Recovery of right ventricular function after bilateral lung transplantation for pediatric pulmonary hypertension

• Published in: Pediatric transplantation Vol. 26; no. 4; pp. e14236 - n/a
• Main Authors: Critser, Paul J., Boyer, Debra, Visner, Gary A., Collins, Shane L., Fynn‐Thompson, Francis, Mullen, Mary P.
• Format: Journal Article
• Language: English
• Published: Denmark Wiley Subscription Services, Inc 01-06-2022
• Subjects: Child; Children; Heart Ventricles; Humans; Hypertension; Hypertension, Pulmonary - surgery; Lung Transplantation; Lung transplants; Patients; pediatric pulmonary hypertension; Pediatrics; Pulmonary hypertension; Recovery (Medical); right ventricular function; Ventricle; Ventricular Dysfunction, Right - surgery; Ventricular Function, Right; lung transplantation; right ventricular function; pediatric pulmonary hypertension
• ISSN: 1397-3142; 1399-3046
• DOI: 10.1111/petr.14236

Background
Lung transplantation is a therapeutic option for end‐stage pediatric pulmonary hypertension (PH). Right ventricular (RV) recovery post‐lung transplant in children with PH has not been well‐described, and questions persist about the peri‐operative course and post‐transplant cardiac function after lung transplantation in medically refractory PH patients with baseline RV dysfunction.
Methods
A single‐center chart review identified patients with childhood PH who subsequently underwent bilateral orthotopic lung transplantation between 2000 and 2020. Twenty‐six patients met criteria; three were excluded due to echocardiograms not available for digital review. RV fractional area change (FAC) and left ventricular eccentricity index (LVEI) were determined prior to transplantation, and at 1, 3, 6, and 12‐month post‐transplantation.
Results
Fourteen of 23 patients had baseline RV dysfunction. The median age at transplantation was 16.5 years and 13.9 years for those with and without baseline RV dysfunction, respectively. Of the 14 with baseline RV dysfunction, 12 (86%) were alive 1‐year post‐transplantation. All patients with baseline RV dysfunction had increased RV‐FAC post‐transplantation with normalization of RV‐FAC in 70% at 3 months and 100% of patients by 12‐month post‐transplantation. Duration of ventilation (p = .4), intensive care unit (p = .5), or hospital stay (p = .9) was not associated with pre‐transplant RV function.
Conclusions
Among pediatric patients with PH and RV dysfunction, pre‐transplantation RV function was not associated with short‐term outcomes. All patients with baseline RV dysfunction had improvement in RV function, justifying consideration of lung transplantation among pediatric patients with end‐stage PH and RV dysfunction.