Zusammenfassung
Die chronisch thrombembolische pulmonale Hypertonie stellt eine häufige Form der pulmonalen Hypertonie dar, die als Spätfolge nach symptomatischen und symptomlosen Lungenarterienembolien auftritt und mit einer hohen Morbidität und Mortalität assoziiert ist. Die Prognose der Erkrankung wird maßgeblich durch das Ausmaß der rechtsventrikulären Dysfunktion bestimmt. Eine unvollständige Revaskularisation des Gefäßbetts sowie möglicherweise auch eine In-situ-Thrombosierung führen zu einer Verminderung des Gefäßbettdurchmessers, aus der eine veränderte Druck- und Volumenbelastung der noch perfundierten Gefäße resultiert. Dies kann in den noch perfundierten Arealen ähnliche Prozesse auslösen, wie sie bei der pulmonalarteriellen Hypertonie beschrieben sind. Allerdings sind die zum Umbau der Pulmonalarterien beitragenden Pathomechanismen noch unzureichend verstanden. Therapeutisch sollte prinzipiell die Endarteriektomie des organisierten thrombotischen Materials bei gegebener Indikation angestrebt werden. Bei inoperablen Patienten oder postoperativ persistierender pulmonaler Hypertonie sind die für die pulmonalarterielle Hypertonie zugelassenen medikamentösen Therapien in Erwägung zu ziehen. Diese könnten Symptomatik und Langzeitüberleben verbessern, allerdings bleibt dies in kontrollierten Studien zu prüfen.
Abstract
Chronic thrombembolic pulmonary hypertension (CTEPH) represents a common type of pulmonary hypertension and is associated with significant morbidity and mortality. The prognosis of this condition reflects the degree of associated right ventricular dysfunction. Unresolved thrombemboli and possible in situ thrombosis lead to obstruction of pulmonary arteries resulting in elevated pressures in those areas of the vasculature that were spared from thromboembolic occlusion. This can resemble pathomechanisms in patients with pulmonary arterial hypertension. However the understanding of pulmonary vascular remodeling in patients with CTEPH is incomplete. Pulmonary endarterectomy of the obstructing thromboembolic material should be performed in patients who are accessible to surgery. In patients who are judged inoperable or with persistent pulmonary hypertension treatment with pulmonary arterial hypertension medications can be considered. This could contribute to improved clinical outcome and survival, whereas further controlled studies are required addressing this question.
Literatur
Bauer M, Wilkens H, Langer F et al. (2002) Selective upregulation of endothelin B receptor gene expression in severe pulmonary hypertension. Circulation 105: 1034–1036
Bonderman D, Nowotny R, Skoro-Sajer N et al. (2005) Bosentan therapy for inoperable chronic thromboembolic pulmonary hypertension. Chest 128: 2599–2603
Bonderman D, Skoro-Sajer N, Jakowitsch J et al. (2007) Predictors of outcome in chronic thromboembolic pulmonary hypertension. Circulation 115: 2153–2158
Condliffe R, Kiely DG, Gibbs JS et al. (2008) Improved outcomes in medically and surgically treated chronic thromboembolic pulmonary hypertension. Am J Respir Crit Care Med 177: 1122–1127
Dartevelle P, Fadel E, Mussot S et al. (2004) Chronic thromboembolic pulmonary hypertension. Eur Respir J 23: 637–648
Dorfmuller P, Zarka V, Durand-Gasselin I et al. (2002) Chemokine RANTES in severe pulmonary arterial hypertension. Am J Respir Crit Care Med 165: 534–539
Fartoukh M, Emilie D, Le Gall C et al. (1998) Chemokine macrophage inflammatory protein-1alpha mRNA expression in lung biopsy specimens of primary pulmonary hypertension. Chest 114 (Suppl): 50S–51S
Fedullo PF, Auger WR, Kerr KM, Rubin LJ (2001) Chronic thromboembolic pulmonary hypertension. N Engl J Med 345: 1465–1472
