Repair of isolated atrial septal defect in infants less than 12 months improves symptoms of chronic lung disease or shunt-related pulmonary hypertension

Charisopoulou, Dafni; Bini, R; Riley, Gillian; Janagarajan, Kalai; Moledina, Shahin; Marek, Jan

doi:10.1017/s1047951120000463

Cited by 12 publications

(16 citation statements)

References 34 publications

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“…These prevalence estimates were quite similar with our study. Meanwhile, in the high-income countries, the prevalence estimates of post-correction PAH were lower than our study: USA (11.1%), Switzerland (0%), Germany (15.4%), and UK (12.5) [ 19 ]. The similarity of the studies in the high-income countries was probably due to the fact that the corrections of CHD were done at an early age (less than 12 months) whereas in our study the median age at intervention was 44 months.…”

Section: Discussioncontrasting

confidence: 65%

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

Murni

Idris

et al. 2021

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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.m 2 ). 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.

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Section: Discussioncontrasting

confidence: 65%

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

Murni

Idris

et al. 2021

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“…13,14 Additional data also supports that many inflammatory mediators known to be abnormally expressed in PAH are also altered in ILD. 15 For example, TGFβ is an inflammatory mediator that is heavily involved in both IPF 16 and PAH. 17 Alterations in VEGF levels, 18 IL-6, 19 as well tumor necrosis factor α 3 have also been reported.…”

Section: Pathophysiology Of Ph-ildmentioning

confidence: 99%

“…1 Furthermore, with PH onset regardless of cause of the PH, there is a trend toward increasing mortality in CTD with an estimated 3-year survival rate of 56%. 15 PH and/or ILD account for 60% of deaths in SSc. 23 In the PHAROS registry (PH Assessment and Recognition of Outcomes in Scleroderma), SSc-PAH populations are found to have poorer long-term survival despite access to vasodilator therapy.…”

Section: Survival: Ph-ild With the Worst Prognosismentioning

confidence: 99%

“…23 Clinical trial data in CTD-PAH demonstrate that PH-specific therapies such as prostacyclins, endothelin receptor antagonists and phosphodiesterase type 5 inhibitors have been shown to improve exercise tolerance, health-related quality of life, and hemodynamic parameters. 15 Unfortunately, not the same can be said with trials that included populations that had ILD. Patients with ILD have been historically excluded, however, with the exception of a few trials in SSc.…”

Section: Treatment: What the Evidence Currently Supports Ph-specif Ic Therapymentioning

confidence: 99%

“…Although an early case series suggested the dual endothelin receptor antagonist bosentan increased exercise capacity in PH-ILD, this finding did not persist at 1 year. 15 Negative results were also seen in B-PHIT, a placebo-controlled RCT of bosentan in fibrotic PH-IIP. Among 60 patients, this medication was not associated with improvements in indexed PVR, exercise capacity, dyspnea, or oxygenation.…”

Section: The Endothelin-1 Pathwaymentioning

confidence: 99%

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2021

Advances in Pulmonary Hypertension

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Atrial septal defect closure is associated with improved clinical status in patients ≤ 10 kg with bronchopulmonary dysplasia

Webb,

Cuevas Guaman,

Sexson Tejtel

et al. 2023

Pulm. circ.

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Patients with bronchopulmonary dysplasia (BPD) have shown clinical improvement after secundum atrial septal defect (ASD) closure. We sought to determine if this post‐ASD closure improvement is secondary to the expected course in BPD patients or related to the closure itself. A novel BPD‐ASD score was created to assess patients' clinical status (higher score = worse disease) and applied to 10 BPD‐ASD inpatients weighing ≤ 10 kg who underwent ASD closure. The score and its subcomponents were retrospectively calculated serially ranging from 8 weeks pre‐ to 8 weeks post‐intervention, and pre‐ and post‐intervention score slopes were created. These slopes were compared using mixed regression modeling with an interaction term. There was a significant difference in pre‐ versus post‐intervention slope with the most score drop the first week post‐intervention (−2.1 + /− 0.8, p = 0.014). The mean score also dropped through weeks 2 (slope −0.8 + /− 0.8, p = 0.013) and 4 (slope −1.0 + /− 0.5, p = 0.001) post‐intervention. There was a significant difference in pre‐ and post‐intervention slopes for diuretics (p = 0.018) and the combined score of respiratory support, FiO2 need, and respiratory symptoms (p = 0.018). This study demonstrated significant improvement in BPD‐ASD score, diuretic need, and respiratory status after ASD closure in BPD‐ASD patients ≤ 10 kg that was outside of the natural course of BPD. Our study was limited by its small, single‐center, retrospective nature. Future studies should be performed in a larger multicenter population to both validate the scoring system and compare to non‐intervention infants.

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Repair of isolated atrial septal defect in infants less than 12 months improves symptoms of chronic lung disease or shunt-related pulmonary hypertension

Cited by 12 publications

References 34 publications

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

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

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Atrial septal defect closure is associated with improved clinical status in patients ≤ 10 kg with bronchopulmonary dysplasia

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