Login Register Cart 0
Generic placeholder image

Current Respiratory Medicine Reviews

Editor-in-Chief

ISSN (Print): 1573-398X
ISSN (Online): 1875-6387

Back
Journal Home About Journal Editorial Board Journal Insight Current Issue Volumes/Issues
Subscribe
Review Article

Pulmonary Arterial Hypertension (PAH) Group 1 (Part A): Overview, Classification, Clinical Subsets, and Workup

Author(s): Maidah Yaqoob*, Thomas W. DeCato and Ronald Oudiz

Volume 20, Issue 2, 2024

Published on: 01 January, 2024

Page: [88 - 106] Pages: 19

DOI: 10.2174/011573398X268623231212180622

Price: $65

Abstract

Pulmonary hypertension is a rare, progressive disease characterized by increased pulmonary arterial pressure and right ventricular failure due to pulmonary vascular remodeling. The disease definition and management have evolved over time. The 6th WSPH now defines it as a mean pulmonary arterial pressure >20mmHg, while recent ESC/ERS guidelines recommend lowering the threshold for pulmonary vascular resistance to 2WU.

Understanding of the disease has improved through registries, classifying it into five distinct groups with similar histology, pathophysiology, and therapeutic approaches. These groups include PAH, with heritable and idiopathic causes, as well as various clinical subsets involving connective tissue disease, HIV, portopulmonary hypertension, congenital heart disease, and schistosomiasis. Long-term responders to calcium channel blockers, PAH with venous/capillaries involvement, and persistent PH of newborns are categorized under Group 1, now re-classified as IPAH.

A comprehensive workup for suspected patients includes various tests like electrocardiogram, pulmonary function testing, autoimmune workup, HIV testing, echocardiogram, right heart catheterization, and cardiopulmonary exercise testing.

This review emphasizes the disease's definition and epidemiology, delving into each subset and providing updated workup guidelines. The subsequent article will focus on risk stratification and treatment strategies.

Keywords: Pulmonary arterial hypertension, HIV testing, echocardiogram, cardiopulmonary, catheterization, autoimmune.

