Congenital Heart Disease and Pulmonary Hypertension

Heart Failure Clinics

Volumn 8, Issue 3, 2012, Pages 427-445

  Gupta, Vedant ^a   Tonelli, Adriano R ^a   Krasuski, Richard A ^a  

^a LERNER RESEARCH INSTITUTE   (United States)

Author keywords

Congenital heart disease; Eisenmenger syndrome; Management; Pulmonary hypertension; Treatment

Indexed keywords

AMBRISENTAN; AMIODARONE; ANTIARRHYTHMIC AGENT; ANTIBIOTIC AGENT; ANTICOAGULANT AGENT; BOSENTAN; CALCIUM CHANNEL BLOCKING AGENT; DIGITALIS; DIURETIC AGENT; ENDOTHELIN RECEPTOR ANTAGONIST; FIOLAN; ILOPROST; IRON; LETARIS; OXYGEN; PHOSPHODIESTERASE V INHIBITOR; PLACEBO; PROGESTERONE; PROSTACYCLIN; PROSTACYCLIN DERIVATIVE; PROSTANOID; SILDENAFIL; SITAXSENTAN; TADALAFIL; UNCLASSIFIED DRUG; UNIPROST; VASODILATOR AGENT;

AEROBIC EXERCISE; ANTIBIOTIC PROPHYLAXIS; ANTICOAGULANT THERAPY; BACTERIAL ENDOCARDITIS; BLEEDING; BLOOD CLOTTING DISORDER; CARDIAC PATIENT; CARDIOPULMONARY EXERCISE TEST; CEREBROVASCULAR DISEASE; CONGENITAL HEART DISEASE; CONNECTIVE TISSUE; CONTRACEPTION; CYANOSIS; DEFIBRILLATION; DEFIBRILLATOR; DISEASE ASSOCIATION; DISEASE CLASSIFICATION; DISEASE COURSE; DISEASE SEVERITY; DYSPNEA; ECHOCARDIOGRAPHY; EISENMENGER COMPLEX; EMPIRICISM; EPIDEMIOLOGICAL DATA; ERYTHROCYTOSIS; EXERCISE TOLERANCE; EXERTIONAL DYSPNEA; FETUS MORTALITY; GALLSTONE; GOUT; GRAFT SURVIVAL; HEART CATHETERIZATION; HEART GRAFT; HEART SUPRAVENTRICULAR ARRHYTHMIA; HEART TRANSPLANTATION; HEART VENTRICLE ARRHYTHMIA; HEMODYNAMICS; HEMOPTYSIS; HIGH RISK PATIENT; HUMAN; HYPERURICEMIA; HYPERVISCOSITY SYNDROME; INFECTION COMPLICATION; IRON DEFICIENCY; IRON THERAPY; KIDNEY INJURY; LABORATORY TEST; LIVER DYSFUNCTION; LUNG EMBOLISM; LUNG VEIN OBSTRUCTION; MATERNAL MORBIDITY; NUCLEAR MAGNETIC RESONANCE IMAGING; OXYGEN THERAPY; PATHOPHYSIOLOGY; PHYSICAL EXAMINATION; PULMONARY ARTERY OCCLUSION; PULMONARY HYPERTENSION; PULMONARY VASCULAR DISEASE; RANDOMIZED CONTROLLED TRIAL (TOPIC); REVIEW; SYSTEMIC CYANOSIS; SYSTEMIC PULMONARY SHUNT; THORAX RADIOGRAPHY; THROMBOCYTE DYSFUNCTION; THROMBOCYTOPENIA;

ARRHYTHMIAS, CARDIAC; CARDIAC CATHETERIZATION; EXERCISE TEST; EXERCISE TOLERANCE; HEART DEFECTS, CONGENITAL; HEMOPTYSIS; HUMANS; HYPERTENSION, PULMONARY; OXYGEN INHALATION THERAPY; PULMONARY EMBOLISM; RISK FACTORS;

EID: 84863084294     PISSN: 15517136     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.hfc.2012.04.002     Document Type: Review

References (161)

1. Wood P. The Eisenmenger syndrome or pulmonary hypertension with reversed central shunt. Br Med J 1958, 2:755-762.
2. Steele P.M., Fuster V., Cohen M., et al. Isolated atrial septal defect with pulmonary vascular obstructive disease-long-term follow-up and prediction of outcome after surgical correction. Circulation 1987, 76:1037-1042.
