Globally, over 4,500 lung transplants are performed annually. Pulmonary Arterial Hypertension represents a unique transplantation indication because:
- It predominantly affects the vascular compartment
- Elevated pulmonary artery pressure and right ventricular dysfunction increase perioperative risk
- Being a rare disease, it accounts for less than 5% of global lung transplants
Given these specific challenges, the authors developed guidelines for managing Pulmonary Arterial Hypertension patients who are lung transplant candidates through a modified Delphi study. The expert panel reached unanimous consensus on several key aspects of lung transplantation for pulmonary arterial hypertension patients:
Referral and Collaboration
- Close collaboration between pulmonary arterial hypertension teams and transplant programs is essential
- Early referral is recommended to allow for education and barrier mitigation
- Patients should be referred despite perceived contraindications
- Referral should not wait until all therapeutic options are exhausted
Referral Criteria
- Intermediate-high risk patients by ESC/ERS model or REVEAL 2.0 score ≥8
- WHO Functional Class III-IV despite therapy
- Patients showing right ventricular dysfunction, disease progression, or hospitalization
- Patients requiring parenteral prostacyclin or with conditions like PVOD/PCH
Contraindications and Considerations
- No specific age limit, though there should be some upper limit
- Drug-associated pulmonary arterial hypertension requires minimum 6 months sobriety
- Mild renal/liver disease acceptable, but severe disease requires combined transplantation
- Critical illness is not a contraindication; ECMO can be used during evaluation
Surgical Approach
- Single lung transplant not recommended for pulmonary arterial hypertension
- Heart-lung transplantation reserved for specific conditions (complex congenital heart disease)
- Bilateral lung transplantation is the preferred approach
Perioperative Management
- Veno-arterial ECMO recommended for bridging
- Prophylactic cannulation beneficial for high-risk patients
- Central veno-arterial ECMO preferred intraoperatively
- Comprehensive monitoring with arterial line and pulmonary artery catheter
- Postoperative ECMO should be guided by hemodynamics and echocardiography
The consensus emphasized that current allocation systems underestimate pulmonary arterial hypertension severity, with agreement that right ventricular function parameters should be incorporated into allocation models.
Read more at this link on Pulmonary Circulation
Citation
Approach to Lung Transplantation in Pulmonary Arterial Hypertension: A Delphi Consensus on Behalf of the Transplant Task Force of the Pulmonary Vascular Research Institute, Nicholas A. Kolaitis, Hayley Barnes, Deborah J. Levine, Howard Castillo, Selim M. Arcasoy, Matthew Bacchetta, Luke Benvenuto, Erika Berman-Rosenzweig, Marisa Cevasco, Caitlin T. Demarest, Celine Dewachter, Michiel E. Erasmus, Allan R. Glanville, John Granton, Shaf Keshavjee, Vikramjit Khangoora, Sheila Krishnan, Olaf Mercier, Andrea N. Miltiades, David Montani, Edward Murphy, Ivan Robbins, Franck F. Rahaghi, Sahar A. Saddoughi, Laurent Savale, Marc A. Simon, Jean-Luc Vachiery, Corey E. Ventetuolo, Helen M. Whitford, Reda E. Girgi, Pulmonary Circulation, First published: 23 April 2025, https://doi.org/10.1002/pul2.70088
