Pulmonary Arterial Hypertension

  • Shinji KatsuragiEmail author
  • Tomoaki Ikeda


Pulmonary arterial hypertension (PAH) is a complex disorder in which pulmonary artery (PA) obstruction leads to elevated PA resistance and right ventricular (RV) failure. Elevation of the pulmonary arterial blood pressure (PABP) is correlated with progressive damage to the pulmonary artery. Before development of surgery for ventricular septal defect (VSD), atrial septal defect (ASD), and patent ductus arteriosus (PDA), most patients died around the age 40, with right-sided cardiac failure being the main cause of death. Deterioration in pregnancy is reported to occur in the second or third trimester and in the postpartum period. Sudden hemodynamic instability is a common cause of death. The risk of cardiac failure during and after pregnancy increases, and sudden cardiac arrest may occur during Cesarean section or soon after birth. The rate of maternal death in pregnancies complicated by PAH is variably reported to be 20–60%. Predictors of cardiac failure during pregnancy are elevated PABP, elevated brain natriuretic peptide (BNP), and elevated RV size. Current PAH-specific therapies have affected survival in nonpregnant PAH patients, and it is important to accumulate data from multiple PAH centers. Prostacyclins became available for PAH treatment in 1996 to the United States and Europe and in 1999 in Japan. Therefore, in this chapter, we classify studies on this condition temporally into pre- and post-PAH therapeutic eras.


Pregnancy Pulmonary arterial hypertension Pulmonary vascular disease Right ventricular failure 


