Skip to main content
Top
Published in: Heart Failure Reviews 3/2016

01-05-2016

Severe pulmonary arterial hypertension: stratification of medical therapies, mechanical support, and lung transplantation

Authors: Sonja Darrell Bartolome, Fernando Torres

Published in: Heart Failure Reviews | Issue 3/2016

Login to get access

Abstract

Severe pulmonary hypertension is recognized by focusing on the clinical signs and tests that demonstrate decompensated right ventricular failure or, in the worst-case scenario, shock. An aggressive treatment regimen including a prostacyclin infusion is required for these patients. Once admitted to the hospital, or the ICU for decompensated right ventricular failure, the short- and long-term outcomes for PAH patients are poor. For those who are candidates, urgent lung transplantation, or extracorporeal membrane oxygenation (ECMO) as a bridge to lung transplantation may be rescue therapy.
Literature
1.
go back to reference Taichman DB, Ornelas J, Chung L et al (2014) Pharmacologic therapy for pulmonary arterial hypertension in adults: CHEST guideline and expert panel report. Chest 146(2):449–475CrossRefPubMedPubMedCentral Taichman DB, Ornelas J, Chung L et al (2014) Pharmacologic therapy for pulmonary arterial hypertension in adults: CHEST guideline and expert panel report. Chest 146(2):449–475CrossRefPubMedPubMedCentral
2.
go back to reference McLaughlin VV, Archer SL, Badesch DB et al (2009) ACCF/AHA 2009 expert consensus document on pulmonary hypertension a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association developed in collaboration with the American College of Chest Physicians; American Thoracic Society, Inc.; and the Pulmonary Hypertension Association. J Am Coll Cardiol 53(17):1573–1619CrossRefPubMed McLaughlin VV, Archer SL, Badesch DB et al (2009) ACCF/AHA 2009 expert consensus document on pulmonary hypertension a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association developed in collaboration with the American College of Chest Physicians; American Thoracic Society, Inc.; and the Pulmonary Hypertension Association. J Am Coll Cardiol 53(17):1573–1619CrossRefPubMed
3.
go back to reference Benza RL, Miller DP, Gomberg-Maitland M et al (2010) Predicting survival in pulmonary arterial hypertension: insights from the registry to evaluate early and long-term pulmonary arterial hypertension disease management (REVEAL). Circulation 122(2):164–172CrossRefPubMed Benza RL, Miller DP, Gomberg-Maitland M et al (2010) Predicting survival in pulmonary arterial hypertension: insights from the registry to evaluate early and long-term pulmonary arterial hypertension disease management (REVEAL). Circulation 122(2):164–172CrossRefPubMed
4.
go back to reference Humbert M, Sitbon O, Chaouat A et al (2010) Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era. Circulation 122(2):156–163CrossRefPubMed Humbert M, Sitbon O, Chaouat A et al (2010) Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era. Circulation 122(2):156–163CrossRefPubMed
5.
6.
go back to reference Raymond RJ, Hinderliter AL, Willis PW et al (2002) Echocardiographic predictors of adverse outcomes in primary pulmonary hypertension. J Am Coll Cardiol 39(7):1214–1219CrossRefPubMed Raymond RJ, Hinderliter AL, Willis PW et al (2002) Echocardiographic predictors of adverse outcomes in primary pulmonary hypertension. J Am Coll Cardiol 39(7):1214–1219CrossRefPubMed
7.
go back to reference Forfia PR, Fisher MR, Mathai SC et al (2006) Tricuspid annular displacement predicts survival in pulmonary hypertension. Am J Respir Crit Care Med 174(9):1034–1041CrossRefPubMed Forfia PR, Fisher MR, Mathai SC et al (2006) Tricuspid annular displacement predicts survival in pulmonary hypertension. Am J Respir Crit Care Med 174(9):1034–1041CrossRefPubMed
8.
