Diagnosis, treatment, and clinical management of pulmonary arterial hypertension in the contemporary era: A review

JAMA Cardiology

Volumn 1, Issue 9, 2016, Pages 1056-1065

  Maron, Bradley A ^a,b   Galiè, Nazzareno ^c  

^a BRIGHAM AND WOMEN S HOSPITAL   (United States)

^b VETERANS AFFAIRS MEDICAL CENTER   (United States)

^c UNIVERSITY OF BOLOGNA   (Italy)

Author keywords

[No Author keywords available]

Indexed keywords

AMBRISENTAN; ANTICOAGULANT AGENT; DIGITALIS; DIURETIC AGENT; ENDOTHELIN A RECEPTOR ANTAGONIST; ENDOTHELIN B RECEPTOR ANTAGONIST; ENDOTHELIN RECEPTOR ANTAGONIST; MACITENTAN; PHOSPHODIESTERASE V INHIBITOR; PLACEBO; PROSTACYCLIN DERIVATIVE; RIOCIGUAT; SELEXIPAG; TADALAFIL;

AEROBIC EXERCISE; CLINICAL FEATURE; COMBINATION DRUG THERAPY; CONNECTIVE TISSUE DISEASE; DELAYED DIAGNOSIS; DIAGNOSTIC ERROR; DIARRHEA; DISEASE SEVERITY; DRUG EFFICACY; DRUG TOLERABILITY; DRUG WITHDRAWAL; ENDARTERECTOMY; FAINTNESS; GERMLINE MUTATION; HEADACHE; HEART INDEX; HEART MUSCLE OXYGEN CONSUMPTION; HIGH RISK POPULATION; HISTOPATHOLOGY; HUMAN; INCIDENCE; JAW PAIN; MONOTHERAPY; NAUSEA; PATHOGENESIS; PATHOPHYSIOLOGY; PERCUTANEOUS TRANSLUMINAL ANGIOPLASTY; PHYSICAL ACTIVITY; PREVALENCE; PRIORITY JOURNAL; PULMONARY HYPERTENSION; REVIEW; SEX DIFFERENCE; SIX MINUTE WALK TEST; SURVIVAL RATE;

EID: 85017627528     PISSN: 23806583     EISSN: 23806591     Source Type: Journal    
DOI: 10.1001/jamacardio.2016.4471     Document Type: Review

References (54)

