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Full recovery of right ventricular systolic function in children undergoing bilateral lung transplantation for severe
Georg Hansmann1, Franziska Diekmann1, Philippe Chouvarine1
1Department of Pediatric Cardiology and Critical Care, Hannover Medical School, Hannover, Germany; European Pediatric Pulmonary Vascular Disease Network, Berlin, Germany.
Insights
Lung transplantation (LuTx) in children with pulmonary arterial hypertension (PAH) leads to full recovery of right ventricular (RV) systolic function within two months. This improvement occurs regardless of pre-transplant condition, highlighting LuTx as a preferred treatment.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Surgery
- Pulmonary Hypertension Research
Background:
- Pulmonary arterial hypertension (PAH) in children often leads to right ventricular (RV) failure.
- Lung transplantation (LuTx) is a critical intervention for end-stage pediatric PAH.
- The potential for RV functional recovery post-LuTx remains an important clinical question.
Purpose of the Study:
- To investigate the recovery of RV systolic function in pediatric patients following lung transplantation for PAH.
- To assess the efficacy of LuTx in reversing RV dysfunction and failure.
- To identify reliable imaging markers for RV function assessment post-transplant.
Main Methods:
- Prospective observational study of 15 children (1.9-17.6 years) undergoing bilateral LuTx for PAH.
- Advanced echocardiography (Echo) and cardiac magnetic resonance imaging (MRI) were performed pre- and ~6 weeks post-LuTx.
- Analysis included conventional imaging, strain analysis, and RV/LV ratios.
Main Results:
- RV volumes and systolic function (RVEF) normalized completely post-LuTx (63% vs 30%, p < 0.05) by MRI.
- RV/LV end-systolic diameter and volume ratios by Echo and MRI were excellent diagnostic tools for RV dysfunction.
- RV 2D longitudinal, radial, and circumferential strain significantly improved post-LuTx.
Conclusions:
- Full recovery of RV systolic function is demonstrated in children within two months after LuTx for severe PAH.
- Recovery is independent of age, weight, or pre-transplant hemodynamic status.
- LuTx should be prioritized over heart-lung transplantation for pediatric end-stage PAH with RV failure.
Background:
We investigated whether RV function recovers in children with pulmonary arterial hypertension (PAH) and RV failure undergoing lung transplantation (LuTx).
Methods:
Prospective observational study of 15 consecutive children, 1.9 to 17.6 years old, with PAH undergoing bilateral LuTx. We performed advanced echocardiography (Echo) and cardiac magnetic resonance imaging (MRI), followed by conventional and strain analysis, pre- and ∼6 weeks post-LuTx.
Results:
After LuTx, RV/LV end-systolic diameter ratio (Echo), RV volumes and systolic RV function (RVEF 63 vs 30 %; p < 0.05) by MRI completely normalized, even in children with severe RV failure (RVEF < 40%). The echocardiographic end-systolic LV eccentricity index nearly normalized post-LuTx (1.0 vs 2.0, p < 0.0001) while RV hypertrophy regressed more slowly and was still evident. We found especially the end-systolic RV/LV ratios by Echo (diameter: 0.6 vs 2.6) or MRI (volumes: 0.8 vs 3.4) excellent diagnostic tools (p < 0.05): Together with RVEF by MRI, these ratios were superior to tricuspid annular plane systolic excursion (TAPSE; p = 0.4551) in assessing global systolic RV dysfunction. Moreover, children with severe PAH had reduced RV 2D longitudinal strain (Echo, MRI; p = 0.0450) and decreased RV 2D radial and circumferential strain (MRI; p = 0.0026 and p = 0.0036 respectively), all of which greatly improved following LuTx.
Conclusion:
We demonstrate full recovery of RV systolic function in children within two months after LuTx for severe PAH, independently of the patients' age, weight, and hemodynamic compromise preceding the LuTx. Even in end-stage pediatric PAH with poor RV function and low cardiac output, LuTx should be preferred over heart-lung transplantation.
