Abstract
Prior to the development of pulmonary endarterectomy (PEA), a surgical removal of organized chronic thrombi, there had long been no efficacious treatment to improve the prognosis of patients with chronic thromboembolic pulmonary hypertension (CTEPH). Anticoagulants are initiated when CTEPH is diagnosed to prevent progression, and treatment of hypoxia and right heart failure are added when these complications occur. In the meantime, because small pulmonary arterioles in CTEPH have the same histological abnormality as those in pulmonary arterial hypertension (PAH), which has been called “small vessel disease,” the pulmonary vasodilators used in PAH have started to be administered to patients with inoperable CTEPH with peripheral lesions. Retrospective studies showed effectiveness of these vasodilators in improving the prognosis of CTEPH, and riociguat, a newly developed soluble guanylate cyclase (sGC) stimulator, demonstrated significant clinical benefit in 6-min walk distance (6MWD) and pulmonary hemodynamics. However, the effectiveness of vasodilators in patients with operable CTEPH is not known.
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References
Piazza G, Goldhaber SZ. Chronic thromboembolic pulmonary hypertension. N Engl J Med. 2011;364:351–60.
Hoeper MM. Pharmacological therapy for patients with chronic thromboembolic pulmonary hypertension. Eur Respir Rev. 2015;136:272–82.
Galiè N, Hoeper MM, Humbert M, Torbicki A, Vachiery J-A, Barbera JA, Beghetti M, Corris P, Gaine S, Gibbs JS, Gomez-Sanchez MA, Jondeau G, Klepetko W, Opitz C, Peacock A, Rubin L, Zellweger M, Simonneau G, Task Force for Diagnosis and Treatment of Pulmonary Hypertension of European Society of Cardiology (ESC), European Respiratory Society (ERS), International Society of Heart and Lung Transplantation (ISHLT). Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J. 2009;34:1219–63.
Limbrey R, Howard L. Developments in the management and treatment of pulmonary embolism. Eur Respir Rev. 2015;24(137):484–97.
Marik PE, Cavallazi R. Extended anticoagulant and aspirin treatment for the secondary prevention of thromboembolic disease: a systematic review and meta-analysis. PLoS One. 2015;10(11).
Yi ES, Kim H, Ahn H, Strother J, Morris T, Masliah E, Hansen LA, Park K, Friedman PJ. Distribution of obstructive intimal lesions and their cellular phenotypes in chronic pulmonary hypertension. A morphometric and immunohistochemical study. Am J Respir Crit Care Med. 2000;162:1577–86.
Galie N, Humbert M, JL V, Gibbs S, Lang I, Torbicki A, Simonneau G, Peacock A, Vonk Noordegraaf A, Beghetti M, Ghofrani A, MA GS, Hansmann G, Klepetko W, Lancellotti P, Matucci M, McDonagh T, LA P, PT T, Zompatori M, Hoeper M. Chronic thromboembolic pulmonary hypertension (group 4) 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Respir J. 2015;46(4):942–5.
Nishimura R, Tanabe N, Sugiura T, Shigeta A, Jujo T, Sekine A, Sakao S, Kasahara Y, Tatsumi K. Improved survival in medically treated chronic thromboembolic pulmonary hypertension. Circ J. 2013;77(8):2110–7.
Freed DH, Thomson BM, Berman M, et al. Survival after pulmonary thromboendarterectomy: effect of residual pulmonary hypertension. J Thorac Cardiovasc Surg. 2011;141:383–7.
Suntharalingam J, Machado RD, Sharples LD, et al. Demographic features, BMPR2 status and outcomes in distal chronic thromboembolic pulmonary hypertension. Thorax. 2007;62:617–22.
Condliffe R, Kiely DG, Gibbs JS, et al. Improved outcomes in medically and surgically treated chronic thromboembolic pulmonary hypertension. Am J Respir Crit Care Med. 2008;177:1122–7.
Riedel M, Stanek V, Widimsky J, Prerovsky I. Longterm follow-up of patients with pulmonary thromboembolism. Late prognosis and evolution of hemodynamic and respiratory data. Chest. 1982;81(2):151–8.
Ghofrani HA, D’Armini AM, Grimminger F, Hoeper MM, Jansa P, Kim NH, Mayer E, Simonneau G, Wilkins MR, Fritsch A, Neuser D, Weimann G, Wang C. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension. N Engl J Med. 2013;369:319–29.
Jaïs X, D’Armini AM, Jansa P, et al. 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. 2008;52:2127–34.
Denninger JW, Marletta MA. Guanylate cyclase and the c NO/cGMP signaling pathway review. Biochim Biophys Acta. 1999;1411:334–50.
Rubin LJ, Galiè N, Grimminger F, Grünig E, Humbert M, Jing ZC, Keogh A, Langleben D, Fritsch A, Menezes F, Davie N, Ghofrani HA. Riociguat for the treatment of pulmonary arterial hypertension: a long-term extension study (PATENT-2). Eur Respir J. 2015;45(5):1303–13.
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Satoh, T. (2017). Medical Therapy for Chronic Thromboembolic Pulmonary Hypertension. In: Fukumoto, Y. (eds) Diagnosis and Treatment of Pulmonary Hypertension. Springer, Singapore. https://doi.org/10.1007/978-981-287-840-3_14
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DOI: https://doi.org/10.1007/978-981-287-840-3_14
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