Eisenmenger syndrome and pulmonary arterial hypertension in adults with congenital heart disease

References

• Brickner ME, Hillis LD, Lange RA. Congenital heart disease in adults. First of two parts. N Eng J Med 2000;342:256–63
   Google Scholar
• Kidd L, Driscoll DJ, Gersony WM, et al. Second natural history study of congenital heart defects. Results of treatment of patients with ventricular septal defects. Circulation 1993;87 (2 Suppl):I38–51
   Google Scholar
• Eisenmenger V. Die angeboren Defekte der Kammerscheidewand des Herzens. Z Klin Med 1897;32:1–28
   Google Scholar
• Vongpatanasin W, Brickner ME, Hillis LD, Lange RA. The Eisenmenger syndrome in adults. Ann Intern Med 1998;128: 745–55
   PubMed Web of Science ®Google Scholar
• Steele PM, 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;76:1037–42
   PubMed Web of Science ®Google Scholar
• Rabinovitch M, Haworth SG, Castaneda AR, et al. Lung biopsy in congenital heart disease: a morphometric approach to pulmonary vascular disease. Circulation 1978;58:1107–122
   PubMed Web of Science ®Google Scholar
• Haworth SG. Pulmonary hypertension in the young. Heart 2002;88:658–64
   PubMed Web of Science ®Google Scholar
• Diller GP, Dimopoulos K, Okonko D, et al. Exercise intolerance in adult congenital heart disease: comparative severity, correlates, and prognostic implication. Circulation 2005;112:828–35
   PubMed Web of Science ®Google Scholar
• Simonneau G, Galie N, Rubin LJ, et al. Clinical classification of pulmonary hypertension. J Am Coll Cardiol 2004;43:5–12S
   Google Scholar
• Wood P. The Eisenmenger syndrome or pulmonary hypertension with reversed central shunt. Br Med J 1958;46:755–62
   Google Scholar
• Oechslin E. Eisenmenger’s syndrome. In: Gatzoulis M, Webb G, Daubeney P, eds. Diagnosis and Management of Adult Congenital Heart Disease. Philadelphia: Churchill Livingstone, 2003:363–78
   Google Scholar
• Galie N, Torbicki A, Barst R, et al. Guidelines on diagnosis and treatment of pulmonary arterial hypertension. The Task Force on Diagnosis and Treatment of Pulmonary Arterial Hypertension of the European Society of Cardiology. Eur Heart J 2004;25:2243–78
   Google Scholar
• Post MC, Janssens S, Van de Werf F, Budts W. 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;25:1651–6
   Google Scholar
• D’Alonzo GE, Barst RJ, Ayres SM, et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med 1991;115:343–9
   Google Scholar
• Daliento L, Sommerville J, Presbitero P, et al. Eisenmenger syndrome. Factors relating to deterioration and death. Eur Heart J 1998;19:1845–55
   PubMed Web of Science ®Google Scholar
• McLaughlin VV, Presberg KW, Doyle RL, et al. Prognosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest 2004;126:78–92S
   Google Scholar
• Hopkins WE, Ochoa LL, Richardson GW, Trulock EP. Comparison of the hemodynamics and survival of adults with severe primary pulmonary hypertension or Eisenmenger syndrome. J Heart Lung Transplant 1996;15:100–5
   PubMed Web of Science ®Google Scholar
• Diller G-P, Dimopoulos K, Broberg CS, et al. Presentation, survival prospects and predictors of death in Eisenmenger syndrome: a combined retrospective and case control study. Eur Heart J 2006; 27:1737–42
   Google Scholar
• Gibbs JSR, Broberg CS, Gatzoulis MA. Idiopathic pulmonary arterial hypertension: current state of play and new treatment modalities. Int J Card 2004;97(Suppl I):7–10
   Google Scholar
• Dimopoulos K, Okonko DO, Diller G-P, et al. Deranged exercise ventilation patterns in adult congenital heart disease: relationship to cyanosis and survival. Circulation 2006; 2006;113:2796–802
   Google Scholar
• Ammash N, Warnes CA. Cerebrovascular events in adult patients with cyanotic congenital heart disease. J Am Coll Cardiol 1996;28:768–72
   PubMed Web of Science ®Google Scholar
• Broberg CS, Bax BE, Rampling MW, et al. Blood viscosity and its relation to iron deficiency, symptoms, and exercise capacity in adults with cyanotic congenital heart disease. J Am Coll Cardiol 2006; 48:356–65
   PubMed Web of Science ®Google Scholar
• Perloff JK, Hart EM, Greaves SM, et al. Proximal pulmonary arterial and intrapulmonary radiologic features of Eisenmenger syndrome and primary pulmonary hypertension. Am J Cardiol 2003;92:182–7
   PubMed Web of Science ®Google Scholar
• Silversides CK, Granton JT, Konen E, et al. Pulmonary thrombosis in adults with Eisenmenger syndrome. J Am Coll Cardiol 2003;42:1982–7
   PubMed Web of Science ®Google Scholar
• 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
   Google Scholar