go back to reference Mathai SC, Sibley CT, Forfia PR et al (2011) Tricuspid annular plane systolic excursion is a robust outcome measure in systemic sclerosis-associated pulmonary arterial hypertension. J Rheumatol 38(11):2410–2418CrossRefPubMed Mathai SC, Sibley CT, Forfia PR et al (2011) Tricuspid annular plane systolic excursion is a robust outcome measure in systemic sclerosis-associated pulmonary arterial hypertension. J Rheumatol 38(11):2410–2418CrossRefPubMed
9.
go back to reference McLaughlin VV, Gaine SP, Howard LS et al (2013) Treatment goals of pulmonary hypertension. J Am Coll Cardiol 62(25 Suppl):D73–D81CrossRefPubMed McLaughlin VV, Gaine SP, Howard LS et al (2013) Treatment goals of pulmonary hypertension. J Am Coll Cardiol 62(25 Suppl):D73–D81CrossRefPubMed
10.
go back to reference van Wolferen SA, Marcus JT, Boonstra A et al (2007) Prognostic value of right ventricular mass, volume, and function in idiopathic pulmonary arterial hypertension. Eur Heart J 28(10):1250–1257CrossRefPubMed van Wolferen SA, Marcus JT, Boonstra A et al (2007) Prognostic value of right ventricular mass, volume, and function in idiopathic pulmonary arterial hypertension. Eur Heart J 28(10):1250–1257CrossRefPubMed
11.
go back to reference Yamada Y, Okuda S, Kataoka M et al (2012) Prognostic value of cardiac magnetic resonance imaging for idiopathic pulmonary arterial hypertension before initiating intravenous prostacyclin therapy. Circ J 76(7):1737–1743CrossRefPubMed Yamada Y, Okuda S, Kataoka M et al (2012) Prognostic value of cardiac magnetic resonance imaging for idiopathic pulmonary arterial hypertension before initiating intravenous prostacyclin therapy. Circ J 76(7):1737–1743CrossRefPubMed
12.
go back to reference van de Veerdonk MC, Kind T, Marcus JT et al (2011) Progressive right ventricular dysfunction in patients with pulmonary arterial hypertension responding to therapy. J Am Coll Cardiol 58(24):2511–2519CrossRefPubMed van de Veerdonk MC, Kind T, Marcus JT et al (2011) Progressive right ventricular dysfunction in patients with pulmonary arterial hypertension responding to therapy. J Am Coll Cardiol 58(24):2511–2519CrossRefPubMed
13.
go back to reference Thenappan T, Shah SJ, Rich S, Tian L, Archer SL, Gomberg-Maitland M (2010) Survival in pulmonary arterial hypertension: a reappraisal of the NIH risk stratification equation. Eur Respir J 35(5):1079–1087CrossRefPubMed Thenappan T, Shah SJ, Rich S, Tian L, Archer SL, Gomberg-Maitland M (2010) Survival in pulmonary arterial hypertension: a reappraisal of the NIH risk stratification equation. Eur Respir J 35(5):1079–1087CrossRefPubMed
14.
go back to reference Humbert M, Sitbon O, Yaici A et al (2010) Survival in incident and prevalent cohorts of patients with pulmonary arterial hypertension. Eur Respir J 36(3):549–555CrossRefPubMed Humbert M, Sitbon O, Yaici A et al (2010) Survival in incident and prevalent cohorts of patients with pulmonary arterial hypertension. Eur Respir J 36(3):549–555CrossRefPubMed
15.
go back to reference Barst RJ, Rubin LJ, Long WA et al (1996) A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. N Engl J Med 334(5):296–301CrossRefPubMed Barst RJ, Rubin LJ, Long WA et al (1996) A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. N Engl J Med 334(5):296–301CrossRefPubMed
16.