« Previous Next »
[1]
Hatano, Shuichi; Strasser, Toma Primary pulmonary hypertension : Report on a WHO meeting; World Health Organization: Geneva, 1975.
[2]
Primary Pulmonary Hypertension. Available from: http://www. wsphassociation.org/wp-content/uploads/2019/04/Primary-Pulmonary-Hypertension-Evian-1998.pdf
[3]
Simonneau, G.; Montani, D.; Celermajer, D.S.; Denton, C.P.; Gatzoulis, M.A.; Krowka, M.; Williams, P.G.; Souza, R. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur. Respir. J., 2019, 53(1), 1801913.
[http://dx.doi.org/10.1183/13993003.01913-2018] [PMID: 30545968]
[4]
Kovacs, G.; Berghold, A.; Scheidl, S.; Olschewski, H. Pulmonary arterial pressure during rest and exercise in healthy subjects: A systematic review. Eur. Respir. J., 2009, 34(4), 888-894.
[http://dx.doi.org/10.1183/09031936.00145608] [PMID: 19324955]
[5]
Assad, T.R.; Maron, B.A.; Robbins, I.M.; Xu, M.; Huang, S.; Harrell, F.E.; Farber-Eger, E.H.; Wells, Q.S.; Choudhary, G.; Hemnes, A.R.; Brittain, E.L. Prognostic effect and longitudinal hemodynamic assessment of borderline pulmonary hypertension. JAMA Cardiol., 2017, 2(12), 1361-1368.
[http://dx.doi.org/10.1001/jamacardio.2017.3882] [PMID: 29071338]
[6]
Douschan, P.; Kovacs, G.; Avian, A.; Foris, V.; Gruber, F.; Olschewski, A.; Olschewski, H. Mild elevation of pulmonary arterial pressure as a predictor of mortality. Am. J. Respir. Crit. Care Med., 2018, 197(4), 509-516.
[http://dx.doi.org/10.1164/rccm.201706-1215OC] [PMID: 29099619]
[7]
Maron, B.A.; Hess, E.; Maddox, T.M.; Opotowsky, A.R.; Tedford, R.J.; Lahm, T.; Joynt, K.E.; Kass, D.J.; Stephens, T.; Stanislawski, M.A.; Swenson, E.R.; Goldstein, R.H.; Leopold, J.A.; Zamanian, R.T.; Elwing, J.M.; Plomondon, M.E.; Grunwald, G.K.; Barón, A.E.; Rumsfeld, J.S.; Choudhary, G. Association of borderline pulmonary hypertension with mortality and hospitalization in a large patient cohort: Insights from the veterans affairs clinical assessment, reporting, and tracking program. Circulation, 2016, 133(13), 1240-1248.
[http://dx.doi.org/10.1161/CIRCULATIONAHA.115.020207] [PMID: 26873944]
[8]
Coghlan, J.G.; Wolf, M.; Distler, O.; Denton, C.P.; Doelberg, M.; Harutyunova, S.; Marra, A.M.; Benjamin, N.; Fischer, C.; Grünig, E. Incidence of pulmonary hypertension and determining factors in patients with systemic sclerosis. Eur. Respir. J., 2018, 51(4), 1701197.
[http://dx.doi.org/10.1183/13993003.01197-2017] [PMID: 29563168]
[9]
Valerio, C.J.; Schreiber, B.E.; Handler, C.E.; Denton, C.P.; Coghlan, J.G. Borderline mean pulmonary artery pressure in patients with systemic sclerosis: transpulmonary gradient predicts risk of developing pulmonary hypertension. Arthritis Rheum., 2013, 65(4), 1074-1084.
[http://dx.doi.org/10.1002/art.37838] [PMID: 23280155]
[10]
Kovacs, G.; Olschewski, A.; Berghold, A.; Olschewski, H. Pulmonary vascular resistances during exercise in normal subjects: A systematic review. Eur. Respir. J., 2012, 39(2), 319-328.
[http://dx.doi.org/10.1183/09031936.00008611] [PMID: 21885394]
[11]
Humbert, M.; Kovacs, G.; Hoeper, M.M.B. ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J, 2022.
[12]
Xanthouli, P.; Jordan, S.; Milde, N.; Marra, A.; Blank, N.; Egenlauf, B.; Gorenflo, M.; Harutyunova, S.; Lorenz, H.M.; Nagel, C.; Theobald, V.; Lichtblau, M.; Berlier, C.; Ulrich, S.; Grünig, E.; Benjamin, N.; Distler, O. Haemodynamic phenotypes and survival in patients with systemic sclerosis: the impact of the new definition of pulmonary arterial hypertension. Ann. Rheum. Dis., 2020, 79(3), 370-378.
[http://dx.doi.org/10.1136/annrheumdis-2019-216476] [PMID: 31818805]
[13]
Beshay, S.; Sahay, S.; Humbert, M. Evaluation and management of pulmonary arterial hypertension. Respir. Med., 2020, 171, 106099.
[http://dx.doi.org/10.1016/j.rmed.2020.106099] [PMID: 32829182]
[14]
Thenappan, T.; Shah, S.J.; Rich, S.; Tian, L.; Archer, S.L.; Gomberg-Maitland, M. Survival in pulmonary arterial hypertension: A reappraisal of the NIH risk stratification equation. Eur. Respir. J., 2010, 35(5), 1079-1087.
[http://dx.doi.org/10.1183/09031936.00072709] [PMID: 20032020]
[15]
Humbert, M.; Sitbon, O.; Chaouat, A.; Bertocchi, M.; Habib, G.; Gressin, V.; Yaïci, A.; Weitzenblum, E.; Cordier, J.F.; Chabot, F.; Dromer, C.; Pison, C.; Reynaud-Gaubert, M.; Haloun, A.; Laurent, M.; Hachulla, E.; Cottin, V.; Degano, B.; Jaïs, X.; Montani, D.; Souza, R.; Simonneau, G. Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era. Circulation, 2010, 122(2), 156-163.
[http://dx.doi.org/10.1161/CIRCULATIONAHA.109.911818] [PMID: 20585011]
[16]
Hoeper, M.M.; Huscher, D.; Ghofrani, H.A.; Delcroix, M.; Distler, O.; Schweiger, C.; Grunig, E.; Staehler, G.; Rosenkranz, S.; Halank, M.; Held, M.; Grohé, C.; Lange, T.J.; Behr, J.; Klose, H.; Wilkens, H.; Filusch, A.; Germann, M.; Ewert, R.; Seyfarth, H.J.; Olsson, K.M.; Opitz, C.F.; Gaine, S.P.; Vizza, C.D.; Vonk-Noordegraaf, A.; Kaemmerer, H.; Gibbs, J.S.R.; Pittrow, D. Elderly patients diagnosed with idiopathic pulmonary arterial hypertension: Results from the COMPERA registry. Int. J. Cardiol., 2013, 168(2), 871-880.
[http://dx.doi.org/10.1016/j.ijcard.2012.10.026] [PMID: 23164592]
[17]
Rådegran, G.; Kjellström, B.; Ekmehag, B.; Larsen, F.; Rundqvist, B.; Blomquist, S.B.; Gustafsson, C.; Hesselstrand, R.; Karlsson, M.; Kornhall, B.; Nisell, M.; Persson, L.; Ryftenius, H.; Selin, M.; Ullman, B.; Wall, K.; Wikström, G.; Willehadson, M.; Jansson, K. Characteristics and survival of adult Swedish PAH and CTEPH patients 2000-2014. Scand. Cardiovasc. J., 2016, 50(4), 243-250.
[http://dx.doi.org/10.1080/14017431.2016.1185532] [PMID: 27146648]
[18]
