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Critical Care Management of the Adult with Eisenmenger Syndrome and Pulmonary Arterial Hypertension Related to Congenital Heart Disease

  • Laura C. Price
  • Rafael Alonso-Gonzalez
  • David Alexander
  • Konstantinos Dimopoulos
Chapter
Part of the Congenital Heart Disease in Adolescents and Adults book series (CHDAA)

Abstract

Major advances in the surgical and medical management of congenital heart disease (CHD) in recent decades have meant that an increasing number of children born with CHD survive to adulthood. In the past, lack of early diagnosis and timely repair resulted in a substantial number of patients developing pulmonary vascular disease, especially those with post-tricuspid shunts. Pulmonary arterial hypertension (PAH) related to congenital heart disease (PAH-CHD) is still one the most common types of PAH.

PAH-CHD shares significant similarities in terms of lung pathology to other types of PAH. All pulmonary hypertension (PH), including PAH-CHD, is a major risk factor for individuals who require critical care due to deterioration of their underlying disease, or because they are undergoing essential surgery or other intervention. A multidisciplinary approach, with significant expertise in CHD, PH, anesthetics, and critical care, is essential in order to optimize the otherwise adverse outcome of PAH-CHD patients who are admitted in intensive care or a high dependency unit.

In this chapter, we provide an overview of the critical care and anesthetic management of adults with PH, in particular PAH-CHD.

Keywords

Congenital heart disease Eisenmenger syndrome Pulmonary arterial hypertension Critical care Anesthesia Cyanosis 

Notes

Disclosures

Dr. Dimopoulos has acted as consultant for Actelion UK, Pfizer UK, and GSK UK and received unrestricted educational grants from Actelion and Pfizer. Dr. Price has acted as a consultant for Actelion UK and GSK UK and has received educational grants from Actelion. Dr Alexander has no disclosures to declare. Dr. Alonso-Gonzalez has acted as consultants for Pfizer Spain and GSK Spain and received unrestricted educational grants from Actelion and GSK.