3. Evans W., Short D.S. Pulmonary hypertension in congenital heart disease. Br Heart J 1958, 20:529-551.
4. Cherian G., Uthaman C.B., Durairaj M., et al. Pulmonary hypertension in isolated secundum atrial septal defect: high frequency in young patients. Am Heart J 1983, 105:952-957.
5. Haworth S.G. Pulmonary vascular bed in children with complete atrioventricular septal defect: relation between structural and hemodynamic abnormalities. Am J Cardiol 1986, 57:833-839.
6. Newfeld E.A., Sher M., Paul M.H., et al. Pulmonary vascular disease in complete atrioventricular canal defect. Am J Cardiol 1977, 39:721-726.
7. Campbell M. Natural history of ventricular septal defect. Br Heart J 1971, 33:246-257.
8. Christman B.W., McPherson C.D., Newman J.H., et al. An imbalance between the excretion of thromboxane and prostacyclin metabolites in pulmonary hypertension. N Engl J Med 1992, 327:70-75.
9. Heath D., Edwards J.E. The pathology of hypertensive pulmonary vascular disease; a description of six grades of structural changes in the pulmonary arteries with special reference to congenital cardiac septal defects. Circulation 1958, 18:533-547.
10. Rabinovitch M., Haworth S.G., Castaneda A.R., et al. Lung biopsy in congenital heart disease: a morphometric approach to pulmonary vascular disease. Circulation 1978, 58:1107-1122.
11. Rabinovitch M., Keane J.F., Norwood W.I., et al. Vascular structure in lung tissue obtained at biopsy correlated with pulmonary hemodynamic findings after repair of congenital heart defects. Circulation 1984, 69:655-667.
12. Besterman E. Atrial septal defect with pulmonary hypertension. Br Heart J 1961, 23:587-598.
13. Diller G.P., Gatzoulis M.A. Pulmonary vascular disease in adults with congenital heart disease. Circulation 2007, 115:1039-1050.
14. Fasules J.W., Tryka F., Chipman C.W., et al. Pulmonary hypertension and arterial changes in calves with a systemic-to-left pulmonary artery connection. J Appl Physiol 1994, 77:867-875.
15. Hoffman J.I., Rudolph A.M. The natural history of ventricular septal defects in infancy. Am J Cardiol 1965, 16:634-653.
16. Humbert M., Morrell N.W., Archer S.L., et al. Cellular and molecular pathobiology of pulmonary arterial hypertension. J Am Coll Cardiol 2004, 43:13S-24S.
17. Kidd L., Driscoll D.J., Gersony W.M., et al. Second natural history study of congenital heart defects. Results of treatment of patients with ventricular septal defects. Circulation 1993, 87:I38-I51.
18. Niwa K., Perloff J.K., Kaplan S., et al. Eisenmenger syndrome in adults: ventricular septal defect, truncus arteriosus, univentricular heart. J Am Coll Cardiol 1999, 34:223-232.
19. Vongpatanasin W., Brickner M.E., Hillis L.D., et al. The Eisenmenger syndrome in adults. Ann Intern Med 1998, 128:745-755.
20. Diller G.P., Dimopoulos K., Okonko D., et al. Exercise intolerance in adult congenital heart disease: comparative severity, correlates, and prognostic implication. Circulation 2005, 112:828-835.
21. Badesch D.B., Champion H.C., Sanchez M.A., et al. Diagnosis and assessment of pulmonary arterial hypertension. J Am Coll Cardiol 2009, 54:S55-S66.
22. 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.
23. Simonneau G., Robbins I.M., Beghetti M., et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2009, 54:S43-S54.
24. Adatia I., Kothari S.S., Feinstein J.A. Pulmonary hypertension associated with congenital heart disease: pulmonary vascular disease: the global perspective. Chest 2010, 137:52S-61S.
25. Broberg C., Ujita M., Babu-Narayan S., et al. Massive pulmonary artery thrombosis with haemoptysis in adults with Eisenmenger's syndrome: a clinical dilemma. Heart 2004, 90:e63.