Ghofrani HA, Schermuly RT, Rose F et al. (2003) Sildenafil for long-term treatment of nonoperable chronic thromboembolic pulmonary hypertension. Am J Respir Crit Care Med 167: 1139–1141
Hoeper MM, Kramm T, Wilkens H et al. (2005) Bosentan therapy for inoperable chronic thromboembolic pulmonary hypertension. Chest 128: 2363–2367
Hoeper MM, Mayer E, Simonneau G, Rubin LJ (2006) Chronic thromboembolic pulmonary hypertension. Circulation 113: 2011–2020
Hughes R, George P, Parameshwar J et al. (2005) Bosentan in inoperable chronic thromboembolic pulmonary hypertension. Thorax 60: 707
Jais X, D’Armini AM, Jansa P et al. (2008) Bosentan for treatment of inoperable chronic thromboembolic pulmonary hypertension: BENEFiT (Bosentan Effects in iNopErable Forms of chronIc Thromboembolic pulmonary hypertension), a randomized, placebo-controlled trial. J Am Coll Cardiol 52: 2127–2134
Jamieson SW, Kapelanski DP, Sakakibara N et al. (2003) Pulmonary endarterectomy: experience and lessons learned in 1,500 cases. Ann Thorac Surg 76: 1457–1462
Kimura H, Okada O, Tanabe N et al. (2001) Plasma monocyte chemoattractant protein-1 and pulmonary vascular resistance in chronic thromboembolic pulmonary hypertension. Am J Respir Crit Care Med 164: 319–324
Klepetko W, Mayer E, Sandoval J et al. (2004) Interventional and surgical modalities of treatment for pulmonary arterial hypertension. J Am Coll Cardiol 43 (Suppl): 73S–80S
Lang IM (2004) Chronic thromboembolic pulmonary hypertension-not so rare after all. N Engl J Med 350: 2236–2238
Lang IM (2008) Chronic thromboembolic pulmonary hypertension (CTEPH). Dtsch Med Wochenschr 133 (Suppl): S206–S208
Lang IM, Klepetko W (2008) Chronic thromboembolic pulmonary hypertension: an updated review. Curr Opin Cardiol 23: 555–559
Moser KM, Auger WR, Fedullo PF (1990) Chronic major-vessel thromboembolic pulmonary hypertension. Circulation 81: 1735–1743
Moser KM, Bloor CM (1993) Pulmonary vascular lesions occurring in patients with chronic major vessel thromboembolic pulmonary hypertension. Chest 103: 685–692
Pengo V, Lensing AW, Prins MH et al. (2004) Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism. N Engl J Med 350: 2257–2264
Ribeiro A, Lindmarker P, Johnsson H et al. (1999) Pulmonary embolism: one-year follow-up with echocardiography doppler and five-year survival analysis. Circulation 99: 1325–1330
Riedel M, Stanek V, Widimsky J, Prerovsky I (1982) Longterm follow-up of patients with pulmonary thromboembolism. Late prognosis and evolution of hemodynamic and respiratory data. Chest 81: 151–158
Thistlethwaite PA, Kaneko K, Madani MM, Jamieson SW (2008) Technique and outcomes of pulmonary endarterectomy surgery. Ann Thorac Cardiovasc Surg 14: 274–282
Wolf M, Boyer-Neumann C, Parent F et al. (2000) Thrombotic risk factors in pulmonary hypertension. Eur Respir J 15: 395–399
Yi ES, Kim H, Ahn H et al. (2000) Distribution of obstructive intimal lesions and their cellular phenotypes in chronic pulmonary hypertension. A morphometric and immunohistochemical study. Am J Respir Crit Care Med 162: 1577–1586
ZuWallack RL, Liss JP, Lahiri B (1976) Acquired continuous murmur associated with acute pulmonary thromboembolism. Chest 70: 557–559
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Drömann, D. Thrombembolien und pulmonale Hypertonie. Internist 50, 1080–1085 (2009). https://doi.org/10.1007/s00108-009-2338-6
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DOI: https://doi.org/10.1007/s00108-009-2338-6
Schlüsselwörter
- Chronisch thrombembolische pulmonale Hypertonie
- Pulmonale Arteriopathie
- Pulmonale Hypertonie
- Embolie
- Pulmonale Endarteriektomie