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.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.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).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 Increased pulmonary blood flow may lead to abnormal lung function in children with left-to-right (L to R) shunt congenital heart disease. This condition has been linked to considerable mortality and morbidity, including reduced lung function.
Objective To assess for lung function abnormality in children with L to R shunt congenital heart disease.
Methods We conducted a cross-sectional study involving children aged 5-18 years and diagnosed with L to R shunt congenital heart disease at Dr. Sardjito Hospital from March to May 2017. Subjects underwent spirometry tests to measure forced expiratory volume-1 (FEV-1), forced vital capacity (FVC), and forced expiratory volume-1 (FEV-1)/forced vital capacity (FVC).
Results Of 61 eligible subjects, 30 (49.2%) children had atrial septal defect (ASD), 25 (41%) children had ventricular septal defect (VSD), and 6 (9.8%) children had patent ductus arteriosus (PDA). Spirometry revealed lung function abnormalities in 37 (60.7%) children. Restrictive lung function was documented in 21/37 children, obstructive lung function in 11/37 children, and mixed pattern of lung function abnormality in 5/37 children. Pulmonary hypertension was found in 21 children. There was no significant difference in lung function among children with and without pulmonary hypertension (P=0.072).
Conclusion Abnormal lung function is prevalent in 60.7% of children with L to R shunt congenital heart disease, of which restrictive lung function is the most common. There was no significant difference in lung function among children with and without pulmonary hypertension.
Latar belakang. Patent duktus arteriosus (PDA) merupakan salah satu penyakit jantung bawaan asianotik yang paling banyakdijumpai pada anak. PDA dapat memengaruhi pertumbuhan anak dan menyebabkan malnutrisi pada anak dan malnutrisi yangterjadi merupakan faktor risiko mortalitas dan morbiditas pada anak.Tujuan. Mengetahui pertambahan berat badan sebelum dan sesudah dilakukan tindakan penutupan duktus pada anak dengan PDAsecara transkateter dan untuk mengetahui faktor-faktor yang berpengaruh terhadap pertambahan berat badan setelah dilakukanpenutupan PDA secara transkateter.Metode. Penelitian observasional pre dan post test design dengan menggunakan data rekam medis. Anak dengan PDA yangdilakukan penutupan secara transkateter. Z-score berat badan menurut umur dinilai sebelum dan pada saat 1, 3, 6, dan 12 bulansetelah penutupan dan dilakukan analisis menggunakan repeated ANOVA test. Faktor-faktor yang memengaruhi pertambahanberat badan 3 bulan setelah penutupan dianalisis menggunakan chi square dan regressi logistik.Hasil. Terdapat 43 anak usia <5 tahun dengan PDA diikutkan dalam penelitian dari Januari 2005 sampai Juni 2011. Sebelumpenutupan 76,7% (33) anak dengan z-score berat badan/umur < -2 SD. Didapatkan perbaikan rerata z-score berat badan/umursebelum dan saat 1, 3, 6, dan 12 bulan setelah penutupan (-2,63 vs -2,41, -2,14, -1,92 and -1,56; p<0,05). Jenis kelamin, umur,z-score berat badan/umur sebelum penutupan, berat lahir, hipertensi pulmonal, gagal jantung, pekerjaan orang tua, pendidikanibu dan penghasilan orang tua tidak berhubungan dengan pertambahan berat badan 3 bulan setelah penutupan.Kesimpulan. Penutupan defek secara transkateter pada anak usia <5 tahun dengan PDA akan memberikan peningkatan z-scoreberat badan berdasarkan umur.
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 Heart failure continues to be a significant contributor to morbidity and mortality in children with congenital heart disease (CHD). Little is known about heart failure in children. Identifying predictors of heart failure in children with CHD can serve to guide preventive strategies to heart failure.
Objective To understand the predictors of heart failure of children with congenital heart disease.
Methods A nested, case-control study was performed using secondary data based on a prospective study previously conducted in Dr Sardjito Tertiary Hospital in Yogyakarta in years 2011-2013. We included children aged 1 month-18 years who had been diagnosed with CHD by echocardiography. Age, sex, type of CHD, CHD complexity, as well as presence of syndrome, no pulmonary obstruction, pneumonia, and malnutrition were analyzed as potential predictors of heart failure. Results are presented as odds ratios (OR) with 95% confidence intervals (95%CI).
Results A total of 2,646 children were hospitalized in Dr Sardjito Tertiary Hospital during the study period. Congenital heart disease was noted in 216 children (8.16%), 200 (7.5%) of whom met the inclusion criteria. The 100 children with heart failure had median age of 1.5 years and 15% died during hospitalization. Multivariate analysis revealed that acyanotic CHD (OR 2.69; 95%CI 1.45 to 5.00), no pulmonary obstruction (OR 3.05; 95%CI 1.33 to 6.99) and the presence of pneumonia (OR 2.04; 95%CI 1.03 to 4.06) were statistically significant as independent predictors of heart failure in children with CHD. However, sex, age, CHD complexity, as well as presence of a syndrome, and malnutrition were not significantly associated with heart failure in children with CHD.
