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Review

How to evaluate patients with congenital heart disease-related pulmonary arterial hypertension

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Pages 11-18 | Received 08 Jun 2018, Accepted 14 Nov 2018, Published online: 06 Dec 2018

References

  • Diller GP, Kempny A, Alonso-Gonzalez R, et al. Survival prospects and circumstances of death in contemporary adult congenital heart disease patients under follow-up at a large tertiary centre. Circulation. 2015 Dec;132(22):2118–2125. PubMed PMID: 26369353.
  • Dimopoulos K, Wort SJ, Gatzoulis MA. Pulmonary hypertension related to congenital heart disease: a call for action. Eur Heart J. 2014 Mar;35(11):691–700. PubMed PMID: 24168793.
  • Mulder BJ. Changing demographics of pulmonary arterial hypertension in congenital heart disease. Eur Respir Rev. 2010 Mar;19(118):308–313. PubMed PMID: 21119189.
  • Engelfriet P, Boersma E, Oechslin E, et al. The spectrum of adult congenital heart disease in Europe: morbidity and mortality in a 5 year follow-up period. The Euro heart survey on adult congenital heart disease. Eur Heart J. 2005 Nov;26(21):2325–2333. PubMed PMID: 15996978.
  • van Riel AC, Schuuring MJ, van Hessen ID, et al. Contemporary prevalence of pulmonary arterial hypertension in adult congenital heart disease following the updated clinical classification. Int J Cardiol. 2014 Jun;174(2):299–305. PubMed PMID: 24794056.
  • Ntiloudi D, Giannakoulas G, Parcharidou D, et al. Adult congenital heart disease: a paradigm of epidemiological change. Int J Cardiol. 2016 Sep;218:269–274. PubMed PMID: 27240150.
  • Moons P, Bovijn L, Budts W, et al. Temporal trends in survival to adulthood among patients born with congenital heart disease from 1970 to 1992 in Belgium. Circulation. 2010 Nov;122(22):2264–2272. PubMed PMID: 21098444.
  • Marelli AJ, Mackie AS, Ionescu-Ittu R, et al. Congenital heart disease in the general population: changing prevalence and age distribution. Circulation. 2007 Jan;115(2):163–172. PubMed PMID: 17210844.
  • Khairy P, Ionescu-Ittu R, Mackie AS, et al. Changing mortality in congenital heart disease. J Am Coll Cardiol. 2010 Sep;56(14):1149–1157. PubMed PMID: 20863956.
  • Marelli AJ, Ionescu-Ittu R, Mackie AS, et al. Lifetime prevalence of congenital heart disease in the general population from 2000 to 2010. Circulation. 2014 Aug;130(9):749–756. PubMed PMID: 24944314.
  • Duffels MG, Engelfriet PM, Berger RM, et al. Pulmonary arterial hypertension in congenital heart disease: an epidemiologic perspective from a Dutch registry. Int J Cardiol. 2007 Aug;120(2):198–204. PubMed PMID: 17182132.
  • Bouzas B, Gatzoulis MA. [Pulmonary arterial hypertension in adults with congenital heart disease]. Rev Esp Cardiol. 2005 May;58(5):465–469. PubMed PMID: 15899189.
  • Giannakoulas G, Vasiliadis K, Frogoudaki A, et al. Adult congenital heart disease in Greece: preliminary data from the CHALLENGE registry. Int J Cardiol. 2017 Oct;245:109–113. PubMed PMID: 28743482.
  • Gatzoulis MA, Alonso-Gonzalez R, Beghetti M. Pulmonary arterial hypertension in paediatric and adult patients with congenital heart disease. Eur Respir Rev. 2009 Sep;18(113):154–161. PubMed PMID: 20956136.
  • Hjortshoj CS, Jensen AS, Sorensen K, et al. Epidemiological changes in Eisenmenger syndrome in the Nordic region in 1977–2012. Heart. 2017 Sep;103(17):1353–1358. PubMed PMID: 28450553.
  • Kempny A, Dimopoulos K, Gatzoulis MA. Declining incidence and prevalence of Eisenmenger syndrome in the developed world: a triumph of modern medicine. Heart. 2017 Sep;103(17):1313–1314. PubMed PMID: 28566473.
  • Van de Bruaene A, Delcroix M, Pasquet A, et al. The Belgian Eisenmenger syndrome registry: implications for treatment strategies? Acta Cardiol. 2009 Aug;64(4):447–453. PubMed PMID: 19725436.
  • Simonneau G, Galie N, Rubin LJ, et al. Clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2004 Jun 16;43(12 Suppl S):5s–12s. PubMed PMID: 15194173.
  • 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 Heart J. 2016 Jan;37(1):67–119. PubMed PMID: 26320113.
