Pulmonary arterial hypertension associated with congenital heart disease

Clinics in Chest Medicine

Volumn 34, Issue 4, 2013, Pages 707-717

  Krishnan, Usha ^a   Rosenzweig, Erika B ^a  

^a NewYork Presbyterian Hospital   (United States)

Author keywords

Congenital heart disease; Eisenmenger syndrome; Fontan; Operability; Pulmonary arterial hypertension

Indexed keywords

CLINICAL FEATURE; CONGENITAL HEART DISEASE; DISEASE ASSOCIATION; EISENMENGER COMPLEX; HEART SINGLE VENTRICLE; HISTOPATHOLOGY; HUMAN; LUNG VASCULAR RESISTANCE; OUTCOME ASSESSMENT; PATHOPHYSIOLOGY; PRIORITY JOURNAL; PROGNOSIS; PULMONARY HYPERTENSION; PULMONARY SHUNT; REVIEW; TREATMENT RESPONSE;

CONGENITAL HEART DISEASE; EISENMENGER SYNDROME; FONTAN; OPERABILITY; PULMONARY ARTERIAL HYPERTENSION;

COMBINED MODALITY THERAPY; HEART DEFECTS, CONGENITAL; HEMODYNAMICS; HUMANS; HYPERTENSION, PULMONARY;

EID: 84888205105     PISSN: 02725231     EISSN: 15578216     Source Type: Journal    
DOI: 10.1016/j.ccm.2013.08.011     Document Type: Review

References (55)

