Abstract
Over the past quarter century, the recognition, evaluation, and management of patients with chronic thromboembolic pulmonary hypertension (CTEPH) have experienced considerable forward strides. This has been accompanied by a better understanding of the risk factors associated with the development of this disorder and an appreciation that CTEPH is more prevalent than previously acknowledged. Investigative efforts have also begun to elucidate the mechanisms by which acute thrombi organize within the pulmonary vascular bed and how this may initiate the pulmonary vascular changes seen in this disorder. These efforts have become “global endeavours” (Jenkins et al. (Eur Respir J 41:735–742, 2013), expanding the ability to care for a greater number of patients afflicted with this previously fatal disorder. However, many challenges remain. The selection criteria for patients who might be considered candidates for thromboendarterectomy, the therapy which offers the greatest chance for a cure, continues to vary considerably. Though the availability of pulmonary hypertensive medical therapy has expanded over the past decade, the patients with CTEPH who would be best treated with these medications remain incompletely defined. And as percutaneous pulmonary angioplasty has resurfaced as a therapeutic option, its role in the care and management of CTEPH patients has to be more clearly established. This chapter provides an overview of the most recent information on the natural history, evaluation, and management of patients with chronic thromboembolic disease.
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Alfakih K, Reid S, Jones T et al (2004) Assessment of ventricular function and mass by cardiac magnetic resonance imaging. Eur Radiol 14:1813–1822
Auger WR, Fedullo PF, Moser KM, Buchbinder M, Peterson KL (1992) Chronic major- vessel chronic thromboembolic pulmonary artery obstruction: appearance at angiography. Radiology 183:393–398
Bailey CL, Channick RN, Auger WR, Fedullo PF, Kerr KM, Yung GL, Rubin LJ (2000) “High probability” perfusion lung scans in pulmonary venoocclusive disease. Am J Respir Crit Care Med 162:1974–1978
Becattini C, Agnelli G, Pesavento R, Silingardi M, Poggio R, Taliani MR, Ageno W (2006) Incidence of chronic thromboembolic pulmonary hypertension after a first episode of pulmonary embolism. Chest 130:172–175
Becattini C, Manina G, Busti C, Gennarini S, Agnelli G (2010) Bosentan for chronic thromboembolic pulmonary hypertension: findings from a systemic review and a meta-analysis. Thromb Res 126:e51–e56
Bernard J, Yi ES (2007) Pulmonary thromboendarterectomy: a clinicopathologic study of 200 consecutive pulmonary thromboendarterectomy cases in one institution. Hum Pathol 38:871–877
Beygui F, Furber A, Delepine S et al (2004) Routine breath-hold gradient echo MRI-derived right ventricular mass, volumes and function: accuracy, reproducibility and coherence study. Int J Cardiovasc Imaging 20:509–516
Bonderman D, Turecek PL, Jakowitsch J, Weltermann A, Adlbrecht C, Schneider B, Kneussl M, Rubun LJ, Kyrle PA, Klepetko W, Maurer G, Lang IM (2003) High prevalence of elevated clotting factor VIII in chronic thromboembolic pulmonary hypertension. Thromb Haemost 90:372–376
Bonderman D, Jakowitsch J, Adlbrecht C, Schemper M, Kyrle PA, Schonauer V, Exner M, Klepetko W, Kneussl MP, Maurer G, Lang I (2005) Medical conditions increasing the risk of chronic thromboembolic pulmonary hypertension. Thromb Haemost 93:512–516
Bonderman D, Skoro-Sajer N, Jakowitsch J, Adlbrecht C, Dunkler D, Taghavi S, Klepetko W, Kneussl M, Lang IM (2007) Predictors of outcome in chronic thromboembolic pulmonary hypertension. Circulation 115:2153–2158