Conclusion The predictors of heart failure in children with CHD were acyanotic CHD, no pulmonary obstruction, and presence of pneumonia.
Background Brain abscess is a severe infection of brain parenchyma, which occurs in 25-46% of cases of uncorrected cyanotic congenital heart disease. Low arterial oxygen saturation is the main risk factor for brain abscess in children with cyanotic congenital heart disease, however, the arterial oxygen saturation test is invasive and not routinely done in our setting.
Objective To evaluate low peripheral oxygen saturation as a risk factor for brain abscess in children with cyanotic congenital heart disease.
Methods We conducted a matched, case-control study at Sardjito Hospital, Yogyakarta for children aged less than 18 years with cyanotic congenital heart disease, from 2010-2016. Case subjects were children with brain abscess complications. The control group had only cyanotic congenital heart disease, and were matched for age and sex to the case group. During hospitalization due to the brain abscess complication in the case group, data regarding peripheral oxygen saturation, polycythemia, pneumonia, sepsis, dental caries and restricted pulmonary blood flow were collected and compared between both groups.
Results During the study period, 18 children with cyanotic congenital heart disease had brain abscesses. This group was compared to the control group of 36 children. Bivariate analysis revealed that the lowest level of peripheral oxygen saturation (OR 0.92; 95%CI 0.85 to 0.98; P=0.02) and dental caries (OR 3.3; 95%CI 1.01 to 11.18; P=0.04) were significant risk factors for brain abscess. However, in the multivariate analysis, the only statistically significant risk factor associated with brain abscess was the lowest level of peripheral oxygen saturation (OR 0.92; 95%CI 0.86 to 0.99; P=0.04).
Conclusion Low peripheral oxygen saturation is a significant risk factor for brain abscess development in children with cyanotic congenital heart disease. A decrease of 1% peripheral oxygen saturation may increase the risk of brain abscess by 8%.
Malnutrition is widely known to affect growth in children. There are many studies focusing on malnutrition globally in relation to limited food access; however, there is only limited research on disease-related malnutrition, especially in chronic conditions and particularly in developing countries. This study aims to review articles on the measurement of malnutrition in pediatric chronic disease, especially in developing countries where there are resource limitations in identifying nutritional status in pediatric chronic disease with complex conditions. This state-of-the-art narrative review was conducted through search of literatures through 2 databases, and identified 31 eligible articles published from 1990 to 2021. This study found no uniformity in malnutrition definitions and no consensus regarding screening tools for the identification of the malnutrition risk in these children. In developing countries where resources are limited, instead of focusing on finding the best tools to identify the malnutrition risk, the approach should be directed toward developing systems that work best according to capacity and allow for a combination of anthropometry assessment, clinical evaluation, and observation of feeding access and tolerance on a regular basis.
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.
Background Ventricle septal defect (VSD) is the most common type of congenital heart disease in children. If definitive therapy delayed, failure to thrive and developmental delays can lead to decreased quality of life. The options for VSD closure include surgical and minimally invasive procedures with transcatheterization. Although transcatheterization is considered to be the safest therapy, the risk of complications can lead to cancellation of procedure.
Objective To determine whether nutritional status, body height, VSD type and size, and type of device used were predictors of cancellation of transcatheter closure of VSD.
Methods A retrospective cohort study using medical records was performed for all children who underwent transcatheter closure of VSD at Dr. Sardjito Hospital, Yogyakarta, Central Java, between January 2017 to March 2020. Cancellation of closure was defined as complications occurring during the procedure, such as cardiac conduction problems, valve regurgitation, and device embolization. Multivariate logistic regression analysis was done to determine independent predictors of closure cancellation.
Results One hundred thirty-four children were enrolled. Independent variables that were significant predictors were doubly committed subarterial (DCSA) VSD type (OR 5.98; 95%CI 1.52 to 23.61; P=0.045), moderate VSD size (OR 15.59; 95%CI 4.67 to 52.06; P=0.001), and types of devices used: symmetric (OR 27.06; 95%CI 2.75 to 266.17; P=0.001), asymmetric (OR 16.46; 95%CI 2.15 to 210.0; P=0.001), and coil (OR 21.26; 95%CI 2.15 to 210.0; P=0.001). Taller body height was a protective factor against cancellation of the procedure (OR 0.98; 95%CI 0.96 to 1.00; P=0.008).
Conclusion Significant predictors of cancellation of transcatheter VSD closure are DCSA VSD, moderate VSD size, as well as coil, symmetric, and asymmetric devices, and increased body height.
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