“LUNG AND HEART-LUNG TRANSPLANTATION IN PULMONARY HYPERTENSION”, March 18, 2024
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ROBERT PLETICHA, MODERATOR
Welcome, and thank you all for joining us today. My name is Rob, a project manager at admedicum. We are thrilled to kick off our first webinar of 2024 in collaboration with the Alliance for Pulmonary Hypertension, for which we serve as the secretariat. The Alliance is a Brussels-registered nonprofit dedicated to knowledge and expertise sharing in pulmonary hypertension. Today, our esteemed speakers will delve into critical aspects of lung and heart-lung transplantation for pulmonary hypertension, including the indications, surgical techniques, post-transplant challenges, and patient perspective.. We encourage you to post your questions in the chat box. After the presentations, we will address these questions collectively. Please look forward to the recording and most slides being available on our knowledge sharing platform in the coming weeks. Our next webinar, scheduled for April 29th, will cover surgical interventions in chronic thromboembolic pulmonary hypertension (CTEPH), featuring world-renowned experts Professors David Jenkins and Hiromi Matsubara. Without further ado, Laurent, the floow is yours.
PROF. LAURENT SAVALE
Thank you, Rob, for that warm introduction. It’s an honor to participate in today’s webinar. I am a pulmonologist specializing in pulmonary hypertension at the French National Reference Center and I oversee the intensive care unit, focusing on preparing patients for lung transplantation. Today, I will discuss the screening process and the journey towards lung transplantation. Despite advancements in medical therapy for pulmonary arterial hypertension, which have significantly improved the prognosis and quality of life, this condition remains irreversible, making lung transplantation a vital option for patients unresponsive to optimal medical therapy. We recommend considering transplantation early in patient care due to its complex nature.
The Challenges and Data on Lung Transplantation
Lung transplantation for pulmonary arterial hypertension patients remains challenging due to specific pre and post-operative strategies, differences in prognostic parameters compared to other conditions, and potential inequities in organ allocation. Recent data from the French National Reference Center indicates that 5% of PH patients underwent lung transplantation over the last decade, highlighting the procedure’s rarity yet importance.
Eligibility and Timing for Transplantation
Determining eligibility and the optimal timing for listing involves a multi-parametric approach and consideration of the patient’s specific context. It’s crucial to introduce the concept of lung transplantation early, especially for patients with severe conditions requiring maximal treatment. The treatment algorithm for pulmonary hypertension [in the 2022 ESC/ERS clinical guidelines], emphasizes risk stratification to identify non-responders to therapy, indicating the need for transplantation consideration.
Lung Transplant Assessment
The lung transplant assessment aims to screen for absolute and relative contraindications, determine the type of transplantation needed, and jointly decide with the patient on proceeding based on informed risks and benefits. Listing for transplantation depends on the patient’s risk and therapeutic situation, with specific conditions warranting immediate consideration.
Navigating Healthcare Systems and Graph Allocation Rules
Access to lung transplantation varies significantly across countries, influenced by healthcare systems and graft allocation rules. It’s essential to navigate these differences to optimize patient outcomes.
Let’s discuss the evolution and challenges of the Lung Allocation Score (LAS) system in providing equitable access to lung transplants for pulmonary arterial hypertension patients. Initially, the LAS system showed disparities in access for pulmonary arterial hypertension patients, with significantly fewer transplants compared to other conditions. However, subsequent revisions incorporated specific parameters for pulmonary arterial hypertension, improving access and outcomes.
Revising the Lung Allocation Score
The initial Lung Allocation Scores (LAS) system’s inadequacies became apparent when analyzing the data ten years post-implementation. Pulmonary arterial hypertension patients had disproportionately lower transplantation rates. Recognizing this, adjustments were made to give greater weight to pulmonary arterial hypertension-specific parameters, enhancing these patients’ access to transplant.
Introduction of the Composite Allocation Score
Recently, a significant shift occurred with the introduction of the Composite Allocation Score, balancing the LAS and recipient-specific biological factors. This new system potentially benefits pulmonary arterial hypertension patients by considering their unique condition more comprehensively, though its long-term impact remains to be assessed.
Urgent Access Systems for PAH Patients
A major advancement has been the implementation of systems granting urgent access to lung transplants for pulmonary arterial hypertension patients experiencing acute decompensation. This approach, combined with extracorporeal life support advancements, has markedly improved outcomes for critically ill pulmonary arterial hypertension patients.