go back to reference Schwaiblmair M, Faul C, von Scheidt W, Berghaus TM (2012) Ventilatory efficiency testing as prognostic value in patients with pulmonary hypertension. BMC Pulm Med 12:23CrossRefPubMedPubMedCentral Schwaiblmair M, Faul C, von Scheidt W, Berghaus TM (2012) Ventilatory efficiency testing as prognostic value in patients with pulmonary hypertension. BMC Pulm Med 12:23CrossRefPubMedPubMedCentral
17.
go back to reference Wensel R, Opitz CF, Anker SD et al (2002) Assessment of survival in patients with primary pulmonary hypertension: importance of cardiopulmonary exercise testing. Circulation 106(3):319–324CrossRefPubMed Wensel R, Opitz CF, Anker SD et al (2002) Assessment of survival in patients with primary pulmonary hypertension: importance of cardiopulmonary exercise testing. Circulation 106(3):319–324CrossRefPubMed
18.
go back to reference Nickel N, Golpon H, Greer M et al (2012) The prognostic impact of follow-up assessments in patients with idiopathic pulmonary arterial hypertension. Eur Respir J 39(3):589–596CrossRefPubMed Nickel N, Golpon H, Greer M et al (2012) The prognostic impact of follow-up assessments in patients with idiopathic pulmonary arterial hypertension. Eur Respir J 39(3):589–596CrossRefPubMed
19.
go back to reference Galie N, Humbert M, Vachiery JL et al (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 46(4):903–975CrossRefPubMed Galie N, Humbert M, Vachiery JL et al (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 46(4):903–975CrossRefPubMed
20.
go back to reference Benza RL, Gomberg-Maitland M, Miller DP et al (2012) The REVEAL Registry risk score calculator in patients newly diagnosed with pulmonary arterial hypertension. Chest 141(2):354–362CrossRefPubMed Benza RL, Gomberg-Maitland M, Miller DP et al (2012) The REVEAL Registry risk score calculator in patients newly diagnosed with pulmonary arterial hypertension. Chest 141(2):354–362CrossRefPubMed
21.
go back to reference Campo A, Mathai SC, Le Pavec J et al (2011) Outcomes of hospitalisation for right heart failure in pulmonary arterial hypertension. Eur Respir J 38(2):359–367CrossRefPubMed Campo A, Mathai SC, Le Pavec J et al (2011) Outcomes of hospitalisation for right heart failure in pulmonary arterial hypertension. Eur Respir J 38(2):359–367CrossRefPubMed
22.
go back to reference Haddad F, Peterson T, Fuh E et al (2011) Characteristics and outcome after hospitalization for acute right heart failure in patients with pulmonary arterial hypertension. Circ Heart Fail 4(6):692–699CrossRefPubMed Haddad F, Peterson T, Fuh E et al (2011) Characteristics and outcome after hospitalization for acute right heart failure in patients with pulmonary arterial hypertension. Circ Heart Fail 4(6):692–699CrossRefPubMed
23.
go back to reference Sztrymf B, Souza R, Bertoletti L et al (2010) Prognostic factors of acute heart failure in patients with pulmonary arterial hypertension. Eur Respir J 35(6):1286–1293CrossRefPubMed Sztrymf B, Souza R, Bertoletti L et al (2010) Prognostic factors of acute heart failure in patients with pulmonary arterial hypertension. Eur Respir J 35(6):1286–1293CrossRefPubMed
24.
go back to reference Ghignone M, Girling L, Prewitt RM (1984) Volume expansion versus norepinephrine in treatment of a low cardiac output complicating an acute increase in right ventricular afterload in dogs. Anesthesiology 60(2):132–135CrossRefPubMed Ghignone M, Girling L, Prewitt RM (1984) Volume expansion versus norepinephrine in treatment of a low cardiac output complicating an acute increase in right ventricular afterload in dogs. Anesthesiology 60(2):132–135CrossRefPubMed
25.
