Early Surgical Closure of Atrial Septal Defect Improves Clinical Status of Symptomatic Young Children with Underlying Pulmonary Abnormalities

Abstract

Elective closure of atrial septal defect (ASD) is usually recommended during preschool ages. However, ASD may contribute to deteriorating health in the presence of significant comorbidity and, thus, may need earlier closure. There is a lack of clarity regarding the indications for and outcomes after ASD closure in infancy and early childhood. We investigated the benefits and safety of surgical ASD closure in symptomatic patients under 2 years of age. Retrospective chart review was conducted in patients who underwent surgical ASD closure within the first 2 years of life. Of 31 symptomatic ASD patients, 22 had persistent respiratory symptoms, 24 failure to thrive, and 9 pulmonary hypertension. Overall, 26 patients (84.0%) showed clinical improvement after ASD closure, including improved respiratory status (17/22; 77.3%), resumption of normal growth (15/24; 62.5%), and resolution of pulmonary hypertension (7/7; 100%, 2 patients unable to assess postoperatively). Two medically complicated patients died a few months after surgery unrelated to surgical complications. Four out of 8 ventilator-dependent patients were weaned from mechanical ventilation within 1 month after ASD closure. Closure of ASD did not improve those patients with highly advanced lung disease and/or medically complex conditions including underlying genetic abnormalities. Surgical complications were uncommon. Postoperative hospital stay was 4 to 298 days (median 8 days). The majority of our patients demonstrated significant clinical improvement after ASD closure. Early ASD closure is safe and beneficial for symptomatic infants and young children with associated underlying pulmonary abnormalities, especially bronchopulmonary dysplasia.

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“…19 While those on background PAH treatment were excluded, 14% of the original cohort had WSPH Group 3 disease on preenrollment right heart catheterization. 20 After 48 weeks of randomized treatment, ambrisentan was not associated with hemodynamic improvements among those who underwent interval right heart catheterization. Moreover, there was a nonsignificant trend among ambrisentan-treated PH patients toward increased ILD progression.…”

“…Prevalence of ILD is estimated to be between 0.0672% 20 (females)/0.0809% (males) and 0.071% 21 in 2 cohort studies. The estimation of the prevalence of PH-ILD is difficult given the variable admixture of causes of ILD, and the inherent bias of the presence of retrospective hemodynamic data only in those patients already suspected of having PH or undergoing transplant work-up.…”

“…CTD-related PH (CTD-PH) occurs as a result of simultaneous mechanisms. 20 Lung predominant CTD with PH (CTD-PH-ILD) is therefore challenging to treat but presents a unique opportunity as these patients have a chance at responding to PH-targeted therapies.…”

“…In the group 3 phenotype, one is distinctly driven by hypoxic pulmo- nary vasoconstriction versus a more hemodynamically/right ventricle (RV) failure group 1 phenotype. 20 There develops a critical point at which PH becomes the larger driver of outcomes, and this is probably more true and possibly earlier realized in CTD patients. King and Shlobin 28 offers a therapeutic approach to PH-ILD where one can view PH as a surrogate marker of disease progression in advanced fibrotic lung disease versus a maladaptive phenotype where PH is the driver of pathophysiologic derangements that is out of proportion to the degree of ILD.…”

“…Graphic illustration of progression in pulmonary hypertension associated with interstitial lung disease where at a critical point, pulmonary hypertension becomes the driver of poor outcomes. Adapted from King and Nathan 20.…”