26. Hopkins W.E. The remarkable right ventricle of patients with Eisenmenger syndrome. Coron Artery Dis 2005, 16:19-25.
27. 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. J Heart Lung Transplant 1996, 15:100-105.
28. van Albada M.E., Berger R.M. Pulmonary arterial hypertension in congenital cardiac disease-the need for refinement of the Evian-Venice classification. Cardiol Young 2008, 18:10-17.
29. Dimopoulos K., Giannakoulas G., Wort S.J., et al. 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, 23:545-554.
30. Beghetti M., Galie N. Eisenmenger syndrome a clinical perspective in a new therapeutic era of pulmonary arterial hypertension. J Am Coll Cardiol 2009, 53:733-740.
31. Marelli A.J., Mackie A.S., Ionescu-Ittu R., et al. Congenital heart disease in the general population: changing prevalence and age distribution. Circulation 2007, 115:163-172.
32. Marelli A.J., Therrien J., Mackie A.S., et al. Planning the specialized care of adult congenital heart disease patients: from numbers to guidelines; an epidemiologic approach. Am Heart J 2009, 157:1-8.
33. 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.
34. Friedman W.F. Proceedings of National Heart, Lung, and Blood Institute pediatric cardiology workshop: pulmonary hypertension. Pediatr Res 1986, 20:811-824.
35. Duffels M.G., Engelfriet P.M., Berger R.M., et al. Pulmonary arterial hypertension in congenital heart disease: an epidemiologic perspective from a Dutch registry. Int J Cardiol 2007, 120:198-204.
36. Lowe B.S., Therrien J., Ionescu-Ittu R., et al. Diagnosis of pulmonary hypertension in the congenital heart disease adult population impact on outcomes. J Am Coll Cardiol 2011, 58:538-546.
37. Rabinovitch M. Pulmonary hypertension: pathophysiology as a basis for clinical decision making. J Heart Lung Transplant 1999, 18:1041-1053.
38. Du L., Sullivan C.C., Chu D., et al. Signaling molecules in nonfamilial pulmonary hypertension. N Engl J Med 2003, 348:500-509.
39. Diller G.P., van Eijl S., Okonko D.O., et al. Circulating endothelial progenitor cells in patients with Eisenmenger syndrome and idiopathic pulmonary arterial hypertension. Circulation 2008, 117:3020-3030.
40. Shah P., Singh W.S., Rose V., et al. Incidence of bacterial endocarditis in ventricular septal defects. Circulation 1966, 34:127-131.
41. Trojnarska O., Gwizdala A., Katarzynski S., et al. The BNP concentrations and exercise capacity assessment with cardiopulmonary stress test in cyanotic adult patients with congenital heart diseases. Int J Cardiol 2010, 139:241-247.
42. Berman E.B., Barst R.J. Eisenmenger's syndrome: current management. Prog Cardiovasc Dis 2002, 45:129-138.
43. Hassoun P.M., Thappa V., Landman M.J., et al. Endothelin 1: mitogenic activity on pulmonary artery smooth muscle cells and release from hypoxic endothelial cells. Proc Soc Exp Biol Med 1992, 199:165-170.
44. Zamora M.R., Stelzner T.J., Webb S., et al. Overexpression of endothelin-1 and enhanced growth of pulmonary artery smooth muscle cells from fawn-hooded rats. Am J Physiol 1996, 270:L101-L109.
45. Cacoub P., Dorent R., Maistre G., et al. Endothelin-1 in primary pulmonary hypertension and the Eisenmenger syndrome. Am J Cardiol 1993, 71:448-450.
46. Giaid A., Saleh D. Reduced expression of endothelial nitric oxide synthase in the lungs of patients with pulmonary hypertension. N Engl J Med 1995, 333:214-221.
47. Cooper C.J., Landzberg M.J., Anderson T.J., et al. Role of nitric oxide in the local regulation of pulmonary vascular resistance in humans. Circulation 1996, 93:266-271.
48. Moncada S., Higgs A. The L-arginine-nitric oxide pathway. N Engl J Med 1993, 329:2002-2012.
49. Michelakis E.D., Tymchak W., Noga M., et al. Long-term treatment with oral sildenafil is safe and improves functional capacity and hemodynamics in patients with pulmonary arterial hypertension. Circulation 2003, 108:2066-2069.