References

  1. 1.
    Dimopoulos K, Wort SJ, Gatzoulis MA. Pulmonary hypertension related to congenital heart disease: a call for action. Eur Heart J. 2014;35(11):691–700.PubMedCrossRefGoogle Scholar
  2. 2.
    Health and Social Care Information Centre 1 Trevelyan Square. National Pulmonary Hypertension Audit [Internet]. 2012 [cited 2014 Oct 27]. http://www.hscic.gov.uk/ph.
  3. 3.
    Heath D, Helmholz HF Jr, Burchell HB, Dushane JW, Edwards JE. Graded pulmonary vascular changes and hemodynamic findings in cases of atrial and ventricular septal defect and patent ductus arteriosus. Circulation. 1958;18(6):1155–66.PubMedCrossRefPubMedCentralGoogle Scholar
  4. 4.
    Dimopoulos K, Giannakoulas G, Wort SJ, Gatzoulis MA. Pulmonary arterial hypertension in adults with congenital heart disease: distinct differences from other causes of pulmonary arterial hypertension and management implications. Curr Opin Cardiol. 2008;23(6):545–54.PubMedCrossRefGoogle Scholar
  5. 5.
    Galiè N, Humbert M, Vachiery J-L, Gibbs S, Lang I, 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 Heart J. 2016;37:67–119.PubMedCrossRefPubMedCentralGoogle Scholar
  6. 6.
    Diller G-P, Dimopoulos K, Broberg CS, Kaya MG, Naghotra US, Uebing A, et al. Presentation, survival prospects, and predictors of death in Eisenmenger syndrome: a combined retrospective and case-control study. Eur Heart J. 2006;27(14):1737–42.PubMedCrossRefPubMedCentralGoogle Scholar
  7. 7.
    Dimopoulos K, Inuzuka R, Goletto S, Giannakoulas G, Swan L, Wort SJ, et al. Improved survival among patients with Eisenmenger syndrome receiving advanced therapy for pulmonary arterial hypertension. Circulation. 2010;121(1):20–5.PubMedCrossRefPubMedCentralGoogle Scholar
  8. 8.
    Wood P. The Eisenmenger syndrome or pulmonary hypertension with reversed central shunt. Br Med J. 1958;2(5098):701–9.PubMedPubMedCentralCrossRefGoogle Scholar
  9. 9.
    Wood P. The Eisenmenger syndrome or pulmonary hypertension with reversed central shunt. Br Med J. 1958;2(5099):755–62.PubMedPubMedCentralCrossRefGoogle Scholar
  10. 10.
    Yamamura K, Nagata H, Ikeda K, Ihara K, Hara T. Efficacy of bosentan therapy for segmental pulmonary artery hypertension due to major aortopulmonary collateral arteries in children. Int J Cardiol. 2012;161(1):e1–3.PubMedCrossRefPubMedCentralGoogle Scholar
  11. 11.
    Schuuring MJ, Bouma BJ, Cordina R, Gatzoulis MA, Budts W, Mullen MP, et al. Treatment of segmental pulmonary artery hypertension in adults with congenital heart disease. Int J Cardiol. 2013;164(1):106–10.PubMedCrossRefPubMedCentralGoogle Scholar
  12. 12.
    Giardini A, Balducci A, Specchia S, Gargiulo G, Bonvicini M, Picchio FM. Effect of sildenafil on haemodynamic response to exercise and exercise capacity in Fontan patients. Eur Heart J. 2008;29(13):1681–7.PubMedCrossRefPubMedCentralGoogle Scholar
  13. 13.
    Hebert A, Mikkelsen UR, Thilen U, Idorn L, Jensen AS, Nagy E, et al. Bosentan improves exercise capacity in adolescents and adults after Fontan operation the TEMPO (treatment with endothelin receptor antagonist in Fontan patients, a randomized, placebo-controlled, double-blind study measuring peak oxygen consumption) study. Circulation. 2014;130(23):2021–30.PubMedCrossRefPubMedCentralGoogle Scholar
  14. 14.
    Dimopoulos K, Diller GP. Pulmonary hypertension in adult congenital heart [Internet]. Springer; 2017 [cited 2017 Sep 2]. http://www.springer.com/us/book/9783319460260.
  15. 15.
    Naeije R, Manes A. The right ventricle in pulmonary arterial hypertension. Eur Respir Rev. 2014;23(134):476–87.PubMedCrossRefPubMedCentralGoogle Scholar
  16. 16.
    Bowater SE, Weaver RA, Beadle RM, Frenneaux MP, Marshall JM, Clift PF. Assessment of the physiological adaptations to chronic hypoxemia in Eisenmenger syndrome. Congenit Heart Dis. 2016;11(4):341–7.PubMedCrossRefPubMedCentralGoogle Scholar
  17. 17.