1. Rose M.L., Strange G., King I., et al. Congenital heart disease-associated pulmonary arterial hypertension: preliminary results from a novel registry. Intern Med J 2012, 42:874-879.
2. Gatzoulis M.A., Alonso-Gonzalez R., Beghetti M. Pulmonary arterial hypertension in paediatric and adult patients with congenital heart disease. Eur Respir Rev 2009, 18:154-161.
3. Fratz S., Geiger R., Kresse H., et al. Pulmonary blood pressure, not flow, is associated with net endothelin-1 production in the lungs of patients with congenital heart disease and normal pulmonary vascular resistance. JThorac Cardiovasc Surg 2003, 126:1724-1729.
4. Simonneau G., Robbins I.M., Beghetti M., et al. Updated clinical classification of pulmonary hypertension. JAm Coll Cardiol 2009, 54(Suppl 1):S43-S54.
5. Humbert M., Sitbon O., Chaouat A., et al. Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med 2006, 173:1023-1030.
6. Badesch D.B., Raskob G.E., Elliott C.G., et al. Pulmonary arterial hypertension: baseline characteristics from the REVEAL registry. Chest 2009, 137:376-387.
7. Haworth S.G., Hislop A.A. Treatment and survival in children with pulmonary arterial hypertension: the UK Pulmonary Hypertension Service for Children 2001-2006. Heart 2009, 95:312-317.
8. Durmowicz A.G., Stenmark K.R. Mechanisms of structural remodeling in chronic pulmonary hypertension. Pediatr Rev 1999, 20:e9-e102.
9. Hopkins W.E., Ochoa L.L., Richardson G.W., et al. Comparison of the hemodynamics and survival of adults with severe primary pulmonary hypertension or Eisenmenger syndrome. JHeart Lung Transplant 1996, 15:100-105.
10. Manes A., Palazzini M., Leci E., et al. Current era survival of patients with pulmonary arterial hypertension associated with congenital heart disease: a comparison between clinical subgroups. Eur Heart J 2013, [Epub ahead of print].
11. Engelfriet P.M., Duffels M.G., Moller T., et al. Pulmonary arterial hypertension in adults born with a heart septal defect: the Euro Heart Survey on adult congenital heart disease. Heart 2007, 93:682-687.
12. McLaughlin V.V., Archer S.L., Badesch D.B., et al. 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. JAm Coll Cardiol 2009, 53:1573-1619.
13. Benza R.L., Miller D.P., 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, 122:164-172.
14. Lindberg L., Olsson A.K., Jogi P., et al. How common is severe pulmonary hypertension after pediatric cardiac surgery?. JThorac Cardiovasc Surg 2002, 123:1155-1163.
15. Bando K., Turrentine M.W., Sharp T.G., et al. Pulmonary hypertension after operations for congenital heart disease: analysis of risk factors and management. JThorac Cardiovasc Surg 1996, 112(6):1600-1607. [discussion: 1607-9].
16. Matsumoto M., Naitoh H., Higashi T., et al. Risk factors for pulmonary hypertensive crisis (PHC) following VSD repair in infants. Masui 1995, 44:1208-1212.
17. Adatia I., Beghetti M. Early postoperative care of patients with pulmonary hypertension associated with congenital cardiac disease. Cardiol Young 2009, 19:315-319.
18. Rosenzweig E.B., Barst R.J. Congenital heart disease and pulmonary hypertension: pharmacology and feasibility of late surgery. Prog Cardiovasc Dis 2012, 55(2):128-133.
19. Beghetti M., Tissot C. Pulmonary hypertension in congenital shunts. Rev Esp Cardiol 2010, 63:1179-1193.
20. Beghetti M., Galie' N., Bonnet D. Can inoperable congenital heart defects become operable in patients with pulmonary arterial hypertension? Dream or reality?. Congenit Heart Dis 2012, 7:3-11.
21. Lopes A.A., O'Leary P.W. Measurement, interpretation and use of haemodynamic parameters in pulmonary hypertension associated with congenital cardiac disease. Cardiol Young 2009, 19:431-435.
22. Viswanathan S., Kumar R.K. Assessment of operability of congenital cardiac shunts with increased pulmonary vascular resistance. Catheter Cardiovasc Interv 2008, 71:665-670.
23. Giglia T.M., Humpl T. Preoperative pulmonary hemodynamics and assessment of operability: is there a pulmonary vascular resistance that precludes cardiac operation?. Pediatr Crit Care Med 2010, 11(Suppl 2):S57-S69.
24. Matamis D., Pampori S., Papathanasiou A., et al. Inhaled NO and sildenafil combination in cardiac surgery patients with out-of-proportion pulmonary hypertension: acute effects on postoperative gas exchange and hemodynamics. Circ Heart Fail 2012, 5:47-53.
25. Galie N., Hoeper M.M., Humbert M., et al. Guidelines for the diagnosis and treatment of pulmonary hypertension: the 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 the International Society of Heart and Lung Transplantation (ISHLT). Eur Heart J 2009, 30:2493-2537.
26. Dimopoulos K., Inuzuka R., Goletto S., et al. Improved survival among patients with Eisenmenger syndrome receiving advanced therapy for pulmonary arterial hypertension. Circulation 2010, 121:20-25.
27. Galie N., Manes A., Palazzini M., et al. Management of pulmonary arterial hypertension associated with congenital systemic-to-pulmonary shunts and Eisenmenger's syndrome. Drugs 2008, 68:1049-1066.