50. Rondelet B., Kerbaul F., Van Beneden R., et al. Signaling molecules in overcirculation-induced pulmonary hypertension in piglets: effects of sildenafil therapy. Circulation 2004, 110:2220-2225.
51. Tuder R.M., Cool C.D., Geraci M.W., et al. Prostacyclin synthase expression is decreased in lungs from patients with severe pulmonary hypertension. Am J Respir Crit Care Med 1999, 159:1925-1932.
52. Roberts K.E., McElroy J.J., Wong W.P., et al. BMPR2 mutations in pulmonary arterial hypertension with congenital heart disease. Eur Respir J 2004, 24:371-374.
53. Newman J.H., Fanburg B.L., Archer S.L., et al. Pulmonary arterial hypertension: future directions: report of a National Heart, Lung and Blood Institute/Office of Rare Diseases workshop. Circulation 2004, 109:2947-2952.
54. Lepetit H., Eddahibi S., Fadel E., et al. Smooth muscle cell matrix metalloproteinases in idiopathic pulmonary arterial hypertension. Eur Respir J 2005, 25:834-842.
55. Petkov V., Mosgoeller W., Ziesche R., et al. Vasoactive intestinal peptide as a new drug for treatment of primary pulmonary hypertension. J Clin Invest 2003, 111:1339-1346.
56. Guignabert C., Izikki M., Tu L.I., et al. Transgenic mice overexpressing the 5-hydroxytryptamine transporter gene in smooth muscle develop pulmonary hypertension. Circ Res 2006, 98:1323-1330.
57. Zaiman A.L., Podowski M., Medicherla S., et al. Role of the TGF-beta/Alk5 signaling pathway in monocrotaline-induced pulmonary hypertension. Am J Respir Crit Care Med 2008, 177:896-905.
58. Morrell N.W., Adnot S., Archer S.L., et al. Cellular and molecular basis of pulmonary arterial hypertension. J Am Coll Cardiol 2009, 54:S20-S31.
59. Erzurum S., Rounds S.I., Stevens T., et al. Strategic plan for lung vascular research: an NHLBI-ORDR Workshop Report. Am J Respir Crit Care Med 2010, 182:1554-1562.
60. 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.
61. Dimopoulos K., Okonko D.O., Diller G.P., et al. Abnormal ventilatory response to exercise in adults with congenital heart disease relates to cyanosis and predicts survival. Circulation 2006, 113:2796-2802.
62. Paciocco G., Martinez F.J., Bossone E., et al. Oxygen desaturation on the six-minute walk test and mortality in untreated primary pulmonary hypertension. Eur Respir J 2001, 17:647-652.
63. Miyamoto S., Nagaya N., Satoh T., et al. Clinical correlates and prognostic significance of six-minute walk test in patients with primary pulmonary hypertension. Comparison with cardiopulmonary exercise testing. Am J Respir Crit Care Med 2000, 161:487-492.
64. Wensel R., Opitz C.F., Anker S.D., et al. Assessment of survival in patients with primary pulmonary hypertension: importance of cardiopulmonary exercise testing. Circulation 2002, 106:319-324.
65. Cantor W.J., Harrison D.A., Moussadji J.S., et al. Determinants of survival and length of survival in adults with Eisenmenger syndrome. Am J Cardiol 1999, 84:677-681.
66. Daliento L., Somerville J., Presbitero P., et al. Eisenmenger syndrome. Factors relating to deterioration and death. Eur Heart J 1998, 19:1845-1855.
67. Sreeram N. Eisenmenger syndrome: towards identifying the risk factors for death. Eur Heart J 2006, 27:1644-1645.
68. Broberg C.S., Ujita M., Prasad S., et al. Pulmonary arterial thrombosis in eisenmenger syndrome is associated with biventricular dysfunction and decreased pulmonary flow velocity. J Am Coll Cardiol 2007, 50:634-642.
69. Silversides C.K., Granton J.T., Konen E., et al. Pulmonary thrombosis in adults with Eisenmenger syndrome. J Am Coll Cardiol 2003, 42:1982-1987.
70. Broberg C.S., Bax B.E., Okonko D.O., et al. Blood viscosity and its relationship to iron deficiency, symptoms, and exercise capacity in adults with cyanotic congenital heart disease. J Am Coll Cardiol 2006, 48:356-365.
