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Eisenmenger syndrome

MedGen UID:
4479
•Concept ID:
C0013743
•
Disease or Syndrome
Synonyms: Complex, Eisenmenger; Complex, Eisenmenger's; Eisenmenger Complex; Eisenmenger Syndrome; Eisenmenger's Complex; Eisenmenger's Syndrome; Eisenmengers Complex; Eisenmengers Syndrome; Syndrome, Eisenmenger; Syndrome, Eisenmenger's
SNOMED CT: Eisenmenger complex (204311009); Eisenmenger syndrome (445928005); Eisenmenger's syndrome (445928005); Eisenmenger's complex (204311009)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0019944
Orphanet: ORPHA97214

Definition

A rare respiratory disease associated with unoperated congenital heart disease and characterized by congenital heart malformations with reversed or bi-directional shunting through an intra-cardiac or intervascular (usually aorto-pulmonary) communication with the development of PAH. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVEisenmenger syndrome

Professional guidelines

PubMed

Evaluation and Management of Pulmonary Arterial Hypertension in Congenital Heart Disease.
Arshad HB, Duarte VE
Methodist Debakey Cardiovasc J 2021;17(2):145-151. Epub 2021 Jul 1 doi: 10.14797/UFEJ2329. PMID: 34326934Free PMC Article
Macitentan in the treatment of pulmonary arterial hypertension.
Zebadúa R, Hernández-Pérez AP, García A, Zayas N, Sandoval J, López J, Pulido T
Future Cardiol 2021 Jan;17(1):49-58. Epub 2020 Jul 17 doi: 10.2217/fca-2020-0012. PMID: 32677463
Eisenmenger syndrome: diagnosis, prognosis and clinical management.
Arvanitaki A, Giannakoulas G, Baumgartner H, Lammers AE
Heart 2020 Nov;106(21):1638-1645. Epub 2020 Jul 20 doi: 10.1136/heartjnl-2020-316665. PMID: 32690623

Recent clinical studies

Etiology

Eisenmenger Syndrome: JACC State-of-the-Art Review.
Arvanitaki A, Gatzoulis MA, Opotowsky AR, Khairy P, Dimopoulos K, Diller GP, Giannakoulas G, Brida M, Griselli M, Grünig E, Montanaro C, Alexander PD, Ameduri R, Mulder BJM, D'Alto M
J Am Coll Cardiol 2022 Mar 29;79(12):1183-1198. doi: 10.1016/j.jacc.2022.01.022. PMID: 35331414
Preventing disease progression in Eisenmenger syndrome.
Barradas-Pires A, Constantine A, Dimopoulos K
Expert Rev Cardiovasc Ther 2021 Jun;19(6):501-518. Epub 2021 May 6 doi: 10.1080/14779072.2021.1917995. PMID: 33853494
Eisenmenger syndrome: diagnosis, prognosis and clinical management.
Arvanitaki A, Giannakoulas G, Baumgartner H, Lammers AE
Heart 2020 Nov;106(21):1638-1645. Epub 2020 Jul 20 doi: 10.1136/heartjnl-2020-316665. PMID: 32690623
Eisenmenger Syndrome: A Multisystem Disorder-Do Not Destabilize the Balanced but Fragile Physiology.
Chaix MA, Gatzoulis MA, Diller GP, Khairy P, Oechslin EN
Can J Cardiol 2019 Dec;35(12):1664-1674. Epub 2019 Oct 10 doi: 10.1016/j.cjca.2019.10.002. PMID: 31813503
Pregnancy and pulmonary hypertension.
Pieper PG, Lameijer H, Hoendermis ES
Best Pract Res Clin Obstet Gynaecol 2014 May;28(4):579-91. Epub 2014 Mar 14 doi: 10.1016/j.bpobgyn.2014.03.003. PMID: 24685319