Bonderman D, Wilkens H, Wakounig S, Schafers H-J, Jansa P, Linder J, Simkova I, Martischnig AM, Dudczak J, Sadushi R, Skoro-Sajer N, Klepetko W, Lang IM (2009) Risk factors for chronic thromboembolic pulmonary hypertension. Eur Respir J 33:325–331
Bresser P, Pepke-Zaba J, Jais X, Humbert M, Hoeper MM (2006) Medical therapies for chronic thromboembolic pulmonary hypertension. An evolving treatment paradigm. Proc Am Thorac Soc 3:594–600
Cabrol S, Souza R, Jais X, Fadel E, Ali RHS, Humbert M, Darteville P, Simonneau G, Sitbon O (2007) Intravenous epoprostenol in inoperable chronic thromboembolic pulmonary hypertension. J Heart Lung Transplant 26:357–362
Cappellini MD, Grespi E, Cassinerio GE, Bignamini D, Fiorelli G (2005) Coagulation and splenectomy: an overview. Ann NY Acad Sci 1054:317–324
Castaner E, Gallardo X, Ballasteros E, Andreu M, Pallardo Y, Mata JM, Riera L (2009) CT diagnosis of chronic pulmonary thromboembolism. Radiographics 29:31–50
Condliffe R, Kiely DG, Gibbs JS, Corris PA, Peacock AJ, Jenkins DP, Hodgkins D, Goldsmith K, Hughes RJ, Sheares K, Tsui SSL, Armstrong IJ, Torpy C, Crackett R, Carlin CM, Das C, Coghlan JG, Pepke-Zaba J (2008) Improved outcomes in medically and surgically treated chronic thromboembolic pulmonary hypertension. Am J Respir Crit Care Med 177:1122–1127
Corsico AG, D’Armini AM, Cerveri I, Klersy C, Ansaldo E, Niniano R, Gatto E, Monterosso C, Morsolini M, Nicolardi S, Tramontin C, Pozzi E, Vigano M (2008) Long term outcome after pulmonary thromboendarterectomy. Am J Respir Crit Care Med 178:419–424
Daily PO, Auger WR (1999) Historical perspective: surgery for chronic thromboembolic disease. Semin Thorac Cardiovasc Surg 11:143–151
Daley MJ, Lat I (2012) Clinical controversies in thrombolytic therapy for the management of acute pulmonary embolism. Pharmacotherapy 32:158–172
Darteville P, Fadel E, Mussot S, Chapelier A, Herve P, de Perrot M, Cerrina J, Ladurie FL, Lehouerou D, Humbert M, Sitbon O, Simmoneau G (2004) Chronic thromboembolic pulmonary hypertension. Eur Respir J 23:637–648
de Perrot M, Fadel E, McRae K, Tan K, Slinger P, Paul N, Mak S, Granton JT (2007) Evaluation of persistent pulmonary hypertension after acute pulmonary embolism. Chest 132:780–785
de Perrot M, McRae K, Shargall Y, Pletsch L, Tan K, Slinger P, Ma M, Paul N, Moric J, Thenganatt J, Mak S, Granton JT (2011) Pulmonary thromboendarterectomy for chronic thromboembolic pulmonary hypertension: the Toronto experience. Can J Cardiol 27:692–697
Dentali F, Donadini M, Gianni M, Bertolini A, Squizzato A, Venco A, Ageno W (2009) Incidence of chronic pulmonary hypertension in patients with previous pulmonary embolism. Thromb Res 124:256–258
Dunne B, van de Broek A, Williams V, Smith G, Revesz T, Edwards M, Gabbay E (2012) Chronic thromboembolic pulmonary hypertension: treat the patient not the haemodynamics. Case Rep Pulmonol 2012:108672
Fedullo PF, Auger WR, Kerr KM, Kim NH (2003) Chronic thromboembolic pulmonary hypertension. Semin Respir Crit Care Med 24(3):273–286
Feinstein JA, Goldhaber SZ, Lock JE, Ferndandes SM, Landzberg MJ (2001) Balloon angioplasty for treatment of chronic thromboembolic pulmonary hypertension. Circulation 103:10–13
Firth AL, Yao W, Ogawa A, Madani MM, Lin GY, Yuan JX (2010) Multipotent mesenchymal progenitor cells are present in endarterectomized tissues from patients with chronic thromboembolic pulmonary hypertension. Am J Physiol Cell Physiol 298:C1217–C1225
Freed DH, Thomson BM, Tsui SSL, Dunning JJ, Sheares KK, Pepke-Zaba J, Jenkins JJ (2008) Functional and haemodynamic outcome 1 year after pulmonary thromboendarterectomy. Eur J Cardiothorac Surg 34:525–530