  • Diller GP, Gatzoulis MA. Pulmonary vascular disease in adults with congenital heart disease. Circulation. 2007 Feb;115(8):1039–1050. PubMed PMID: 17325254.
  • Beghetti M, Galie N. Eisenmenger syndrome a clinical perspective in a new therapeutic era of pulmonary arterial hypertension. J Am Coll Cardiol. 2009 Mar;53(9):733–740. PubMed PMID: 19245962.
  • Hopkins WE, Ochoa LL, Richardson GW, et al. Comparison of the hemodynamics and survival of adults with severe primary pulmonary hypertension or Eisenmenger syndrome. J Heart Lung Transplant. 1996 Jan;15(1 Pt 1):100–105. PubMed PMID: 8820089.
  • Diller GP, Kempny A, Inuzuka R, et al. Survival prospects of treatment naive patients with Eisenmenger: a systematic review of the literature and report of own experience. Heart. 2014 Sep;100(17):1366–1372. PubMed PMID: 25099652.
  • Giannakoulas G, Gatzoulis MA. Pulmonary arterial hypertension in congenital heart disease: current perspectives and future challenges. Hellenic J Cardiol. 2016 Aug;57(4):218–222. PubMed PMID: 27642135.
  • Barst RJ, Ivy DD, Foreman AJ, et al. Four- and seven-year outcomes of patients with congenital heart disease-associated pulmonary arterial hypertension (from the REVEAL Registry). Am J Cardiol. 2014 Jan;113(1):147–155. PubMed PMID: 24176071.
  • Lange RA, Brickner ME. Improving survival in patients with eisenmenger syndrome: are we any closer? Circulation. 2017 Apr;135(15):1441–1443. PubMed PMID: 28396379.
  • Engelfriet PM, Duffels MG, 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 Jun;93(6):682–687. PubMed PMID: 17164490.
  • Moceri P, Kempny A, Liodakis E, et al. Physiological differences between various types of Eisenmenger syndrome and relation to outcome. Int J Cardiol. 2015 Jan;179:455–460. PubMed PMID: 25465305.
  • Kempny A, Hjortshoj CS, Gu H, et al. Predictors of death in contemporary adult patients with Eisenmenger syndrome: a multicenter study. Circulation. 2017 Apr;135(15):1432–1440. PubMed PMID: 27979875.
  • Kempny A, Dimopoulos K, Alonso-Gonzalez R, et al. Six-minute walk test distance and resting oxygen saturations but not functional class predict outcome in adult patients with Eisenmenger syndrome. Int J Cardiol. 2013 Oct;168(5):4784–4789. PubMed PMID: 23962785.
  • Ntiloudi D, Apostolopoulou S, Vasiliadis K, et al. Hospitalisations for heart failure predict mortality in pulmonary hypertension related to congenital heart disease. Heart. 2018 Sept; pii: heartjnl-2018-313613. [Epub ahead of print]. PubMed PMID: 30269081. DOI:10.1136/heartjnl-2018-313613
  • Hjortshoj CMS, Kempny A, Jensen AS, et al. Past and current cause-specific mortality in Eisenmenger syndrome. Eur Heart J. 2017 Jul;38(26):2060–2067. PubMed PMID: 28430906.
  • 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. 2014 Mar;35(11):716–724. PubMed PMID: 23455361.
  • Dimopoulos K, Giannakoulas G, Wort SJ, 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 Nov;23(6):545–554. PubMed PMID: 18830068.
  • Dimopoulos K, Diller GP, Petraco R, et al. Hyponatraemia: a strong predictor of mortality in adults with congenital heart disease. Eur Heart J. 2010 Mar;31(5):595–601. PubMed PMID: 19933692.
  • Dimopoulos K, Diller GP, Koltsida E, et al. Prevalence, predictors, and prognostic value of renal dysfunction in adults with congenital heart disease. Circulation. 2008 May;117(18):2320–2328. PubMed PMID: 18443238.
  • Shiina Y, Toyoda T, Kawasoe Y, et al. The prevalence and risk factors for cholelithiasis and asymptomatic gallstones in adults with congenital heart disease. Int J Cardiol. 2011 Oct;152(2):171–176. PubMed PMID: 20675000.
  • Perloff JK. Systemic complications of cyanosis in adults with congenital heart disease. Hematologic derangements, renal function, and urate metabolism. Cardiol Clin. 1993 Nov;11(4):689–699. PubMed PMID: 8252568.
  • Oya H, Nagaya N, Satoh T, et al. Haemodynamic correlates and prognostic significance of serum uric acid in adult patients with Eisenmenger syndrome. Heart. 2000 Jul;84(1):53–58. PubMed PMID: 10862589.
