Eisenmenger syndrome
Shunt reversal from pulmonary vascular disease, recognising it, the multisystem effects of cyanosis, what not to do (closure, venesection by numbers, pregnancy) and PAH therapy.
Tier 2Condition
Written with AI assistance and fact-checked against the sources below. For exam study, not clinical decisions. How notes are made
At a glance
- An unrepaired large shunt (VSD, PDA, AVSD, less often ASD) exposes the pulmonary bed to high flow and pressure. Pulmonary arterial hypertension develops, PVR rises to systemic levels and the shunt reverses.
- It is the extreme end of PAH associated with congenital heart disease, group 1 in the pulmonary hypertension classification.
- Now uncommon in Australian-born adults because shunts are closed in infancy. Expect it in migrants from places with limited paediatric cardiac surgery and in adults with Down syndrome (AVSD; see Down syndrome).
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Sources
- Nicolae M, Gentles T, Strange G et al. Adult congenital heart disease in Australia and New Zealand: a call for optimal care. Heart Lung Circ 2019;28(4):521-529. doi:10.1016/j.hlc.2018.10.015AU/NZCardiac Society of Australia and New Zealand (Paediatric and Congenital Council), 2019
- 2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for the Management of Adults With Congenital Heart Disease. J Am Coll Cardiol 2026;87(7):822-976. doi:10.1016/j.jacc.2025.09.006American College of Cardiology and American Heart Association, 2025 (published February 2026)
- Baumgartner H, De Backer J, Babu-Narayan SV et al. 2020 ESC Guidelines for the management of adult congenital heart disease. Eur Heart J 2021;42:563-645. doi:10.1093/eurheartj/ehaa554European Society of Cardiology, 2020
- De Backer J, Haugaa KH, Hasselberg NE et al. 2025 ESC Guidelines for the management of cardiovascular disease and pregnancy. Eur Heart J 2025;46(43):4462-4568. doi:10.1093/eurheartj/ehaf193European Society of Cardiology, 2025
- Humbert M, Kovacs G, Hoeper MM et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J 2022;43:3618-3731European Society of Cardiology and European Respiratory Society, 2022
- Galiè N, Beghetti M, Gatzoulis MA et al. Bosentan therapy in patients with Eisenmenger syndrome (BREATHE-5). Circulation 2006;114:48-54Circulation, 2006
- Last updated
- 14 November 2026
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