Freed DH, Thomson BM, Berman M, Tsui SSL, Dunning J, Sheares KK, Pepke-Zaba J, Jenkins DP (2011) Survival after pulmonary thromboendarterectomy: effect of residual pulmonary hypertension. J Thorac Cardiovasc Surg 141:383–387
Ghofrani HA, D’Armini AM, Grimminger F, Hoeper MM, Kim NH, Mayer E, Simminneau G, Wilkins MR, Fritsch A, Neuser D, Weimann G, Wang C (2013) Riociguat for the treatment of chronic thromboembolic pulmonary hypertension. N Engl J Med 369:319–329
Hagl C, Khaladj N, Peters T, Hoeper MM, Logemann F, Haverich A, Macchiarini P (2003) Technical advances of pulmonary thromboendarterectomy for chronic thromboembolic pulmonary hypertension. Eur J Cardiothorac Surg 23:776–781
Hartz RS, Byme JG, Levitsky S, Park J, Rich S (1996) Predictors of mortality in pulmonary thromboendarterectomy. Ann Thorac Surg 62:1255–1260
Ishida K, Masuda M, Tanabe N, Matsumiya G, Tatsumi K, Nakajima N (2012) Long-term outcome after pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension. J Thorac Cardiovasc Surg 144:321–326
Jais X, Ioos V, Jardim C, Sitbon O, Parent F, Hamid A, Fadel E, Darteville P, Simonneau G, Humbert M (2005) Splenectomy and chronic thromboembolic pulmonary hypertension. Thorax 60:1031–1034
Jais X, D’Armini A, Jansa P, Torbicki A, Delcroix M, Ghofrani HA, Hoeper MM, Lang IM, Mayer E, Pepke-Zapa J, Perchenet L, Morganti A, Simonneau G, Rubin LJ (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
Jensen KW, Kerr KM, Fedullo PF, Kim NH, Test VJ, Ben-Yehuda O, Auger WR (2009) Pulmonary hypertensive medical therapy in chronic thromboembolic pulmonary hypertension before pulmonary thromboendarterectomy. Circulation 120:1248–1254
Kapitan KS, Buchbinder M, Wagner PD, Moser KM (1989) Mechanisms of hypoxemia in chronic thromboembolic pulmonary hypertension. Am Rev Respir Dis 139:1149–1154
Kataoka M, Inami T, Hayashida K, Shimura N, Ishiguro H, Abe T, Tamura Y, Ando M, Fukuda K, Yoshino H, Satoh T (2012) Percutaneous transluminal pulmonary angioplasty for the treatment of chronic thromboembolic pulmonary hypertension. Circ Cardiovasc Interv 5:756–762
Kerr KM (2005) Pulmonary artery sarcoma masquerading as chronic thromboembolic pulmonary hypertension. Nat Clin Pract Cardiovasc Med 2:108–112
Kerr KM, Auger WR, Fedullo PF, Channick RN, Yi ES, Moser KM (1995) Large vessel pulmonary arteritis mimicking chronic thromboembolic disease. Am J Respir Crit Care Med 152:367–373
Kim HS, Fesler P, Channick RN, Knowlton KU, Ben-Yehuda O, Lee SH, Naeije R, Rubin LJ (2004) Preoperative partitioning of pulmonary vascular resistance correlates with early outcome after thromboendarterectomy for chronic thromboembolic pulmonary hypertension. Circulation 109:18–22
Kline JA, Steuerwald MT, Marchik MR, Hernandez-Nino J, Rose GA (2009) Prospective evaluation of right ventricular function and functional status 6 months after acute submassive pulmonary embolism: frequency of persistent or subsequent elevation in estimated pulmonary artery pressure. Chest 136:1202–1210
Klok FA, van Kralingen KW, van Dijk APJ, Heyning FH, Vliegen HW (2010) Prospective cardiopulmonary screening program to detect chronic thromboembolic pulmonary hypertension in patients after acute pulmonary embolism. Haematologica 95:970–975
Konstantinides S, Tiede N, Geibel A, Olschewski M, Just H, Kasper W (1998) Comparison of alteplase versus heparin for resolution of major pulmonary embolism. Am J Cardiol 82:966–970
Korkmaz A, Ozlu T, Ozsu S, Kazaz Z, Bulbul Y (2012) Long-term outcomes in acute pulmonary thromboembolism: the incidence of chronic thromboembolic pulmonary hypertension and associated risk factors. Clin Appl Thromb Hemost 18:281–288
Kreitner K-F, Ley S, Kauczor H-U, Mayer E, Kramm T, Pitton MB, Krummenauer F, Thelen M (2004) Chronic thromboembolic pulmonary hypertension: pre- and postoperative assessment with breath-hold magnetic resonance techniques. Radiology 32:535–543
