Eisenmenger

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Eisenmenger Syndrome

Eisenmenger syndrome refers to any untreated congenital cardiac defect with intracardiac

communication that leads to pulmonaryhypertension, reversal of flow, and cyanosis. [1, 2, 3] The

previous left-to-right shunt is converted into a right-to-left shunt secondary to elevated

pulmonary artery pressures and associated pulmonary vascular disease. !ee Etiology,

Treatment, and "edication.#

$esions in Eisenmenger syndrome, such as large septal defects, are characteri%ed &y high

pulmonary pressure and'or a high pulmonary flow state. (evelopment of the syndrome

represents a point at which pulmonary hypertension is irreversi&le and is an indication that the

cardiac lesion is li)ely inopera&le see the image &elow#. !ee Etiology, *or)up, and

Treatment.#

Eisenmenger syndrome was initially descri&ed in 1+, when ictor Eisenmengerreported on a patient with symptoms of dyspnea and cyanosis from infancy whosu&se/uently developedheart failure and succum&ed to massive hemoptysis.[0] nautopsy revealed a large ventricular septal defect !(# and an overriding aorta. Thiswas the first description of a lin) &etween a large congenital cardiac shunt defect andthe development of pulmonary hypertension. !ee resentation and *or)up.#

dvances in the medical treatment of patients with severe pulmonary hypertension mayimprove survival in patients with Eisenmenger syndrome and may potentially reversethe process in selected patients to a point at which they again &ecome candidates forsurgical repair. !ee Treatment and "edication.#[]

Pulmonary hypertensionulmonary hypertension is defined as a mean pulmonary artery pressure of more than 2 mm 4g at restor more than 35 mm 4g during e6ercise. The *orld 4ealth 7rgani%ation *47# has pu&lished aclassification system of various etiologies of pulmonary hypertension8 the most recent update waspu&lished in 2513.[9]Eisenmenger syndrome is considered part of the group 1 causes of pulmonaryhypertension, according to the enice classification.

Intracardiac communication

n intracardiac communication allows high pulmonary artery pressures to develop and produces right-to-left intracardiac &lood flow. 7riginally descri&ed in association with a large !(, Eisenmenger syndromecan also manifest with a patent ductus arteriosus (# or, less fre/uently, with other congenital cardiacanomalies.

E6amples of congenital heart disease su&types that may cause pulmonary vascular disease and proceedto Eisenmenger syndrome include the following:

• ;ncreased pulmonary arterial flow - trial septal defect !(#, systemic arteriovenous fistulae,

total anomalous pulmonary venous return

• ;ncreased pulmonary arterial pressure and flow - $arge !(, large (, truncus arteriosus,

single ventricle with uno&structed pulmonary &lood flow

• Elevated pulmonary venous pressure - "itral stenosis, cor triatriatum, o&structed pulmonary

venous return

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Epidemiology

Eisenmenger syndrome usually develops &efore pu&erty &ut may develop in adolescence and early

adulthood.

atients in underdeveloped countries are more li)ely to present late with uncorrected congenital cardiac

lesions and a mar)edly elevated pulmonary vascular resistance


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Blinically, patients gradually develop the following complications of advanced pulmonary vascular

disease:

• (yspnea upon e6ertion

• !yncope

• Bhest pain

• !tro)e

• Crain a&scess

• Byanosis

• Bongestive heart failure

• (ysrhythmia

• 4yperviscosity complications

• ulmonary hemorrhage'hemoptysis

• Endocarditis

The time frame for this process depends on the anatomic nature of the lesion and whether conditions,

such as trisomy 21 (own syndrome#, that are )nown to accelerate the development of 7( are

present.[15]

*ithout intervention, reversal of flow may happen in early childhood or around pu&erty, andprogression of symptoms may lead to death &y the second or third decade of life. [11, 12];nterestingly, adult

patients with Eisenmenger syndrome may have a &etter prognosis compared with those with other

causes of pulmonary hypertension.[13]

Bauses

Bauses of Eisenmenger syndrome include the following:

• $arge, uncorrected cardiac shunt or palliative, surgically created systemic-to-pulmonary shunt for

congenital heart disease

• $arge, nonrestrictive !(

• ?onrestrictive (

• trioventricular septal defect, including a large ostium primum !( without a ventricular

component

• ortopulmonary window

• alliative, surgically created systemic-to-pulmonary anastomosis for treatment of congenital heart

disease

rognosis

Eisenmenger syndrome is uniformly fatal8 however, some patients survive into the si6th decade of life.