  1. 1.
    Hoeper MM (2013) Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol 62:D42–D50CrossRefGoogle Scholar
  2. 2.
    Anna RH, David GK, Barbara AC, Cockrill ZS, Victoria JW, Manak AH et al (2015) Statement on pregnancy in pulmonary hypertension from the pulmonary vascular research institute. Pulm Circ 5(3):435–465CrossRefGoogle Scholar
  3. 3.
    Bonnin M, Mercier FJ, Sitbon O, Roger-Christoph S, Jaïs X, Humbert M, Audibert F, Frydman R, Simonneau G, Benhamou D (2005) Severe pulmonary hypertension during pregnancy: mode of delivery and anesthetic management of 15 consecutive cases. Anesthesiology 102(6):1133–1137. discussion 5A–6ACrossRefGoogle Scholar
  4. 4.
    Katsuragi S, Yamanaka K, Neki R, Kamiya C, Sasaki Y, Osato K, Miyoshi T et al (2012) Maternal outcome in pregnancy complicated with pulmonary arterial hypertension. Circ J 76(9):2249–2254CrossRefGoogle Scholar
  5. 5.
    Bédard E, Dimopoulos K, Gatzoulis MA (2009) Has there been any progress made on pregnancy outcomes among women with pulmonary arterial hypertension? Eur Heart J 30(3):256–265CrossRefGoogle Scholar
  6. 6.
    Monnery L, Nanson J, Charlton G (2001) Primary pulmonary hypertension in pregnancy; a role for novel vasodilators. Br J Anaesth 87(2):295–298CrossRefGoogle Scholar
  7. 7.
    Duarte AG, Thomas S, Safdar Z, Torres F, Pacheco LD, Feldman J, deBoisblanc B (2013) Management of pulmonary arterial hypertension during pregnancy: a retrospective., multicenter experience. Chest 143(5):1330–1336CrossRefGoogle Scholar
  8. 8.
    Ma L, Liu W, Huang Y (2012) Perioperative management for parturients with pulmonary hypertension: experience with 30 consecutive cases. Front Med 6(3):307–310CrossRefGoogle Scholar
  9. 9.
    Rosengarten D, Blieden LC, Kramer MR (2012) Pregnancy outcomes in pulmonary arterial hypertension in the modern management era. Eur Respir J 40(5):1304–1305CrossRefGoogle Scholar
  10. 10.
    Jaïs X, Olsson KM, Barberà JA, Blanco B, Torbicki A, Peacock A, Vizza CD, Macdonald P, Humbert M, Hoeper MM (2012) Pregnancy outcomes in pulmonary arterial hypertension in the modern management era. Eur Respir J 40(4):881–885CrossRefGoogle Scholar
  11. 11.
    Kiely DG, Condliffe R, Webster V, Mills GH, Wrench I, Gandhi SV, Selby K et al (2010) Improved survival in pregnancy and pulmonary hypertension using a multiprofessional approach. BJOG 117(5):565–574CrossRefGoogle Scholar
  12. 12.
    Curry RA, Fletcher C, Gelson E, Gatzoulis MA, Woolnough M, Richards N, Swan L, Steer PJ, Johnson MR (2012) Pulmonary hypertension and pregnancy—a review of 12 pregnancies in nine women. BJOG 119(6):752–761CrossRefGoogle Scholar
  13. 13.
    Regitz-Zagrosek V, Lundqvist CB, Borghi C, Cifkova R, Ferreira R, Foidart JM, Gibbs JSR et al (2011) ESC guidelines on the management of cardiovascular diseases during pregnancy: the task force on the Management of Cardiovascular Diseases during pregnancy of the European Society of Cardiology (ESC). Eur Heart J 32(24):3147–3197CrossRefGoogle Scholar
  14. 14.
    Pieper PG, Hoendermis ES (2011) Pregnancy in women with pulmonary hypertension. Neth Heart J 19(12):504–508CrossRefGoogle Scholar
  15. 15.
    Wexler ID, Johannesson M, Edenborough FP, Sufian BS, Kerem E (2007) Pregnancy and chronic progressive pulmonary disease. Am J Respir Crit Care Med 175(4):300–305CrossRefGoogle Scholar
  16. 16.
    Jain JK, Mishell DR Jr (1994) A comparison of intravaginal misoprostol with prostaglandin E2 for termination of second-trimester pregnancy. N Engl J Med 331(5):290–293CrossRefGoogle Scholar
  17. 17.
    Pitkin RM, Perloff JK, Koos BJ, Beall MH (1990) Pregnancy and congenital heart disease. Ann Intern Med 112:445–454CrossRefGoogle Scholar
  18. 18.
    Svetlichnaya J, Janmohammed M, De Marco T (2016) Special situations in pulmonary hypertension: pregnancy and right ventricular failure. Cardiol Clin 34:473–487CrossRefGoogle Scholar
  19. 19.
    Liu S, Liston RM, Joseph KS, Heaman M, Sauve R, Kramer MS (2007) Maternal mortality and severe morbidity associated with low-risk planned cesarean delivery versus planned vaginal delivery at term. Can Med Assoc J 176(4):455–460CrossRefGoogle Scholar
  20. 20.
    Heerdt PM, Caldwell RW (1989) The mechanism of nitrous oxide-induced changes in pulmonary vascular resistance in a dog model of left atrial outflow obstruction. J Cardiothorac Anesth 3(5):568–573CrossRefGoogle Scholar
  21. 21.
    Sørensen MB, Jacobsen E (1977) Pulmonary hemodynamics during induction of anesthesia. Anesthesiology 46(4):246–251CrossRefGoogle Scholar
  22. 22.
    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):1114–1124CrossRefGoogle Scholar
  23. 23.
    Duggan AB, Katz SG (2003) Combined spinal and epidural anaesthesia for caesarean section in a parturient with severe primary pulmonary hypertension. Anaesth Intensive Care 31(5):565–569PubMedGoogle Scholar