Jansa, P.; Jarkovsky, J.; Al-Hiti, H.; Popelova, J.; Ambroz, D.; Zatocil, T.; Votavova, R.; Polacek, P.; Maresova, J.; Aschermann, M.; Brabec, P.; Dusek, L.; Linhart, A. Epidemiology and long-term survival of pulmonary arterial hypertension in the Czech Republic: A retrospective analysis of a nationwide registry. BMC Pulm. Med., 2014, 14(1), 45.
[http://dx.doi.org/10.1186/1471-2466-14-45] [PMID: 24629043]
[19]
Chung, W.J.; Park, Y.B.; Jeon, C.H.; Jung, J.W.; Ko, K.P.; Choi, S.J.; Seo, H.S.; Lee, J.S.; Jung, H.O. Baseline characteristics of the korean registry of pulmonary arterial hypertension. J. Korean Med. Sci., 2015, 30(10), 1429-1438.
[http://dx.doi.org/10.3346/jkms.2015.30.10.1429] [PMID: 26425039]
[20]
Alves, J.L., Jr; Gavilanes, F.; Jardim, C.; Fernandes, C.J.C.S.; Morinaga, L.T.K.; Dias, B.; Hoette, S.; Humbert, M.; Souza, R. Pulmonary arterial hypertension in the southern hemisphere: Results from a registry of incident Brazilian cases. Chest, 2015, 147(2), 495-501.
[http://dx.doi.org/10.1378/chest.14-1036] [PMID: 25317567]
[21]
Humbert, M.; Sitbon, O.; Chaouat, A.; Bertocchi, M.; Habib, G.; Gressin, V.; Yaici, A.; Weitzenblum, E.; Cordier, J.F.; Chabot, F.; Dromer, C.; Pison, C.; Reynaud-Gaubert, M.; Haloun, A.; Laurent, M.; Hachulla, E.; Simonneau, G. Pulmonary arterial hypertension in France: Results from a national registry. Am. J. Respir. Crit. Care Med., 2006, 173(9), 1023-1030.
[http://dx.doi.org/10.1164/rccm.200510-1668OC] [PMID: 16456139]
[22]
Escribano-Subias, P.; Blanco, I.; López-Meseguer, M.; Lopez-Guarch, C.J.; Roman, A.; Morales, P.; Castillo-Palma, M.J.; Segovia, J.; Gómez-Sanchez, M.A.; Barberà, J.A. Survival in pulmonary hypertension in Spain: insights from the Spanish registry. Eur. Respir. J., 2012, 40(3), 596-603.
[http://dx.doi.org/10.1183/09031936.00101211] [PMID: 22362843]
[23]
Rich, S.; Kaufmann, E.; Levy, P.S. The effect of high doses of calcium-channel blockers on survival in primary pulmonary hypertension. N. Engl. J. Med., 1992, 327(2), 76-81.
[http://dx.doi.org/10.1056/NEJM199207093270203] [PMID: 1603139]
[24]
Sitbon, O.; Humbert, M.; Jaïs, X.; Ioos, V.; Hamid, A.M.; Provencher, S.; Garcia, G.; Parent, F.; Hervé, P.; Simonneau, G. Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation, 2005, 111(23), 3105-3111.
[http://dx.doi.org/10.1161/CIRCULATIONAHA.104.488486] [PMID: 15939821]
[25]
Hemnes, A.R.; Zhao, M.; West, J.; Newman, J.H.; Rich, S.; Archer, S.L.; Robbins, I.M.; Blackwell, T.S.; Cogan, J.; Loyd, J.E.; Zhao, Z.; Gaskill, C.; Jetter, C.; Kropski, J.A.; Majka, S.M.; Austin, E.D. Critical genomic networks and vasoreactive variants in idiopathic pulmonary arterial hypertension. Am. J. Respir. Crit. Care Med., 2016, 194(4), 464-475.
[http://dx.doi.org/10.1164/rccm.201508-1678OC] [PMID: 26926454]
[26]
Cogan, J.D.; Pauciulo, M.W.; Batchman, A.P.; Prince, M.A.; Robbins, I.M.; Hedges, L.K.; Stanton, K.C.; Wheeler, L.A.; Phillips, J.A., III; Loyd, J.E.; Nichols, W.C. High frequency of BMPR2 exonic deletions/duplications in familial pulmonary arterial hypertension. Am. J. Respir. Crit. Care Med., 2006, 174(5), 590-598.
[http://dx.doi.org/10.1164/rccm.200602-165OC] [PMID: 16728714]
[27]
Aldred, M.A.; Vijayakrishnan, J.; James, V.; Soubrier, F.; Gomez-Sanchez, M.A.; Martensson, G.; Galie, N.; Manes, A.; Corris, P.; Simonneau, G.; Humbert, M.; Morrell, N.W.; Trembath, R.C. BMPR2 gene rearrangements account for a significant proportion of mutations in familial and idiopathic pulmonary arterial hypertension. Hum. Mutat., 2006, 27(2), 212-213.
[http://dx.doi.org/10.1002/humu.9398] [PMID: 16429403]
[28]
Shintani, M.; Yagi, H.; Nakayama, T.; Saji, T.; Matsuoka, R. A new nonsense mutation of SMAD8 associated with pulmonary arterial hypertension. J. Med. Genet., 2009, 46(5), 331-337.
[http://dx.doi.org/10.1136/jmg.2008.062703] [PMID: 19211612]
[29]
Austin, E.D.; Ma, L.; LeDuc, C.; Berman Rosenzweig, E.; Borczuk, A.; Phillips, J.A., III; Palomero, T.; Sumazin, P.; Kim, H.R.; Talati, M.H.; West, J.; Loyd, J.E.; Chung, W.K. Whole exome sequencing to identify a novel gene (caveolin-1) associated with human pulmonary arterial hypertension. Circ. Cardiovasc. Genet., 2012, 5(3), 336-343.
[http://dx.doi.org/10.1161/CIRCGENETICS.111.961888] [PMID: 22474227]
[30]
Ryanto, G.R.T.; Musthafa, A.; Hara, T.; Emoto, N. Inactivating the uninhibited: The tale of activins and inhibins in pulmonary arterial hypertension. Int. J. Mol. Sci., 2023, 24(4), 3332.
[http://dx.doi.org/10.3390/ijms24043332] [PMID: 36834742]
[31]
Machado, R.D.; Aldred, M.A.; James, V.; Harrison, R.E.; Patel, B.; Schwalbe, E.C.; Gruenig, E.; Janssen, B.; Koehler, R.; Seeger, W.; Eickelberg, O.; Olschewski, H.; Gregory Elliott, C.; Glissmeyer, E.; Carlquist, J.; Kim, M.; Torbicki, A.; Fijalkowska, A.; Szewczyk, G.; Parma, J.; Abramowicz, M.J.; Galie, N.; Morisaki, H.; Kyotani, S.; Nakanishi, N.; Morisaki, T.; Humbert, M.; Simonneau, G.; Sitbon, O.; Soubrier, F.; Coulet, F.; Morrell, N.W.; Trembath, R.C. Mutations of the TGF-β type II receptorBMPR2 in pulmonary arterial hypertension. Hum. Mutat., 2006, 27(2), 121-132.
[http://dx.doi.org/10.1002/humu.20285] [PMID: 16429395]
[32]
Thomson, J.R.; Machado, R.D.; Pauciulo, M.W.; Morgan, N.V.; Humbert, M.; Elliott, G.C.; Ward, K.; Yacoub, M.; Mikhail, G.; Rogers, P.; Newman, J.; Wheeler, L.; Higenbottam, T.; Gibbs, J.S.; Egan, J.; Crozier, A.; Peacock, A.; Allcock, R.; Corris, P.; Loyd, J.E.; Trembath, R.C.; Nichols, W.C. Sporadic primary pulmonary hypertension is associated with germline mutations of the gene encoding BMPR-II, a receptor member of the TGF-beta family. J. Med. Genet., 2000, 37(10), 741-745.
[http://dx.doi.org/10.1136/jmg.37.10.741] [PMID: 11015450]
[33]
