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

Arafuri, Nadya; Murni, Indah Kartika; Idris, Nikmah Salamia; Uiterwaal, Cuno S.P.M.; Savitri, Ary I.; Nugroho, Sasmito; Noormanto, Noormanto

doi:10.5334/gh.831

Cited by 4 publications

(5 citation statements)

References 35 publications

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“…9 In this study, transthoracic echocardiography and cardiac catheterization were performed by pediatric cardiologists, with good Kappa values. 3 The Kappa value of 0.89 in our study was within the reference study target range. 1,3 As such, echocardiography and cardiac catheterization findings should closely approximate the actual condition of the patient and uniform technique.…”

Section: Discussionsupporting

confidence: 67%

“…3 The Kappa value of 0.89 in our study was within the reference study target range. 1,3 As such, echocardiography and cardiac catheterization findings should closely approximate the actual condition of the patient and uniform technique.…”

Section: Discussionsupporting

confidence: 67%

“…1,2 Early diagnosis of pulmonary hypertension is needed in CHD patients because repair of the defect during late stage pulmonary hypertension has the poorest prognosis amongst other types of pulmonary hypertension. [3][4][5] The gold standard of pulmonary artery pressure measurement is by cardiac catheterization. However, this procedure is invasive and has risks, related to the procedure itself, as well as the risk of allergy to the contrast material used.…”

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confidence: 99%

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Tricuspid regurgitation pressure gradient to diagnose pulmonary hypertension: a diagnostic accuracy study

Murni¹,

Siagian²,

Nurnaningsih

et al. 2022

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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.

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Section: Discussionsupporting

confidence: 67%

Section: Discussionsupporting

confidence: 67%

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confidence: 99%

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Tricuspid regurgitation pressure gradient to diagnose pulmonary hypertension: a diagnostic accuracy study

Murni¹,

Siagian²,

Nurnaningsih

et al. 2022

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“…Early diagnosis or early treatment of LTRS or PAH-LTRS will effectively reduce the progression and mortality rate of PAH ( 19 ). Closure of cardiac defects in early childhood could effectively prevent the occurrence and progression of PAH and has a favorable outcome ( 20 ). However, not all children can be repaired timely and successfully, especially in developing countries ( 20 ).…”

Section: Introductionmentioning

confidence: 99%

“…Closure of cardiac defects in early childhood could effectively prevent the occurrence and progression of PAH and has a favorable outcome ( 20 ). However, not all children can be repaired timely and successfully, especially in developing countries ( 20 ). For them, they may continue to suffer from PAH their whole life.…”

Section: Introductionmentioning

confidence: 99%

Altered Lung Microbiome and Metabolome Profile in Children With Pulmonary Arterial Hypertension Associated With Congenital Heart Disease

Cheng²,

Song³

et al. 2022

Front. Med.

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BackgroundsPulmonary arterial hypertension (PAH) is characterized by progressive pulmonary vascular functional and structural changes, resulting in increased pulmonary vascular resistance and eventually right heart failure and death. Congenital Left-to-Right shunts (LTRS) is one type of congenital heart disease (CHD) and PAH associated with the congenital Left-to-Right shunt (PAH-LTRS) is a severe disease in children. However, changes in the lung microbiome and their potential impact on PAH-LTRS have not been not fully studied. We hypothesized that lung microbiota and their derived metabolites have been disturbed in children with PAH-LTRS, which might contribute to the progression and outcomes of PAH-LTRS.MethodsIn this study, 68 age- and sex-matched children of three different groups (patients with PAH-LTRS cohort, patients with LTRS but have no pathologic features of PAH cohort, and healthy reference cohort) were enrolled in the current study. Bronchoalveolar lavage fluid samples from these participants were conducted for multi-omics analysis, including 16S rRNA sequencing and metabolomic profiling. Data progressing and integration analysis were performed to identify pulmonary microbial and metabolic characteristics of PAH-LTRS in children.ResultsWe found that microbial community density was not significantly altered in PAH-LTRS based on α-diversity analysis. Microbial composition analysis indicated phylum of Bacteroidetes was that less abundant while Lactobacillus, Alicycliphilus, and Parapusillimonas were significantly altered and might contribute to PAH in children with LTRS. Moreover, metabolome profiling data showed that metabolites involved in Purine metabolism, Glycerophospholipid metabolism, Galactose metabolism, and Pyrimidine metabolism were also significantly disturbed in the PAH-LTRS cohort. Correlation analysis between microbes and metabolites indicated that alterations in the microbial composition from the lung microbiota could eventually result in the disturbance in certain metabolites, and might finally contribute to the pathology of PAH-LTRS.ConclusionLung microbial density was not significantly altered in patients with PAH-LTRS. Composition analysis results showed that the relative microbiome abundance was different between groups. Metabolome profiling and correlation analysis with microbiota showed that metabolome also altered in children with PAH-LTRS. This study indicated that pulmonary microbes and metabolites disturbed in PAH-LTRS could be potentially effective biomarkers and provides valuable perspectives on clinical diagnosis, treatment, and prognosis of pediatric PAH-LTRS.

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

et al. 2023

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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.

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

Cited by 4 publications

References 35 publications

Tricuspid regurgitation pressure gradient to diagnose pulmonary hypertension: a diagnostic accuracy study

Tricuspid regurgitation pressure gradient to diagnose pulmonary hypertension: a diagnostic accuracy study

Altered Lung Microbiome and Metabolome Profile in Children With Pulmonary Arterial Hypertension Associated With Congenital Heart Disease

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

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