Julian E. Cameron, Doff B. McElhinney, Esther Liu, Rachel K. Hopper, Ritu Asija, Manchula Navaratnam, Frank L. Hanley, Jeffrey A. Feinstein
Lucile Salter Packard Children’s Hospital and Stanford University School of Medicine. Children’s Hospital Los Angeles and Keck School of Medicine University of Southern California.
United States
Pulmonary Circulation
Pulm Circ 2025; 15:
DOI: 10.1002/pul2.70134
Abstract
Segmental pulmonary hypertension (PH) in congenital heart disease remains poorly understood with data limited to case studies. We performed a retrospective, single center study in children treated with PH medications after unifocalization/pulmonary artery reconstruction for major aortopulmonary collaterals (MAPCA). Drug response was determined by hemodynamic changes across at least two cardiac catheterizations. Mechanical properties of the segmental arteries were quantified by distensibility, stiffness, and augmentation indices. Twenty-five patients were included (8 surgical shunt, 17 complete repair), with 76% considered responsive to PH medications based on the relative decrease in maximum segmental mean pulmonary artery pressure (mPAP). At a median duration of 14 months (Q1-Q3 9.5-29), mPAP decreased from 33 mmHg (28-38) to 23 mmHg (21-32) (p < 0.001) with no significant change in blood flow distribution by lung perfusion scintigraphy. Subgroup analysis demonstrated a trend towards a larger percent decrease in mPAP of 35% (18-45) on dual therapy compared to 23% (Q1-Q3 3-36) on monotherapy (p = 0.16). In repaired patients, arterial distensibility at initial catheterization correlated with residual elevation in mPAP at follow-up (R 2 0.687, p < 0.001), with distensibility < 1.7%/mmHg associated with treatment failure. Among the lowest distensibility values were patients with JAG1 mutations, and among patients with extended follow-up, progressive increase in mPAP was identified only in those with hereditary PH-associated mutations. Children with segmental PH following pulmonary artery reconstruction can be successfully treated with PH medications. Although treatment efficacy may be limited in patients with high vascular stiffness and those with pathologic vascular mutations.
Category
Segmental Pulmonary Arterial Disease
Diagnostic Testing for Pulmonary Vascular Disease. Invasive Testing
Medical Therapy. Efficacy or Lack of Efficacy
Genetic Factors Associated with Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
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Free PDF File or Full Text Article Available Through PubMed or DOI: Yes