Hostname: page-component-78c5997874-94fs2 Total loading time: 0 Render date: 2024-11-10T21:00:40.120Z Has data issue: false hasContentIssue false
  • English
  • Français

Pulmonary arterial hypertension in congenital cardiac disease – the need for refinement of the Evian-Venice classification

Published online by Cambridge University Press:  21 January 2008

Mirjam E. van Albada  and
Rolf M.F. Berger
Mirjam E. van Albada
Affiliation:
Department of Paediatric and Congenital Cardiology, University Medical Center Groningen, University of Groningen, The Netherlands
Rolf M.F. Berger*
Affiliation:
Department of Paediatric and Congenital Cardiology, University Medical Center Groningen, University of Groningen, The Netherlands
*
University Medical Center Groningen, PO Box 30001, 9700 RB Groningen, The Netherlands. Tel: +31 50 3612800; Fax: +31 50 3614235; E-mail: r.m.f.berger@bkk.umcg.nl
Get access Rights & Permissions [Opens in a new window]

Abstract

Pulmonary hypertension associated with congenital systemic-to-pulmonary shunts has been classified, in the Evian-Venice classification, as Pulmonary Arterial Hypertension, which includes a heterogeneous group of conditions. Emerging options for treatment of patients with pulmonary arterial hypertension are related to those with the idiopathic form of the disease, but may also improve quality of life and survival in patients with pulmonary arterial hypertension associated with congenital cardiac disease. Despite the evident similarities in pulmonary vascular disease, it is important also to recognise the differences between patients in whom pulmonary arterial hypertension is the consequence of systemic-to-pulmonary shunts as opposed to those with other conditions. Patients with pulmonary hypertension associated with congenital cardiac disease themselves constitute a heterogeneous population, in which generalisation may be hazardous. Specific considerations need to be given to the type of cardiac diagnosis, the prognosis and evolution of pulmonary vascular disease, and the circulatory physiology before embarking on new strategies for medical treatment in the individual patient. In this review, we highlight the features that require specific attention in these patients. In addition, we discuss briefly the data currently available on the effectiveness of the new anti-proliferative drugs in patients with the Eisenmenger syndrome.

Keywords

Congenital cardiac malformationscongenital shuntsEisenmenger syndrome
Type
Review Article
Information
Cardiology in the Young , Volume 18 , Issue 1 , February 2008 , pp. 10 - 17
Copyright
Copyright © Cambridge University Press 2008

