition is important to improve clinical outcomes. We aimed to evaluate the outcome and factors associated with undernutrition among children with CHD. MATERIAL AND METHODS: A prospective cohort study was conducted among children with CHD at Dr. Sardjito Hospital, Yogyakarta, Indonesia during February 2016 to June 2018. Clinical and demographic data were collected at the time of diagnosis. Multivariate logistic regression analysis was used to identify independent factors associated with undernutrition using odds ratio (OR) and 95% confidence interval (CI). Significance was set as p<0.05. RESULTS: We recruited 1,149 children with CHD, of those, 563 (49%) were underweight, 549 (47.8%) were stunting, and 361 (31.4%) were wasting. In the multivariate analysis, cyanotic CHD, delayed diagnosis, congestive heart failure, pulmonary hypertension, syndrome, young maternal age, history of low birth weight, and being first child were independently associated with undernutrition. Underweight and stunting were significantly associated with increased mortality with OR of 3.54 (95% CI: 1.62-7.74), p<0.001 and OR 3.31 (95% CI: 1.65-6.64), p<0.001, respectively. CONCLUSIONS: About half of the children with CHD were categorized with undernutrition. An increased risk of undernutrition was associated with cyanotic CHD, delayed diagnosis, congestive heart failure, pulmonary hypertension, syndrome, low birth weight, and being first child. Underweight and stunting were significantly associated with increased risk of death.

Survival of Rheumatic Heart Disease in Indonesian Children.

Background: Rheumatic heart disease (RHD) remains a preventable cause of cardiovascular deaths in children in low- and middle-income countries. However, long-term outcome data of children with RHD is still lacking in Indonesia. Objective: To investigate the long-term outcomes of RHD, particularly the survival rates and the predictors. Methods: A retrospective cohort study was done in children aged less than 18 years old admitted with RHD at a tertiary hospital (Dr. Sardjito Hospital, Indonesia) from 2011-2021. Survival time was estimated from the date of first diagnosis of RHD to the survival endpoint (date of mortality or censoring). Kaplan-Meier curves, log-rank test and Cox-regression analysis were used for survival analysis and the predictors. Results: A total of 155 patients admitted with RHD during the study period. Of these, 14 (9.0%) deaths were reported as RHD related mortality with the mean age ± standard deviation of 11.9 ± 2.9 years. Median follow up period was 16 months. The survival rate at 1, 4, and 8 years were 93%, 86.1% and 60%, respectively. Survival was lower in patients with severe valve lesions and NYHA functional class III-IV at the time of diagnosis. Multivariate Cox-regression analysis showed the significant predictors for mortality were poor adherence to penicillin and congestive heart failure, HR 12.7 (95%CI 1.4-113.5) and 18.6 (95%CI 1.8-185.0) respectively. Conclusion: Approximately, only six in 10 children are able to survive at eight years after diagnosis. Poor adherence to penicillin and congestive heart failure were predictors for death. This study emphasizes the needs to improve the adherence of children with RHD and early detection of subclinical carditis in children.

Feasibility of screening for critical congenital heart disease using pulse oximetry in Indonesia.

BACKGROUND: Screening of critical congenital heart disease (CCHD) using pulse oximetry is a routine procedure in many countries, but not in Indonesia. This study aimed to evaluate the feasibility of implementing CCHD screening with pulse oximetry for newborns in Yogyakarta, Indonesia. METHODS: A cross-sectional study was conducted at four hospitals in Yogyakarta, Indonesia. Newborns aged 24-48 hours who met the inclusion criteria were screened on the right hand and left or right foot using a pulse oximeter. Positive results were indicated by: either (1) SpO2 level < 90% in one extremity, (2) SpO2 level of 90-94% in both right hand and either foot on three measurements conducted 1 hour apart, or (3) a saturation difference > 3% between the upper and lower extremity on three measurements conducted 1 hour apart. Positive findings were confirmed by echocardiography. RESULTS: Of 1452 newborns eligible for screening, 10 had positive results and were referred for echocardiographic evaluation. Of those, 8 (6 per 1000 live birth, 8/1452) had CCHD. Barriers found during screening processes were associated with hospital procedures, equipment, healthcare personnel, and condition of the newborn. CONCLUSION: Pulse oximetry screening might be feasible to be implemented within the routine newborn care setting for CCHD in Indonesia. In order to successfully implement pulse oximetry screening to identify CCHD in Indonesia, the barriers will need to be addressed.

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.

Survival of Left-to-Right Shunt Repair in Children with Pulmonary Arterial Hypertension at a Tertiary Hospital in a Low-to-Middle-Income Country.