1. Barst RJ, Rubin LJ, Long WA, et al; Primary Pulmonary Hypertension Study Group. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. N Engl J Med. 1996;334(5):296-301.
2. Galiè N, Palazzini M, Manes A. Pulmonary arterial hypertension: from the kingdom of the near-dead to multiple clinical trial meta-analyses. Eur Heart J. 2010;31(17):2080-2086.
3. Lau EM, Manes A, Celermajer DS, Galiè N. Early detection of pulmonary vascular disease in pulmonary arterial hypertension: time to move forward. Eur Heart J. 2011;32(20):2489-2498.
4. McLaughlin VV, Langer A, Tan M, et al; Pulmonary Arterial Hypertension-Quality Enhancement Research Initiative. Contemporary trends in the diagnosis and management of pulmonary arterial hypertension: an initiative to close the care gap. Chest. 2013;143(2):324-332.
5. Austin ED, Kawut SM, Gladwin MT, Abman SH. Pulmonary hypertension: NHLBI workshop on the primary prevention of chronic lung diseases. Ann Am Thorac Soc. 2014;11(suppl 3):S178-S185.
6. Galiè N, Humbert M, Vachiery JL, et al. 2016 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 Heart J. 2016;37(1): 67-119.
7. Galiè 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. 2015;46(4): 903-975.
8. Lang IM, Pesavento R, Bonderman D, Yuan JX. Risk factors and basic mechanisms of chronic thromboembolic pulmonary hypertension: a current understanding. Eur Respir J. 2013;41(2): 462-468.
9. Montani D, Achouh L, Dorfmüller P, et al. Pulmonary veno-occlusive disease: clinical, functional, radiologic, and hemodynamic characteristics and outcome of 24 cases confirmed by histology. Medicine (Baltimore). 2008;87(4): 220-233.
10. Maron BA, Choudhary G, Khan UA, et al. Clinical profile and underdiagnosis of pulmonary hypertension in US veteran patients. Circ Heart Fail. 2013;6(5):906-912.
11. Deaño RC, Glassner-Kolmin C, Rubenfire M, et al. Referral of patients with pulmonary hypertension diagnoses to tertiary pulmonary hypertension centers: the multicenter Repherral study. JAMA Intern Med. 2013;173(10):887-893.
12. Brown LM, Chen H, Halpern S, et al. Delay in recognition of pulmonary arterial hypertension: factors identified from the REVEAL registry. Chest. 2011;140(1):19-26.
13. Ryan JJ, Butrous G, Maron BA. The heterogeneity of clinical practice patterns among an international cohort of pulmonary arterial hypertension experts. Pulm Circ. 2014;4(3): 441-451.
14. Kovacs G, Maier R, Aberer E, et al. Borderline pulmonary artery pressure is associated with decreased exercise capacity in scleroderma. Am J Respir Crit Care Med. 2009;180(9):881-886.
15. Kovacs G, Avian A, Tscherner M, et al. Characterization of patients with borderline pulmonary arterial pressure. Chest. 2014;146(6): 1486-1493.
16. Pagnamenta A, Dewachter C, McEntee K, Fesler P, Brimioulle S, Naeije R. Early right ventriculo-arterial uncoupling in borderline pulmonary hypertension on experimental heart failure. J Appl Physiol (1985). 2010;109(4): 1080-1085.
17. Humbert M, Sitbon O, Chaouat A, et al. Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era. Circulation. 2010;122(2):156-163.
18. Benza RL, Miller DP, Gomberg-Maitland M, et al. Predicting survival in pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management (REVEAL). Circulation. 2010;122(2):164-172.
19. Hoeper MM, Huscher D, Ghofrani HA, et al. Elderly patients diagnosed with idiopathic pulmonary arterial hypertension: results from the COMPERA registry. Int J Cardiol. 2013;168(2): 871-880.
20. Rich S, Dantzker DR, Ayres SM, et al. Primary pulmonary hypertension: a national prospective study. Ann Intern Med. 1987;107(2):216-223.
21. Austin ED, Lahm T, West J, et al. Gender, sex hormones and pulmonary hypertension. Pulm Circ. 2013;3(2):294-314.
22. Ventetuolo CE, Praestgaard A, Palevsky HI, Klinger JR, Halpern SD, Kawut SM. Sex and haemodynamics in pulmonary arterial hypertension. Eur Respir J. 2014;43(2):523-530.
23. D’Alonzo GE, Barst RJ, Ayres SM, et al. Survival in patients with primary pulmonary hypertension: results from a national prospective registry. Ann Intern Med. 1991;115(5):343-349.
24. Farber HW, Miller DP, Poms AD, et al. Five-year outcomes of patients enrolled in the REVEAL Registry. Chest. 2015;148(4):1043-1054.
25. Sitbon O, Sattler C, Bertoletti L, et al. Initial dual oral combination therapy in pulmonary arterial hypertension. Eur Respir J. 2016;47(6):1727-1736.
26. Levy D, Kenchaiah S, Larson MG, et al. Long-term trends in the incidence of and survival with heart failure. N Engl J Med. 2002;347(18): 1397-1402.
27. Montani D, Lau EM, Dorfmüller P, et al. Pulmonary veno-occlusive disease. Eur Respir J. 2016;47(5):1518-1534.