Diagnosis

Eisenmenger Syndrome: JACC State-of-the-Art Review.
Arvanitaki A, Gatzoulis MA, Opotowsky AR, Khairy P, Dimopoulos K, Diller GP, Giannakoulas G, Brida M, Griselli M, Grünig E, Montanaro C, Alexander PD, Ameduri R, Mulder BJM, D'Alto M
J Am Coll Cardiol 2022 Mar 29;79(12):1183-1198. doi: 10.1016/j.jacc.2022.01.022. PMID: 35331414
Congenital Heart Disease-Associated Pulmonary Hypertension.
Rosenzweig EB, Krishnan U
Clin Chest Med 2021 Mar;42(1):9-18. Epub 2021 Jan 12 doi: 10.1016/j.ccm.2020.11.005. PMID: 33541620
Patent foramen ovale and atrial septal defect.
Kheiwa A, Hari P, Madabhushi P, Varadarajan P
Echocardiography 2020 Dec;37(12):2172-2184. doi: 10.1111/echo.14646. PMID: 33368546
Eisenmenger syndrome: diagnosis, prognosis and clinical management.
Arvanitaki A, Giannakoulas G, Baumgartner H, Lammers AE
Heart 2020 Nov;106(21):1638-1645. Epub 2020 Jul 20 doi: 10.1136/heartjnl-2020-316665. PMID: 32690623
Eisenmenger Syndrome in Pregnancy.
Yuan SM
Braz J Cardiovasc Surg 2016 Jul-Sep;31(4):325-329. doi: 10.5935/1678-9741.20160062. PMID: 27849306Free PMC Article

Therapy

Oxygen and pulmonary arterial hypertension: effects, mechanisms, and therapeutic benefits.
Green S, Stuart D
Eur J Prev Cardiol 2021 Mar 23;28(1):127-136. doi: 10.1093/eurjpc/zwaa001. PMID: 33623970
Macitentan in the treatment of pulmonary arterial hypertension.
Zebadúa R, Hernández-Pérez AP, García A, Zayas N, Sandoval J, López J, Pulido T
Future Cardiol 2021 Jan;17(1):49-58. Epub 2020 Jul 17 doi: 10.2217/fca-2020-0012. PMID: 32677463
Advanced Therapy in Eisenmenger Syndrome: A Systematic Review.
Hjortshøj CS, Jensen AS, Søndergaard L
Cardiol Rev 2017 May/Jun;25(3):126-132. doi: 10.1097/CRD.0000000000000107. PMID: 27054605
Pulmonary hypertension in pregnancy.
Martínez MV, Rutherford JD
Cardiol Rev 2013 Jul-Aug;21(4):167-73. doi: 10.1097/CRD.0b013e318275cf01. PMID: 23018670
Eisenmenger syndrome: not always inoperable.
Huang JB, Liang J, Zhou LY
Respir Care 2012 Sep;57(9):1488-95. Epub 2012 Feb 17 doi: 10.4187/respcare.01418. PMID: 22348268

Prognosis

Eisenmenger Syndrome: JACC State-of-the-Art Review.
Arvanitaki A, Gatzoulis MA, Opotowsky AR, Khairy P, Dimopoulos K, Diller GP, Giannakoulas G, Brida M, Griselli M, Grünig E, Montanaro C, Alexander PD, Ameduri R, Mulder BJM, D'Alto M
J Am Coll Cardiol 2022 Mar 29;79(12):1183-1198. doi: 10.1016/j.jacc.2022.01.022. PMID: 35331414
Eisenmenger syndrome: diagnosis, prognosis and clinical management.
Arvanitaki A, Giannakoulas G, Baumgartner H, Lammers AE
Heart 2020 Nov;106(21):1638-1645. Epub 2020 Jul 20 doi: 10.1136/heartjnl-2020-316665. PMID: 32690623
Congenital Heart Disease and Pulmonary Hypertension.
Constantine A, Dimopoulos K, Opotowsky AR
Cardiol Clin 2020 Aug;38(3):445-456. Epub 2020 Jun 8 doi: 10.1016/j.ccl.2020.04.008. PMID: 32622496
Eisenmenger Syndrome in Pregnancy.
Yuan SM
Braz J Cardiovasc Surg 2016 Jul-Sep;31(4):325-329. doi: 10.5935/1678-9741.20160062. PMID: 27849306Free PMC Article
Pregnancy and pulmonary hypertension.
Pieper PG, Lameijer H, Hoendermis ES
Best Pract Res Clin Obstet Gynaecol 2014 May;28(4):579-91. Epub 2014 Mar 14 doi: 10.1016/j.bpobgyn.2014.03.003. PMID: 24685319