    Broberg CS, Ujita M, Prasad S, Li W, Rubens M, Bax BE, et al. Pulmonary arterial thrombosis in Eisenmenger syndrome is associated with biventricular dysfunction and decreased pulmonary flow velocity. J Am Coll Cardiol. 2007;50(7):634–42.PubMedCrossRefPubMedCentralGoogle Scholar
  18. 18.
    Daliento L, Somerville J, Presbitero P, Menti L, Brach-Prever S, Rizzoli G, et al. Eisenmenger syndrome. Factors relating to deterioration and death. Eur Heart J. 1998;19(12):1845–55.PubMedCrossRefPubMedCentralGoogle Scholar
  19. 19.
    Dimopoulos K, Diller G-P, Petraco R, Koltsida E, Giannakoulas G, Tay EL, et al. Hyponatraemia: a strong predictor of mortality in adults with congenital heart disease. Eur Heart J. 2010;31(5):595–601.PubMedCrossRefPubMedCentralGoogle Scholar
  20. 20.
    Dimopoulos K, Diller G-P, Koltsida E, Pijuan-Domenech A, Papadopoulou SA, Babu-Narayan SV, et al. Prevalence, predictors, and prognostic value of renal dysfunction in adults with congenital heart disease. Circulation. 2008;117(18):2320–8.PubMedPubMedCentralCrossRefGoogle Scholar
  21. 21.
    Kempny A, Diller G-P, Alonso-Gonzalez R, Uebing A, Rafiq I, Li W, et al. Hypoalbuminaemia predicts outcome in adult patients with congenital heart disease. Heart Br Card Soc. 2015;101(9):699–705.Google Scholar
  22. 22.
    Diller G-P, Alonso-Gonzalez R, Kempny A, Dimopoulos K, Inuzuka R, Giannakoulas G, et al. B-type natriuretic peptide concentrations in contemporary Eisenmenger syndrome patients: predictive value and response to disease targeting therapy. Heart. 2012;98(9):736–42.PubMedCrossRefPubMedCentralGoogle Scholar
  23. 23.
    Hjortshøj CMS, Kempny A, Jensen AS, Sørensen K, Nagy E, Dellborg M, et al. Past and current cause-specific mortality in Eisenmenger syndrome. Eur Heart J. 2017;38(26):2060–7.PubMedCrossRefPubMedCentralGoogle Scholar
  24. 24.
    Kempny A, Hjortshøj CS, Gu H, Li W, Opotowsky AR, Landzberg MJ, et al. Predictors of death in contemporary adult patients with Eisenmenger syndrome: a multicenter study. Circulation. 2017;135(15):1432–40.PubMedCrossRefPubMedCentralGoogle Scholar
  25. 25.
    Kempny A, Dimopoulos K, Uebing A, Moceri P, Swan L, Gatzoulis MA, et al. Reference values for exercise limitations among adults with congenital heart disease. Relation to activities of daily life—single centre experience and review of published data. Eur Heart J. 2012;33(11):1386–96.PubMedCrossRefPubMedCentralGoogle Scholar
  26. 26.
    Kempny A, Dimopoulos K, Alonso-Gonzalez R, Alvarez-Barredo M, Tutarel O, Uebing A, et al. Six-minute walk test distance and resting oxygen saturations but not functional class predict outcome in adult patients with Eisenmenger syndrome. Int J Cardiol. 2013;168:4784–9.PubMedCrossRefPubMedCentralGoogle Scholar
  27. 27.
    Dimopoulos K, Okonko DO, Diller G-P, Broberg CS, Salukhe TV, Babu-Narayan SV, et al. Abnormal ventilatory response to exercise in adults with congenital heart disease relates to cyanosis and predicts survival. Circulation. 2006;113(24):2796–802.PubMedCrossRefPubMedCentralGoogle Scholar
  28. 28.
    Inuzuka R, Diller G-P, Borgia F, Benson L, Tay ELW, Alonso-Gonzalez R, et al. Comprehensive use of cardiopulmonary exercise testing identifies adults with congenital heart disease at increased mortality risk in the medium termclinical perspective. Circulation. 2012;125(2):250–9.CrossRefPubMedPubMedCentralGoogle Scholar
  29. 29.
    Alonso-Gonzalez R, Borgia F, Diller G-P, Inuzuka R, Kempny A, Martinez-Naharro A, et al. Abnormal lung function in adults with congenital heart disease: prevalence, relation to cardiac anatomy, and association with survival. Circulation. 2013;127(8):882–90.CrossRefPubMedPubMedCentralGoogle Scholar
  30. 30.
    Brida M, Dimopoulos K, Kempny A, Liodakis E, Alonso-Gonzalez R, Swan L, et al. Body mass index in adult congenital heart disease. Heart Br Card Soc. 2017;103(16):1250–7.Google Scholar
  31. 31.
    Dimopoulos K, Kempny A. Patients with Down syndrome and congenital heart disease: survival is improving, but challenges remain. Heart Br Card Soc. 2016;102(19):1515–7.Google Scholar