  24. 24.
    Le RJ, Fenstad ER, Maradit-Kremers H, McCully RB, Frantz RP, MD MG, Kane GC (2011) Syncope in adults with pulmonary arterial hypertension [retracted in J am Coll Cardiol 59(21):1919]. J Am Coll Cardiol 58(8):863–867CrossRefGoogle Scholar
  25. 25.
    Brignole M, Alboni P, Benditt DG, Bergfeldt L, Blanc JJ, Bloch Thomsen PE, van Dijk JG et al (2004) Guidelines on management (diagnosis and treatment) of syncope—update 2004: executive summary. Eur Heart J 25(22):2054–2072CrossRefGoogle Scholar
  26. 26.
    Yarlagadda S, Poma PA, Green LS, Katz V (2010) Syncope during pregnancy. Obstet Gynecol 115(2):377–380CrossRefGoogle Scholar
  27. 27.
    Elliot CA, Stewart P, Webster VJ, Mills GH, Hutchinson SP, Howarth ES, Bu’Lock FA, Lawson RA, Armstrong IJ, Kiely DG (2005) The use of iloprost in early pregnancy in patients with pulmonary arterial hypertension. Eur Respir J 26(1):168–173CrossRefGoogle Scholar
  28. 28.
    Bédard E, McCarthy KP, Dimopoulos K, Giannakoulas G, Gatzoulis MA, Ho SY (2009) Structural abnormalities of the pulmonary trunk in tetralogy of Fallot and potential clinical implications: a morphological study. J Am Coll Cardiol 54(20):1883–1890CrossRefGoogle Scholar
  29. 29.
    Brittain EL, Pugh ME, Wheeler LA, Robbins IM, Loyd JE, Newman JH, Austin ED, Hemnes AR (2013) Prostanoids but not oral therapies improve right ventricular function in pulmonary arterial hypertension. JACC Heart Failure 1(4):300–307CrossRefGoogle Scholar
  30. 30.
    Waxman AB, Zamanian RT (2013) Pulmonary arterial hypertension: new insights into the optimal role of current and emerging prostacyclin therapies. Am J Cardiol 111(5):1A–16A. quiz 17A–19ACrossRefGoogle Scholar
  31. 31.
    Barst RJ, Gibbs JS, Ghofrani HA, Hoeper MM, McLaughlin VV, Rubin LJ, Sitbon O, Tapson VF, Galiè N (2009) Updated evidence-based treatment algorithm in pulmonary arterial hypertension. J Am Coll Cardiol 54(1 suppl):S78–S84CrossRefGoogle Scholar
  32. 32.
    McLaughlin VV, Archer SL, Badesch DB, Barst RJ, Farber HW, Lindner JR, Mathier MA 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):1573–1619CrossRefGoogle Scholar
  33. 33.
    Galiè N, Hoeper MM, Humbert M, Torbicki A, Vachiéry JL, Barberà JA, Beghetti M et al (2009) Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J 34(6):1219–1263CrossRefGoogle Scholar
  34. 34.
    Hsu CH, Gomberg-Maitland M, Glassner C, Chen JH (2011) The management of pregnancy and pregnancy-related medical conditions in pulmonary arterial hypertension patients. Int J Clin Pract Suppl 65:6–14CrossRefGoogle Scholar
  35. 35.
    Decoene C, Bourzoufi K, Moreau D, Narducci F, Crepin F, Krivosic-Horber R (2001) Use of inhaled nitric oxide for emergency cesarean section in a woman with unexpected primary pulmonary hypertension. Can J Anaesth 48(6):584–587CrossRefGoogle Scholar
  36. 36.
    Goland S, Tsai F, Habib M, Janmohamed M, Goodwin TM, Elkayam U (2010) Favorable outcome of pregnancy with an elective use of epoprostenol and sildenafil in women with severe pulmonary hypertension. Cardiology 115(3):205–208CrossRefGoogle Scholar
  37. 37.
    LeVarge BL (2015) Prostanoid therapies in the management of pulmonary arterial hypertension. Ther Clin Risk Manag 11:535–547CrossRefGoogle Scholar
  38. 38.
    Sitbon O, Humbert M, Jaïs X, Ioos V, Hamid AM, Provencher S, Garcia G, Parent F, Hervé P, Simonneau G (2005) Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation 111(23):3105–3111CrossRefGoogle Scholar
  39. 39.
    Simhan HN, Caritis SN (2007) Prevention of preterm delivery. N Engl J Med 357(5):477–487CrossRefGoogle Scholar
  40. 40.
    Kiely DG, Elliot CA, Wilson VJ, Stewart P (2006) Pregnancy and pulmonary hypertension: new approaches to the management of a life threatening condition. In: Steer PH, Gatzoulis MA, Baker P (eds) Heart disease and pregnancy. RCOG Press, London, pp 211–229Google Scholar
  41. 41.
    Jonas MM, Tanser SJ (2002) Lithium dilution measurement of cardiac output and arterial pulse waveform analysis: an indicator dilution calibrated beat-by-beat system for continuous estimation of cardiac output. Curr Opin Crit Care 8(3):257–261CrossRefGoogle Scholar
  42. 42.
    Kiely DG, Condliffe R, Wilson VJ, Gandhi SV, Elliot CA (2013) Pregnancy and pulmonary hypertension: a practical approach to management. Obstet Med 6(4):144–154CrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media Singapore 2019

Authors and Affiliations

  1. 1.Department of Obstetrics and GynecologySakakibara Heart InstituteFuchuJapan
  2. 2.Department of Obstetrics and GynecologyMie University Graduate School of MedicineTsuJapan

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