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1. Rich, S, Dantzker, DR, Ayres, SM, et al. . Primary pulmonary hypertension. A national prospective study. Ann Intern Med 1987; 107: 216–223.CrossRefGoogle ScholarPubMed
2. Simonneau, G, Galie, N, Rubin, LJ, et al. . Clinical classification of pulmonary hypertension. J Am Coll Cardiol 2004; 43 (12 Suppl S): 5S–12S.CrossRefGoogle ScholarPubMed
3. Craig, RJ, Selzer, A. Natural history and prognosis of atrial septal defect. Circulation 1968; 37: 805–815.CrossRefGoogle ScholarPubMed
4. Ikawa, S, Shimazaki, Y, Nakano, S, Kobayashi, J, Matsuda, H, Kawashima, Y. Pulmonary vascular resistance during exercise late after repair of large ventricular septal defects. Relation to age at the time of repair. J Thorac Cardiovasc Surg 1995; 109: 1218–1224.CrossRefGoogle ScholarPubMed
5. Wagenvoort, CA, Mooi, WJ. Plexogenic arteriopathy. In: Munro Neville, A, Walker, F, Gottlieb, LS (eds). Biopsy Pathology of the Pulmonary Vasculature. Chapman and Hall Medical, London, 1989, pp 56–113.Google Scholar
6. McLaughlin, VV, Presberg, KW, Doyle, RL, et al. . Prognosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest 2004; 126 (1 Suppl): 78S–92S.CrossRefGoogle ScholarPubMed
7. Daliento, L, Somerville, J, Presbitero, P, et al. . Eisenmenger syndrome. Factors relating to deterioration and death. Eur Heart J 1998; 19: 1845–1855.CrossRefGoogle ScholarPubMed
8. Rudolph, AM, Nadas, AS. The pulmonary circulation and congenital heart disease. Considerations of the role of the pulmonary circulation in certain systemic-pulmonary communications. N Engl J Med 1962; 267: 1022–1029.CrossRefGoogle ScholarPubMed
9. Hornberger, LK, Sahn, DJ, Krabill, KA, et al. . Elucidation of the natural history of ventricular septal defects by serial Doppler color flow mapping studies. J Am Coll Cardiol 1989; 13: 1111–1118.CrossRefGoogle ScholarPubMed
10. Somerville, J. How to manage the Eisenmenger syndrome. Int J Cardiol 1998; 63: 1–8.CrossRefGoogle ScholarPubMed
11. de Leval, MR. The Fontan circulation: a challenge to William Harvey? Nat Clin Pract Cardiovasc Med 2005; 2: 202–208.CrossRefGoogle ScholarPubMed
12. BissetGS, III GS, III, Hirschfeld, SS. Severe pulmonary hypertension associated with a small ventricular septal defect. Circulation 1983; 67: 470–473.CrossRefGoogle ScholarPubMed
13. Roberts, KE, McElroy, JJ, Wong, WP, et al. . BMPR2 mutations in pulmonary arterial hypertension with congenital heart disease. Eur Respir J 2004; 24: 371–374.CrossRefGoogle ScholarPubMed
14. Yamaki, S, Horiuchi, T, Sekino, Y. Quantitative analysis of pulmonary vascular disease in simple cardiac anomalies with the Down syndrome. Am J Cardiol 1983; 51: 1502–1506.CrossRefGoogle ScholarPubMed
15. Galie, N. Classification of patients with congenital systemic-to-pulmonary shunts associated with pulmonary arterial hypertension: current status and future directions. In: Beghetti, M (ed). Pulmonary arterial hypertension related to congenital heart disease. Elsevier, Munich, 2006, pp 11–17.Google Scholar
16. Galie, N, Ghofrani, HA, Torbicki, A, et al. . Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 2005; 353: 2148–2157.CrossRefGoogle ScholarPubMed
17. Galie, N, Humbert, M, Vachiery, JL, et al. . Effects of beraprost sodium, an oral prostacyclin analogue, in patients with pulmonary arterial hypertension: a randomized, double-blind, placebo-controlled trial. J Am Coll Cardiol 2002; 39: 1496–1502.CrossRefGoogle ScholarPubMed
18. Simonneau, G, Barst, RJ, Galie, N, et al. . Continuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension: a double-blind, randomized, placebo-controlled trial. Am J Respir Crit Care Med 2002; 165: 800–804.CrossRefGoogle ScholarPubMed
19. Wood, P. The Eisenmenger syndrome or pulmonary hypertension with reversed central shunt. Br Med J 1958; 2: 755–762.CrossRefGoogle ScholarPubMed
20. Diller, GP, Gatzoulis, MA. Pulmonary vascular disease in adults with congenital heart disease. Circulation 2007; 115: 1039–1050.CrossRefGoogle ScholarPubMed
21. Fuster, V, Steele, PM, Edwards, WD, Gersh, BJ, McGoon, MD, Frye, RL. Primary pulmonary hypertension: natural history and the importance of thrombosis. Circulation 1984; 70: 580–587.CrossRefGoogle ScholarPubMed
22. Rich, S, Kaufmann, E, Levy, PS. The effect of high doses of calcium-channel blockers on survival in primary pulmonary hypertension. N Engl J Med 1992; 327: 76–81.CrossRefGoogle ScholarPubMed