  32. 32.
    Tay ELW, Peset A, Papaphylactou M, Inuzuka R, Alonso-Gonzalez R, Giannakoulas G, et al. Replacement therapy for iron deficiency improves exercise capacity and quality of life in patients with cyanotic congenital heart disease and/or the Eisenmenger syndrome. Int J Cardiol. 2011;151(3):307–12.PubMedCrossRefPubMedCentralGoogle Scholar
  33. 33.
    Van De Bruaene A, Delcroix M, Pasquet A, De Backer J, De Pauw M, Naeije R, et al. Iron deficiency is associated with adverse outcome in Eisenmenger patients. Eur Heart J. 2011;32(22):2790–9.CrossRefGoogle Scholar
  34. 34.
    Ammash N, Warnes CA. Cerebrovascular events in adult patients with cyanotic congenital heart disease. J Am Coll Cardiol. 1996;28(3):768–72.PubMedCrossRefPubMedCentralGoogle Scholar
  35. 35.
    Jensen AS, Idorn L, Thomsen C, von der Recke P, Mortensen J, Sørensen KE, et al. Prevalence of cerebral and pulmonary thrombosis in patients with cyanotic congenital heart disease. Heart Br Card Soc. 2015;101(19):1540–6.Google Scholar
  36. 36.
    Habib G, Lancellotti P, Antunes MJ, Bongiorni MG, Casalta J-P, et al. 2015 ESC Guidelines for the management of infective endocarditis: the task force for the Management of Infective Endocarditis of the European Society of Cardiology (ESC) endorsed by: European Association for Cardio-Thoracic Surgery (EACTS), the European Association of Nuclear Medicine (EANM). Eur Heart J. 2015;36:3075–128.PubMedCrossRefGoogle Scholar
  37. 37.
    Bédard E, Dimopoulos K, Gatzoulis MA. Has there been any progress made on pregnancy outcomes among women with pulmonary arterial hypertension? Eur Heart J. 2009;30(3):256–65.PubMedCrossRefGoogle Scholar
  38. 38.
    European Society of Gynecology (ESG), Association for European Paediatric Cardiology (AEPC), German Society for Gender Medicine (DGesGM), Regitz-Zagrosek V, Blomstrom Lundqvist C, Borghi C, et al. 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. 2011;32(24):3147–97.CrossRefGoogle Scholar
  39. 39.
    Galiè N, Beghetti M, Gatzoulis MA, Granton J, Berger RMF, Lauer A, et al. Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, double-blind, randomized, placebo-controlled study. Circulation. 2006;114(1):48–54.CrossRefPubMedPubMedCentralGoogle Scholar
  40. 40.
    Gatzoulis MA, Beghetti M, Galiè N, Granton J, Berger RMF, Lauer A, et al. Longer-term bosentan therapy improves functional capacity in Eisenmenger syndrome: results of the BREATHE-5 open-label extension study. Int J Cardiol. 2008;127(1):27–32.PubMedCrossRefGoogle Scholar
  41. 41.
    Berger RMF, Beghetti M, Galiè N, Gatzoulis MA, Granton J, Lauer A, et al. Atrial septal defects versus ventricular septal defects in BREATHE-5, a placebo-controlled study of pulmonary arterial hypertension related to Eisenmenger’s syndrome: a subgroup analysis. Int J Cardiol. 2010;144:373–8.PubMedCrossRefGoogle Scholar
  42. 42.
    Mukhopadhyay S, Nathani S, Yusuf J, Shrimal D, Tyagi S. Clinical efficacy of phosphodiesterase-5 inhibitor tadalafil in Eisenmenger syndrome—a randomized, placebo-controlled, double-blind crossover study. Congenit Heart Dis. 2011;6(5):424–31.PubMedCrossRefGoogle Scholar
  43. 43.
    Tay ELW, Papaphylactou M, Diller GP, Alonso-Gonzalez R, Inuzuka R, Giannakoulas G, et al. Quality of life and functional capacity can be improved in patients with Eisenmenger syndrome with oral sildenafil therapy. Int J Cardiol. 2011;149(3):372–6.PubMedCrossRefGoogle Scholar
  44. 44.
    D’Alto M, Romeo E, Argiento P, Sarubbi B, Santoro G, Grimaldi N, et al. Bosentan-sildenafil association in patients with congenital heart disease-related pulmonary arterial hypertension and Eisenmenger physiology. Int J Cardiol. 2012;155(3):378–82.PubMedCrossRefGoogle Scholar
  45. 45.
    Fernandes SM, Newburger JW, Lang P, Pearson DD, Feinstein JA, Gauvreau K, et al. Usefulness of epoprostenol therapy in the severely ill adolescent/adult with Eisenmenger physiology. Am J Cardiol. 2003;91(5):632–5.PubMedCrossRefGoogle Scholar