The French High Priority Allocation Program
In France, the establishment of a high-priority allocation program for pulmonary arterial hypertension patients in life-threatening conditions has significantly improved access to lung transplants. Post-program data shows a dramatic increase in transplant access, from 50% to over 80% for listed patients.
Conclusion
In conclusion, while accessing lung transplantation for pulmonary arterial hypertension remains complex and highly variable across healthcare systems, considerable progress has been made. Early and ongoing discussion about transplantation as a treatment option is crucial. The decision to refer a patient for transplantation should be a joint one, made in consultation with the patient and medical team, taking into account a comprehensive risk assessment and the anticipated waiting times. Urgent transplant programs have been pivotal in improving access for pulmonary arterial hypertension patients. Our collective efforts continue to push the boundaries, ensuring better outcomes for those in need. Thank you for your attention.
PROF. KONRAD HOETZENECKER
I’m Professor Konrad Hoetzenecker, the surgical head of the Vienna Lung Transplant Program at the Medical University of Vienna. Before I start, I must disclose my consultancy with Medtronic, unrelated to pulmonary arterial hypertension treatments. All patients mentioned have consented to the use of their photographs. My talk is structured into three parts: indications and timing for lung transplantation, choice of procedure, and post-operative management focusing on cardiac remodeling challenges in the Intensive Care Unit.
The Vienna Lung Transplant Program
We perform between 100 and 120 lung transplants annually, making us one of Europe’s largest centers. Pulmonary arterial hypertension patients constitute about 8-9% of our cases, significantly higher than the global average reported by the International Society of Heart and Lung Transplantation, where pulmonary arterial hypertension accounts for only 2.8% of lung transplants.
Indications and Optimal Timing for Transplantation
Lung transplantation is reserved for chronic or acute end-stage lung diseases that have exhausted all other medical options. It’s crucial not to refer patients too early or too late, especially in pulmonary arterial hypertension, where timely referral can significantly impact outcomes. Recent guidelines have increasingly emphasized lung transplantation in the treatment algorithm for pulmonary arterial hypertension, reflecting its growing importance.
Challenges with Late Referrals
Late referrals present significant challenges, as demonstrated by cases of patients in severe right heart failure. Early referral, when the patient is more stable, significantly enhances transplant success rates.
Choosing the Right Procedure
Historically, pulmonary arterial hypertension was treated with heart-lung transplantation, but advancements have made double lung transplants the standard of care, avoiding the need for heart transplantation. This approach relies on the heart’s ability to undergo remodeling post-transplant. Single lung transplants are not recommended for PAH due to hemodynamic instability and the risk of early graft dysfunction.
Cardiac Remodeling Post-Transplant
Cardiac remodeling is a critical phase of recovery, focusing on the right ventricle’s adaptation post-transplant. Successful management of this phase is essential for the patient’s recovery and long-term outcomes.
Post transplant Management
Addressing post-transplant management, our focus extends beyond the right ventricle to how the left ventricle adapts to new conditions, and the overall graft health. A pivotal advancement in post-transplant care for PAH patients has been the implementation of prolonged veno-arterial ECMO (Extra Corporeal Membrane Oxygenation). This technique, essentially a more compact form of cardiopulmonary bypass, drains blood from the venous system and reintroduces it into the arterial system, bypassing the pulmonary circulation. This support allows the heart previously damaged by pulmonary arterial hypertension to recover.
A typical setup involves an additional small incision for inserting two cannulas into the femoral artery and vein, with a third cannula to ensure leg perfusion. Patients may require this ECMO support for two to three weeks post-operation, significantly stabilizing cardiac function and facilitating smoother recovery.
Our experience, supported by recent studies, shows that a post-operative prolonged ECMO strategy significantly improves cumulative survival. We now achieve one-year survival rates of 90% for pulmonary arterial hypertension patients undergoing lung transplantation. This approach, echoed by other transplant centers, has also shown a reduction in early post-transplant mortality, offering five-year survival rates of 70-75%.
However, survival rates alone don’t capture the full picture. Quality of life improvements post-transplant are dramatic, with patients resuming activities previously impossible due to pulmonary arterial hypertension. An illustrative example of the potential for post-transplant life quality is the ascent of Mount Kilimanjaro by a group of lung transplant recipients, including pulmonary arterial hypertension patients, led by our lead pulmonologist, Peter Jaksch, in 2017. We’re now planning an expedition to Aconcagua, further highlighting the remarkable outcomes possible post-transplant.
In conclusion, lung transplantation for pulmonary arterial hypertension in experienced centers now sees one-year survival rates above 90%, with five-year rates approaching 80%. Early referral to a lung transplant center post-initial treatment failure is crucial to determine candidacy and ensure optimal outcomes for PAH patients.
