Pulmonary Hypertension and Lung Transplantation

Abstract

Purpose

Pulmonary artery hypertension (PAH), a subtype of pulmonary hypertension, is a rare end-stage lung disease. Bilateral lung and combined heart and lung transplantation have long been considered as a gold standard therapy for PAH.

Methods

This manuscript reviewed the most up-to-date literature on lung transplantation for PAH, focusing particularly on risk stratification, donor allocation, bridging to transplantation (BTT), and intra- and postoperative management in both adult and pediatric patients.

Results

The last two decades have witnessed an important shift in the transplant indications and strategy for PAH. Newly introduced antihypertensive drugs have postponed the time of transplantation in eligible patients, thus reserving transplantation for severely ill high-risk patients on triple antihypertensive therapy. Furthermore, the widespread peritransplant use of veno-arterial extracorporeal membrane oxygenation (ECMO) enables cardiac remodeling after bilateral lung transplantation.

Conclusion

The change in transplant strategy from combined heart and lung transplantation to bilateral lung transplantation renders more organs available for transplantation in an era of organ shortage. ECMO-bridging is a life-saving tool in selected PAH patients although associated with higher complications. Better organ allocation in PAH patients at high risk of decompensation may reduce the need for ECMO-bridging and may further improve outcomes.

Zusammenfassung

Hintergrund

Die pulmonal arterielle Hypertonie (PAH) ist als Untergruppe der pulmonalen Hypertonie eine seltene terminale Lungenerkrankung. Die bilaterale Lungentransplantation und kombinierte Herz-Lungen-Transplantation stellten langzeitig den Goldstandard der Therapie dar.

Methoden

Dieses Review fasst die aktuelle Literatur zur Lungentransplantation bei PAH-Patienten zusammen, fokussiert auf Risikostratifizierungen, Allokation der Spenderorgane, Möglichkeiten der Überbrückung zur Transplantation – die sog. Bridge-to-Transplant-Verfahren (BTT) – sowie das intra- und postoperative Management bei adulten und pädiatrischen Patienten.

Ergebnisse

Basierend auf der Einführung neuer Therapieoptionen bei PAH-Patienten konnte der Zeitpunkt der Transplantation innerhalb der letzten Jahrzehnte verzögert werden. Neue antihypertensive Medikamente ermöglichen bei geeigneten PAH-Patienten eine verlängerte Therapie ohne Transplantation, sodass diese für Patienten mit fehlendem Ansprechen auf die medikamentöse 3-fach-Kombination vorbehalten ist. Ausgehend von den zunehmenden Erfahrungen in der postoperativ verlängerten ECMO konnte ein kardiales Remodeling nach bilateraler Lungentransplantation gezeigt werden.

Schlussfolgerung

Aktuelle Fortschritte in der PAH-Therapie ermöglichen eine bilaterale Lungentransplantation anstelle der kombinierten Herz-Lungen-Transplantation, sodass in dem aktuell herrschenden Organmangel mehr Organe für andere Patienten alloziert werden können. ECMO-Bridging stellt eine lebensrettende Maßnahme bei akuter Dekompensation dar, geht jedoch mit signifikant höheren Komplikationen einher. Eine adäquate Berücksichtigung der Hochrisiko-PAH-Patienten in der Organverteilung könnte die Notwendigkeit eines ECMO-Bridgings minimieren und das Transplantations-Outcome weiter verbessern.

Keywords

transplantation surgery - transplantation - thoracic surgery - long-term survival

Schlüsselwörter

Transplantationschirurgie - Transplantation - Thoraxchirurgie - Langzeitüberleben

Publication History

Received: 17 January 2025

Accepted after revision: 07 April 2025

Article published online:
14 May 2025

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