Kreitner K-F, Kunz RP, Ley S, Oberholzer K, Neeb D, Gast KK, Heussel C-P, Eberle B, Mayer E, Kauczor H-U, Duber C (2007) Chronic thromboembolic pulmonary hypertension – assessment by magnetic resonance imaging. Eur Radiol 17:11–21
Krug S, Hammerschmidt S, Pankau H, Wirtz H, Seyfarth HJ (2008) Acute improved hemodynamics following inhaled iloprost in chronic thromboembolic pulmonary hypertension. Respiration 76:154–159
Lafci G, Tasoglu I, Ulas MM, Yalcinkaya A, Cagli K (2012) Pulmonary endarterectomy with use of moderate hypothermia and antegrade cerebral perfusion without circulatory arrest. Tex Heart Inst J 39:65–67
Lang IM, Pesavento R, Bonderman D, Yuan JX-J (2013) Risk factors and basic mechanisms of chronic thromboembolic pulmonary hypertension: a current understanding. Eur Respir J 41:462–468
Lewczuk J, Piszko P, Jagas J, Porada A, Wojciak S, Sobkowicz B, Wrabec K (2001) Prognostic factors in medically treated patients with chronic pulmonary embolism. Chest 119:818–823
Ley S, Ley-Zaporozhan J, Pitton MB, Schneider J, Wirth GM, Mayer E, Duber C, Kreitner K-F (2012) Diagnostic performance of state-of-the-art imaging techniques for morphological assessment of vascular abnormalities in patients with chronic thromboembolic pulmonary hypertension (CTEPH). Eur Radiol 22:607–616
Li J-F, Lin Y, Yang Y-H, Gan H-L, Liang Y, Liu J, Yang S-Q, Zhang W-J, Cui N, Zhao L, Zhai Z-G, Wang J, Wang C (2013) Fibrinogen Aα Thr312Ala polymorphism specifically contributes to chronic thromboembolic pulmonary hypertension by increasing fibrin resistance. PLoS ONE 8:e69635
Lisbona R, Kreisman H, Novales-Diaz J, Derbekyan V (1985) Perfusion lung scanning: differentiation of primary from thromboembolic pulmonary hypertension. Am J Roentgenol 144:27–30
Madani MM, Jamieson SW (2006) Technical advances of pulmonary endarterectomy for chronic thromboembolic disease. Semin Thorac Cardiovasc Surg 18:243–249
Madani MM, Auger WR, Pretorius V, Sakakibara N, Kerr KM, Kim NK, Fedullo PF, Jamieson SW (2012) Pulmonary endarterectomy: recent changes in a single institution’s experience of more than 2,700 patients. Ann Thorac Surg 94:97–103
Mahmud E, Sadehgi HM, Raisinghani A, Hassankhani A, Strachan GM, Auger WR, DeMaria AN, Blanchard DG (2002) Correlation of left ventricular diastolic filling characteristics with right ventricular overload and pulmonary artery pressure in chronic thromboembolic pulmonary hypertension. J Am Coll Cardiol 40:318–324
Maliyasena VA, Hopkins PMA, Thomson BM, Dunning J, Wall DA, Nq BJ, McNeil KD, Mullany D, Kermeen FD (2012) An Australian tertiary referral center experience of the management of chronic thromboembolic pulmonary hypertension. Pulm Circ 2:359–364
Maruoka M, Sakao S, Kantake M, Tanabe N, Kasahara Y, Kurosu K, Takiguchi Y, Masuda M, Yoshino I, Voelkel NF, Tatsumi K (2011) Characterization of myofibroblasts in chronic thromboembolic pulmonary hypertension. Int J Cardiol 159:119–127
Mayer E, Jenkins D, Lindner J, D’Armini A, Kloek J, Meyns B, Ilkjaer LB, Klepetko W, Delcroix M, Lang I, Pepke-Zaba J, Simonneau G, Darteville P (2011) Surgical management and outcome of patients with chronic thromboembolic pulmonary hypertension: results from an international prospective registry. J Thorac Cardiovasc Surg 141:702–710
McCann C, Gopalan D, Sheares K, Screaton N (2012) Imaging in pulmonary hypertension, part 2: large vessel diseases. Postgrad Med J 88:317–325
McKie SJ, Hardwick DJ, Reid JH, Murchison JT (2005) Features of cardiac disease demonstrated on CT angiography. Clin Radiol 60:31–38