  46. 46.
    Cha KS, Cho KI, Seo JS, Choi JH, Park YH, Yang DH, et al. Effects of inhaled iloprost on exercise capacity, quality of life, and cardiac function in patients with pulmonary arterial hypertension secondary to congenital heart disease (the Eisenmenger syndrome) (from the EIGER Study). Am J Cardiol. 2013;112(11):1834–9.PubMedCrossRefGoogle Scholar
  47. 47.
    Yang SI, Chung WJ, Jung SH, Choi DY. Effects of inhaled iloprost on congenital heart disease with Eisenmenger syndrome. Pediatr Cardiol. 2012;33(5):744–8.PubMedCrossRefGoogle Scholar
  48. 48.
    Pulido T, Adzerikho I, Channick RN, Delcroix M, Galiè N, Ghofrani H-A, et al. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med. 2013;369(9):809–18.PubMedCrossRefGoogle Scholar
  49. 49.
    Rosenkranz S, Ghofrani H-A, Beghetti M, Ivy D, Frey R, Fritsch A, et al. Riociguat for pulmonary arterial hypertension associated with congenital heart disease. Heart Br Card Soc. 2015;101(22):1792–9.Google Scholar
  50. 50.
    Budhiraja R, Tuder RM, Hassoun PM. Endothelial dysfunction in pulmonary hypertension. Circulation. 2004;109(2):159–65.PubMedCrossRefGoogle Scholar
  51. 51.
    Stojnic BB, Brecker SJ, Xiao HB, Helmy SM, Mbaissouroum M, Gibson DG. Left ventricular filling characteristics in pulmonary hypertension: a new mode of ventricular interaction. Br Heart J. 1992;68(1):16–20.PubMedPubMedCentralCrossRefGoogle Scholar
  52. 52.
    Vizza CD, Lynch JP, Ochoa LL, Richardson G, Trulock EP. Right and left ventricular dysfunction in patients with severe pulmonary disease. Chest. 1998;113(3):576–83.PubMedCrossRefGoogle Scholar
  53. 53.
    Via G, Braschi A. Pathophysiology of severe pulmonary hypertension in the critically ill patient. Minerva Anestesiol. 2004;70(4):233–7.PubMedGoogle Scholar
  54. 54.
    West JB. Invited review: pulmonary capillary stress failure. J Appl Physiol Bethesda MD 1985. 2000;89(6):2483–9. discussion 2497.Google Scholar
  55. 55.
    Bando K, Turrentine MW, Sharp TG, Sekine Y, Aufiero TX, Sun K, et al. Pulmonary hypertension after operations for congenital heart disease: analysis of risk factors and management. J Thorac Cardiovasc Surg. 1996;112(6):1600–7. discussion 1607–1609.PubMedCrossRefGoogle Scholar
  56. 56.
    Jardin F, Dubourg O, Bourdarias JP. Echocardiographic pattern of acute cor pulmonale. Chest. 1997;111(1):209–17.PubMedCrossRefGoogle Scholar
  57. 57.
    Hickey PR, Retzack SM. Acute right ventricular failure after pulmonary hypertensive responses to airway instrumentation: effect of fentanyl dose. Anesthesiology. 1993;78(2):372–6.PubMedCrossRefGoogle Scholar
  58. 58.
    Jardin F, Brun-Ney D, Cazaux P, Dubourg O, Hardy A, Bourdarias JP. Relation between transpulmonary pressure and right ventricular isovolumetric pressure change during respiratory support. Cathet Cardiovasc Diagn. 1989;16(4):215–20.PubMedCrossRefGoogle Scholar
  59. 59.
    Alfonsi P, Vieillard-Baron A, Coggia M, Guignard B, Goeau-Brissonniere O, Jardin F, et al. Cardiac function during intraperitoneal CO2 insufflation for aortic surgery: a transesophageal echocardiographic study. Anesth Analg. 2006;102(5):1304–10.PubMedCrossRefGoogle Scholar
  60. 60.
    Wort SJ, Evans TW. The role of the endothelium in modulating vascular control in sepsis and related conditions. Br Med Bull. 1999;55(1):30–48.PubMedCrossRefGoogle Scholar
  61. 61.
    Dávila-Román VG, Waggoner AD, Hopkins WE, Barzilai B. Right ventricular dysfunction in low output syndrome after cardiac operations: assessment by transesophageal echocardiography. Ann Thorac Surg. 1995;60(4):1081–6.PubMedCrossRefGoogle Scholar
  62. 62.
    Denault A, Deschamps A, Tardif J-C, Lambert J, Perrault L. Pulmonary hypertension in cardiac surgery. Curr Cardiol Rev. 2010;6(1):1–14.PubMedPubMedCentralCrossRefGoogle Scholar
  63. 63.