71. Gidding S.S., Stockman J.A. Erythropoietin in cyanotic heart disease. Am Heart J 1988, 116:128-132.
72. Kontras S.B., Bodenbender J.G., Craenen J., et al. Hyperviscosity in congenital heart disease. J Pediatr 1970, 76:214-220.
73. Perloff J.K., Rosove M.H., Child J.S., et al. Adults with cyanotic congenital heart disease: hematologic management. Ann Intern Med 1988, 109:406-413.
74. Rosove M.H., Perloff J.K., Hocking W.G., et al. Chronic hypoxaemia and decompensated erythrocytosis in cyanotic congenital heart disease. Lancet 1986, 2:313-315.
75. Lill M.C., Perloff J.K., Child J.S. Pathogenesis of thrombocytopenia in cyanotic congenital heart disease. Am J Cardiol 2006, 98:254-258.
76. Perloff J.K. Systemic complications of cyanosis in adults with congenital heart disease. Hematologic derangements, renal function, and urate metabolism. Cardiol Clin 1993, 11:689-699.
77. Ammash N., Warnes C.A. Cerebrovascular events in adult patients with cyanotic congenital heart disease. J Am Coll Cardiol 1996, 28:768-772.
78. Perloff J.K., Latta H., Barsotti P. Pathogenesis of the glomerular abnormality in cyanotic congenital heart disease. Am J Cardiol 2000, 86:1198-1204.
79. Perloff J.K., Marelli A.J., Miner P.D. Risk of stroke in adults with cyanotic congenital heart disease. Circulation 1993, 87:1954-1959.
80. Kaemmerer H., Fratz S., Braun S.L., et al. Erythrocyte indexes, iron metabolism, and hyperhomocysteinemia in adults with cyanotic congenital cardiac disease. Am J Cardiol 2004, 94:825-828.
81. Diller G.P., Alonso-Gonzalez R., Kempny A., et al. B type natriuretic peptide concentrations in contemporary Eisenmenger syndrome patients: predictive value and response to disease targeting therapy. Heart 2012, 98(9):736-742.
82. Knirsch W., Hausermann E., Fasnacht M., et al. Plasma B-type natriuretic peptide levels in children with heart disease. Acta Paediatr 2011, 100:1213-1216.
83. Sheehan R., Perloff J.K., Fishbein M.C., et al. Pulmonary neovascularity: a distinctive radiographic finding in Eisenmenger syndrome. Circulation 2005, 112:2778-2785.
84. Warnes C.A., Williams R.G., Bashore T.M., et al. ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines on the management of adults with congenital heart disease). Circulation 2008, 118:e714-e833.
85. 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. Circulation 2009, 119:2250-2294.
86. Balzer D.T., Kort H.W., Day R.W., et al. Inhaled nitric oxide as a preoperative test (INOP test I): the INOP Test Study Group. Circulation 2002, 106:I76-I81.
87. Post M.C., Janssens S., Van de Werf F., et al. Responsiveness to inhaled nitric oxide is a predictor for mid-term survival in adult patients with congenital heart defects and pulmonary arterial hypertension. Eur Heart J 2004, 25:1651-1656.
88. D'Alto M., Romeo E., Argiento P., et al. Pulmonary vasoreactivity predicts long-term outcome in patients with Eisenmenger syndrome receiving bosentan therapy. Heart 2010, 96:1475-1479.
89. Budts W., Van Pelt N., Gillyns H., et al. Residual pulmonary vasoreactivity to inhaled nitric oxide in patients with severe obstructive pulmonary hypertension and Eisenmenger syndrome. Heart 2001, 86:553-558.
90. Goodwin T.M., Gherman R.B., Hameed A., et al. Favorable response of Eisenmenger syndrome to inhaled nitric oxide during pregnancy. Am J Obstet Gynecol 1999, 180:64-67.
91. ATS statement: guidelines for the six-minute walk test. Am J Respir Crit Care Med 2002, 166:111-117.
92. 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.
93. Deanfield J., Thaulow E., Warnes C., et al. Management of grown up congenital heart disease. Eur Heart J 2003, 24:1035-1084.