go back to reference Zamanian RT, Haddad F, Doyle RL, Weinacker AB (2007) Management strategies for patients with pulmonary hypertension in the intensive care unit. Crit Care Med 35(9):2037–2050CrossRefPubMed Zamanian RT, Haddad F, Doyle RL, Weinacker AB (2007) Management strategies for patients with pulmonary hypertension in the intensive care unit. Crit Care Med 35(9):2037–2050CrossRefPubMed
26.
go back to reference Tayama E, Ueda T, Shojima T et al (2007) Arginine vasopressin is an ideal drug after cardiac surgery for the management of low systemic vascular resistant hypotension concomitant with pulmonary hypertension. Interact CardioVasc Thorac Surg 6(6):715–719CrossRefPubMed Tayama E, Ueda T, Shojima T et al (2007) Arginine vasopressin is an ideal drug after cardiac surgery for the management of low systemic vascular resistant hypotension concomitant with pulmonary hypertension. Interact CardioVasc Thorac Surg 6(6):715–719CrossRefPubMed
27.
go back to reference Price LC, Forrest P, Sodhi V et al (2007) Use of vasopressin after Caesarean section in idiopathic pulmonary arterial hypertension. Br J Anaesth 99(4):552–555CrossRefPubMed Price LC, Forrest P, Sodhi V et al (2007) Use of vasopressin after Caesarean section in idiopathic pulmonary arterial hypertension. Br J Anaesth 99(4):552–555CrossRefPubMed
28.
go back to reference Kerbaul F, Rondelet B, Motte S et al (2004) Effects of norepinephrine and dobutamine on pressure load-induced right ventricular failure. Crit Care Med 32(4):1035–1040CrossRefPubMed Kerbaul F, Rondelet B, Motte S et al (2004) Effects of norepinephrine and dobutamine on pressure load-induced right ventricular failure. Crit Care Med 32(4):1035–1040CrossRefPubMed
29.
go back to reference Gordon C, Collard CD, Pan W (2010) Intraoperative management of pulmonary hypertension and associated right heart failure. Curr Opin Anaesthesiol 23(1):49–56CrossRefPubMed Gordon C, Collard CD, Pan W (2010) Intraoperative management of pulmonary hypertension and associated right heart failure. Curr Opin Anaesthesiol 23(1):49–56CrossRefPubMed
30.
go back to reference Pritts CD, Pearl RG (2010) Anesthesia for patients with pulmonary hypertension. Curr Opin Anaesthesiol 23(3):411–416CrossRefPubMed Pritts CD, Pearl RG (2010) Anesthesia for patients with pulmonary hypertension. Curr Opin Anaesthesiol 23(3):411–416CrossRefPubMed
31.
go back to reference Hoeper MM, Granton J (2011) Intensive care unit management of patients with severe pulmonary hypertension and right heart failure. Am J Respir Crit Care Med 184(10):1114–1124CrossRefPubMed Hoeper MM, Granton J (2011) Intensive care unit management of patients with severe pulmonary hypertension and right heart failure. Am J Respir Crit Care Med 184(10):1114–1124CrossRefPubMed
32.
go back to reference Simonneau G, Rubin LJ, Galie N et al (2008) Addition of sildenafil to long-term intravenous epoprostenol therapy in patients with pulmonary arterial hypertension: a randomized trial. Ann Intern Med 149(8):521–530CrossRefPubMed Simonneau G, Rubin LJ, Galie N et al (2008) Addition of sildenafil to long-term intravenous epoprostenol therapy in patients with pulmonary arterial hypertension: a randomized trial. Ann Intern Med 149(8):521–530CrossRefPubMed
33.
go back to reference Sitbon O, Jais X, Savale L et al (2014) Upfront triple combination therapy in pulmonary arterial hypertension: a pilot study. Eur Respir J 43(6):1691–1697CrossRefPubMed Sitbon O, Jais X, Savale L et al (2014) Upfront triple combination therapy in pulmonary arterial hypertension: a pilot study. Eur Respir J 43(6):1691–1697CrossRefPubMed
34.