  • Rosove MH, Perloff JK, Hocking WG, et al. Chronic hypoxaemia and decompensated erythrocytosis in cyanotic congenital heart disease. Lancet. 1986 Aug;2(8502):313–315. PubMed PMID: 2874330.
  • Tay EL, Peset A, Papaphylactou M, et al. Replacement therapy for iron deficiency improves exercise capacity and quality of life in patients with cyanotic congenital heart disease and/or the Eisenmenger syndrome. Int J Cardiol. 2011 Sep;151(3):307–312. PubMed PMID: 20580108.
  • Kaemmerer H, Fratz S, Braun SL, et al. Erythrocyte indexes, iron metabolism, and hyperhomocysteinemia in adults with cyanotic congenital cardiac disease. Am J Cardiol. 2004 Sep;94(6):825–828. PubMed PMID: 15374802.
  • Martin-Garcia AC, Arachchillage DR, Kempny A, et al. Platelet count and mean platelet volume predict outcome in adults with Eisenmenger syndrome. Heart. 2018 Jan;104(1):45–50. PubMed PMID: 28663364.
  • Dimopoulos K, Giannakoulas G, Bendayan I, et al. Cardiothoracic ratio from postero-anterior chest radiographs: a simple, reproducible and independent marker of disease severity and outcome in adults with congenital heart disease. Int J Cardiol. 2013 Jun;166(2):453–457. PubMed PMID: 22137450.
  • Diller GP, Dimopoulos K, Okonko D, et al. Exercise intolerance in adult congenital heart disease: comparative severity, correlates, and prognostic implication. Circulation. 2005 Aug;112(6):828–835. PubMed PMID: 16061735.
  • Dimopoulos K, Okonko DO, Diller GP, et al. Abnormal ventilatory response to exercise in adults with congenital heart disease relates to cyanosis and predicts survival. Circulation. 2006 Jun;113(24):2796–2802. PubMed PMID: 16769913.
  • Niedeggen A, Skobel E, Haager P, et al. Comparison of the 6-minute walk test with established parameters for assessment of cardiopulmonary capacity in adults with complex congenital cardiac disease. Cardiol Young. 2005 Aug;15(4):385–390. PubMed PMID: 16014186.
  • Vis JC, Thoonsen H, Duffels MG, et al. Six-minute walk test in patients with down syndrome: validity and reproducibility. Arch Phys Med Rehabil. 2009 Aug;90(8):1423–1427. PubMed PMID: 19651279.
  • Kempny A, Dimopoulos K, Uebing A, et al. Reference values for exercise limitations among adults with congenital heart disease. Relation to activities of daily life–single centre experience and review of published data. Eur Heart J. 2012 Jun;33(11):1386–1396. PubMed PMID: 22199119.
  • Inuzuka R, Diller GP, Borgia F, et al. Comprehensive use of cardiopulmonary exercise testing identifies adults with congenital heart disease at increased mortality risk in the medium term. Circulation. 2012 Jan;125(2):250–259. PubMed PMID: 22147905.
  • Yasue H, Yoshimura M, Sumida H, et al. Localization and mechanism of secretion of B-type natriuretic peptide in comparison with those of A-type natriuretic peptide in normal subjects and patients with heart failure. Circulation. 1994 Jul;90(1):195–203. PubMed PMID: 8025996.
  • Reardon LC, Williams RJ, Houser LS, et al. Usefulness of serum brain natriuretic peptide to predict adverse events in patients with the Eisenmenger syndrome. Am J Cardiol. 2012 Nov;110(10):1523–1526. PubMed PMID: 22863176.
  • Giannakoulas G, Mouratoglou SA, Gatzoulis MA, et al. Blood biomarkers and their potential role in pulmonary arterial hypertension associated with congenital heart disease. A systematic review. Int J Cardiol. 2014 Jul;174(3):618–623. PubMed PMID: 24814894.
  • Hopkins WE. The remarkable right ventricle of patients with Eisenmenger syndrome. Coron Artery Dis. 2005 Feb;16(1):19–25. PubMed PMID: 15654195.
  • Diller GP, 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 May;98(9):736–742. PubMed PMID: 22397941.
  • Schuijt MTU, Blok IM, Zwinderman AH, et al. Mortality in pulmonary arterial hypertension due to congenital heart disease: serial changes improve prognostication. Int J Cardiol. 2017 Sep;243:449–453. PubMed PMID: 28606655.
  • D’Alto M, Dimopoulos K, Budts W, et al. Multimodality imaging in congenital heart disease-related pulmonary arterial hypertension. Heart. 2016 Jun;102(12):910–918. PubMed PMID: 27013702.
