The use of pulmonary arterial hypertension therapies in Eisenmenger syndrome

References

• Arvanitaki A, Giannakoulas G, Baumgartner H, et al., Eisenmenger syndrome: diagnosis, prognosis and clinical management. Heart. 106(21): 1638–1645. 2020.
   PubMed Web of Science ®Google Scholar
• Dimopoulos K, Inuzuka R, Goletto S, et al. Improved survival among patients with Eisenmenger syndrome receiving advanced therapy for pulmonary arterial hypertension. Circulation. 2010 Jan 5;121(1):20–25.
   PubMed Web of Science ®Google Scholar
• Diller GP, Korten MA, Bauer UM, et al. Current therapy and outcome of Eisenmenger syndrome: data of the German National Register for congenital heart defects. Eur Heart J. 2016 May 7;37(18):1449–1455.
   PubMed Web of Science ®Google Scholar
• Hascoet S, Fournier E, Jais X, et al. Outcome of adults with Eisenmenger syndrome treated with drugs specific to pulmonary arterial hypertension: a French multicentre study. Arch Cardiovasc Dis. 2017 May;110(5):303–316.
   PubMed Web of Science ®Google Scholar
• Heath D, Edwards JE. 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 Oct;18(4 Part 1):533–547.
   PubMed Web of Science ®Google Scholar
• Kempny A, Hjortshoj CS, Gu H, et al. Predictors of Death in Contemporary Adult Patients With Eisenmenger Syndrome: a Multicenter Study. Circulation. 2017 Apr 11;135(15):1432–1440.
   PubMed Web of Science ®Google Scholar
• Sanz J, Sanchez-Quintana D, Bossone E, et al. Anatomy, Function, and Dysfunction of the Right Ventricle: JACC State-of-the-Art Review. J Am Coll Cardiol. 2019 Apr 2;73(12):1463–1482.
   PubMed Web of Science ®Google Scholar
• Cacoub P, Dorent R, Maistre G, et al. Endothelin-1 in primary pulmonary hypertension and the Eisenmenger syndrome. Am J Cardiol. 1993 Feb 15;71(5):448–450.
   PubMed Web of Science ®Google Scholar
• 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 Jul 4;114(1):48–54.
   PubMed Web of Science ®Google Scholar
• Berger RM, 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 Oct 29;144(3):373–378.
   PubMed Web of Science ®Google Scholar
• Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: executive Summary: a Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol. 2019 Apr 2;73(12):1494–1563.
   PubMed Web of Science ®Google Scholar
• Baumgartner H, De Backer J, Babu-Narayan SV, et al. 2020 ESC Guidelines for the management of adult congenital heart disease. Eur Heart J. 2020 Aug 29;41(43):4153–4154.
   PubMed Web of Science ®Google Scholar
• Hjortshøj CS, Jensen AS, Søndergaard L. Advanced Therapy in Eisenmenger Syndrome. Cardiol Rev. 2017;25(3):126–132.
   PubMed Web of Science ®Google Scholar
• Vis JC, Duffels MG, 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. 2013 Mar 20;164(1):64–69.
   PubMed Web of Science ®Google Scholar
• Duffels MGJ, Vis JC, van Loon RLE, et al. Effect of Bosentan on Exercise Capacity and Quality of Life in Adults With Pulmonary Arterial Hypertension Associated With Congenital Heart Disease With and Without Down’s Syndrome. Am J Cardiol. 2009 May 1;103(9):1309–1315.
   PubMed Web of Science ®Google Scholar
• Diller GP, Dimopoulos K, Kaya MG, et al. Long-term safety, tolerability and efficacy of bosentan in adults with pulmonary arterial hypertension associated with congenital heart disease. Heart. 2007 Aug;93(8):974–976.
   PubMed Web of Science ®Google Scholar
• Gatzoulis MA, Landzberg M, Beghetti M, et al. Evaluation of Macitentan in Patients With Eisenmenger Syndrome. Circulation. 2019 Jan 2;139(1):51–63.
   PubMed Web of Science ®Google Scholar
• Arvind B, Relan J, Kothari SS. “Treat and repair” strategy for shunt lesions: a critical review. Pulm Circ. 2020 Apr-Jun;10(2):2045894020917885.