23. Barst, RJ, Rubin, LJ, Long, WA, et al. . A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. The Primary Pulmonary Hypertension Study Group. N Engl J Med 1996; 334: 296–302.CrossRefGoogle Scholar
24. Rubin, LJ, Badesch, DB, Barst, RJ, et al. . Bosentan therapy for pulmonary arterial hypertension. N Engl J Med 2002; 346: 896–903.CrossRefGoogle ScholarPubMed
25. Channick, RN, Simonneau, G, Sitbon, O, et al. . Effects of the dual endothelin-receptor antagonist bosentan in patients with pulmonary hypertension: a randomised placebo-controlled study. Lancet 2001; 358: 1119–1123.CrossRefGoogle ScholarPubMed
26. Galie, N, Ghofrani, HA, Torbicki, A, et al. . Sildenafil Use in Pulmonary Arterial Hypertension (SUPER) Study Group. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 2005; 353: 2148–2157.CrossRefGoogle Scholar
27. de P S Soares, R, Maeda, NY, Bydlowski, SP, Lopes, AA. Markers of endothelial dysfunction and severity of hypoxaemia in the Eisenmenger syndrome. Cardiol Young 2005; 15: 504–513.CrossRefGoogle ScholarPubMed
28. Silversides, CK, Granton, JT, Konen, E, Hart, MA, Webb, GD, Therrien, J. Pulmonary thrombosis in adults with Eisenmenger syndrome. J Am Coll Cardiol 2003; 42: 1982–1987.CrossRefGoogle ScholarPubMed
29. Sitbon, O, Humbert, M, Jais, X, et al. . Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation 2005; 111: 3105–3111.CrossRefGoogle ScholarPubMed
30. Wimmer, M, Schlemmer, M. Long-term hemodynamic effects of nifedipine on congenital heart disease with Eisenmenger’s mechanism in children. Cardiovasc Drugs Ther 1992; 6: 183–186.CrossRefGoogle ScholarPubMed
31. Rosenzweig, EB, Kerstein, D, Barst, RJ. Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects. Circulation 1999; 99: 1858–1865.CrossRefGoogle ScholarPubMed
32. McLaughlin, VV, Genthner, DE, Panella, MM, Hess, DM, Rich, S. Compassionate use of continuous prostacyclin in the management of secondary pulmonary hypertension: a case series. Ann Intern Med 1999; 130: 740–743.CrossRefGoogle ScholarPubMed
33. Fernandes, SM, Newburger, JW, Lang, P, et al. . Usefulness of epoprostenol therapy in the severely ill adolescent/adult with Eisenmenger physiology. Am J Cardiol 2003; 91: 632–635.CrossRefGoogle ScholarPubMed
34. Beghetti, M, Black, SM, Fineman, JR. Endothelin-1 in congenital heart disease. Pediatr Res 2005; 57 (5 Pt 2): 16R–20R.CrossRefGoogle ScholarPubMed
35. Galie, N, Beghetti, M, Gatzoulis, MA, et al. . Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, double-blind, randomized, placebo-controlled study. Circulation 2006; 114: 48–54.CrossRefGoogle ScholarPubMed
36. Gatzoulis MA, Beghetti M, Galie N, et al. Longer-term bosentan therapy improves functional capacity in Eisenmenger syndrome: Results of the BREATHE-5 open-label extension study. Int J Cardiol (2007), doi:10.1016/j.ijcard.2007.04.078CrossRefGoogle Scholar
37. Singh, TP, Rohit, M, Grover, A, Malhotra, S, Vijayvergiya, R. A randomized, placebo-controlled, double-blind, crossover study to evaluate the efficacy of oral sildenafil therapy in severe pulmonary artery hypertension. Am Heart J 2006; 151: 851–855.CrossRefGoogle ScholarPubMed
38. Lim, ZS, Salmon, AP, Vettukattil, JJ, Veldtman, GR. Sildenafil therapy for pulmonary arterial hypertension associated with atrial septal defects. Int J Cardiol 2007; 118: 178–182.CrossRefGoogle ScholarPubMed
39. Mukhopadhyay, S, Sharma, M, Ramakrishnan, S, et al. . Phosphodiesterase-5 inhibitor in Eisenmenger syndrome. A preliminary observational study. Circulation 2006; 114: 1807–1810.CrossRefGoogle ScholarPubMed
40. Maurer, JR, Frost, AE, Estenne, M, Higenbottam, T, Glanville, AR. International guidelines for the selection of lung transplant candidates. The International Society for Heart and Lung Transplantation, the American Thoracic Society, the American Society of Transplant Physicians, the European Respiratory Society. Transplantation 1998; 66: 951–956.CrossRefGoogle Scholar
41. Trulock, EP. Lung transplantation. Am J Respir Crit Care Med 1997; 155: 789–818.CrossRefGoogle ScholarPubMed
42. Charman, SC, Sharples, LD, McNeil, KD, Wallwork, J. Assessment of survival benefit after lung transplantation by patient diagnosis. J Heart Lung Transplant 2002; 21: 226–232.CrossRefGoogle ScholarPubMed
43. Waddell, TK, Bennett, L, Kennedy, R, Todd, TR, Keshavjee, SH. Heart-lung or lung transplantation for Eisenmenger syndrome. J Heart Lung Transplant 2002; 21: 731–737.CrossRefGoogle ScholarPubMed