Boutet, K.; Frachon, I.; Jobic, Y.; Gut-Gobert, C.; Leroyer, C.; Carlhant-Kowalski, D.; Sitbon, O.; Simonneau, G.; Humbert, M. Fenfluramine-like cardiovascular side-effects of benfluorex. Eur. Respir. J., 2009, 33(3), 684-688.
[http://dx.doi.org/10.1183/09031936.00086308] [PMID: 19251806]
[34]
Ramirez, R.L., III; Thomas, C.A.; Anderson, R.J.; Bernardo, R.J.; Al-Motarreb, A.; Al-Suwaidi, J.; Zamanian, R.T.; de Jesus Perez, V.A. Drug- and toxin-induced pulmonary arterial hypertension: Current state of the literature. Glob. Cardiol. Sci. Pract., 2019, 19
[http://dx.doi.org/10.21542/gcsp.2019.19]
[35]
Kilbourne, E.M.; Rigau-Perez, J.G.; Heath, C.W., Jr; Zack, M.M.; Falk, H.; Martin-Marcos, M.; de Carlos, A. Clinical epidemiology of toxic-oil syndrome. Manifestations of a New Illness. N. Engl. J. Med., 1983, 309(23), 1408-1414.
[http://dx.doi.org/10.1056/NEJM198312083092302] [PMID: 6633617]
[36]
Gomez-Sanchez, M.A.; de la Calzada, C.S.; Gomez-Pajuelo, C.; Martinez-Tello, F.J.; de Juan, M.J.M.; James, T.N. Clinical and pathologic manifestations of pulmonary vascular disease in the toxic oil syndrome. J. Am. Coll. Cardiol., 1991, 18(6), 1539-1545.
[http://dx.doi.org/10.1016/0735-1097(91)90688-6] [PMID: 1939959]
[37]
Douglas, J.G.; Munro, J.F.; Kitchin, A.H.; Muir, A.L.; Proudfoot, A.T. Pulmonary hypertension and fenfluramine. BMJ, 1981, 283(6296), 881-883.
[http://dx.doi.org/10.1136/bmj.283.6296.881] [PMID: 6793158]
[38]
Manson, J.E.; Faich, G.A. Pharmacotherapy for obesity -- do the benefits outweigh the risks? N. Engl. J. Med., 1996, 335(9), 659-660.
[http://dx.doi.org/10.1056/NEJM199608293350910] [PMID: 8687523]
[39]
Gruenewald, P.J.; Ponicki, W.R.; Remer, L.G.; Waller, L.A.; Zhu, L.; Gorman, D.M. Mapping the spread of methamphetamine abuse in California from 1995 to 2008. Am. J. Public Health, 2013, 103(7), 1262-1270.
[http://dx.doi.org/10.2105/AJPH.2012.300779] [PMID: 23078474]
[40]
Orcholski, M.E.; Khurshudyan, A.; Shamskhou, E.A.; Yuan, K.; Chen, I.Y.; Kodani, S.D.; Morisseau, C.; Hammock, B.D.; Hong, E.M.; Alexandrova, L.; Alastalo, T.P.; Berry, G.; Zamanian, R.T.; de Jesus Perez, V.A. Reduced carboxylesterase 1 is associated with endothelial injury in methamphetamine-induced pulmonary arterial hypertension. Am. J. Physiol. Lung Cell. Mol. Physiol., 2017, 313(2), L252-L266.
[http://dx.doi.org/10.1152/ajplung.00453.2016] [PMID: 28473326]
[41]
Yakel, D.L., Jr; Eisenberg, M.J. Pulmonary artery hypertension in chronic intravenous cocaine users. Am. Heart J., 1995, 130(2), 398-399.
[http://dx.doi.org/10.1016/0002-8703(95)90459-X] [PMID: 7631626]
[42]
Savale, L.; Sattler, C.; Günther, S.; Montani, D.; Chaumais, M.C.; Perrin, S.; Jaïs, X.; Seferian, A.; Jovan, R.; Bulifon, S.; Parent, F.; Simonneau, G.; Humbert, M.; Sitbon, O. Pulmonary arterial hypertension in patients treated with interferon. Eur. Respir. J., 2014, 44(6), 1627-1634.
[http://dx.doi.org/10.1183/09031936.00057914] [PMID: 25323231]
[43]
Perros, F.; Günther, S.; Ranchoux, B.; Godinas, L.; Antigny, F.; Chaumais, M.C.; Dorfmüller, P.; Hautefort, A.; Raymond, N.; Savale, L.; Jaïs, X.; Girerd, B.; Cottin, V.; Sitbon, O.; Simonneau, G.; Humbert, M.; Montani, D. Mitomycin-induced pulmonary veno-occlusive disease. Circulation, 2015, 132(9), 834-847.
[http://dx.doi.org/10.1161/CIRCULATIONAHA.115.014207] [PMID: 26130118]
[44]
Gupta, S.; Gupta, A.; Rehman, S.; Ocak, I.; Domsic, R.T.; Schneider, F.; George, M.P. Pulmonary veno-occlusive disease is highly prevalent in scleroderma patients undergoing lung transplantation. ERJ Open Res., 2019, 5(1), 00168-2018.
[http://dx.doi.org/10.1183/23120541.00168-2018] [PMID: 30775371]
[45]
Kherbeck, N.; Tamby, M.C.; Bussone, G.; Dib, H.; Perros, F.; Humbert, M.; Mouthon, L. The role of inflammation and autoimmunity in the pathophysiology of pulmonary arterial hypertension. Clin. Rev. Allergy Immunol., 2013, 44(1), 31-38.
[http://dx.doi.org/10.1007/s12016-011-8265-z] [PMID: 21394427]
[46]
Riemekasten, G.; Philippe, A.; Näther, M.; Slowinski, T.; Müller, D.N.; Heidecke, H.; Matucci-Cerinic, M.; Czirják, L.; Lukitsch, I.; Becker, M.; Kill, A.; van Laar, J.M.; Catar, R.; Luft, F.C.; Burmester, G.R.; Hegner, B.; Dragun, D. Involvement of functional autoantibodies against vascular receptors in systemic sclerosis. Ann. Rheum. Dis., 2011, 70(3), 530-536.
[http://dx.doi.org/10.1136/ard.2010.135772] [PMID: 21081526]
[47]
Negi, V.S.; Tripathy, N.K.; Misra, R.; Nityanand, S. Antiendothelial cell antibodies in scleroderma correlate with severe digital ischemia and pulmonary arterial hypertension. J. Rheumatol., 1998, 25(3), 462-466.
[PMID: 9517764]
[48]
Bordron, A.; Dueymes, M.; Levy, Y.; Jamin, C.; Leroy, J.P.; Piette, J.C.; Shoenfeld, Y.; Youinou, P.Y. The binding of some human antiendothelial cell antibodies induces endothelial cell apoptosis. J. Clin. Invest., 1998, 101(10), 2029-2035.
[http://dx.doi.org/10.1172/JCI2261] [PMID: 9593758]
[49]
Tamby, M.C.; Humbert, M.; Guilpain, P.; Servettaz, A.; Dupin, N.; Christner, J.J.; Simonneau, G.; Fermanian, J.; Weill, B.; Guillevin, L.; Mouthon, L. Antibodies to fibroblasts in idiopathic and scleroderma-associated pulmonary hypertension. Eur. Respir. J., 2006, 28(4), 799-807.
[http://dx.doi.org/10.1183/09031936.06.00152705] [PMID: 16774952]
[50]
Wipff, J.; Kahan, A.; Hachulla, E.; Sibilia, J.; Cabane, J.; Meyer, O.; Mouthon, L.; Guillevin, L.; Junien, C.; Boileau, C.; Allanore, Y. Association between an endoglin gene polymorphism and systemic sclerosis-related pulmonary arterial hypertension. Rheumatology, 2006, 46(4), 622-625.
[http://dx.doi.org/10.1093/rheumatology/kel378] [PMID: 17166870]
[51]
Kim, K.K.; Factor, S.M. Membranoproliferative glomerulonephritis and plexogenic pulmonary arteriopathy in a homosexual man with acquired immunodeficiency syndrome. Hum. Pathol., 1987, 18(12), 1293-1296.