The usual life e6pectancy of a patient with Eisenmenger syndrome is 25-5 years if the syndrome isdiagnosed promptly and treated with vigilance. The onset of pulmonary hemorrhage is usually the

hallmar) of a rapid progression of the disease.[10]

The complications of chronic cyanotic heart disease affect multiple organ systems, including the

hematologic, s)eletal, renal, and neurologic systems, causing significant mor&idity and mortality.

http://emedicine.medscape.com/article/943216-overviewhttp://emedicine.medscape.com/article/943216-overview


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The /uality of life is poor in patients with Eisenmenger syndrome &ecause e6ercise tolerance is e6tremely

limited due to limited o6ygen upta)e resulting from an ina&ility to increase pulmonary &lood flow# and

complications are profound. oor prognosis is predicted &y syncope, elevated-right sided pressures, and

hypo6emia.

study &y !alehian et al reported that left ventricular dysfunction defined as left ventricular eDection

fraction [$E@] 5A#, right ventricular hypertrophy, and signs and symptoms of heart failure predict

mortality in patients with Eisenmenger syndrome. [1] simple echocardiographic score relying on right

ventricular and right atrial characteristics was found to predict adverse outcomes in patients with

Eisenmenger syndrome that is not associated with comple6 congenital heart disease.[19]

Fncorrected congenital heart disease with development of Eisenmenger comple6 portends an insidious

progression to near complete physical disa&ility.

7ccurrence

The fre/uency of pulmonary hypertension and the su&se/uent development of reversed shunting vary

depending on the specific heart defect and operative intervention. !uch variations include the following:

• $arge, nonrestrictive !( or ( - ppro6imately 5A of infants with one of these defects

develop pulmonary hypertension &y early childhood

• !( or ( and transposition of the great arteries - @orty percent of patients develop pulmonary

hypertension within the first year of life

• $arge secundum !( - The natural history of a large secundum !( differs in that the 15A of

cases that progress to pulmonary hypertension do so more slowly and usually not until after the thirddecade of life

• ersistent truncus arteriosus and unrestricted pulmonary &lood flow - ll patients develop severe

pulmonary hypertension &y the second year of life

• Bommon atrioventricular canal - lmost all patients develop severe pulmonary hypertension &y

the second year of life• !urgically created systemic-to-pulmonary shunt - The fre/uency of pulmonary hypertension

varies depending on si%e and anatomy

• Claloc)-Taussig anastomosis su&clavian artery to pulmonary artery# - Ten percent of patients

develop pulmonary hypertension

• *aterston ascending aorta to pulmonary artery# or otts descending aorta to pulmonary artery#

shunt - Thirty percent of patients develop pulmonary hypertension.

"or&idity

Bomplications in Eisenmenger syndrome include the following:

•

4ematologic complications - These include hyperviscosity syndromes related to secondaryerythrocytosis and &leeding diatheses

• ?ervous system complications G These include &rain a&scess, transient cere&ral ischemia,

throm&otic stro)e, and intracere&ral hemorrhage

• 4yper&iliru&inemia - ;ncreases the ris) of gallstones

• 4yperuricemia - Ban cause nephrolithiasis and secondary gout

• 4ypertrophic osteoarthropathy - Bauses &one pain and tenderness

• ision loss -


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• Bongestive heart failure

• (ysrhythmia

• ulmonary infarction and hemorrhage

• ;nfective endocarditis

• !yncope - The systemic vascular &ed is prone to vasodilation and su&se/uent systemic arterial

hypotension, which can cause syncope

• !udden death

"ortality

atients with Eisenmenger syndrome usually do not survive &eyond the second or third decade. $ong-

term survival depends on the patientHs age at the onset of pulmonary hypertension and the coe6istence of

additional adverse features, such as (own syndrome. !urvival predominantly depends on right ventricular

function. The mortality rate in pregnant patients with Eisenmenger syndrome is reported to &e

appro6imately 5A, although it may &e higher.

The most fre/uent terminal event in this syndrome is a com&ination of hypo6emia and arrhythmia in the

setting of rapid increases in pulmonary vascular resistance or decreases in systemic vascular resistance

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