28. Ranchoux B, Antigny F, Rucker-Martin C, et al. Endothelial-to-mesenchymal transition in pulmonary hypertension. Circulation. 2015;131(11): 1006-1018.
29. Marsboom G, Toth PT, Ryan JJ, et al. Dynamin-related protein 1–mediated mitochondrial mitotic fission permits hyperproliferation of vascular smooth muscle cells and offers a novel therapeutic target in pulmonary hypertension. Circ Res. 2012;110(11):1484-1497.
30. Paulin R, Dromparis P, Sutendra G, et al. Sirtuin 3 deficiency is associated with inhibited mitochondrial function and pulmonary arterial hypertension in rodents and humans. Cell Metab. 2014;20(5):827-839.
31. Maron BA, Leopold JA. Emerging concepts in the molecular basis of pulmonary arterial hypertension, II: neurohormonal signaling contributes to the pulmonary vascular and right ventricular pathophenotype of pulmonary arterial hypertension. Circulation. 2015;131(23): 2079-2091.
32. Manders E, Rain S, Bogaard HJ, et al. The striated muscles in pulmonary arterial hypertension: adaptations beyond the right ventricle. Eur Respir J. 2015;46(3):832-842.
33. Opotowsky AR, Ojeda J, Rogers F, et al. A simple prediction rule for hemodynamics in pulmonary hypertension. Circ Cardiovasc Imaging. 2012;5:765-775.
34. Vanderpool RR, Pinsky MR, Naeije R, et al. RV-pulmonary arterial coupling predicts outcome in patients referred for pulmonary hypertension. Heart. 2015;101(1):37-43.
35. Hsu S, Houston BA, Tampakakis E, et al. Right ventricular functional reserve in pulmonary arterial hypertension. Circulation. 2016;133(24):2413-2422.
36. Newman JH, Wheeler L, Lane KB, et al. Mutation in the gene for bone morphogenetic protein receptor II as a cause of primary pulmonary hypertension in a large kindred. N Engl J Med. 2001; 345(5):319-324.
37. Austin ED, Loyd JE. The genetics of pulmonary arterial hypertension. Circ Res. 2014;115(1): 189-202.
38. Ma L, Roman-Campos D, Austin ED, et al. A novel channelopathy in pulmonary arterial hypertension. N Engl J Med. 2013;369(4):351-361.
39. Eyries M, Montani D, Girerd B, et al. EIF2AK4 mutations cause pulmonary veno-occlusive disease, a recessive form of pulmonary hypertension. Nat Genet. 2014;46(1):65-69.
40. Maron BA, Loscalzo J. Pulmonary hypertension: pathophysiology and signaling pathways. Handb Exp Pharmacol. 2013;218:31-58.
41. Pulido T, Adzerikho I, Channick RN, et al; SERAPHIN Investigators. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med. 2013;369(9):809-818.
42. Ghofrani HA, Galiè N, Grimminger F, et al; PATENT-1 Study Group. Riociguat for the treatment of pulmonary arterial hypertension. N Engl J Med. 2013;369(4):330-340.
43. Sitbon O, Channick R, Chin KM, et al; GRIPHON Investigators. Selexipag for the treatment of pulmonary arterial hypertension. N Engl J Med. 2015;373(26):2522-2533.
44. Galiè N, Barberà JA, Frost AE, et al; AMBITION Investigators. Initial use of ambrisentan plus tadalafil in pulmonary arterial hypertension. N Engl J Med. 2015;373(9):834-844.
45. Galiè N, Negro L, Simonneau G. The use of combination therapy in pulmonary arterial hypertension: new developments. Eur Respir J. 2009;18(113):148-153.
46. Galiè N, Muller K, Scalise AV, Grunig E. PATENT PLUS: a blinded, randomised and extension study of riociguat plus sildenafil in pulmonary arterial hypertension. Eur Respir J. 2015; 45(5):1314-1322.
47. Wilson ME, Vakil AP, Kandel P, Undavalli C, Dunlay SM, Kennedy CC. Pretransplant frailty is associated with decreased survival after lung transplantation. J Heart Lung Transplant. 2016;35 (2):173-178.
48. Mereles D, Ehlken N, Kreuscher S, et al. Exercise and respiratory training improve exercise capacity and quality of life in patients with severe chronic pulmonary hypertension. Circulation. 2006;114(14):1482-1489.
49. Pandey A, Garg S, Khunger M, et al. Efficacy and safety of exercise training in chronic pulmonary hypertension: systematic review and meta-analysis. Circ Heart Fail. 2015;8(6):1032-1043.
50. Arena R. Exercise testing and training in chronic lung disease and pulmonary arterial hypertension. Prog Cardiovasc Dis. 2011;53(6): 454-463.
51. Galie N, Manes A, Palazzini M. Exercise training in pulmonary hypertension: improving performance but waiting for outcome. Eur Heart J. 2016;37(1):45-48.
52. Heresi GA, Minai OA, Tonelli AR, et al. Clinical characterization and survival of patients with borderline elevation in pulmonary artery pressure. Pulm Circ. 2013;3(4):916-925.
53. Maron BA, Hess E, Maddox TM, et al. 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.
54. Opitz CF, Hoeper MM, Gibbs JS, et al. Pre-capillary, combined, and post-capillary pulmonary hypertension: a pathophysiological continuum. J Am Coll Cardiol. 2016;68(4): 368-378.