[http://dx.doi.org/10.1016/S0046-8177(87)80417-3] [PMID: 3679202]
[52]
Butrous, G. Human immunodeficiency virus-associated pulmonary arterial hypertension: considerations for pulmonary vascular diseases in the developing world. Circulation, 2015, 131(15), 1361-1370.
[http://dx.doi.org/10.1161/CIRCULATIONAHA.114.006978] [PMID: 25869003]
[53]
Degano, B.; Guillaume, M.; Savale, L.; Montani, D.; Jaïs, X.; Yaici, A.; Le Pavec, J.; Humbert, M.; Simonneau, G.; Sitbon, O. HIV-associated pulmonary arterial hypertension: survival and prognostic factors in the modern therapeutic era. AIDS, 2010, 24(1), 67-75.
[http://dx.doi.org/10.1097/QAD.0b013e328331c65e] [PMID: 19770696]
[54]
Asrani, S.K.; Devarbhavi, H.; Eaton, J.; Kamath, P.S. Burden of liver diseases in the world. J. Hepatol., 2019, 70(1), 151-171.
[http://dx.doi.org/10.1016/j.jhep.2018.09.014] [PMID: 30266282]
[55]
Colle, I.; Moreau, R.; Godinho, E.; Belghiti, J.; Ettori, F.; Cohen-Solal, A.; Mal, H.; Bernuau, J.; Marty, J.; Lebrec, D.; Valla, D.; Durand, F. Diagnosis of portopulmonary hypertension in candidates for liver transplantation: A prospective study. Hepatology, 2003, 37(2), 401-409.
[http://dx.doi.org/10.1053/jhep.2003.50060] [PMID: 12540791]
[56]
Krowka, M.J.; Miller, D.P.; Barst, R.J.; Taichman, D.; Dweik, R.A.; Badesch, D.B.; McGoon, M.D. Portopulmonary hypertension. Chest, 2012, 141(4), 906-915.
[http://dx.doi.org/10.1378/chest.11-0160] [PMID: 21778257]
[57]
Khaderi, S.; Khan, R.; Safdar, Z.; Stribling, R.; Vierling, J.M.; Goss, J.A.; Sussman, N.L. Long-term follow-up of portopulmonary hypertension patients after liver transplantation. Liver Transpl., 2014, 20(6), 724-727.
[http://dx.doi.org/10.1002/lt.23870] [PMID: 24648168]
[58]
Rodríguez-Roisin, R.; Krowka, M.J.; Hervé, P.; Fallon, M.B. Pulmonary-Hepatic vascular Disorders (PHD). Eur. Respir. J., 2004, 24(5), 861-880.
[http://dx.doi.org/10.1183/09031936.04.00010904] [PMID: 15516683]
[59]
Saleemi, S. Portopulmonary hypertension. Ann. Thorac. Med., 2010, 5(1), 5-9.
[http://dx.doi.org/10.4103/1817-1737.58953] [PMID: 20351954]
[60]
Kawut, S.M.; Krowka, M.J.; Trotter, J.F.; Roberts, K.E.; Benza, R.L.; Badesch, D.B.; Taichman, D.B.; Horn, E.M.; Zacks, S.; Kaplowitz, N.; Brown, R.S., Jr; Fallon, M.B. Clinical risk factors for portopulmonary hypertension. Hepatology, 2008, 48(1), 196-203.
[http://dx.doi.org/10.1002/hep.22275] [PMID: 18537192]
[61]
Swanson, K.L.; Wiesner, R.H.; Nyberg, S.L.; Rosen, C.B.; Krowka, M.J. Survival in portopulmonary hypertension: Mayo Clinic experience categorized by treatment subgroups. Am. J. Transplant., 2008, 8(11), 2445-2453.
[http://dx.doi.org/10.1111/j.1600-6143.2008.02384.x] [PMID: 18782292]
[62]
Krowka, M.J.; Swanson, K.L.; Frantz, R.P.; McGoon, M.D.; Wiesner, R.H. Portopulmonary hypertension: Results from a 10-year screening algorithm. Hepatology, 2006, 44(6), 1502-1510.
[http://dx.doi.org/10.1002/hep.21431] [PMID: 17133488]
[63]
Schwartz, S.S.; Madsen, N.; Laursen, H.B.; Hirsch, R.; Olsen, M.S. Incidence and mortality of adults with pulmonary hypertension and congenital heart disease. Am. J. Cardiol., 2018, 121(12), 1610-1616.
[http://dx.doi.org/10.1016/j.amjcard.2018.02.051] [PMID: 29655882]
[64]
Simonneau, G.; Galiè, N.; Rubin, L.J.; Langleben, D.; Seeger, W.; Domenighetti, G.; Gibbs, S.; Lebrec, D.; Speich, R.; Beghetti, M.; Rich, S.; Fishman, A. Clinical classification of pulmonary hypertension. J. Am. Coll. Cardiol., 2004, 43(12), S5-S12.
[http://dx.doi.org/10.1016/j.jacc.2004.02.037] [PMID: 15194173]
[65]
del Cerro, M.J.; Abman, S.; Diaz, G.; Freudenthal, A.H.; Freudenthal, F.; Harikrishnan, S.; Haworth, S.G.; Ivy, D.; Lopes, A.A.; Raj, J.U.; Sandoval, J.; Stenmark, K.; Adatia, I. A consensus approach to the classification of pediatric pulmonary hypertensive vascular disease: Report from the PVRI Pediatric Taskforce, Panama 2011. Pulm. Circ., 2011, 1(2), 286-298.
[http://dx.doi.org/10.4103/2045-8932.83456] [PMID: 21874158]
[66]
Steele, P.M.; Fuster, V.; Cohen, M.; Ritter, D.G.; McGoon, D.C. Isolated atrial septal defect with pulmonary vascular obstructive disease--long-term follow-up and prediction of outcome after surgical correction. Circulation, 1987, 76(5), 1037-1042.
[http://dx.doi.org/10.1161/01.CIR.76.5.1037] [PMID: 3664992]
[67]
Barst, R.J.; McGoon, M.D.; Elliott, C.G.; Foreman, A.J.; Miller, D.P.; Ivy, D.D. Survival in childhood pulmonary arterial hypertension: Insights from the registry to evaluate early and long-term pulmonary arterial hypertension disease management. Circulation, 2012, 125(1), 113-122.
[http://dx.doi.org/10.1161/CIRCULATIONAHA.111.026591] [PMID: 22086881]
[68]
Kozlik-Feldmann, R.; Hansmann, G.; Bonnet, D.; Schranz, D.; Apitz, C.; Michel-Behnke, I. Pulmonary hypertension in children with congenital heart disease (PAH-CHD, PPHVD-CHD). Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK. Heart, 2016, 102(Suppl. 2), ii42-ii48.
[http://dx.doi.org/10.1136/heartjnl-2015-308378] [PMID: 27053697]
[69]
Lapa, M.; Dias, B.; Jardim, C.; Fernandes, C.J.C.; Dourado, P.M.M.; Figueiredo, M.; Farias, A.; Tsutsui, J.; Terra-Filho, M.; Humbert, M.; Souza, R. Cardiopulmonary manifestations of hepatosplenic schistosomiasis. Circulation, 2009, 119(11), 1518-1523.
[http://dx.doi.org/10.1161/CIRCULATIONAHA.108.803221] [PMID: 19273723]
[70]
Da Silva, L.C.; Carrilho, F.J. Hepatosplenic schistosomiasis. Pathophysiology and treatment. Gastroenterol. Clin. North Am., 1992, 21(1), 163-177.
[http://dx.doi.org/10.1016/S0889-8553(21)00183-7] [PMID: 1568771]
[71]