Mikus PM, Mikus E, Martin-Suarez S, Galie N, Manes A, Pastore S, Arpesella G (2008) Pulmonary endarterectomy: an alternative to circulatory arrest and deep hypothermia: mid-term results. Eur J Cardiothorac Surg 34:159–163
Miniati M, Monti S, Bottai M, Scoscia E, Bauleo C, Tonelli L, Dainelli A, Giuntini C (2006) Survival and restoration of pulmonary perfusion in a long-term follow-up of patients after acute pulmonary embolism. Medicine 85:253–262
Mizoguchi H, Ogawa A, Munemasa M, Mikouchi H, Ito H, Matsubara H (2012) Refined balloon pulmonary angioplasty for inoperable patients with chronic thromboembolic pulmonary hypertension. Circ Cardiovasc Interv 5:748–755
Morris TA, Auger WR, Ysrael MZ, Olson LK, Channick RN, Fedullo PF, Moser KM (1996) Parenchymal scarring is associated with restrictive spirometric defects in patients with chronic thromboembolic pulmonary hypertension. Chest 110:399–403
Morris TA, Marsh JJ, Chiles PG, Magana MM, Liang NC, Soler X, Desantis DJ, Ngo D, Woods VL Jr (2009) High prevalence of dysfibrinogenemia among patients with chronic thromboembolic pulmonary hypertension. Blood 114:1929–1936
Morsolini M, Nicolardi S, Milanesi E, Sarchi E, Mattiucci G, Klersy C, D’Armini AM (2012) Evolving surgical techniques for pulmonary endarterectomy according to the changing features of chronic thromboembolic pulmonary hypertension patients during 17-year single-center experience. J Thorac Cardiovasc Surg 144:100–107
Moser KM, Bloor CM (1993) Pulmonary vascular lesions occurring in patients with chronic major vessel thromboembolic pulmonary hypertension. Chest 103:685–692
Nagaya N, Sasaki N, Ando M, Ogino H, Sakamaki F, Kyotani S, Nakanishi N (2003) Prostacyclin therapy before pulmonary thromboendarterectomy in patients with chronic thromboembolic pulmonary hypertension. Chest 123:338–343
Nicod P, Peterson K, Levine M, Dittrich H, Buchbinder M, Chappuis F, Moser K (1987) Pulmonary angiography in severe chronic pulmonary hypertension. Ann Intern Med 107:565–568
Nijkeuter M, Hovens MMC, Davidson BL, Huisman MV (2006) Resolution of thromboemboli in patients with acute pulmonary embolism. Chest 129:192–197
Nikolaou K, Schoenberg SO, Attenberger U, Scheidler J, Dietrich O, Kuehn B, Rosa F, Huber A, Leuchte H, Baumgartner R, Behr J, Reiser MF (2005) Pulmonary arterial hypertension: diagnosis with fast perfusion imaging and high-spatial- resolution MR angiography – preliminary experience. Radiology 236:694–703
Ogino H, Ando M, Matsuda H, Minatoya K, Sasaki H, Nakanishi N, Kyotani S, Imanaka H, Kitamura S (2006) Japanese single-center experience of surgery for chronic thromboembolic pulmonary hypertension. Ann Thorac Surg 82:630–636
Olman MA, Marsh JJ, Lang IM, Moser KM, Binder BR, Schleef RR (1992) Endogenous fibrinolytic system in chronic large-vessel thromboembolic pulmonary hypertension. Circulation 86:1241–1248
Pengo V, Lensing ASA, Prins MH, Marchiori A, Davidson BL, Tiozzo F, Albanese P, Biasiolo A, Pegoraro C, Iliceto S, Prandoni P (2004) Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism. N Engl J Med 350:2257–2264
Pepke-Zaba J, Delcroix M, Lang I, Mayer E, Jansa P, Ambroz D, Treacy C, D’Armini AM, Morsolini M, Snijder R, Bresser P, Torbicki A, Kristensen B, Lewczuk J, Simkova I, Barbera JA, de Perrot M, Hoeper MM, Gaine S, Speich R, Gomez-Sanchez MA, Kovacs G, Hamid AM, Jais X, Simonneau G (2011) Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry. Circulation 124:1973–1981
Pineda LA, Hathwar VS, Grant BJ (2001) Clinical suspicion of fatal pulmonary embolism. Chest 20:791–795
Pitton MB, Kemmerich G, Herber S, Schweden F, Mayer E, Thelen M (2002) Chronic thromboembolic pulmonary hypertension: diagnostic impact of multislice CT and selective pulmonary DSA. Rofo 174:474–479