94. Antibiotic prophylaxis of infective endocarditis. Recommendations from the Endocarditis Working Party of the British Society for Antimicrobial Chemotherapy. Lancet 1990, 335:88-89.
95. Wilson W., Taubert K.A., Gewitz M., et al. Prevention of infective endocarditis: guidelines from the American Heart Association. Circulation 2007, 116:1736-1754.
96. Montani D., Savale L., Natali D., et al. Long-term response to calcium-channel blockers in non-idiopathic pulmonary arterial hypertension. Eur Heart J 2010, 31:1898-1907.
97. Avila W.S., Grinberg M., Snitcowsky R., et al. Maternal and fetal outcome in pregnant women with Eisenmenger's syndrome. Eur Heart J 1995, 16:460-464.
98. Uebing A., Steer P.J., Yentis S.M., et al. Pregnancy and congenital heart disease. BMJ 2006, 332:401-406.
99. Yentis S.M., Steer P.J., Plaat F. Eisenmenger's syndrome in pregnancy: maternal and fetal mortality in the 1990s. Br J Obstet Gynaecol 1998, 105:921-922.
100. Opotowsky A.R., Siddiqi O.K., D'Souza B., et al. Maternal cardiovascular events during childbirth among women with congenital heart disease. Heart 2012, 98:145-151.
101. Apostolopoulou S.C., Manginas A., Cokkinos D.V., et al. Effect of the oral endothelin antagonist bosentan on the clinical, exercise, and haemodynamic status of patients with pulmonary arterial hypertension related to congenital heart disease. Heart 2005, 91:1447-1452.
102. Benza R.L., Rayburn B.K., Tallaj J.A., et al. Efficacy of bosentan in a small cohort of adult patients with pulmonary arterial hypertension related to congenital heart disease. Chest 2006, 129:1009-1015.
103. Channick R.N., 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.
104. Christensen D.D., McConnell M.E., Book W.M., et al. Initial experience with bosentan therapy in patients with the Eisenmenger syndrome. Am J Cardiol 2004, 94:261-263.
105. Gatzoulis M.A., Rogers P., Li W., et al. Safety and tolerability of bosentan in adults with Eisenmenger physiology. Int J Cardiol 2005, 98:147-151.
106. Gilbert N., Luther Y.C., Miera O., et al. Initial experience with bosentan (Tracleer) as treatment for pulmonary arterial hypertension (PAH) due to congenital heart disease in infants and young children. Z Kardiol 2005, 94:570-574.
107. Rubin L.J., Badesch D.B., Barst R.J., et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med 2002, 346:896-903.
108. Schulze-Neick I., Gilbert N., Ewert R., et al. Adult patients with congenital heart disease and pulmonary arterial hypertension: first open prospective multicenter study of bosentan therapy. Am Heart J 2005, 150:716.
109. 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.
110. Berger R.M., Beghetti M., Galie N., 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, 144:373-378.
111. 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, 127:27-32.
112. Apostolopoulou S.C., Manginas A., Cokkinos D.V., et al. Long-term oral bosentan treatment in patients with pulmonary arterial hypertension related to congenital heart disease: a 2-year study. Heart 2007, 93:350-354.
113. D'Alto M., Vizza C.D., Romeo E., et al. Long term effects of bosentan treatment in adult patients with pulmonary arterial hypertension related to congenital heart disease (Eisenmenger physiology): safety, tolerability, clinical, and haemodynamic effect. Heart 2007, 93:621-625.
114. Diaz-Caraballo E., Gonzalez-Garcia A.E., Renones M., et al. Long-term bosentan treatment of complex congenital heart disease and Eisenmenger's syndrome. Rev Esp Cardiol 2009, 62:1046-1049.
115. Diller G.P., Dimopoulos K., Kaya M.G., et al. Long-term safety, tolerability and efficacy of bosentan in adults with pulmonary arterial hypertension associated with congenital heart disease. Heart 2007, 93:974-976.
116. Mehta P.K., Simpson L., Lee E.K., et al. Endothelin receptor antagonists improve exercise tolerance and oxygen saturations in patients with Eisenmenger syndrome and congenital heart defects. Tex Heart Inst J 2008, 35:256-261.