   Web of Science ®Google Scholar
• Zuckerman WA, Leaderer D, Rowan CA, et al. Ambrisentan for pulmonary arterial hypertension due to congenital heart disease. Am J Cardiol. 2011 May 1;107(9):1381–1385.
   PubMed Web of Science ®Google Scholar
• Zhao Q, Guo N, Chen J, et al. Comparative assessment of efficacy and safety of ambrisentan and bosentan in patients with pulmonary arterial hypertension: a meta‐analysis. J Clin Pharm Ther. 2021. DOI:https://doi.org/10.1111/jcpt.13481.
   Google Scholar
• Cary SPL, Winger JA, Marletta MA. Tonic and acute nitric oxide signaling through soluble guanylate cyclase is mediated by nonheme nitric oxide, ATP, and GTP. Proc Nat Acad Sci. 2005;102(37):13064–13069.
   PubMed Web of Science ®Google Scholar
• Klinger JR, Kadowitz PJ. The Nitric Oxide Pathway in Pulmonary Vascular Disease. Am J Cardiol. 2017;120(8):S71–S79.
   PubMedGoogle Scholar
• Chau EM, Fan KY, Chow WH. Effects of chronic sildenafil in patients with Eisenmenger syndrome versus idiopathic pulmonary arterial hypertension. Int J Cardiol. 2007 Sep 3;120(3):301–305.
   PubMed Web of Science ®Google Scholar
• Zhang ZN, Jiang X, Zhang R, et al. Oral sildenafil treatment for Eisenmenger syndrome: a prospective, open-label, multicentre study. Heart. 2011 Nov;97(22):1876–1881.
   PubMed Web of Science ®Google Scholar
• Singh TP, 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 Apr;151(4):851 e1–5.
   Web of Science ®Google Scholar
• Sun Y-J, Yang T, Zeng W-J, et al. Impact of Sildenafil on Survival of Patients With Eisenmenger Syndrome. J Clin Pharmacol. 2013;53(6):611–618.
   PubMed Web of Science ®Google Scholar
• 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 Sep-Oct;6(5):424–431.
   PubMed Web of Science ®Google Scholar
• Papamichalis M, Xanthopoulos A, Papamichalis P, et al. Adult congenital heart disease with pulmonary arterial hypertension: mechanisms and management. Heart Fail Rev. 2019;25(5):773–794.
   Web of Science ®Google Scholar
• Rosenkranz S, Ghofrani HA, Beghetti M, et al. Riociguat for pulmonary arterial hypertension associated with congenital heart disease. Heart. 2015 Nov;101(22):1792–1799.
   PubMed Web of Science ®Google Scholar
• Del Pozo R, Hernandez Gonzalez I, Escribano-Subias P. The prostacyclin pathway in pulmonary arterial hypertension: a clinical review. Expert Rev Respir Med. 2017;11(6):491–503.
   PubMed Web of Science ®Google Scholar
• Thomas IC, Glassner-Kolmin C, Gomberg-Maitland M. Long-term effects of continuous prostacyclin therapy in adults with pulmonary hypertension associated with congenital heart disease. Int J Cardiol. 2013 Oct 9;168(4):4117–4121.
   PubMed Web of Science ®Google Scholar
• Nashat H, Kempny A, Harries C, et al. A single-centre, placebo-controlled, double-blind randomised cross-over study of nebulised iloprost in patients with Eisenmenger syndrome: a pilot study. Int J Cardiol. 2020 Jan 15;299:131–135.
   PubMed Web of Science ®Google Scholar
• Yang SI, Chung WJ, Jung SH, et al. Effects of Inhaled Iloprost on Congenital Heart Disease With Eisenmenger Syndrome. Pediatr Cardiol. 2012;33(5):744–748.
   PubMed Web of Science ®Google Scholar
• 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(5):632–635.
   PubMed Web of Science ®Google Scholar
• Rosenzweig EB, Kerstein D, Barst RJ. Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects. Circulation. 1999 Apr 13;99(14):1858–1865.
   PubMed Web of Science ®Google Scholar
• Cha KS, Cho KI, Seo JS, 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 Dec 1;112(11):1834–1839.