Powe JE, Palevsky HI, McCarthy KE, Alavi A (1987) Pulmonary arterial hypertension: value of perfusion scintigraphy. Radiology 164:727–730
Quarck R, Wynants M, Ronisz A, Sepulveda MR, Wuytack F, Van Raemdonck D, Meyns B, Delcroix M (2012) Characterization of proximal pulmonary arterial cells from chronic thromboembolic pulmonary hypertension patients. Respir Res 13:27
Raisinghani A, Ben-Yehuda O (2006) Echocardiography in chronic thromboembolic pulmonary hypertension. Semin Thorac Cardiovasc Surg 18:230–235
Rajaram S, Swift AJ, Capener D, Telfer A, Davies C, Hill C, Condliffe R, Elliot C, Hurdman J, Kiely DG, Wild JM (2012) Diagnostic accuracy of contrast-enhanced MR angiography and unenhanced proton MR imaging compared with CT angiography in chronic thromboembolic pulmonary hypertension. Eur Radiol 22:310–317
Reesink HJ, Surie S, Kloek JJ, Tan HL, Tepaske R, Fedullo PF, Bresser P (2010) Bosentan as a bridge to pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension. J Thorac Cardiovasc Surg 139:85–91
Reichenberger F, Voswinckel R, Enke B, Rutsch M, El Fechtail E, Schmehl T, Olschewski H, Schermuly R, Weissmann N, Ghofrani HA, Grimminger F, Mayer E, Seeger W (2007) Long-term treatment with sildenafil in chronic thromboembolic pulmonary hypertension. Eur Respir J 30:922–927
Ribeiro A, Lindmarker P, Johnsson H, Juhlin-Dannfelt A, Jorfeldt L (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 hemodynamics and respiratory data. Chest 81:151–158
Rossi SE, McAdams HP, Rosado-de-Christenson ML, Franks TJ, Galvin JR (2001) Fibrosing mediastinitis. Radiographics 21:737–757
Rush C, Langleben D, Schlesinger RD, Stern J, Wang NS, Lamoureux E (1991) Lung scintigraphy in pulmonary capillary hemangiomatosis: a rare disorder causing primary pulmonary hypertension. Clin Nucl Med 16:913–917
Ryan KL, Fedullo PF, Davis GB, Vasquez TE, Moser KM (1988) Perfusion scan findings understate the severity of angiographic and hemodynamic compromise in chronic thromboembolic pulmonary hypertension. Chest 93:1180–1185
Sakao S, Hao H, Tanabe N, Kasahara Y, Kurosu K, Tatsumi K (2011) Endothelial-like cells in chronic thromboembolic pulmonary hypertension: crosstalk with myofibroblast-like cells. Respir Res 12:109
Saouti N, Morshuis WJ, Heijmen RH, Snijder RJ (2009) Long-term outcome after pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension: a single institution experience. Eur J Cardiothorac Surg 35:947–952
Satoh T, Kyotani S, Okano Y, Nakanishi N, Kunieda T (2005) Descriptive patterns of severe chronic pulmonary hypertension by chest radiography. Respir Med 99:329–336
Scholzel BE, Post MC, Thijs Plokker HW, Snijder RJ (2012) Clinical worsening during long-term follow-up in inoperable chronic thromboembolic pulmonary hypertension. Lung 190:161–167
Skoro-Sajer N, Bonderman D, Wiesbauer F, Harja E, Jakowitsch J, Klepetko W, Kneussl MP, Lang IM (2007) Treprostinil for severe inoperable chronic thromboembolic pulmonary hypertension. J Thromb Haemost 5:483–489
Soler X, Kerr KM, Marsh JJ, Renner JW, Hoh CK, Test VJ, Morris TA (2012) Pilot study comparing SPECT perfusion scintigraphy with CT pulmonary angiography in chronic thromboembolic pulmonary hypertension. Respirology 17:180–184
Steenhuis LH, Groen HJM, Koeter GH, van der Mark TW (2000) Diffusion capacity and haemodynamics in primary and chronic thromboembolic pulmonary hypertension. Eur Respir J 16:276–281
Stein PD, Matta F, Mussani MH (2010) Silent pulmonary embolism in patients with deep venous thrombosis. A systematic review. Am J Med 123:426–431
Stevinson BG, Hernandez-Nino J, Rose G, Kline JA (2007) Echocardiographic and functional cardiopulmonary problems 6 months after first-time pulmonary embolism in previously healthy patients. Eur Heart J 28:2517–2524
