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Eisenmenger syndrome
Dr Aditya Shetty and Dr Gagandeep Singh et al.
Eisenmenger syndrome is a complication of an uncorrected high-flow, high-pressure congenital heart anomaly leading
to chronic pulmonary arterial hypertension and shunt reversal.
Epidemiology
In general the shunts that lead to Eisenmenger syndrome share two features
3
:
high flow
high pressures
As such the three lesions that account for most cases are:
ventricular septal defect (VSD)
most common
Eisenmenger syndrome in the setting of a VSD is refered to as Eisenmenger complex
5
atrioventricular septal defect (AVSD)
patent ductus arteriosus (PDA)
In this group of patients, gender distribution is equal, and it uncommon to develop Eisenmenger syndrome before the
age of 2
4-5
.
In contrast, atrial septal defects (ASD) which are commonly not apparent until adulthood, are generally well tolerated
due to the low pressures involved. In this setting Eisenmenger syndrome is uncommon, but when it does occur is more
common in females
3-4
.
Pathology
Exposure of the right heart and pulmonary circulation to increased flow and pressure results in changes including
5
:
13/7/2014 Eisenmenger syndrome | Radiology Reference Article | Radiopaedia.org
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arteriolar medial hypertrophy
intimal proliferation and fibrosis, and eventual
capillary and arteriolar occlusion due to development of pelxiform lesions and necrotising arteritis.
Concurrently, and in response to increase vascular resistance in the pulmonary vascular bed, the right ventricle
hypertrophies, to generate ever increasing pressures. Eventually a critical balance is reached at which point the pressures
generated on the right are higher than those on the left, and the shunt reverses, with de-oxygenated blood passing into
the systemic circulation and resulting in cyanosis.
Clinical presentation
In general by the time true Eisenmenger syndrome develops patients are already symptomatic with easy fatigue,
dyspnoea, chest pain and syncope being common
5
. With shunt reversal, patients become cyanotic and gradually there
is a compensatory increase in haematocrit (erythrocytosis).
Haemoptysis may develop due to rupture of small pulmonary arteriolar aneurysms.
Radiographic features
No specific imaging findings can distinguish between a patient with severe pulmonary arterial hypertension and one with
superimposed Eisenmenger syndrome. As such the findings are those of pulmonary arterial hypertension.
Cardiac imaging is useful in assessing for the presence of an intra-cardiac shunt.
Treatment and prognosis
Treatment for Eisenmenger phenomenon has historically been palliative, and closure of the underlying shunt is contra-
indicated (remember that right to left shunts have been used to palliative patients with severe pulmonary arterial
hypertension)
2
.
More recently some pharmacological agents show the prospect of being beneficial in pulmonary arterial hypertension,
including Eisenmenger syndrome (see PAH article).
In selected cases combined heart and lung transplantation can be performed.
Although Eisenmenger phenomenon is one of the most severe manifestations of pulmonary arterial hypertension,
prognosis is better than that of idiopathic pulmonary arterial hypertension
2
.
Survival, as measured by age reached
2
:
30 years of age: 75%
40 years of age: 70%
55 years of age: 55%
Complications include:
haemoptysis
syncope and sudden death
Etymology
The phenomenon was first described by Victor Eisenmenger (1864 - 1932) Austrian physician, Vienna in 1897 in a
32 year old patient with a large VSD, but his name was only associated with the syndrome in 1958
6-7
.
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Differential diagnosis
The radiographic differential is essentially that of pulmonary arterial hypertension (see causes of pulmonary arterial
hypertension).
References
1. Sheehan R, Perloff JK, Fishbein MC et-al. Pulmonary neovascularity: a distinctive radiographic finding in
Eisenmenger syndrome. Circulation. 2005;112 (18): 2778-85. doi:10.1161/CIRCULATIONAHA.104.509869
- Pubmed citation
2. Diller, Gerhard-Paul; Dimopoulos, Konstantinos; Kafka, Henryk; Ho, Siew Yen; Gatzoulis, Michael A.
Model of chronic adaptation: right ventricular function in Eisenmenger syndrome European Heart Journal
Supplements. 9 (suppl H): H54. doi:10.1093/eurheartj/sum019
3. McMillan JA, Feigin RD, DeAngelis C et-al. Oski's pediatrics, principles & practice. Williams & Wilkins.
(2006) ISBN:0781738946. Read it at Google Books - Find it at Amazon
4. Nixon JV, Alpert JS. The AHA clinical cardiac consult. Lippincott Williams & Wilkins. (2006)
ISBN:0781764904. Read it at Google Books - Find it at Amazon
5. Hillis LD, Lange RA, Winniford MD et-al. Manual of clinical problems in cardiology, with annotated key
references. Lippincott Williams & Wilkins. (2003) ISBN:0781723817. Read it at Google Books - Find it at
Amazon
6. Victor Eisenmenger from whonamedit.com, the dictionary of medical eponyms. Victor Eisenmenger
7. Wood P. The Eisenmenger syndrome or pulmonary hypertension with reversed central shunt. Br Med J.
1958;2 (5099): 755-62. - Free text at pubmed - Pubmed citation
Synonyms & Alternative Spellings
Synonyms or Alternative Spelling Include in Listings?
Eisenmenger's syndrome
Eisenmenger phenomenon
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