  • D’Alto M, Romeo E, Argiento P, et al. Accuracy and precision of echocardiography versus right heart catheterization for the assessment of pulmonary hypertension. Int J Cardiol. 2013 Oct;168(4):4058–4062. PubMed PMID: 23890907.
  • D’Alto M, Romeo E, Argiento P, et al. Pulmonary arterial hypertension: the key role of echocardiography. Echocardiography. 2015 Jan;32(Suppl 1):S23–S37. PubMed PMID: 25244441.
  • Moceri P, Dimopoulos K, Liodakis E, et al. Echocardiographic predictors of outcome in eisenmenger syndrome. Circulation. 2012 Sept;126(12):1461–1468. PubMed PMID: 22899772.
  • Pirat B, McCulloch ML, Zoghbi WA. Evaluation of global and regional right ventricular systolic function in patients with pulmonary hypertension using a novel speckle tracking method. Am J Cardiol. 2006 Sep;98(5):699–704. PubMed PMID: 22899772.
  • Motoji Y, Tanaka H, Fukuda Y, et al. Efficacy of right ventricular free-wall longitudinal speckle-tracking strain for predicting long-term outcome in patients with pulmonary hypertension. Circ J. 2013 Mar;77(3):756–763. Pubmed PMID: 23220860.
  • Moceri P, Iriart X, Bouvier P, et al. Speckle-tracking imaging in patients with Eisenmenger syndrome. Arch Cardiovasc Dis. 2016 Feb;109(2):104–112. PubMed PMID: 26868160.
  • Moceri P, Bouvier P, Baudouy D, et al. Cardiac remodelling amongst adults with various aetiologies of pulmonary arterial hypertension including Eisenmenger syndrome-implications on survival and the role of right ventricular transverse strain. Eur Heart J Cardiovasc Imaging. 2016 Nov;18(11):1262–1270. PubMed PMID: 28011668.
  • Jensen AS, Broberg CS, Rydman R, et al. Impaired right, left, or biventricular function and resting oxygen saturation are associated with mortality in Eisenmenger syndrome: a clinical and cardiovascular magnetic resonance study. Circ Cardiovasc Imaging. 2015 Dec;8(12). PubMed PMID: 26659374.
  • Broberg CS, 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 Aug;50(7):634–642. PubMed PMID: 17692749.
  • Freed BH, Gomberg-Maitland M, Chandra S, et al. Late gadolinium enhancement cardiovascular magnetic resonance predicts clinical worsening in patients with pulmonary hypertension. J Cardiovasc Magn Reson. 2012 Feb;14:11. PubMed PMID: 22296860.
  • Broberg CS, Prasad SK, Carr C, et al. Myocardial fibrosis in Eisenmenger syndrome: a descriptive cohort study exploring associations of late gadolinium enhancement with clinical status and survival. J Cardiovasc Magn Reson. 2014 May;16:32. PubMed PMID: 24886403.
  • Devaraj A, Hansell DM. Computed tomography signs of pulmonary hypertension: old and new observations. Clin Radiol. 2009 Aug;64(8):751–760. PubMed PMID: 19589413.
  • Babu-Narayan SV, Giannakoulas G, Valente AM, et al. Imaging of congenital heart disease in adults. Eur Heart J. 2016 Apr;37(15):1182–1195. PubMed PMID: 26424866.
  • Simonneau G, Gatzoulis MA, Adatia I, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2013 Dec;62(25 Suppl):D34–D41. PubMed PMID: 24355639.
  • Kozlik-Feldmann R, Hansmann G, Bonnet D, et al. Pulmonary hypertension in children with congenital heart disease (PAH-CHD, PPHVD-CHD). Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European paediatric pulmonary vascular disease network, endorsed by ISHLT and DGPK. Heart. 2016 May;102(Suppl 2):ii42–ii48. PubMed PMID: 27053697.
  • Apitz C, Hansmann G, Schranz D. Hemodynamic assessment and acute pulmonary vasoreactivity testing in the evaluation of children with pulmonary vascular disease. Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European paediatric pulmonary vascular disease network, endorsed by ISHLT and DGPK. Heart. 2016 May;102(Suppl 2):ii23–ii29. PubMed PMID: 27053694.
  • Post MC, 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 Sep;25(18):1651–1656. PubMed PMID: 15351165.
  • 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;114(1):48–54. PubMed PMID: 16801459.
  • Gatzoulis MA, Beghetti M, Berger RM, et al. Evaluation of Macitentan in patients with Eisenmenger syndrome: results from the randomized, controlled MAESTRO study. Circulation. 2018. DOI:10.1161/CIRCULATIONAHA.118.033575
  • 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 PMID: 20202971.
  • 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 PMID: 21914136.

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