117. Galie N., Rubin L., Hoeper M., et al. Treatment of patients with mildly symptomatic pulmonary arterial hypertension with bosentan (EARLY study): a double-blind, randomised controlled trial. Lancet 2008, 371:2093-2100.
118. Vis J.C., Duffels M.G., Mulder P., et al. Prolonged beneficial effect of bosentan treatment and 4-year survival rates in adult patients with pulmonary arterial hypertension associated with congenital heart disease. Int J Cardiol 2011, 10.1016/j.ijcard.2011.06.064.
119. Avellana P., Segovia J., Sufrate E., et al. Long-term (5 years) effects of bosentan in patients with pulmonary arterial hypertension. Rev Esp Cardiol 2011, 64:667-673.
120. Monfredi O., Griffiths L., Clarke B., et al. Efficacy and safety of bosentan for pulmonary arterial hypertension in adults with congenital heart disease. Am J Cardiol 2011, 108:1483-1488.
121. van Loon R.L., Hoendermis E.S., Duffels M.G., et al. Long-term effect of bosentan in adults versus children with pulmonary arterial hypertension associated with systemic-to-pulmonary shunt: does the beneficial effect persist?. Am Heart J 2007, 154:776-782.
122. Galie N., Olschewski H., Oudiz R.J., et al. Ambrisentan for the treatment of pulmonary arterial hypertension: results of the ambrisentan in pulmonary arterial hypertension, randomized, double-blind, placebo-controlled, multicenter, efficacy (ARIES) study 1 and 2. Circulation 2008, 117:3010-3019.
123. Oudiz R.J., Galie N., Olschewski H., et al. Long-term ambrisentan therapy for the treatment of pulmonary arterial hypertension. J Am Coll Cardiol 2009, 54:1971-1981.
124. Zuckerman W.A., Leaderer D., Rowan C.A., et al. Ambrisentan for pulmonary arterial hypertension due to congenital heart disease. Am J Cardiol 2011, 107:1381-1385.
125. McGoon M.D., Frost A.E., Oudiz R.J., et al. Ambrisentan therapy in patients with pulmonary arterial hypertension who discontinued bosentan or sitaxsentan due to liver function test abnormalities. Chest 2009, 135:122-129.
126. Galie N., Ghofrani H.A., Torbicki A., et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 2005, 353:2148-2157.
127. Chau E.M., Fan K.Y., Chow W.H. Effects of chronic sildenafil in patients with Eisenmenger syndrome versus idiopathic pulmonary arterial hypertension. Int J Cardiol 2007, 120:301-305.
128. Garg N., Sharma M.K., Sinha N. Role of oral sildenafil in severe pulmonary arterial hypertension: clinical efficacy and dose response relationship. Int J Cardiol 2007, 120:306-313.
129. Humpl T., Reyes J.T., Holtby H., et al. Beneficial effect of oral sildenafil therapy on childhood pulmonary arterial hypertension: twelve-month clinical trial of a single-drug, open-label, pilot study. Circulation 2005, 111:3274-3280.
130. Lim Z.S., Salmon A.P., Vettukattil J.J., et al. Sildenafil therapy for pulmonary arterial hypertension associated with atrial septal defects. Int J Cardiol 2007, 118:178-182.
131. Mikhail G.W., Prasad S.K., Li W., et al. Clinical and haemodynamic effects of sildenafil in pulmonary hypertension: acute and mid-term effects. Eur Heart J 2004, 25:431-436.
132. Mukhopadhyay S., Sharma M., Ramakrishnan S., et al. Phosphodiesterase-5 inhibitor in Eisenmenger syndrome: a preliminary observational study. Circulation 2006, 114:1807-1810.
133. Singh T.P., Rohit M., Grover A., et al. 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. e851-5.
134. Rosenzweig E.B., Kerstein D., Barst R.J. Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects. Circulation 1999, 99:1858-1865.
135. Fernandes S.M., Newburger J.W., Lang P., et al. Usefulness of epoprostenol therapy in the severely ill adolescent/adult with Eisenmenger physiology. Am J Cardiol 2003, 91:632-635.
136. Badesch D.B., McLaughlin V.V., Delcroix M., et al. Prostanoid therapy for pulmonary arterial hypertension. J Am Coll Cardiol 2004, 43:56S-61S.
