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Takayasu arteritis (TA) is a rare chronic granulomatous vasculitis mainly affecting the aorta and its main branches. Clinical presentations of TA are non-specific, especially in the initial phase, which likely contributes to delayed diagnosis besides the rarity of the disorder. Childhood-onset of TA is associated with significant morbidity and mortality. This case report aimed to present two rare cases of acute symptomatic severe hypertension in children due to TA.

Abstract 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.

Background Pulmonary hypertension carries significant morbidity and mortality in children. Early diagnosis and management may improve outcomes in children with pulmonary hypertension. Heart catheterization, a gold standard for diagnosing pulmonary hypertension, is an invasive procedure and not widely available. Echocardiography can be used as an alternative diagnostic tool for pulmonary hypertension. Objective To determine the diagnostic value of tricuspid regurgitation pressure gradient on echocardiography compared to heart catheterization to diagnose pulmonary hypertension in children. Methods This diagnostic test study was done with medical record data of children with acyanotic congenital heart disease who underwent cardiac catheterization and echocardiography procedures from January 2018 to December 2020 at Dr. Sardjito Hospital, Yogyakarta, Indonesia. Data were analyzed to obtain sensitivity, specificity, positive and negative predictive values, as well as positive and negative likelihood ratios. Results A total of 98 children with acyanotic congenital heart disease were included. The sensitivity and specificity of the tricuspid regurgitation pressure gradient to determine pulmonary artery pressure compared to heart catheterization were 64.4% and 54.5%, respectively. The positive likelihood ratio was 1.42. The pre-test and post-test probability of this study were 88.7% and 91.7%, respectively. Conclusion Tricuspid regurgitation pressure gradient measured using echocardiography has poor sensitivity and specificity to diagnose pulmonary hypertension.

Abstract 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 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.

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 pResults 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), pConclusions 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.

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.

Abstract 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

Kawasaki disease (KD), or mucocutaneous syndrome, is an acute, systemic vasculitis of small- and medium-sized arteries that predominantly affects patients younger than five years.1 KD is the leading cause of childhood acquired heart disease in the developed world.2 The incidence in those aged under 5 years varies widely throughout the world, accounting for 8.4 per 100,000 in the UK, 17.5 to 20.8 per 100,000 in the USA, and 239.6 per 100,000 in Japan.2 The diagnosis of classic KD is based on the simultaneous presence of high fever for 5 or more days with at least four of five other symptoms (bilateral conjunctival hyperemia, ulcerations of the lips and inflammation of the oral cavity, polymorphous rash, edema and desquamation of the extremities, and cervical lymphadenopathy), or fever associated with less than 4 of the diagnostic criteria and echocardiographic abnormalities of the coronary arteries.3

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.

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.

Background Albumin transfusion for the treatment of neonatal hypoalbuminemia may reduce morbidity. In conditions with disrupted endothelial integrity (e.g., sepsis and critical illness), the administered albumin may leak into the interstitial space, hence, serum albumin levels may fall below expected levels after transfusion. To date, few studies have been done to evaluate the risk factors for failure to achieve normal neonatal albumin levels after transfusion. Objectives To determine the risk factors for failure to achieve normal neonatal albumin levels after transfusion. Methods We performed a case-control study in the Neonatal Ward of Dr. Sardjito Hospital from 2007 to 2012. Normal albumin level was defined as above 3 g/dL. The case group included neonates with post-transfusion albumin levels 24-hour interval between albumin examination and transfusion (OR 0.06; 95%CI 0.01 to 0.37) were significant risk factors affecting the failure to achieve normal albumin levels. Conclusions Failure to achieve normal albumin levels after transfusion in neonates was significantly associated with low albumin level prior to transfusion, critical illness, sepsis, and >24-hour interval between transfusion and post-transfusion albumin examination.[Paediatr Indones. 2016;56:129-33.].