• Broberg C, Ujita M, Bouzas B, et al. Pulmonary arterial thrombosis in Eisenmenger syndrome is associated with ventricular dysfunction, neurohormonal activation, and poorer exercise capacity. J Am Coll Cardiol 2007 (in press)
   Google Scholar
• Rubens C, Ewert R, Halank M, et al. Big endothelin-1 and endothelin-1 plasma levels are correlated with the severity of primary pulmonary hypertension. Chest 2001;120:1562–9
   PubMed Web of Science ®Google Scholar
• Channick RN, Simonneau G, Sitbon O, et al. Effects of the dual endothelin-receptor antagonist bosentan in patients with pulmonary hypertension: a randomised placebocontrolled study. Lancet 2001;358:1119–23
   PubMed Web of Science ®Google Scholar
• Rubin LJ, Badesch DB, Barst RJ, et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med 2002;346: 896–903
   PubMed Web of Science ®Google Scholar
• Gatzoulis MA, Rogers P, Li W, et al. Safety and tolerability of bosentan in adults with Eisenmenger physiology. Int J Cardiol 2005;98:147–51
   PubMed Web of Science ®Google Scholar
• Schulze-Neick I, Gilbert N, Ewert R, et al. Bosentan therapy for pulmonary arterial hypertension (PAH) in adult patients with congenital heart disease: a multicenter cooperation. Am Heart J 2005;150:716e7–12
   Google Scholar
• Maiya S, Hislop AA, Flynn Y, Haworth SG. Response to bosentan in children with pulmonary hypertension. Heart 2006;92:664–70
   PubMed Web of Science ®Google Scholar
• Apostolopoulou SC, Rammos S, Kyriakides ZS, et al. Acute endothelin A receptor antagonism improves pulmonary and systemic haemodynamics in patients with pulmonary arterial hypertension that is primary or autoimmune and related to congenital heart disease. Heart 2003;89:1221–7
   PubMed Web of Science ®Google Scholar
• Galie N, Beghetti M, Gatzoulis MA, et al. on behalf of the BREATHE-5 Investigators: Bosentan therapy in patients with Eisenmenger syndrome: a multi-centre, double-blind randomised placebo-controlled study. Circulation 2006; 114:48–54
   PubMed Web of Science ®Google Scholar
• Hoeper MM, Maier R, Tongers J, et al. Determination of cardiac output by the Fick method, thermodilution, and acetylene rebreathing in pulmonary hypertension. Am J Respir Crit Care Med 1999;160:535–41
   PubMed Web of Science ®Google Scholar
• Kuehne T, Yilmaz S, Schulze-Neick I, et al. Magnetic resonance imaging guided catheterisation for assessment of pulmonary vascular resistance: in vivo validation and clinical application in patients with pulmonary hypertension. Heart 2005;91;1064–9
   Google Scholar
• Paciocco G, Martinez F, Bossone E, et al. Oxygen desaturation on the six-minute wall test and mortality in untreated primary pulmonary hypertension. Eur Respir J 2001;17:647–52
   PubMed Web of Science ®Google Scholar
• Galie N, Hinderliter AL, Torbicki A, et al. Effects of the oral endothelin-receptor antagonist bosentan on echocardiographic and Doppler measures in patients with pulmonary arterial hypertension. J Am Coll Cardiol 2003;41:1380–6
   PubMed Web of Science ®Google Scholar
• Diller G-P, Gatzoulis MA. Pulmonary vascular disease in adult congenital heart disease. Circulation 2007;115:1039–50
   Google Scholar
• Rosenzweig EB, Kerstein D, Barst RJ. Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects. Circulation 1999;99:1858–65
   Google Scholar
• Mikhail GW, Prasad SK, Li W, et al. Clinical and haemodynamic effects of sildenafil in pulmonary hypertension: acute and mid-term effects. Eur Heart J 2004;25:431–6
   PubMed Web of Science ®Google Scholar
• Humpl T, Reyes JT, 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;111:3274–80
   PubMed Web of Science ®Google Scholar
• Wilkins MR, Paul GP, Strange JW, et al. Sildenafil versus endothelin receptor antagonist for pulmonary hypertension (SERAPH) study. Am J Respir Crit Care Med 2005;171: 1292–7
   PubMed Web of Science ®Google Scholar
• Galiè N, Ghofrani HA, Torbicki A, et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 2005;353:2148–57
   Google Scholar
• Stoica SC, McNeil KD, Perreas K, et al. Heart-lung transplantation for Eisenmenger syndrome: early and long-term results. Ann Thorac Surg 2001;72:1887–91
   PubMed Web of Science ®Google Scholar
• Waddell TK, Bennett L, Kennedy R, et al. Heart-lung or lung transplantation for Eisenmenger syndrome. J Heart Lung Transplant 2002;21:731–7
   PubMed Web of Science ®Google Scholar
• Khambadkone S, Li J, de Leval MR, et al. Basal pulmonary vascular resistance and nitric oxide responsiveness late after Fontan-type operation. Circulation 2003;107: 3204–8
   PubMed Web of Science ®Google Scholar