Critser PJ; Department of Cardiology, Boston Children’s Hospital, Boston, MA.
Buchmiller TL; Gauvreau K; Zalieckas JM; Sheils CA; Visner GA;
Shafer KM; Chen MH; Mullen MP

Journal of Pediatrics. 271:114034, 2024 Aug.

OBJECTIVE: To determine the prevalence of exercise-induced pulmonary
hypertension (PH) among long-survivors of congenital diaphragmatic hernia (CDH)
repair.

STUDY DESIGN: This is a single-center, retrospective cohort study of CDH
survivors who underwent exercise stress echocardiography (ESE) at Boston
Children’s Hospital from January 2006 to June 2020. PH severity was
assessed by echocardiogram at baseline and after exercise. Patients were
categorized by right ventricular systolic pressure (RVSP) after exercise:
Group 1 – no or mild PH; and Group 2 – moderate or severe PH (RVSP >= 60
mmHg or >= 1/2 systemic blood pressure).

RESULTS: Eighty-four patients with CDH underwent 173 ESE with median age
8.1 (4.8 – 19.1) years at first ESE. Sixty-four patients were classified
as Group 1, 11 as Group 2, and 9 had indeterminate RVSP with ESE. Moderate
to severe PH after exercise was found in 8 (10%) patients with no or mild
PH at rest. Exercise-induced PH was associated with larger CDH defect
size, patch repair, use of ECMO, supplemental oxygen at discharge, and
higher WHO functional class. Higher VE/VCO2 slope, lower peak oxygen
saturation, and lower percent predicted FEV1, and FEV1/FVC ratio were
associated with Group 2 classification. ESE changed management in 9/11
Group 2 patients. PH was confirmed in all 5 Group 2 patients undergoing
cardiac catheterization after ESE.

CONCLUSIONS: Among long-term CDH survivors, 10% had moderate-severe
exercise-induced PH on ESE, indicating ongoing pulmonary vascular
abnormalities. Further studies are needed