go back to reference Bhorade S, Christenson J, O’Connor M, Lavoie A, Pohlman A, Hall JB (1999) Response to inhaled nitric oxide in patients with acute right heart syndrome. Am J Respir Crit Care Med 159(2):571–579CrossRefPubMed Bhorade S, Christenson J, O’Connor M, Lavoie A, Pohlman A, Hall JB (1999) Response to inhaled nitric oxide in patients with acute right heart syndrome. Am J Respir Crit Care Med 159(2):571–579CrossRefPubMed
35.
go back to reference Hardy JD, Webb WR, Dalton ML Jr, Walker GR Jr (1963) Lung homotransplantation in Man. JAMA 186:1065–1074CrossRefPubMed Hardy JD, Webb WR, Dalton ML Jr, Walker GR Jr (1963) Lung homotransplantation in Man. JAMA 186:1065–1074CrossRefPubMed
36.
go back to reference Reitz BA, Wallwork JL, Hunt SA et al (1982) Heart-lung transplantation: successful therapy for patients with pulmonary vascular disease. N Engl J Med 306(10):557–564CrossRefPubMed Reitz BA, Wallwork JL, Hunt SA et al (1982) Heart-lung transplantation: successful therapy for patients with pulmonary vascular disease. N Engl J Med 306(10):557–564CrossRefPubMed
37.
go back to reference Toronto Lung Transplant Group (1986) Unilateral lung transplantation for pulmonary fibrosis. N Engl J Med 314(18):1140–1145CrossRef Toronto Lung Transplant Group (1986) Unilateral lung transplantation for pulmonary fibrosis. N Engl J Med 314(18):1140–1145CrossRef
38.
go back to reference Pasque MK, Cooper JD, Kaiser LR, Haydock DA, Triantafillou A, Trulock EP (1990) Improved technique for bilateral lung transplantation: rationale and initial clinical experience. Ann Thorac Surg 49(5):785–791CrossRefPubMed Pasque MK, Cooper JD, Kaiser LR, Haydock DA, Triantafillou A, Trulock EP (1990) Improved technique for bilateral lung transplantation: rationale and initial clinical experience. Ann Thorac Surg 49(5):785–791CrossRefPubMed
39.
go back to reference Yusen RD, Christie JD, Edwards LB et al (2013) The registry of the International Society for heart and lung transplantation: thirtieth adult lung and heart-lung transplant report–2013; focus theme: age. J Heart Lung Transpl 32(10):965–978CrossRef Yusen RD, Christie JD, Edwards LB et al (2013) The registry of the International Society for heart and lung transplantation: thirtieth adult lung and heart-lung transplant report–2013; focus theme: age. J Heart Lung Transpl 32(10):965–978CrossRef
40.
go back to reference Yusen RD, Edwards LB, Kucheryavaya AY et al (2014) The registry of the International Society for Heart and Lung Transplantation: thirty-first adult lung and heart-lung transplant report–2014; focus theme: retransplantation. J Heart Lung Transpl 33(10):1009–1024CrossRef Yusen RD, Edwards LB, Kucheryavaya AY et al (2014) The registry of the International Society for Heart and Lung Transplantation: thirty-first adult lung and heart-lung transplant report–2014; focus theme: retransplantation. J Heart Lung Transpl 33(10):1009–1024CrossRef
41.
go back to reference Paden ML, Conrad SA, Rycus PT, Thiagarajan RR (2013) Extracorporeal life support organization registry report 2012. ASAIO J 59(3):202–210CrossRefPubMed Paden ML, Conrad SA, Rycus PT, Thiagarajan RR (2013) Extracorporeal life support organization registry report 2012. ASAIO J 59(3):202–210CrossRefPubMed
42.