   PubMed Web of Science ®Google Scholar
• Sitbon O, Channick R, Chin KM, et al. Selexipag for the Treatment of Pulmonary Arterial Hypertension. N Engl J Med. 2015 Dec 24;373(26):2522–2533.
   PubMed Web of Science ®Google Scholar
• Apostolopoulou SC, Karyofyllis P, Demerouti EA. Use of the Prostacyclin-Receptor Agonist Selexipag in Pulmonary Arterial Hypertension Associated with Eisenmenger Syndrome. Can J Cardiol. 2021 Feb 1;37(8):1286–1288.
   PubMed Web of Science ®Google Scholar
• Galie N, Channick RN, Frantz RP, et al. Risk stratification and medical therapy of pulmonary arterial hypertension. Eur Respir J. 2019 Jan;53(1):1801889.
   PubMed Web of Science ®Google Scholar
• Iversen K, Jensen AS, Jensen TV, et al. Combination therapy with bosentan and sildenafil in Eisenmenger syndrome: a randomized, placebo-controlled, double-blinded trial. Eur Heart J. 2010 May;31(9):1124–1131.
   PubMed Web of Science ®Google Scholar
• 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 Mar 22;155(3):378–382.
   PubMed Web of Science ®Google Scholar
• D’Alto M, Constantine A, Balint OH, et al. The effects of parenteral prostacyclin therapy as add-on treatment to oral compounds in Eisenmenger syndrome. Eur Respir J. 2019 Nov;54(5). DOI:https://doi.org/10.1183/13993003.01401-2019.
   Google Scholar
• Diller GP, Alonso-Gonzalez R, Dimopoulos K, et al. Disease targeting therapies in patients with Eisenmenger syndrome: response to treatment and long-term efficiency. Int J Cardiol. 2013 Aug 10;167(3):840–847.
   PubMed Web of Science ®Google Scholar
• Pradegan N, Pesce R, Castaldi B, et al. Reversing Inoperability in Eisenmenger Syndrome: the “Drug-and-Banding. World J Pediatric. 2020 Sep;11(5):646–648.
   Google Scholar
• Manuel L, Freeman L, Nashef SA. Surgery for Eisenmenger syndrome: time for a rethink? J R Soc Med. 2019 Dec;112(12):512–513.
   PubMed Web of Science ®Google Scholar
• Kempny A, Hjortshøj CS, Søndergaard L, et al. Mortality in adult patients with Eisenmenger Syndrome: 5-Years perspective. Int J Cardiol Congenital Heart Dis. 2021; 2: 100070.
   Google Scholar
• Chambers DC, Cherikh WS, Harhay MO, et al. The International Thoracic Organ Transplant Registry of the International Society for Heart and Lung Transplantation: thirty-sixth adult lung and heart-lung transplantation Report-2019; Focus theme: donor and recipient size match. J Heart Lung Transplant. 2019 Oct;38(10):1042–1055.
   PubMedGoogle Scholar
• Kearney K, Lau EM, Darley D, et al. Waitlist and post-transplant outcomes for eisenmenger syndrome: a comparison of transplant strategies. J Heart Lung Transplant. 2021 Aug;40(8):841–849.
   PubMed Web of Science ®Google Scholar
• Hascoet S, Pontailler M, Le Pavec J, et al. Transplantation for pulmonary arterial hypertension with congenital heart disease: impact on outcomes of the current therapeutic approach including a high-priority allocation program. Am J Transplant. 2021 Oct;21(10):3388–3400.
   PubMed Web of Science ®Google Scholar
• Hjortshoj CS, Gilljam T, Dellgren G, et al. Outcome after heart-lung or lung transplantation in patients with Eisenmenger syndrome. Heart. 2020 Jan;106(2):127–132.
   PubMed Web of Science ®Google Scholar
• Sertic F, Han J, Diagne D, et al. Not All Septal Defects Are Equal: outcomes of Bilateral Lung Transplant With Cardiac Defect Repair vs Combined Heart-Lung Transplant in Patients With Eisenmenger Syndrome in the United States. Chest. 2020 Nov;158(5):2097–2106.