Clinical prediction guides

Management and outcomes of pulmonary artery hypertension and Eisenmenger syndrome during pregnancy: a prospective observational cohort study.
Jha N, Divya MB, Jha AK
BJOG 2023 Sep;130(10):1258-1268. Epub 2023 Apr 11 doi: 10.1111/1471-0528.17474. PMID: 37039249
Evolving Indications for Heart-Lung Transplant in Spain.
López-Vilella R, Gómez Bueno M, González Vílchez F, Solé Jover A, Laporta Hernández R, Vicente Guillén R, González Román AI, Sánchez-Lázaro I, Hernández Pérez F, Sales Badía G, Córdoba Peláez MDM, Torregrosa Puerta S, Forteza Gil A, Donoso Trenado V, Martínez Dolz L, Segovia Cubero J, Almenar Bonet L
Transplant Proc 2022 Nov;54(9):2500-2502. Epub 2022 Oct 29 doi: 10.1016/j.transproceed.2022.10.003. PMID: 36319494
Eisenmenger syndrome: diagnosis, prognosis and clinical management.
Arvanitaki A, Giannakoulas G, Baumgartner H, Lammers AE
Heart 2020 Nov;106(21):1638-1645. Epub 2020 Jul 20 doi: 10.1136/heartjnl-2020-316665. PMID: 32690623
RV pressure overload: from hypertrophy to failure.
van der Bruggen CEE, Tedford RJ, Handoko ML, van der Velden J, de Man FS
Cardiovasc Res 2017 Oct 1;113(12):1423-1432. doi: 10.1093/cvr/cvx145. PMID: 28957530
Pregnancy and Adult Congenital Heart Disease.
Bhatt AB, DeFaria Yeh D
Cardiol Clin 2015 Nov;33(4):611-23, ix. Epub 2015 Aug 30 doi: 10.1016/j.ccl.2015.07.008. PMID: 26471824

Recent systematic reviews

What is the position of pulmonary arterial hypertension-specific drug therapy in patients with Eisenmenger syndrome: A systematic review and meta-analysis.
Li Q, Kuang HY, Wu YH, Lu TW, Yi QJ
Medicine (Baltimore) 2019 May;98(20):e15632. doi: 10.1097/MD.0000000000015632. PMID: 31096477Free PMC Article
The Effect of Endothelin Receptor Antagonists in Patients with Eisenmenger Syndrome: A Systematic Review.
Elshafay A, Truong DH, AboElnas MM, Idrees H, Metwali HG, Vuong NL, Saad OA, Hirayama K, Huy NT
Am J Cardiovasc Drugs 2018 Apr;18(2):93-102. doi: 10.1007/s40256-017-0240-5. PMID: 28660556
Advanced Therapy in Eisenmenger Syndrome: A Systematic Review.
Hjortshøj CS, Jensen AS, Søndergaard L
Cardiol Rev 2017 May/Jun;25(3):126-132. doi: 10.1097/CRD.0000000000000107. PMID: 27054605
Survival prospects of treatment naïve patients with Eisenmenger: a systematic review of the literature and report of own experience.
Diller GP, Kempny A, Inuzuka R, Radke R, Wort SJ, Baumgartner H, Gatzoulis MA, Dimopoulos K
Heart 2014 Sep;100(17):1366-72. doi: 10.1136/heartjnl-2014-305690. PMID: 25099652
Pregnancy and pulmonary hypertension.
Pieper PG, Lameijer H, Hoendermis ES
Best Pract Res Clin Obstet Gynaecol 2014 May;28(4):579-91. Epub 2014 Mar 14 doi: 10.1016/j.bpobgyn.2014.03.003. PMID: 24685319

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