Sugimura K, Fukumoto Y, Satoh K, Nochioka K, Miura Y, Aoki T, Tatebe S, Miyamichi-Yamamoto S, Shimokawa H (2012) Percutaneous transluminal pulmonary angioplasty markedly improves pulmonary hemodynamics and long-term prognosis in patients with chronic thromboembolic pulmonary hypertension. Circ J 76:485–488
Sugiura T, Tanabe N, Matsuura Y, Shigeta A, Kawata N, Jujo T, Yanagawa N, Sakao S, Kasahara Y, Tatsumi K (2013) Role of 320-slice computed tomography in the diagnostic workup of patients with chronic thromboembolic pulmonary hypertension. Chest 143:1070–1077
Sun X-G, Hansen JE, Oudiz RJ, Wasserman K (2001) Exercise pathophysiology in patients with primary pulmonary hypertension. Circulation 104:429–435
Suntharalingam J, Goldsmith K, Toshner M, Doughty N, Sheares KK, Hughes R, Jenkins D, Pepke-Zaba J (2007) Role of NT-proBNP and 6MWD in chronic thromboembolic pulmonary hypertension. Respir Med 101:2254–2262
Suntharalingam J, Goldsmith K, van Marion V, Long L, Treacy CM, Dudbridge F, Toshner MR, Pepke-Zaba J, Eikenboom JC, Morrell NW (2008a) Fibrinogen Aα Thr312Ala polymorphism is associated with chronic thromboembolic pulmonary hypertension. Eur Respir J 31:736–741
Suntharalingam J, Treacy CM, Doughty NJ, Goldsmith K, Soon E, Toshner MR, Sheares KK, Hughes R, Morrell NW, Pepke-Zaba J (2008b) Long-term use of sildenafil in inoperable chronic thromboembolic pulmonary hypertension. Chest 134:229–236
Surie S, Reesink HJ, Marcus JT, van der Plas MN, Kloek JJ, Vonk-Noordegraaf A, Bresser P (2013) Bosentan treatment is associated with improvement of right heart function and remodeling in chronic thromboembolic pulmonary hypertension. Clin Cardiol 36:698–703
Tanabe N, Okada O, Abe Y, Masuda M, Nakajima N, Kuriyama T (2001) The influence of fractional pulse pressure on the outcome of pulmonary thromboendarterectomy. Eur Respir J 17:653–659
Thistlethwaite PA, Auger WR, Madani MM, Pradhan S, Kapelanski DP, Jamieson SW (2001) Pulmonary thromboendarterectomy combined with other cardiac operations: indications, surgical approach, and outcome. Ann Thorac Surg 72:13–19
Thistlethwaite PA, Kemp A, Du L, Madani MM, Jamieson SW (2006) Outcomes of pulmonary thromboendarterectomy for treatment of extreme thromboembolic pulmonary hypertension. J Thorac Cardiovasc Surg 131:307–313
Thompson B, Tsui SSL, Dunning J, Goodwin A, Vuylsteke A, Latimer R, Pepke-Zaba J, Jenkins DP (2008) Pulmonary endarterectomy is possible and effective without the use of complete circulatory arrest – the UK experience in over 150 patients. Eur J Cardiothorac Surg 33:157–163
Toshner M, Suntharalingam J, Fesler P, Soon E, Sheares KK, Jenkins D, White P, Morrell NW, Naeije R, Pepke-Zaba J (2012) Occlusion pressure analysis role in partitioning of pulmonary vascular resistance in CTEPH. Eur Respir J 40:612–617
Tunariu N, Gibbs SJ, Win Z, Gin-Sing W, Graham A, Gishen P, Al-Nahhas A (2007) Ventilation-perfusion scintigraphy is more sensitive than multidetector CTPA in detecting chronic thromboembolic disease as a treatable cause of pulmonary hypertension. J Nucl Med 48:680–684
Ulrich S, Fischler M, Speich R, Popov V, Maggiorini M (2006) Chronic thromboembolic and pulmonary arterial hypertension share acute vasoreactivity properties. Chest 130:841–846
van der Plas MN, Reesink HJ, Roos CM (2010) Pulmonary endarterectomy improves dyspnea by the relief of dead space ventilation. Ann Thorac Surg 89:347–352
Vuylsteke A, Sharples L, Charman G, Kneeshaw J, Tsui S, Dunning J, Wheaton E, Klein A, Arrowsmith J, Hall R, Jenkins D (2011) Circulatory arrest versus cerebral perfusion during pulmonary endarterectomy surgery (PEACOG): a randomized controlled trial. Lancet 378:1379–1387
