Related Experiment Video
Updated: Oct 5, 2025
Left Lung Orthotopic Transplantation in a Juvenile Porcine Model for ESLP
Published on: February 14, 2022
Recovery of right ventricular function after bilateral lung transplantation for pediatric pulmonary hypertension
Paul J Critser1, Debra Boyer2,3, Gary A Visner2,3
1Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts, USA.
Insights
Lung transplantation improves right ventricular (RV) function in children with pulmonary hypertension (PH) and baseline RV dysfunction. Post-transplant RV recovery is significant, supporting lung transplantation as a viable option for these patients.
Area of Science:
- Cardiology
- Pulmonology
- Pediatric Critical Care
Background:
- Pediatric pulmonary hypertension (PH) often leads to end-stage disease requiring lung transplantation.
- Right ventricular (RV) dysfunction is a common complication in pediatric PH.
- Limited data exists on RV recovery and post-transplant cardiac function in children with PH and baseline RV dysfunction.
Purpose of the Study:
- To evaluate RV recovery and cardiac function post-lung transplantation in pediatric patients with PH and baseline RV dysfunction.
- To determine the association between pre-transplant RV function and peri-operative outcomes.
- To assess the long-term impact of lung transplantation on RV function in this population.
Main Methods:
- Single-center chart review of pediatric patients with PH undergoing bilateral orthotopic lung transplantation (2000-2020).
- Analysis of echocardiographic data to determine RV fractional area change (FAC) and left ventricular eccentricity index (LVEI) pre- and post-transplantation.
- Comparison of outcomes between patients with and without baseline RV dysfunction.
Main Results:
- 14 of 23 patients had baseline RV dysfunction; 12 (86%) survived 1 year post-transplantation.
- All patients with baseline RV dysfunction showed improved RV-FAC post-transplant, with 70% normalization by 3 months and 100% by 12 months.
- Pre-transplant RV function was not associated with duration of ventilation, ICU stay, or hospital stay.
Conclusions:
- Pediatric lung transplantation leads to significant RV functional improvement in patients with PH and baseline RV dysfunction.
- Pre-transplant RV function does not predict short-term outcomes after lung transplantation.
- Lung transplantation is a justifiable therapeutic option for pediatric end-stage PH with RV dysfunction.
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.