go back to reference Marasco SF, Lukas G, McDonald M, McMillan J, Ihle B (2008) Review of ECMO (extra corporeal membrane oxygenation) support in critically ill adult patients. Heart Lung Circ 17(Suppl 4):S41–S47CrossRefPubMed Marasco SF, Lukas G, McDonald M, McMillan J, Ihle B (2008) Review of ECMO (extra corporeal membrane oxygenation) support in critically ill adult patients. Heart Lung Circ 17(Suppl 4):S41–S47CrossRefPubMed
43.
go back to reference Jackson A, Cropper J, Pye R, Junius F, Malouf M, Glanville A (2008) Use of extracorporeal membrane oxygenation as a bridge to primary lung transplant: 3 consecutive, successful cases and a review of the literature. J Heart Lung Transpl 27(3):348–352CrossRef Jackson A, Cropper J, Pye R, Junius F, Malouf M, Glanville A (2008) Use of extracorporeal membrane oxygenation as a bridge to primary lung transplant: 3 consecutive, successful cases and a review of the literature. J Heart Lung Transpl 27(3):348–352CrossRef
44.
go back to reference Santambrogio L, Nosotti M, Palleschi A et al (2009) Use of venovenous extracorporeal membrane oxygenation as a bridge to urgent lung transplantation in a case of acute respiratory failure. Transpl Proc 41(4):1345–1346CrossRef Santambrogio L, Nosotti M, Palleschi A et al (2009) Use of venovenous extracorporeal membrane oxygenation as a bridge to urgent lung transplantation in a case of acute respiratory failure. Transpl Proc 41(4):1345–1346CrossRef
45.
go back to reference Gibbon JH Jr (1954) Application of a mechanical heart and lung apparatus to cardiac surgery. Minn Med 37(3):171–185PubMed Gibbon JH Jr (1954) Application of a mechanical heart and lung apparatus to cardiac surgery. Minn Med 37(3):171–185PubMed
46.
go back to reference Hill JD, O’Brien TG, Murray JJ et al (1972) Prolonged extracorporeal oxygenation for acute post-traumatic respiratory failure (shock-lung syndrome). Use of the Bramson membrane lung. N Engl J Med 286(12):629–634CrossRefPubMed Hill JD, O’Brien TG, Murray JJ et al (1972) Prolonged extracorporeal oxygenation for acute post-traumatic respiratory failure (shock-lung syndrome). Use of the Bramson membrane lung. N Engl J Med 286(12):629–634CrossRefPubMed
47.
go back to reference Bartlett RH, Roloff DW, Custer JR, Younger JG, Hirschl RB (2000) Extracorporeal life support: the University of Michigan experience. JAMA 283(7):904–908CrossRefPubMed Bartlett RH, Roloff DW, Custer JR, Younger JG, Hirschl RB (2000) Extracorporeal life support: the University of Michigan experience. JAMA 283(7):904–908CrossRefPubMed
48.
go back to reference Hoopes CW, Gurley JC, Zwischenberger JB, Diaz-Guzman E (2012) Mechanical support for pulmonary veno-occlusive disease: combined atrial septostomy and venovenous extracorporeal membrane oxygenation. Semin Thorac Cardiovasc Surg 24(3):232–234CrossRefPubMed Hoopes CW, Gurley JC, Zwischenberger JB, Diaz-Guzman E (2012) Mechanical support for pulmonary veno-occlusive disease: combined atrial septostomy and venovenous extracorporeal membrane oxygenation. Semin Thorac Cardiovasc Surg 24(3):232–234CrossRefPubMed
Metadata
Title
Severe pulmonary arterial hypertension: stratification of medical therapies, mechanical support, and lung transplantation
Authors
Sonja Darrell Bartolome
Fernando Torres
Publication date
01-05-2016
Publisher
Springer US
Published in
Heart Failure Reviews / Issue 3/2016
Print ISSN: 1382-4147
Electronic ISSN: 1573-7322
DOI
https://doi.org/10.1007/s10741-016-9562-7

Other articles of this Issue 3/2016

Heart Failure Reviews 3/2016 Go to the issue