    Wessel DL, Adatia I, Giglia TM, Thompson JE, Kulik TJ. Use of inhaled nitric oxide and acetylcholine in the evaluation of pulmonary hypertension and endothelial function after cardiopulmonary bypass. Circulation. 1993;88(5 Pt 1):2128–38.PubMedCrossRefGoogle Scholar
  64. 64.
    Conzen PF, Habazettl H, Gutmann R, Hobbhahn J, Goetz AE, Peter K, et al. Thromboxane mediation of pulmonary hemodynamic responses after neutralization of heparin by protamine in pigs. Anesth Analg. 1989;68(1):25–31.PubMedCrossRefGoogle Scholar
  65. 65.
    Reich DL, Bodian CA, Krol M, Kuroda M, Osinski T, Thys DM. Intraoperative hemodynamic predictors of mortality, stroke, and myocardial infarction after coronary artery bypass surgery. Anesth Analg. 1999;89(4):814–22.PubMedGoogle Scholar
  66. 66.
    Roques F, Nashef SA, Michel P, Gauducheau E, de Vincentiis C, Baudet E, et al. Risk factors and outcome in European cardiac surgery: analysis of the EuroSCORE multinational database of 19030 patients. Eur J Cardio-Thorac Surg Off J Eur Assoc Cardio-Thorac Surg. 1999;15(6):816–22. discussion 822–823.CrossRefGoogle Scholar
  67. 67.
    Ghoreishi M, Evans CF, DeFilippi CR, Hobbs G, Young CA, Griffith BP, et al. Pulmonary hypertension adversely affects short- and long-term survival after mitral valve operation for mitral regurgitation: implications for timing of surgery. J Thorac Cardiovasc Surg. 2011;142(6):1439–52.PubMedCrossRefGoogle Scholar
  68. 68.
    Romano MA, Cowger J, Aaronson KD, Pagani FD. Diagnosis and management of right-sided heart failure in subjects supported with left ventricular assist devices. Curr Treat Options Cardiovasc Med. 2010;12(5):420–30.PubMedCrossRefGoogle Scholar
  69. 69.
    Miller OI, Tang SF, Keech A, Pigott NB, Beller E, Celermajer DS. Inhaled nitric oxide and prevention of pulmonary hypertension after congenital heart surgery: a randomised double-blind study. Lancet Lond Engl. 2000;356(9240):1464–9.CrossRefGoogle Scholar
  70. 70.
    Lindberg L, Olsson AK, Jögi P, Jonmarker C. How common is severe pulmonary hypertension after pediatric cardiac surgery? J Thorac Cardiovasc Surg. 2002;123(6):1155–63.PubMedCrossRefGoogle Scholar
  71. 71.
    Pagowska-Klimek I, Pychynska-Pokorska M, Krajewski W, Moll JJ. Predictors of long intensive care unit stay following cardiac surgery in children. Eur J Cardio-Thorac Surg Off J Eur Assoc Cardio-Thorac Surg. 2011;40(1):179–84.CrossRefGoogle Scholar
  72. 72.
    Lundgren R, Häggmark S, Reiz S. Hemodynamic effects of flexible fiberoptic bronchoscopy performed under topical anesthesia. Chest. 1982;82(3):295–9.PubMedCrossRefGoogle Scholar
  73. 73.
    Riley DJ. Risk of surgical lung biopsy in idiopathic interstitial pneumonias. Chest. 2005;127(5):1485–6.PubMedCrossRefGoogle Scholar
  74. 74.
    Kreider ME, Hansen-Flaschen J, Ahmad NN, Rossman MD, Kaiser LR, Kucharczuk JC, et al. Complications of video-assisted thoracoscopic lung biopsy in patients with interstitial lung disease. Ann Thorac Surg. 2007;83(3):1140–4.PubMedCrossRefGoogle Scholar
  75. 75.
    Hoeper MM, Lee SH, Voswinckel R, Palazzini M, Jais X, Marinelli A, et al. Complications of right heart catheterization procedures in patients with pulmonary hypertension in experienced centers. J Am Coll Cardiol. 2006;48(12):2546–52.PubMedCrossRefGoogle Scholar
  76. 76.
    Taylor CJ, Derrick G, McEwan A, Haworth SG, Sury MRJ. Risk of cardiac catheterization under anaesthesia in children with pulmonary hypertension. Br J Anaesth. 2007;98(5):657–61.PubMedCrossRefGoogle Scholar
  77. 77.
    Gottlieb EA, Andropoulos DB. Anesthesia for the patient with congenital heart disease presenting for noncardiac surgery. Curr Opin Anaesthesiol. 2013;26(3):318–26.PubMedCrossRefGoogle Scholar
  78. 78.
    Baehner T, Ellerkmann RK. Anesthesia in adults with congenital heart disease. Curr Opin Anaesthesiol. 2017;30(3):418–25.PubMedCrossRefGoogle Scholar
  79. 79.
    Bennett JM, Ehrenfeld JM, Markham L, Eagle SS. Anesthetic management and outcomes for patients with pulmonary hypertension and intracardiac shunts and Eisenmenger syndrome: a review of institutional experience. J Clin Anesth. 2014;26(4):286–93.PubMedCrossRefGoogle Scholar