137. Frost A.E., Quinones M.A., Zoghbi W.A., et al. Reversal of pulmonary hypertension and subsequent repair of atrial septal defect after treatment with continuous intravenous epoprostenol. J Heart Lung Transplant 2005, 24:501-503.
138. Simonneau G., Barst R.J., 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.
139. Barst R.J., McGoon M., McLaughlin V., et al. Beraprost therapy for pulmonary arterial hypertension. J Am Coll Cardiol 2003, 41:2119-2125.
140. Galie N., Humbert M., Vachiery J.L., 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.
141. Oudiz R.J., Schilz R.J., Barst R.J., et al. Treprostinil, a prostacyclin analogue, in pulmonary arterial hypertension associated with connective tissue disease. Chest 2004, 126:420-427.
142. D'Alto M., Romeo E., Argiento P., et al. Bosentan-sildenafil association in patients with congenital heart disease-related pulmonary arterial hypertension and Eisenmenger physiology. Int J Cardiol 2012, 155(3):378-382.
143. Hoeper M.M., Markevych I., Spiekerkoetter E., et al. Goal-oriented treatment and combination therapy for pulmonary arterial hypertension. Eur Respir J 2005, 26:858-863.
144. Lunze K., Gilbert N., Mebus S., et al. First experience with an oral combination therapy using bosentan and sildenafil for pulmonary arterial hypertension. Eur J Clin Invest 2006, 36(Suppl 3):32-38.
145. McLaughlin V.V., Oudiz R.J., Frost A., et al. Randomized study of adding inhaled iloprost to existing bosentan in pulmonary arterial hypertension. Am J Respir Crit Care Med 2006, 174:1257-1263.
146. Okyay K., Cemri M., Boyac B., et al. Use of long-term combined therapy with inhaled iloprost and oral sildenafil in an adult patient with eisenmenger syndrome. Cardiol Rev 2005, 13:312-314.
147. 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.
148. Duffels M., van Loon L., Berger R., et al. Pulmonary arterial hypertension associated with a congenital heart defect: advanced medium-term medical treatment stabilizes clinical condition. Congenit Heart Dis 2007, 2:242-249.
149. 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.
150. Trulock E.P. Lung transplantation for primary pulmonary hypertension. Clin Chest Med 2001, 22:583-593.
151. Stoica S.C., McNeil K.D., Perreas K., et al. Heart-lung transplantation for Eisenmenger syndrome: early and long-term results. Ann Thorac Surg 2001, 72:1887-1891.
152. Waddell T.K., Bennett L., Kennedy R., et al. Heart-lung or lung transplantation for Eisenmenger syndrome. J Heart Lung Transplant 2002, 21:731-737.
153. Klepetko W., Mayer E., Sandoval J., et al. Interventional and surgical modalities of treatment for pulmonary arterial hypertension. J Am Coll Cardiol 2004, 43:73S-80S.
154. Dimopoulos K., Diller G.P., Koltsida E., et al. Prevalence, predictors, and prognostic value of renal dysfunction in adults with congenital heart disease. Circulation 2008, 117:2320-2328.
155. Dimopoulos K., Peset A., Gatzoulis M.A. Evaluating operability in adults with congenital heart disease and the role of pretreatment with targeted pulmonary arterial hypertension therapy. Int J Cardiol 2008, 129:163-171.
156. Rame J.E. Pulmonary hypertension complicating congenital heart disease. Curr Cardiol Rep 2009, 11:314-320.
157. 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.
158. Kidd L., Rose V., Collins G., et al. The hemodynamics in ventricular septal defect in childhood. Am Heart J 1965, 70:732-738.
159. Yeager S.B., Freed M.D., Keane J.F., et al. Primary surgical closure of ventricular septal defect in the first year of life: results in 128 infants. J Am Coll Cardiol 1984, 3:1269-1276.
160. Moller J.H., Patton C., Varco R.L., et al. Late results (30 to 35 years) after operative closure of isolated ventricular septal defect from 1954 to 1960. Am J Cardiol 1991, 68:1491-1497.
161. Neutze J.M., Ishikawa T., Clarkson P.M., et al. Assessment and follow-up of patients with ventricular septal defect and elevated pulmonary vascular resistance. Am J Cardiol 1989, 63:327-331.