Crosby, A.; Soon, E.; Jones, F.M.; Southwood, M.R.; Haghighat, L.; Toshner, M.R.; Raine, T.; Horan, I.; Yang, P.; Moore, S.; Ferrer, E.; Wright, P.; Ormiston, M.L.; White, R.J.; Haight, D.A.; Dunne, D.W.; Morrell, N.W. Hepatic shunting of eggs and pulmonary vascular remodeling in Bmpr2+/− Mice with Schistosomiasis. Am. J. Respir. Crit. Care Med., 2015, 192(11), 1355-1365.
[http://dx.doi.org/10.1164/rccm.201412-2262OC] [PMID: 26308618]
[72]
Graham, B.B.; Bandeira, A.P.; Morrell, N.W.; Butrous, G.; Tuder, R.M. Schistosomiasis-associated pulmonary hypertension: Psulmonary vascular disease: the global perspective. Chest, 2010, 137(6), 20S-29S.
[http://dx.doi.org/10.1378/chest.10-0048] [PMID: 20522577]
[73]
Thadani, U.; Burrow, C.; Whitaker, W.; Heath, D. Pulmonary veno-occlusive disease. Q. J. Med., 1975, 44(173), 133-159.
[PMID: 1153689]
[74]
Huertas, A.; Girerd, B.; Dorfmuller, P.; O’Callaghan, D.; Humbert, M.; Montani, D. Pulmonary veno-occlusive disease: Advances in clinical management and treatments. Expert Rev. Respir. Med., 2011, 5(2), 217-231.
[http://dx.doi.org/10.1586/ers.11.15] [PMID: 21510732]
[75]
Montani, D.; Achouh, L.; Dorfmüller, P.; Le Pavec, J.; Sztrymf, B.; Tchérakian, C.; Rabiller, A.; Haque, R.; Sitbon, O.; Jaïs, X.; Dartevelle, P.; Maître, S.; Capron, F.; Musset, D.; Simonneau, G.; Humbert, M. Pulmonary veno-occlusive disease: Clinical, functional, radiologic, and hemodynamic characteristics and outcome of 24 cases confirmed by histology. Medicine, 2008, 87(4), 220-233.
[http://dx.doi.org/10.1097/MD.0b013e31818193bb] [PMID: 18626305]
[76]
Heath, D.; Segel, N.; Bishop, J. Pulmonary veno-occlusive disease. Circulation, 1966, 34(2), 242-248.
[http://dx.doi.org/10.1161/01.CIR.34.2.242] [PMID: 5969356]
[77]
Eyries, M.; Montani, D.; Girerd, B.; Perret, C.; Leroy, A.; Lonjou, C.; Chelghoum, N.; Coulet, F.; Bonnet, D.; Dorfmüller, P.; Fadel, E.; Sitbon, O.; Simonneau, G.; Tregouët, D.A.; Humbert, M.; Soubrier, F. EIF2AK4 mutations cause pulmonary veno-occlusive disease, a recessive form of pulmonary hypertension. Nat. Genet., 2014, 46(1), 65-69.
[http://dx.doi.org/10.1038/ng.2844] [PMID: 24292273]
[78]
Tenorio, J.; Navas, P.; Barrios, E.; Fernández, L.; Nevado, J.; Quezada, C.A.; López-Meseguer, M.; Arias, P.; Mena, R.; Lobo, J.L.; Alvarez, C.; Heath, K.; Escribano-Subías, P.; Lapunzina, P. A founder EIF2AK4 mutation causes an aggressive form of pulmonary arterial hypertension in Iberian Gypsies. Clin. Genet., 2015, 88(6), 579-583.
[http://dx.doi.org/10.1111/cge.12549] [PMID: 25512148]
[79]
Montani, D.; Lau, E.M.; Descatha, A.; Jaïs, X.; Savale, L.; Andujar, P.; Bensefa-Colas, L.; Girerd, B.; Zendah, I.; Le Pavec, J.; Seferian, A.; Perros, F.; Dorfmüller, P.; Fadel, E.; Soubrier, F.; Sitbon, O.; Simonneau, G.; Humbert, M. Occupational exposure to organic solvents: A risk factor for pulmonary veno-occlusive disease. Eur. Respir. J., 2015, 46(6), 1721-1731.
[http://dx.doi.org/10.1183/13993003.00814-2015] [PMID: 26541523]
[80]
Dorfmüller, P.; Montani, D.; Humbert, M. Beyond arterial remodelling: Pulmonary venous and cardiac involvement in patients with systemic sclerosis-associated pulmonary arterial hypertension. Eur. Respir. J., 2010, 35(1), 6-8.
[http://dx.doi.org/10.1183/09031936.00081009] [PMID: 20044455]
[81]
Johnson, S.R.; Patsios, D.; Hwang, D.M.; Granton, J.T. Pulmonary veno-occlusive disease and scleroderma associated pulmonary hypertension. J. Rheumatol., 2006, 33(11), 2347-2350.
[PMID: 16981286]
[82]
Hoffstein, V.; Ranganathan, N.; Mullen, J.B. Sarcoidosis simulating pulmonary veno-occlusive disease. Am. Rev. Respir. Dis., 1986, 134(4), 809-811.
[http://dx.doi.org/10.1164/arrd.1986.134.4.809] [PMID: 3767134]
[83]
Hamada, K.; Teramoto, S.; Narita, N.; Yamada, E.; Teramoto, K.; Kobzik, L. Pulmonary veno-occlusive disease in pulmonary Langerhans′ cell granulomatosis. Eur. Respir. J., 2000, 15(2), 421-423.
[http://dx.doi.org/10.1034/j.1399-3003.2000.15b33.x] [PMID: 10706515]
[84]
Kokturk, N.; Demir, N.; Demircan, S.; Memis, L.; Kurul, C.; Akyurek, N.; Turktas, H. Pulmonary veno-occlusive disease in a patient with a history of Hashimoto’s thyroiditis. Indian J. Chest Dis. Allied Sci., 2005, 47(4), 289-292.
[PMID: 16255401]
[85]
Frazier, A.A.; Franks, T.J.; Mohammed, T.L.H.; Ozbudak, I.H.; Galvin, J.R. From the Archives of the AFIP: pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis. Radiographics, 2007, 27(3), 867-882.
[http://dx.doi.org/10.1148/rg.273065194] [PMID: 17495297]
[86]
Resten, A.; Maitre, S.; Humbert, M.; Rabiller, A.; Sitbon, O.; Capron, F.; Simonneau, G.; Musset, D. Pulmonary hypertension: CT of the chest in pulmonary venoocclusive disease. AJR Am. J. Roentgenol., 2004, 183(1), 65-70.
[http://dx.doi.org/10.2214/ajr.183.1.1830065] [PMID: 15208112]
[87]
Dufour, B.; Maître, S.; Humbert, M.; Capron, F.; Simonneau, G.; Musset, D. High-resolution CT of the chest in four patients with pulmonary capillary hemangiomatosis or pulmonary venoocclusive disease. AJR Am. J. Roentgenol., 1998, 171(5), 1321-1324.
[http://dx.doi.org/10.2214/ajr.171.5.9798872] [PMID: 9798872]
[88]
Swensen, S.J.; Tashjian, J.H.; Myers, J.L.; Engeler, C.E.; Patz, E.F.; Edwards, W.D.; Douglas, W.W. Pulmonary venoocclusive disease: CT findings in eight patients. AJR Am. J. Roentgenol., 1996, 167(4), 937-940.
[http://dx.doi.org/10.2214/ajr.167.4.8819387] [PMID: 8819387]
[89]
Lantuéjoul, S.; Sheppard, M.N.; Corrin, B.; Burke, M.M.; Nicholson, A.G. Pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis: A clinicopathologic study of 35 cases. Am. J. Surg. Pathol., 2006, 30(7), 850-857.
[http://dx.doi.org/10.1097/01.pas.0000209834.69972.e5] [PMID: 16819327]
[90]
