Eisenmenger’s syndrome natural history
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Abdelrahman Ibrahim Abushouk, MD[2]
Overview
Eisenmenger's syndrome passes through different stages, including the causative left-to-right shunt, development of pulmonary hypertension, polycythemia, and shunt reversal. The complications of Eisenmenger's syndrome include intracranial hemorrhage, stroke, congestive heart failure, angina pectoris, hyperviscosity syndrome, infection (cerebral abscess), renal failure, and sudden death. How well the infant or child does depends onwhether another medical condition is present and the age at which high blood pressure develops in the lungs. Patients with Eisenmenger's syndrome can live 20 to 50 years.
Natural History
Patients with Eisenmenger's syndrome pass through the following stages[1]:
- An underlying heart defect that initially allows a left-to-right shunt between the left and right sides of the heart.
- The development of pulmonary hypertension.
- Polycythemia, an increase in the number of red blood cells.
- Finally, a reversal of the left-to-right shunt so that there is a right-to-left shunt.
- At the final stage, the patient is liable to the complications mentioned below.
Therefore, they may present with a history of symptoms for each of these stages.
Complications
The complications of Eisenmenger's syndrome include[2][3]:
- Intracranial hemorrhage
- Stroke
- Congestive heart failure
- Angina Pectoris
- Hyperviscosity syndrome
- Infection (cerebral abscess)
- Renal failure
- High risk pregnancy and fetal loss.
- Sudden death
Prognosis
- How well the infant or child does depends on[4]:
- whether another medical condition is present
- the age at which high blood pressure develops in the lungs.
- Patients with Eisenmenger's syndrome can live 20 to 50 years.
- The predictors of mortality in Eisenmenger's syndrome include[5]:
- Age
- Pretricuspid shunt
- Oxygen saturation at rest
- Presence of sinus rhythm
- Presence of pericardial effusion
References
- ↑ Vongpatanasin W, Brickner ME, Hillis LD, Lange RA (1998). "The Eisenmenger syndrome in adults". Ann Intern Med. 128 (9): 745–55. doi:10.7326/0003-4819-128-9-199805010-00008. PMID 9556469.
- ↑ Vongpatanasin W, Brickner ME, Hillis LD, Lange RA (1998). "The Eisenmenger syndrome in adults". Ann Intern Med. 128 (9): 745–55. doi:10.7326/0003-4819-128-9-199805010-00008. PMID 9556469.
- ↑ Katsurahgi S, Kamiya C, Yamanaka K, Neki R, Miyoshi T, Iwanaga N; et al. (2019). "Maternal and fetal outcomes in pregnancy complicated with Eisenmenger syndrome". Taiwan J Obstet Gynecol. 58 (2): 183–187. doi:10.1016/j.tjog.2019.01.002. PMID 30910135.
- ↑ Hjortshøj CMS, Kempny A, Jensen AS, Sørensen K, Nagy E, Dellborg M; et al. (2017). "Past and current cause-specific mortality in Eisenmenger syndrome". Eur Heart J. 38 (26): 2060–2067. doi:10.1093/eurheartj/ehx201. PMID 28430906.
- ↑ Kempny A, Hjortshøj CS, Gu H, Li W, Opotowsky AR, Landzberg MJ; et al. (2017). "Predictors of Death in Contemporary Adult Patients With Eisenmenger Syndrome: A Multicenter Study". Circulation. 135 (15): 1432–1440. doi:10.1161/CIRCULATIONAHA.116.023033. PMID 27979875.