  80. 80.
    Weiss BM, Zemp L, Seifert B, Hess OM. Outcome of pulmonary vascular disease in pregnancy: a systematic overview from 1978 through 1996. J Am Coll Cardiol. 1998;31(7):1650–7.PubMedCrossRefPubMedCentralGoogle Scholar
  81. 81.
    Jaïs X, Olsson KM, Barbera JA, Blanco I, Torbicki A, Peacock A, et al. Pregnancy outcomes in pulmonary arterial hypertension in the modern management era. Eur Respir J. 2012;40(4):881–5.PubMedCrossRefPubMedCentralGoogle Scholar
  82. 82.
    Bonnin M, Mercier FJ, Sitbon O, Roger-Christoph S, Jaïs X, Humbert M, et al. Severe pulmonary hypertension during pregnancy: mode of delivery and anesthetic management of 15 consecutive cases. Anesthesiology. 2005;102(6):1133–7. discussion 5A–6A.PubMedCrossRefPubMedCentralGoogle Scholar
  83. 83.
    Kiely DG, Condliffe R, Webster V, Mills GH, Wrench I, Gandhi SV, et al. Improved survival in pregnancy and pulmonary hypertension using a multiprofessional approach. BJOG. 2010;117(5):565–74.PubMedCrossRefPubMedCentralGoogle Scholar
  84. 84.
    Kiely DG, Condliffe R, Wilson VJ, Gandhi SV, Elliot CA. Pregnancy and pulmonary hypertension: a practical approach to management. Obstet Med. 2013;6(4):144–54.PubMedPubMedCentralCrossRefGoogle Scholar
  85. 85.
    Curry RA, Fletcher C, Gelson E, Gatzoulis MA, Woolnough M, Richards N, et al. Pulmonary hypertension and pregnancy—a review of 12 pregnancies in nine women. BJOG. 2012;119(6):752–61.PubMedCrossRefGoogle Scholar
  86. 86.
    Cauldwell M, Von Klemperer K, Uebing A, Swan L, Steer PJ, Gatzoulis M, et al. Why is post-partum haemorrhage more common in women with congenital heart disease? Int J Cardiol. 2016;218:285–90.PubMedCrossRefGoogle Scholar
  87. 87.
    Martin JT, Tautz TJ, Antognini JF. Safety of regional anesthesia in Eisenmenger’s syndrome. Reg Anesth Pain Med. 2002;27(5):509–13.PubMedGoogle Scholar
  88. 88.
    Pritts CD, Pearl RG. Anesthesia for patients with pulmonary hypertension. Curr Opin Anaesthesiol. 2010;23(3):411–6.PubMedCrossRefGoogle Scholar
  89. 89.
    Price LC, Wort SJ, Finney SJ, Marino PS, Brett SJ. Pulmonary vascular and right ventricular dysfunction in adult critical care: current and emerging options for management: a systematic literature review. Crit Care Lond Engl. 2010;14(5):R169.CrossRefGoogle Scholar
  90. 90.
    Rajek A, Pernerstorfer T, Kastner J, Mares P, Grabenwöger M, Sessler DI, et al. Inhaled nitric oxide reduces pulmonary vascular resistance more than prostaglandin E(1) during heart transplantation. Anesth Analg. 2000;90(3):523–30.PubMedCrossRefPubMedCentralGoogle Scholar
  91. 91.
    Ardehali A, Hughes K, Sadeghi A, Esmailian F, Marelli D, Moriguchi J, et al. Inhaled nitric oxide for pulmonary hypertension after heart transplantation. Transplantation. 2001;72(4):638–41.PubMedCrossRefPubMedCentralGoogle Scholar
  92. 92.
    Theodoraki K, Tsiapras D, Tsourelis L, Zarkalis D, Sfirakis P, Kapetanakis E, et al. Inhaled iloprost in eight heart transplant recipients presenting with post-bypass acute right ventricular dysfunction. Acta Anaesthesiol Scand. 2006;50(10):1213–7.PubMedCrossRefPubMedCentralGoogle Scholar
  93. 93.
    Kramm T, Eberle B, Krummenauer F, Guth S, Oelert H, Mayer E. Inhaled iloprost in patients with chronic thromboembolic pulmonary hypertension: effects before and after pulmonary thromboendarterectomy. Ann Thorac Surg. 2003;76(3):711–8.PubMedCrossRefPubMedCentralGoogle Scholar
  94. 94.
    Winterhalter M, Fischer S, Tessmann R, Goerler A, Piepenbrock S, Haverich A, et al. Using inhaled iloprost to wean from cardiopulmonary bypass after implanting a left ventricular assist device. Anesth Analg. 2006;103(2):515–6.PubMedCrossRefPubMedCentralGoogle Scholar
  95. 95.