Montani, D.; Lau, E.M.; Dorfmüller, P.; Girerd, B.; Jaïs, X.; Savale, L.; Perros, F.; Nossent, E.; Garcia, G.; Parent, F.; Fadel, E.; Soubrier, F.; Sitbon, O.; Simonneau, G.; Humbert, M. Pulmonary veno-occlusive disease. Eur. Respir. J., 2016, 47(5), 1518-1534.
[http://dx.doi.org/10.1183/13993003.00026-2016] [PMID: 27009171]
[91]
Hansmann, G. Pulmonary hypertension in infants, children, and young adults. J. Am. Coll. Cardiol., 2017, 69(20), 2551-2569.
[http://dx.doi.org/10.1016/j.jacc.2017.03.575] [PMID: 28521893]
[92]
Gao, Y.; Raj, J.U. Regulation of the pulmonary circulation in the fetus and newborn. Physiol. Rev., 2010, 90(4), 1291-1335.
[http://dx.doi.org/10.1152/physrev.00032.2009] [PMID: 20959617]
[93]
Abman, S.H. Recent advances in the pathogenesis and treatment of persistent pulmonary hypertension of the newborn. Neonatology, 2007, 91(4), 283-290.
[http://dx.doi.org/10.1159/000101343] [PMID: 17575471]
[94]
Konduri, GG; Kim, UO Advances in the diagnosis and management of persistent pulmonary hypertension of the newborn. Pediatr Clin North Am, 2009, 56(3), 579.
[http://dx.doi.org/10.1016/j.pcl.2009.04.004]
[95]
Abman, S.H. Impaired vascular endothelial growth factor signaling in the pathogenesis of neonatal pulmonary vascular disease. Adv. Exp. Med. Biol., 2010, 661, 323-335.
[http://dx.doi.org/10.1007/978-1-60761-500-2_21] [PMID: 20204740]
[96]
Yoder, B.A.; Kirsch, E.A.; Barth, W.H., Jr; Gordon, M.C. Changing obstetric practices associated with decreasing incidence of meconium aspiration syndrome. Obstet. Gynecol., 2002, 99(5, Part 1), 731-739.
[http://dx.doi.org/10.1097/00006250-200205000-00011] [PMID: 11978280]
[97]
’t Jong, G.W.; Einarson, T.; Koren, G.; Einarson, A. Antidepressant use in pregnancy and persistent pulmonary hypertension of the newborn (PPHN): A systematic review. Reprod. Toxicol., 2012, 34(3), 293-297.
[http://dx.doi.org/10.1016/j.reprotox.2012.04.015] [PMID: 22564982]
[98]
Galiè, N.; Humbert, M.; Vachiery, J.L.; Gibbs, S.; Lang, I.; Torbicki, A.; Simonneau, G.; Peacock, A.; Vonk Noordegraaf, A.; Beghetti, M.; Ghofrani, A.; Gomez Sanchez, M.A.; Hansmann, G.; Klepetko, W.; Lancellotti, P.; Matucci, M.; McDonagh, T.; Pierard, L.A.; Trindade, P.T.; Zompatori, M.; Hoeper, M. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur. Respir. J., 2015, 46(4), 903-975.
[http://dx.doi.org/10.1183/13993003.01032-2015] [PMID: 26318161]
[99]
Galiè, N.; Saia, F.; Palazzini, M.; Manes, A.; Russo, V.; Bacchi Reggiani, M.L.; Dall’Ara, G.; Monti, E.; Dardi, F.; Albini, A.; Rinaldi, A.; Gotti, E.; Taglieri, N.; Marrozzini, C.; Lovato, L.; Zompatori, M.; Marzocchi, A. Left main coronary artery compression in patients with pulmonary arterial hypertension and angina. J. Am. Coll. Cardiol., 2017, 69(23), 2808-2817.
[http://dx.doi.org/10.1016/j.jacc.2017.03.597] [PMID: 28595696]
[100]
Jing, Z.C.; Xu, X.Q.; Han, Z.Y.; Wu, Y.; Deng, K.W.; Wang, H.; Wang, Z.W.; Cheng, X.S.; Xu, B.; Hu, S.S.; Hui, R.T.; Yang, Y.J. Registry and survival study in chinese patients with idiopathic and familial pulmonary arterial hypertension. Chest, 2007, 132(2), 373-379.
[http://dx.doi.org/10.1378/chest.06-2913] [PMID: 17400671]
[101]
Rich, S.; Dantzker, D.R.; Ayres, S.M.; Bergofsky, E.H.; Brundage, B.H.; Detre, K.M.; Fishman, A.P.; Goldring, R.M.; Groves, B.M.; Koerner, S.K. Primary pulmonary hypertension. A national prospective study. Ann. Intern. Med., 1987, 107(2), 216-223.
[http://dx.doi.org/10.7326/0003-4819-107-2-216] [PMID: 3605900]
[102]
Henkens, I.R.; Mouchaers, K.T.B.; Vonk-Noordegraaf, A.; Boonstra, A.; Swenne, C.A.; Maan, A.C.; Man, S.C.; Twisk, J.W.R.; van der Wall, E.E.; Schalij, M.J.; Vliegen, H.W. Improved ECG detection of presence and severity of right ventricular pressure load validated with cardiac magnetic resonance imaging. Am. J. Physiol. Heart Circ. Physiol., 2008, 294(5), H2150-H2157.
[http://dx.doi.org/10.1152/ajpheart.01312.2007] [PMID: 18310513]
[103]
Romano, A.M.; Tomaselli, S.; Gualtieri, G.; Zoia, M.C.; Fanfulla, F.; Berrayah, L.; Cerveri, I. Respiratory function in precapillary pulmonary hypertension. Monaldi Arch. Chest Dis., 1993, 48(3), 201-204.
[PMID: 8369783]
[104]
Meyer, F.J.; Ewert, R.; Hoeper, M.M.; Olschewski, H.; Behr, J.; Winkler, J.; Wilkens, H.; Breuer, C.; Kübler, W.; Borst, M.M. Peripheral airway obstruction in primary pulmonary hypertension. Thorax, 2002, 57(6), 473-476.
[http://dx.doi.org/10.1136/thorax.57.6.473] [PMID: 12037220]
[105]
Sun, X.G.; Hansen, J.E.; Oudiz, R.J.; Wasserman, K. Pulmonary function in primary pulmonary hypertension. J. Am. Coll. Cardiol., 2003, 41(6), 1028-1035.
[http://dx.doi.org/10.1016/S0735-1097(02)02964-9] [PMID: 12651053]
[106]
Farha, S.; Laskowski, D.; George, D.; Park, M.M.; Tang, W.H.W.; Dweik, R.A.; Erzurum, S.C. Loss of alveolar membrane diffusing capacity and pulmonary capillary blood volume in pulmonary arterial hypertension. Respir. Res., 2013, 14(1), 6.
[http://dx.doi.org/10.1186/1465-9921-14-6] [PMID: 23339456]
[107]
He, J.; Fang, W.; Lv, B.; He, J.G.; Xiong, C.M.; Liu, Z.H.; He, Z.X. Diagnosis of chronic thromboembolic pulmonary hypertension. Nucl. Med. Commun., 2012, 33(5), 459-463.
[http://dx.doi.org/10.1097/MNM.0b013e32835085d9] [PMID: 22262242]
[108]
Tunariu, N.; Gibbs, S.J.R.; Win, Z.; Gin-Sing, W.; Graham, A.; Gishen, P.; AL-Nahhas, A. Ventilation-perfusion scintigraphy is more sensitive than multidetector CTPA in detecting chronic thromboembolic pulmonary disease as a treatable cause of pulmonary hypertension. J. Nucl. Med., 2007, 48(5), 680-684.
[http://dx.doi.org/10.2967/jnumed.106.039438] [PMID: 17475953]
[109]
Soler, X.; Hoh, C.K.; Test, V.J.; Kerr, K.M.; Marsh, J.J.; Morris, T.A. Single photon emission computed tomography in chronic thromboembolic pulmonary hypertension. Respirology, 2011, 16(1), 131-137.