Wartski M, Collignon M-A for the THESEE Study Group (2000) Incomplete recovery of lung perfusion after 3 months in patients with acute pulmonary embolism treated with antithrombotic agents. J Nucl Med 41:1043–1048
Wijesuriya S, Chandratreya L, Medford AR (2013) Chronic pulmonary emboli and radiologic mimics on CT pulmonary angiography. A diagnostic challenge. Chest 143:1460–1471
Willemink MJ, van Es HW, Koobs L, Morshuis WJ, Snijder RJ, van Heesewijk JPM (2012) CT evaluation of chronic thromboembolic pulmonary hypertension. Clin Radiol 67:277–285
Winkler MH, Rohrer CH, Ratty SC, Jamieson S, Dembitsky W, Moser K, Auger W (1990) Perfusion techniques of profound hypothermia and circulatory arrest for pulmonary thromboendarterectomy. J Extra-Corpor Technol 22:57–60
Wolf M, Boyer-Neumann C, Parent F, Eschwege V, Jaillet H, Meyer D, Simonneau G (2000) Thrombotic risk factors in pulmonary hypertension. Eur Respir J 15:395–399
Wong CL, Szydio R, Gibbs S, Laffan M (2010) Hereditary and acquired thrombotic risk factors for chronic thromboembolic pulmonary hypertension. Blood Coagul Fibrinolysis 21:201–206
Woodruff WW III, Hoeck BE, Chitwood WR Jr, Lyerly HK, Sabiston DC Jr, Chen JTT (1985) Radiographic findings in pulmonary hypertension from unresolved embolism. Am J Roentgenol 144:681–686
Wynants M, Quarck R, Ronisz A, Alfaro-Moreno E, Van Raemdonck D, Meyns B, Delcroix M (2012) Effects of C-reactive protein on human pulmonary vascular cells in chronic thromboembolic pulmonary hypertension. Eur Respir J 40:886–894
Yao W, Firth AL, Sacks RS, Ogawa A, Auger WR, Fedullo PF, Madani MM, Lin GY, Sakakibara N, Thistlethwaite PA, Jamieson SW, Rubin LJ, Yuan JX-J (2009) Identification of putative endothelial cells (CD34+CD133+Flk-1+) in endarterectomized tissue of patients with chronic thromboembolic pulmonary hypertension. Am J Physiol Lung Cell Mol Physiol 296:L870–L878
Yi ES, Kim H, Ahn H, Strother J, Morris T, Masliah E, Hansen LA, Park K, Friedman PJ (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
Zabini D, Nagaraj C, Stacher E, Lang IM, Nierlich P, Klepetko W, Heinemann A, Olschewski H, Balint Z, Olschewski A (2012) Angiostatic factors in the pulmonary endarterectomy material from chronic thromboembolic pulmonary hypertension patients cause endothelial dysfunction. PLoS ONE 7(8):e43793
Zhai Z, Murphy K, Tighe H, Wang C, Wilkins MR, Gibbs JS, Howard LS (2011) Differences in ventilatory inefficiency between pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. Chest 140:1284–1291
Further Reading
Lang IM, Pesavento R, Bonderman D, Yuan JX-J (2013) Risk factors and basic mechanisms of chronic thromboembolic pulmonary hypertension: a current understanding, Eur Respir J 41:462–468
Pepke-Zaba J, Delcroix M, Lang I, Mayer E, Jansa P, Ambroz D, Treacy C, D’Armini AM, Morsolini M, Snijder R, Bresser P, Torbicki A, Kristensen B, Lewczuk J, Simkova I, Barbera JA, de Perrot M, Hoeper MM, Gaine S, Speich R, Gomez-Sanchez MA, Kovacs G, Hamid AM, Jais X, Simonneau (2011) Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry. Circulation 124:1973–1981
Madani MM, Auger WR, Pretorius V, Sakakibara N, Kerr KM, Kim NK, Fedullo PF, Jamieson SW (2012) Pulmonary endarterectomy: recent changes in a single institution’s experience of more than 2,700 patients. Ann Thorac Surg 94:97–103
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Auger, W.R., Fedullo, P.F. (2014). Chronic Thromboembolic Pulmonary Hypertension. In: Lanzer, P. (eds) PanVascular Medicine. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-37393-0_204-1
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DOI: https://doi.org/10.1007/978-3-642-37393-0_204-1
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