Zusammenfassung
Die pulmonale Hypertonie (PH) ist die hämodynamische Konsequenz multipler Ätiologien und verschiedener Pathomechanismen im Rahmen vieler pulmonaler und extrapulmonaler Erkrankungen, die zu einer Zunahme des Lungengefäßwiderstandes und bei chronischem Verlauf zu Veränderungen der Lungengefäßstruktur führen. Diese hämodynamische Abnormalität übt ihre klinischen Effekte vorwiegend auf den rechten Ventrikel und den pulmonalen Gasaustausch aus. Klinische Manifestationen, Verlauf, Reversibilität und Therapie der PH sind abhängig von Ätiologie, Art der pulmonal-arteriellen Läsionen und Schweregrad der Erkrankung. Die chirurgische Behandlung der PH zielt grundsätzlich immer auf die zugrunde liegende Erkrankung der Lungen, der Gefäße, oder des linken Herzens. Bei schwerer thromboembolischer Obstruktion größerer Lungengefäße sind die Embolektomie (bei akuter Lungenembolie) und die Thrombendarteriektomie (bei chronischer thromboembolischer PH) etablierte Verfahren. In fortgeschrittenen lebensbedrohlichen Stadien der chronischen PH, unabhängig davon, ob es sich um eine primäre pulmonale Hypertonie oder eine sekundäre Form bei rezidivierenden Lungenembolien oder angeborenen Shunt-Vitien handelt, ist nach Ausschöpfung aller konservativer Therapieoptionen die Lungentransplantation (ggf. Herz-Lungen-Transplantation) die einzig mögliche Therapie, die rund 50% dieser ansonsten nicht überlebensfähigen Patienten eine Überlebenschance von über 5 Jahren bietet. Bei angeborenen Shunt-Vitien kann eine rechtzeitige operative Korrektur, die Entwicklung einer sekundären PH verhindern. Bei der chirurgischen Therapie der Linksherzinsuffizienz spielen Ausmaß und potentielle Reversibilität der sekundären PH eine maßgebende Rolle bei der Entscheidung für eine Transplantation oder den Einsatz mechanischer linksventrikulärer (LVAD) oder biventrikulärer (BVAD) Unterstützungssysteme. Auch bei Patienten mit PH, wo die chirurgische Therapie für das Langzeitüberleben der Patienten entscheidend ist, kann eine zumindest vorübergehende spezifische pulmonalgefäßerweiternde Therapie mit NO, Prostanoiden und/oder Endothelin- Rezeptorantagonisten, (insbesondere als „bridging to transplant“ oder bei residueller PH nach Thrombendarteriektomie oder LVAD ) im Einzelfall entscheidend für den gesamten Therapieerfolg sein.
Summary
Pulmonary hypertension (PH) is the final hemodynamic result of changes in vascular tone and structure during many pulmonary and extrapulmonary diseases, with the relative contribution of each dependent upon the etiology and mechanisms of the increased vascular resistance. This hemodynamic abnormality exerts its clinical effects mainly on the right ventricle and pulmonary gas exchange. Clinical manifestations, natural history, reversibility and treatment depend mainly on the etiology and level of PH and also on the nature of the pulmonary arterial lesions. Surgical treatment of PH is basically directed at the pulmonary, vascular or left heart disease responsible for the development of PH. For severe thromboembolic obstruction of larger pulmonary arteries, embolectomy (for acute pulmonary emboli) and thromboendarterectomy (for chronic thromboembolic PH) become the therapeutic approaches of choice. For life-threatening end-stage chronic PH, regardless of whether it is primary PH or secondary PH associated with recurrent acute pulmonary emboli, collagen vascular disease or congenital systemic to pulmonary shunts, lung transplantation (or heart-lung transplantation) is the only possible therapy and provides about 50% survival chance for at least 5 years. In young patients with congenital systemic to pulmonary shunts, early surgical repair will impede the development of PH.
For successful surgical treatment in patients with end-stage heart failure, as well as for the decision in favor of transplantation or mechanical circulatory support (left ventricular or biventricular assist device), both severity and reversibility of secondary PH are of paramount importance. Even in patients with PH in whom surgical treatment is necessary for long-term survival, specific pulmonary vasodilative therapy with nitric oxide, prostanoids and/or endothelin receptor antagonists (especially as “bridge to transplant”; or for the treatment of residual PH after thromboendarterectomy or LVAD) may be decisive for the success of therapy. Treatment of severe primary PH should always be initiated in a specialist center, preferably with the capacity for thoracic surgery and transplantation; otherwise early collaboration with a transplant center is vital.
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Dandel, M., Hetzer, R. Chirurgische Aspekte des pulmonalen Hochdrucks. Z Herz- Thorax- Gefäßchir 19, 57–74 (2005). https://doi.org/10.1007/s00398-005-0491-3
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DOI: https://doi.org/10.1007/s00398-005-0491-3
Schlüsselwörter
- Pulmonal-arterielle Hypertonie
- Akute Lungenembolie
- chronische thromboembolische pulmonale Hypertonie
- pulmonale Hypertonie bei Linksherzinsuffizienz
- Transplantation