[http://dx.doi.org/10.1111/j.1440-1843.2010.01867.x] [PMID: 20920137]
[110]
Seferian, A.; Helal, B.; Jaïs, X.; Girerd, B.; Price, L.C.; Günther, S.; Savale, L.; Dorfmüller, P.; Parent, F.; Sitbon, O.; Humbert, M.; Simonneau, G.; Montani, D. Ventilation/perfusion lung scan in pulmonary veno-occlusive disease. Eur. Respir. J., 2012, 40(1), 75-83.
[http://dx.doi.org/10.1183/09031936.00097911] [PMID: 22088969]
[111]
Giordano, J.; Khung, S.; Duhamel, A.; Hossein-Foucher, C.; Bellèvre, D.; Lamblin, N.; Remy, J.; Remy-Jardin, M. Lung perfusion characteristics in pulmonary arterial hypertension (PAH) and peripheral forms of chronic thromboembolic pulmonary hypertension (pCTEPH): Dual-energy CT experience in 31 patients. Eur. Radiol., 2017, 27(4), 1631-1639.
[http://dx.doi.org/10.1007/s00330-016-4500-6] [PMID: 27480438]
[112]
Ende-Verhaar, Y.M.; Meijboom, L.J.; Kroft, L.J.M.; Beenen, L.F.M.; Boon, G.J.A.M.; Middeldorp, S.; Nossent, E.J.; Symersky, P.; Huisman, M.V.; Bogaard, H.J.; Vonk Noordegraaf, A.; Klok, F.A. Usefulness of standard computed tomography pulmonary angiography performed for acute pulmonary embolism for identification of chronic thromboembolic pulmonary hypertension: results of the InShape III study. J. Heart Lung Transplant., 2019, 38(7), 731-738.
[http://dx.doi.org/10.1016/j.healun.2019.03.003] [PMID: 30962147]
[113]
Sanz, J.; Kuschnir, P.; Rius, T.; Salguero, R.; Sulica, R.; Einstein, A.J.; Dellegrottaglie, S.; Fuster, V.; Rajagopalan, S.; Poon, M. Pulmonary arterial hypertension: Noninvasive detection with phase-contrast MR imaging. Radiology, 2007, 243(1), 70-79.
[http://dx.doi.org/10.1148/radiol.2431060477] [PMID: 17329691]
[114]
Sun, X.G.; Hansen, J.E.; Oudiz, R.J.; Wasserman, K. Exercise pathophysiology in patients with primary pulmonary hypertension. Circulation, 2001, 104(4), 429-435.
[http://dx.doi.org/10.1161/hc2901.093198] [PMID: 11468205]
[115]
Hansen, J.E.; Ulubay, G.; Chow, B.F.; Sun, X.G.; Wasserman, K. Mixed-expired and end-tidal CO2 distinguish between ventilation and perfusion defects during exercise testing in patients with lung and heart diseases. Chest, 2007, 132(3), 977-983.
[http://dx.doi.org/10.1378/chest.07-0619] [PMID: 17573506]
[116]
Sun, X.G.; Hansen, J.E.; Oudiz, R.J.; Wasserman, K. Gas exchange detection of exercise-induced right-to-left shunt in patients with primary pulmonary hypertension. Circulation, 2002, 105(1), 54-60.
[http://dx.doi.org/10.1161/hc0102.101509] [PMID: 11772876]
[117]
Grapsa, J.; Pereira Nunes, M.C.; Tan, T.C.; Cabrita, I.Z.; Coulter, T.; Smith, B.C.F.; Dawson, D.; Gibbs, J.S.R.; Nihoyannopoulos, P. Echocardiographic and hemodynamic predictors of survival in precapillary pulmonary hypertension. Circ. Cardiovasc. Imaging, 2015, 8(6), e002107.
[http://dx.doi.org/10.1161/CIRCIMAGING.114.002107] [PMID: 26063743]
[118]
Lang, R.M.; Badano, L.P.; Mor-Avi, V.; Afilalo, J.; Armstrong, A.; Ernande, L.; Flachskampf, F.A.; Foster, E.; Goldstein, S.A.; Kuznetsova, T.; Lancellotti, P.; Muraru, D.; Picard, M.H.; Rietzschel, E.R.; Rudski, L.; Spencer, K.T.; Tsang, W.; Voigt, J.U. Recommendations for cardiac chamber quantification by echocardiography in adults: An update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging. J. Am. Soc. Echocardiogr., 2015, 28(1), 1-39.e14.
[http://dx.doi.org/10.1016/j.echo.2014.10.003] [PMID: 25559473]
[119]
Hoeper, M.M.; Lee, S.H.; Voswinckel, R.; Palazzini, M.; Jais, X.; Marinelli, A.; Barst, R.J.; Ghofrani, H.A.; Jing, Z.C.; Opitz, C.; Seyfarth, H.J.; Halank, M.; McLaughlin, V.; Oudiz, R.J.; Ewert, R.; Wilkens, H.; Kluge, S.; Bremer, H.C.; Baroke, E.; Rubin, L.J. Complications of right heart catheterization procedures in patients with pulmonary hypertension in experienced centers. J. Am. Coll. Cardiol., 2006, 48(12), 2546-2552.
[http://dx.doi.org/10.1016/j.jacc.2006.07.061] [PMID: 17174196]
[120]
Halpern, S.D.; Taichman, D.B. Misclassification of pulmonary hypertension due to reliance on pulmonary capillary wedge pressure rather than left ventricular end-diastolic pressure. Chest, 2009, 136(1), 37-43.
[http://dx.doi.org/10.1378/chest.08-2784] [PMID: 19255293]
[121]
Krishnan, A.; Markham, R.; Savage, M.; Wong, Y.W.; Walters, D. Right heart catheterisation: How to do it. Heart Lung Circ., 2019, 28(4), e71-e78.
[http://dx.doi.org/10.1016/j.hlc.2018.08.005] [PMID: 30253970]
[122]
Argueta, E.E.; Paniagua, D. Thermodilution cardiac output. Cardiol. Rev., 2019, 27(3), 138-144.
[http://dx.doi.org/10.1097/CRD.0000000000000223] [PMID: 30946701]
[123]
Herve, P.; Lau, E.M.; Sitbon, O.; Savale, L.; Montani, D.; Godinas, L.; Lador, F.; Jaïs, X.; Parent, F.; Günther, S.; Humbert, M.; Simonneau, G.; Chemla, D. Criteria for diagnosis of exercise pulmonary hypertension. Eur. Respir. J., 2015, 46(3), 728-737.
[http://dx.doi.org/10.1183/09031936.00021915] [PMID: 26022955]
[124]
Eisman, A.S.; Shah, R.V.; Dhakal, B.P.; Pappagianopoulos, P.P.; Wooster, L.; Bailey, C.; Cunningham, T.F.; Hardin, K.M.; Baggish, A.L.; Ho, J.E.; Malhotra, R.; Lewis, G.D. Pulmonary capillary wedge pressure patterns during exercise predict exercise capacity and incident heart failure. Circ. Heart Fail., 2018, 11(5), e004750.
[http://dx.doi.org/10.1161/CIRCHEARTFAILURE.117.004750] [PMID: 29695381]
[125]
D’Alto, M.; Romeo, E.; Argiento, P.; Motoji, Y.; Correra, A.; Di Marco, G.M.; Iacono, A.M.; Barracano, R.; D’Andrea, A.; Rea, G.; Sarubbi, B.; Russo, M.G.; Naeije, R. Clinical relevance of fluid challenge in patients evaluated for pulmonary hypertension. Chest, 2017, 151(1), 119-126.
[http://dx.doi.org/10.1016/j.chest.2016.08.1439] [PMID: 27575357]

Rights & Permissions Print Cite
Article Metrics
10
1
© 2024 Bentham Science Publishers | Privacy Policy