Background: In low-to-middle-income countries, repair of the left-to-right shunts congenital heart disease (CHD) are often done with existing pulmonary arterial hypertension (PAH). Long-term outcomes data of this condition in either low-to-middle or high-income countries are limited. We conducted a study to evaluate the outcomes of children with PAH related to left-to-right shunt CHD who underwent surgical or transcatheter repair. Methods: All cases of PAH related to left-to-right shunt CHD repairs from 2015-2018 were retrospectively reviewed with additional new patients who underwent repair within our study period (2019-2020). Cases with complex congenital heart disease and incomplete hemodynamic echocardiography or catheterization measurements were excluded. Kaplan-Meier curves, log-rank test, Cox regression with Firth's correction and restricted mean survival time were used for survival analysis. Results: Of the 118 patients, 103 patients were enrolled and 15 patients were excluded due to complex congenital heart disease and missing hemodynamic measurements prior to repair. Overall, median age at intervention was 44 months, mPAP mean was 43.17 ± 16.05 mmHg and Pulmonary Vascular Resistance index (PVRi) mean was 2.84 ± 2.09 (WU.m2). Nine patients died after repair. The survival rate for patients with PAH-CHD at 1 day, 30 days and 1400 days (4 years) was 96.1%, 92.1%, and 91.0% respectively. Patients with persisting PAH after correction had -476.1 days (95% confidence interval [CI]: -714.4, -237,8) shorter survival over 4 years of follow up compared to patients with reversed PAH. PVRi was found to be the influencing covariate of the difference of restricted mean survival time between these groups. Conclusion: In low-to-middle income settings, with accurate PAH reversibility assessment prior to intervention, repair of left-to-right shunt CHD with existing PAH in children has a favourable outcome. Inferior survival is found in patients with persistence of PAH. PVRi at baseline predicts between-group survival difference.

Delayed diagnosis in children with congenital heart disease: a mixed-method study.

BACKGROUND: Delayed diagnosis of congenital heart disease (CHD) causes significant morbidity and mortality. We aimed to determine the proportion of delayed diagnosis of CHD and factors related to the delayed diagnosis. METHODS: A prospective cohort study with mixed-methods was conducted in Dr. Sardjito Hospital, Yogyakarta, Indonesia. Patients aged < 18 years with newly diagnosed CHD and echocardiography confirmed CHD were included. Data were recorded from medical records and interviews from direct caregivers. Logistic regression was used to identify independent factors associated with the delay. RESULTS: A total of 838 patients were included with median age of 2.9 years (0-17.7 years), with female predominance (54.2%, n = 454). The proportions of delayed diagnosis were 60.8% (510), 54.9% (373) and 86.2% (137) in all children with CHD, acyanotic and cyanotic CHD, respectively. Delayed diagnosis by doctor was the most common cause, followed by delayed diagnosis related to midwifery care, financial, referral/follow-up, and social factors. In multivariate analysis, cyanotic CHD, residence outside the city, non-syndromic, low family income, normal labour and at term gestation at birth were independently associated with the delay. At diagnosis, heart failure and pulmonary hypertension occurred in 414 (49.4%) and 132 (15.8%) children with CHD, respectively. CONCLUSIONS: Six in ten children with CHD were diagnosed with significant delay. Delayed diagnosis by doctor was the most common cause. Children with cyanotic CHD, residence outside the city, non-syndromic, low family income, normal labour and at term gestation at birth were independently associated with the delay. Comorbid complications in delayed diagnosis of CHD were prevalent.

Outcome in children with newly diagnosed rheumatic heart disease in Indonesia.

BACKGROUND: Rheumatic heart disease (RHD) is associated with high morbidity and mortality, especially in those with severe RHD or progression of valvular disease (VD). Evaluation of the factors that predict valvular progression is important in order to improve clinical outcome. AIM: To evaluate outcome and clinical predictors of valvular progression in children with newly diagnosed RHD. METHODS: A retrospective cohort study was conducted in children with newly diagnosed RHD at Dr Sardjito Hospital, Yogyakarta, Indonesia during 2013-2020. Clinical and echocardiography data at the time of diagnosis were collected and patients were followed up for 1 year. Echocardiography evaluations were undertaken to determine the progression of VD. Independent predictors of valvular progression were identified by Cox regression analysis. RESULTS: A total of 77 patients were recruited, 36 (46.7%) of whom were male, and the median age (range) was 12.3 years (5.9-17.8). Thirty-three (42.8%) had progression of VD in the year after diagnosis. By multivariable analysis, an age at diagnosis of >10 years and high C-reactive protein (CRP) were independently associated with an increased risk of valvular progression with an adjusted hazard ratio (95% CI) of 3.23 (1.09-9.60) and 3.69 (1.45-9.67), respectively. CONCLUSION: After only 1 year of follow-up, approximately four in 10 children with newly diagnosed RHD developed progression of VD. An increased risk of valvular progression was associated with being over 10 years of age and a high level of CRP.