DR. ANDREAS REIMANN, MODERATOR
Thank you, Professor Hoetzenecker, for sharing these impressive results and inspiring stories. It’s encouraging to hear about the transformational impact lung transplantation can have on pulmonary arterial hypertension patients. Florence, as someone who has gone through this journey, could you share your experience? How has the transplant changed your quality of life and your perspective on your condition?
FLORENCE PERRIN-SZABO
Indeed, I have quite the story to share. Hello, everyone. My name is Florence Zsabo. Before my life took a dramatic turn, I was a nearly 40-year-old married mother of three, working as a financing pre-sales manager at a major computer company in Paris. My ordeal began with sudden breathlessness one month after the birth of my third child in August 2005. By October, the diagnosis was swift: probable pulmonary arterial hypertension, confirmed shortly after by Professor Simonneau at the hospital in France.
After five years of triple therapy without significant improvement, my condition worsened. By September 2010, I was listed for transplantation. A pre-transplant evaluation revealed high levels of antibodies, complicating my case. By January 2011, despite undergoing plasmapheresis in an attempt to manage these antibodies, my condition did not improve. That month, as I faced the prospect of returning home without a solution, I began writing my “Chronicles of Declaire,” detailing my hospital stay and profiling the medical team. This not only helped me pass the time but also enriched my interactions with family, friends, and the medical team.
In a twist of fate, the transplantation team, inspired by new approaches discussed at an international congress, proposed a novel strategy involving plasmapheresis during the operation, supported by ECMO. Miraculously, a graft became available on February 8th, and I underwent transplantation that night.
Waking up ten days later, I faced my greatest challenge yet: rebuilding my life. Despite the fear and the multitude of tubes, I felt no pain. My recovery involved learning to eat, gaining weight, regaining muscle strength, and understanding the plethora of medications prescribed to me. My family’s unwavering support was my pillar during this time.
Despite a few health setbacks, including a mild transplant rejection and an aspergillus infection, my journey over the past 13 years has been one of gratitude and resilience. Today, my life is vastly different. I no longer work, focusing instead on enjoying every moment of this second chance at life. I actively participate in my medical care, understanding and managing my medications and their side effects.
In summary, my lung transplantation journey has been transformative, marked by challenges, learning, and immense gratitude. I cherish each day, committed to making the most of this extraordinary gift of life.
My life post-transplant is a blend of medical vigilance and embracing normalcy. My regimen now includes minimal anti-rejection medication, occasional antibiotics, and regular check-ups with various specialists. However, my life isn’t defined by my transplant. I engage in activities like yoga, Nordic walking, biking, and even skiing. I also pursue education, volunteer, and participate actively in our national pulmonary hypertension association. This morning’s check-up was reassuring, and my pulmonologist encouraged my skiing plans. I am profoundly grateful to my donor, whose generosity has enabled this second chance at life.
DR. ANDREA REIMANN
Florence, your openness and emotional depth in sharing your journey are truly moving. It’s a poignant reminder of the profound impact donors have on recipients’ lives. Today also marks the launch of Germany’s voluntary organ transplant registry, a significant step towards improving transplant rates.
LIVE Q & A
Discussion on Healthcare System Variations and Genetic Factors in Transplant Success
Questions from the audience addressed the disparities in lung allocation scores across Europe and the potential genetic influences on transplant success and rejection. The responses highlighted the complexity of healthcare systems, the need for centralized expertise in treating pulmonary arterial hypertension, and the current lack of evidence linking genetic factors to transplant outcomes.
Advice for pulmonary arterial hypertension patients considering transplantation
Florence, Laure, and Konrad shared insights on the importance of early, well-considered referrals to transplantation centers. They emphasized the patient’s mindset and determination as crucial to recovery and the invaluable role of centers of excellence in managing complex cases like Florence’s.
Collaboration with Patient Organizations
The discussion also touched on the collaboration with patient organizations, underscoring the benefits of connecting prospective transplant recipients with those who have undergone the procedure. Such interactions can provide invaluable support and practical insights into life post-transplant.
Closing Remarks
The webinar concluded with reminders of upcoming events and thanks to all participants for their contributions. Florence’s story, alongside expert insights from Laure and Conrad, offered a comprehensive overview of the challenges and triumphs associated with lung transplantation for pulmonary arterial hypertension, highlighting the essential collaboration between patients, medical professionals, and support networks in navigating this life-changing process.