    Rex S, Schaelte G, Metzelder S, Flier S, de Waal EEC, Autschbach R, et al. Inhaled iloprost to control pulmonary artery hypertension in patients undergoing mitral valve surgery: a prospective, randomized-controlled trial. Acta Anaesthesiol Scand. 2008;52(1):65–72.PubMedCrossRefPubMedCentralGoogle Scholar
  96. 96.
    Sztrymf B, Coulet F, Girerd B, Yaici A, Jais X, Sitbon O, et al. Clinical outcomes of pulmonary arterial hypertension in carriers of BMPR2 mutation. Am J Respir Crit Care Med. 2008;177(12):1377–83.PubMedCrossRefPubMedCentralGoogle Scholar
  97. 97.
    Hoeper MM, Granton J. Intensive care unit management of patients with severe pulmonary hypertension and right heart failure. Am J Respir Crit Care Med. 2011;184(10):1114–24.PubMedCrossRefPubMedCentralGoogle Scholar
  98. 98.
    Zamanian RT, Haddad F, Doyle RL, Weinacker AB. Management strategies for patients with pulmonary hypertension in the intensive care unit. Crit Care Med. 2007;35(9):2037–50.PubMedCrossRefPubMedCentralGoogle Scholar
  99. 99.
    Lamarche Y, Perrault LP, Maltais S, Tétreault K, Lambert J, Denault AY. Preliminary experience with inhaled milrinone in cardiac surgery. Eur J Cardio-Thorac Surg Off J Eur Assoc Cardio-Thorac Surg. 2007;31(6):1081–7.CrossRefGoogle Scholar
  100. 100.
    Winterhalter M, Simon A, Fischer S, Rahe-Meyer N, Chamtzidou N, Hecker H, et al. Comparison of inhaled iloprost and nitric oxide in patients with pulmonary hypertension during weaning from cardiopulmonary bypass in cardiac surgery: a prospective randomized trial. J Cardiothorac Vasc Anesth. 2008;22(3):406–13.PubMedCrossRefPubMedCentralGoogle Scholar
  101. 101.
    Fraisse A, Butrous G, Taylor MB, Oakes M, Dilleen M, Wessel DL. Intravenous sildenafil for postoperative pulmonary hypertension in children with congenital heart disease. Intensive Care Med. 2011;37(3):502–9.PubMedCrossRefPubMedCentralGoogle Scholar
  102. 102.
    Mydin M, Berman M, Klein A, Tsui S, Dunning J, Valchanov K, et al. Extracorporeal membrane oxygenation as a bridge to pulmonary endarterectomy. Ann Thorac Surg. 2011;92(5):e101–3.PubMedCrossRefPubMedCentralGoogle Scholar
  103. 103.
    Srivastava MC, Ramani GV, Garcia JP, Griffith BP, Uber PA, Park MH. Veno-venous extracorporeal membrane oxygenation bridging to pharmacotherapy in pulmonary arterial hypertensive crisis. J Heart Lung Transplant Off Publ Int Soc Heart Transplant. 2010;29(7):811–3.CrossRefGoogle Scholar
  104. 104.
    Rosenzweig EB, Brodie D, Abrams DC, Agerstrand CL, Bacchetta M. Extracorporeal membrane oxygenation as a novel bridging strategy for acute right heart failure in group 1 pulmonary arterial hypertension. ASAIO J Am Soc Artif Intern Organs 1992. 2014;60(1):129–33.CrossRefGoogle Scholar
  105. 105.
    Keogh AM, Mayer E, Benza RL, Corris P, Dartevelle PG, Frost AE, et al. Interventional and surgical modalities of treatment in pulmonary hypertension. J Am Coll Cardiol. 2009;54(1 Suppl):S67–77.PubMedCrossRefPubMedCentralGoogle Scholar
  106. 106.
    Strueber M, Hoeper MM, Fischer S, Cypel M, Warnecke G, Gottlieb J, et al. Bridge to thoracic organ transplantation in patients with pulmonary arterial hypertension using a pumpless lung assist device. Am J Transplant Off J Am Soc Transplant Am Soc Transpl Surg. 2009;9(4):853–7.CrossRefGoogle Scholar
  107. 107.
    Schmid C, Philipp A, Hilker M, Arlt M, Trabold B, Pfeiffer M, et al. Bridge to lung transplantation through a pulmonary artery to left atrial oxygenator circuit. Ann Thorac Surg. 2008;85(4):1202–5.PubMedCrossRefPubMedCentralGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2019

Authors and Affiliations

  • Laura C. Price
    • 1
    • 2
  • Rafael Alonso-Gonzalez
    • 1
    • 2
  • David Alexander
    • 1
    • 2
  • Konstantinos Dimopoulos
    • 1
    • 2
  1. 1.Adult Congenital Heart Centre and National Centre for Pulmonary HypertensionNational Heart and Lung Institute, Imperial College School of MedicineLondonUK
  2. 2.Department of AnaestheticsRoyal Brompton and Harefield NHS Foundation Trust, Royal Brompton HospitalLondonUK

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