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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

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
Review of evidence for bosentan therapy for treatment of Eisenmenger syndrome.
Hartwig BJ, Schultze B
J Am Assoc Nurse Pract 2019 Jan;31(1):72-77. doi: 10.1097/JXX.0000000000000104. PMID: 30475252

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
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
Evaluation of Macitentan in Patients With Eisenmenger Syndrome.
Gatzoulis MA, Landzberg M, Beghetti M, Berger RM, Efficace M, Gesang S, He J, Papadakis K, Pulido T, Galiè N; MAESTRO Study Investigators
Circulation 2019 Jan 2;139(1):51-63. doi: 10.1161/CIRCULATIONAHA.118.033575. PMID: 30586694Free PMC Article
Pulmonary arterial hypertension in adult congenital heart disease.
Brida M, Gatzoulis MA
Heart 2018 Oct;104(19):1568-1574. Epub 2018 May 2 doi: 10.1136/heartjnl-2017-312106. PMID: 29720395

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
Pulmonary arterial hypertension in adult congenital heart disease.
Brida M, Gatzoulis MA
Heart 2018 Oct;104(19):1568-1574. Epub 2018 May 2 doi: 10.1136/heartjnl-2017-312106. PMID: 29720395

Therapy

Evaluation of Macitentan in Patients With Eisenmenger Syndrome.
Gatzoulis MA, Landzberg M, Beghetti M, Berger RM, Efficace M, Gesang S, He J, Papadakis K, Pulido T, Galiè N; MAESTRO Study Investigators
Circulation 2019 Jan 2;139(1):51-63. doi: 10.1161/CIRCULATIONAHA.118.033575. PMID: 30586694Free PMC Article
Review of evidence for bosentan therapy for treatment of Eisenmenger syndrome.
Hartwig BJ, Schultze B
J Am Assoc Nurse Pract 2019 Jan;31(1):72-77. doi: 10.1097/JXX.0000000000000104. PMID: 30475252
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
Pulmonary arterial hypertension in adult congenital heart disease.
Brida M, Gatzoulis MA
Heart 2018 Oct;104(19):1568-1574. Epub 2018 May 2 doi: 10.1136/heartjnl-2017-312106. PMID: 29720395
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

Clinical prediction guides

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
Waitlist and post-transplant outcomes for eisenmenger syndrome: A comparison of transplant strategies.
Kearney K, Lau EM, Darley D, Romfh A, Bart N, Kotlyar E, Hayes D Jr, Khush K, Keogh A
J Heart Lung Transplant 2021 Aug;40(8):841-849. Epub 2021 Apr 21 doi: 10.1016/j.healun.2021.04.005. PMID: 34112578
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
Eisenmenger syndrome a clinical perspective in a new therapeutic era of pulmonary arterial hypertension.
Beghetti M, Galiè N
J Am Coll Cardiol 2009 Mar 3;53(9):733-40. doi: 10.1016/j.jacc.2008.11.025. PMID: 19245962

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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