   PubMed Web of Science ®Google Scholar
• Galie N, Humbert M, Vachiery JL, 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 Respir J. 2015 Oct;46(4):903–975.
   PubMed Web of Science ®Google Scholar
• Hoeper MM, Kramer T, Pan Z, et al. Mortality in pulmonary arterial hypertension: prediction by the 2015 European pulmonary hypertension guidelines risk stratification model. Eur Respir J. 2017 Aug;50(2). DOI:https://doi.org/10.1183/13993003.00740-2017.
   Web of Science ®Google Scholar
• Kylhammar D, Kjellstrom B, Hjalmarsson C, et al. A comprehensive risk stratification at early follow-up determines prognosis in pulmonary arterial hypertension. Eur Heart J. 2018 Dec 14;39(47):4175–4181.
   PubMed Web of Science ®Google Scholar
• Benza RL, Gomberg-Maitland M, Elliott CG, et al. Predicting Survival in Patients With Pulmonary Arterial Hypertension: the REVEAL Risk Score Calculator 2.0 and Comparison With ESC/ERS-Based Risk Assessment Strategies. Chest. 2019 Aug;156(2):323–337.
   PubMed Web of Science ®Google Scholar
• Moceri P, Dimopoulos K, Liodakis E, et al. Echocardiographic predictors of outcome in eisenmenger syndrome. Circulation. 2012 Sep 18;126(12):1461–1468.
   PubMed Web of Science ®Google Scholar
• D’Alto M, Diller G-P. Pulmonary hypertension in adults with congenital heart disease and Eisenmenger syndrome: current advanced management strategies. Heart. 2014 Sep;100(17):1322–1328.
   PubMed Web of Science ®Google Scholar
• Van De Bruaene A, De Meester P, Voigt JU, et al. Worsening in oxygen saturation and exercise capacity predict adverse outcome in patients with Eisenmenger syndrome. Int J Cardiol. 2013 Sep 30;168(2):1386–1392.
   PubMed Web of Science ®Google Scholar
• Arvanitaki A, Ntiloudi D, Giannakoulas G, et al. Prediction Models and Scores in Adult Congenital Heart Disease. Curr Pharm Des. 2021 Jan 11;27(10):1232–1244.
   PubMed Web of Science ®Google Scholar
• Kempny A, Fraisse A, Dimopoulos K. Risk stratification in congenital heart disease - A call for protocolised assessment and multicentre collaboration. Int J Cardiol. 2019 Feb 1;276:114–115.
   PubMed Web of Science ®Google Scholar
• Rubinstein I. Big Data: the End of Privacy or a New Beginning? SSRN Electron J. 2012. DOI:https://doi.org/10.2139/ssrn.2157659
   Google Scholar
• Orwat S, Arvanitaki A, Diller G-P. A new approach to modelling in adult congenital heart disease: artificial intelligence. Revista Española de Cardiología. 2021;74(7):573–575.
   PubMed Web of Science ®Google Scholar
• Diller GP, Kempny A, Babu-Narayan SV, et al. Machine learning algorithms estimating prognosis and guiding therapy in adult congenital heart disease: data from a single tertiary centre including 10 019 patients. Eur Heart J. 2019 Apr 1;40(13):1069–1077.
   PubMed Web of Science ®Google Scholar
• Marelli A. The Future of Adult Congenital Heart Disease Research: precision Health Services Delivery for the Next Decade. Can J Cardiol. 2019 Dec;35(12):1609–1619.
   PubMed Web of Science ®Google Scholar
• Diller GP, Arvanitaki A, Opotowsky AR, et al. Lifespan Perspective on Congenital Heart Disease Research: JACC State-of-the-Art Review. J Am Coll Cardiol. 2021 May 4;77(17):2219–2235.
   PubMed Web of Science ®Google Scholar
• Sommer N, Ghofrani HA, Pak O, et al. Current and future treatments of pulmonary arterial hypertension. Br J Pharmacol. 2020;1:548.
   Google Scholar
• Wirtz VJ, Kaplan WA, Kwan GF, et al. Access to Medications for Cardiovascular Diseases in Low- and Middle-Income Countries. Circulation. 2016 May 24;133(21):2076–2085.
   PubMed Web of Science ®Google Scholar