28. Lopes A.A., Bandeira A.P., Flores P.C., et al. Pulmonary hypertension in Latin America: pulmonary vascular disease: the global perspective. Chest 2010, 137(Suppl 6):78S-84S.
29. Diller G.P., Dimopoulos K., Broberg C.S., et al. Presentation, survival prospects, and predictors of death in Eisenmenger syndrome: a combined retrospective and case-control study. Eur Heart J 2006, 27:1737-1742.
30. Swan L., Lupton M., Anthony J., et al. Controversies in pregnancy and congenital heart disease. Congenit Heart Dis 2006, 1:27-34.
31. Bedard E., Dimopoulos K., Gatzoulis M.A. Has there been any progress made on pregnancy outcomes among women with pulmonary arterial hypertension?. Eur Heart J 2009, 30:256-265.
32. Rosenzweig E.B., Kerstein D., Barst R.J. Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects. Circulation 1999, 99:1858-1865.
33. Barst R.J., Simonneau G., Rich S., for the Uniprost PAH Study Group, et al. Efficacy and safety of chronic subcutaneous infusion of UT-15 (Uniprost) in pulmonary arterial hypertension (PAH). Circulation 2000, 102:100-101.
34. Galie N., Beghetti M., Gatzoulis M.A., et al. Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, double-blind, randomized, placebo-controlled study. Circulation 2006, 114:48-54.
35. Gatzoulis M.A., 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 2008, 127(1):27-32.
36. Zuckerman W.A., Leaderer D., Rowan C.A., et al. Ambrisentan for pulmonary arterial hypertension due to congenital heart disease. Am J Cardiol 2011, 107(9):1381-1385.
37. Schulze-Neick I., Hartenstein P., Li J. Intravenous Sildenafil is a potent pulmonary vasodilator in children with congenital heart disease. Circulation 2003, 108:II167-II173.
38. Zhang Z.N., Jiang X., Zhang R., et al. Oral sildenafil treatment for Eisenmenger syndrome: a prospective, open-label, multicentre study. Heart 2011, 97(22):1876-1881.
39. Mukhopadhyay S., Nathani S., Yusuf J., et al. Clinical efficacy of phosphodiesterase-5 inhibitor tadalafil in Eisenmenger syndrome-a randomized, placebo-controlled, double-blind crossover study. Congenit Heart Dis 2011, 6(5):424-431.
40. Ofori-Amanfo G., Hsu D., Lamour J.M., et al. Heart transplantation in children with markedly elevated pulmonary vascular resistance: impact of right ventricular failure on outcome. JHeart Lung Transplant 2011, 30(6):659-666.
41. Lang G., Taghavi S., Aigner C. Primary lung transplantation after bridge with extracorporeal membrane oxygenation: a plea for a shift in our paradigms for indications. Transplantation 2012, 93:729-736.
42. Trulock E.P., Edwards L.B., Taylor D.O., et al. The Registry of the International Society for Heart and Lung Transplantation: Twentieth Official Adult Lung and Heart-Lung transplant report-2003. JHeart Lung Transplant 2003, 22:625-635.
43. Khambadkone S., Li J., de Leval M.R., et al. Basal pulmonary vascular resistance and nitric oxide responsiveness late after Fontan-type operation. Circulation 2003, 107(25):3204-3208.
44. Beghetti M. Fontan and the pulmonary circulation: a potential role for new pulmonary hypertension therapies. Heart 2010, 96(12):911-916.
45. Goldberg D.J., Shaddy R.E., Ravishankar C., et al. The failing Fontan: etiology, diagnosis and management. Expert Rev Cardiovasc Ther 2011, 9(6):785-793.
46. Ryerson L., Goldberg C., Rosenthal A., et al. Usefulness of heparin therapy in protein-losing enteropathy associated with single ventricle palliation. Am J Cardiol 2008, 101:248-251.
47. Ringel R.E., Peddy S.B. Effect of high-dose spironolactone on protein-losing enteropathy in patients with Fontan palliation of complex congenital heart disease. Am J Cardiol 2003, 91:1031-1032. A1039.
48. Yoshimura N., Yamaguchi M., Oka S., et al. Inhaled nitric oxide therapy after Fontan-type operations. Surg Today 2005, 35:31-35.
49. Cai J., Su Z., Shi Z., et al. Nitric oxide and milrinone: combined effect on pulmonary circulation after Fontan-type procedure: a prospective, randomized study. Ann Thorac Surg 2008, 86:882-888.
50. Miyaji K., Nagata N., Miyamoto T., et al. Combined therapy with inhaled nitric oxide and intravenous epoprostenol (prostacyclin) for critical pulmonary perfusion after the Fontan procedure. JThorac Cardiovasc Surg 2003, 125:437-439.
51. Trachte A.L., Lobato E.B., Urdaneta F., et al. Oral sildenafil reduces pulmonary hypertension after cardiac surgery. Ann Thorac Surg 2005, 79:194-197.
52. Haseyama K., Satomi G., Yasukochi S., et al. Pulmonary vasodilation therapy with sildenafil citrate in a patient with plastic bronchitis after the Fontan procedure for hypoplastic left heart syndrome. JThorac Cardiovasc Surg 2006, 132(5):1232-1233.
53. Giardini A., Balducci A., Specchia S., et al. Effect of sildenafil on haemodynamic response to exercise and exercise capacity in Fontan patients. Eur Heart J 2008, 29:1681-1687.
54. Apostolopoulou S.C., Papagiannis J., Rammos S. Bosentan induces clinical, exercise and hemodynamic improvement in a pre-transplant patient with plastic bronchitis after Fontan operation. JHeart Lung Transplant 2005, 24:1174-1176.
55. Ovaert C., Thijs D., Dewolf D., et al. The effect of bosentan in patients with a failing Fontan circulation. Cardiol Young 2009, 19:331-339.