Eisenmenger Syndrome: Background

Eisenmenger Syndrome: Background, Epidemiology, Etiology
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http://emedicine.medscape.com/article/154555-overview#showall
Eisenmenger Syndrome
Author: Mikhael F El-Chami, MD; Chief Editor: Park W Willis IV, MD more...
Updated: Nov 23, 2014
Background
Eisenmenger syndrome refers to any untreated congenital cardiac defect with

intracardiac communication that leads to pulmonary hypertension, 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. (See Etiology, Treatment, and Medication.)
Lesions in Eisenmenger syndrome, such as large septal defects, are characterized

by high pulmonary pressure and/or a high pulmonary flow state. Development of the
syndrome represents a point at which pulmonary hypertension is irreversible and is
an indication that the cardiac lesion is likely inoperable (see the image below). (See
Etiology, Workup, and Treatment.)
This radiograph reveals an enlarged right heart and pulmonary artery dilatation in a
24-year-old woman with an unrestricted patent ductus arteriosus (PDA) and Eisenmenger
syndrome.
Eisenmenger syndrome was initially described in 1897, when Victor Eisenmenger

reported on a patient with symptoms of dyspnea and cyanosis from infancy who
subsequently developed heart failure and succumbed to massive hemoptysis.[4] An
autopsy revealed a large ventricular septal defect (VSD) and an overriding aorta.
This was the first description of a link between a large congenital cardiac shunt
defect and the development of pulmonary hypertension. (See Presentation and
Workup.)
Advances in the medical treatment of patients with severe pulmonary hypertension
may improve survival in patients with Eisenmenger syndrome and may potentially
reverse the process in selected patients to a point at which they again become
candidates for surgical repair. (See Treatment and Medication.)[5]
Pulmonary hypertension
Pulmonary hypertension is defined as a mean pulmonary artery pressure of more

than 25 mm Hg at rest or more than 30 mm Hg during exercise. The World Health
Organization (WHO) has published a classification system of various etiologies of
pulmonary hypertension; the most recent update was published in 2013.[6]
Eisenmenger syndrome is considered part of the group 1 causes of pulmonary
hypertension, according to the Venice classification.
Intracardiac communication
An intracardiac communication allows high pulmonary artery pressures to develop

and produces right-to-left intracardiac blood flow. Originally described in association
with a large VSD, Eisenmenger syndrome can also manifest with a patent ductus
arteriosus (PDA) or, less frequently, with other congenital cardiac anomalies. (See
the images below.)
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This transesophageal image is from the midesophagus of a patient with Eisenmenger

syndrome secondary to an unrestricted patent ductus arteriosus (PDA). It shows a severely
dilated pulmonary artery. PA = pulmonary artery, Asc Ao = ascending aorta.
This computed tomography (CT) chest scan shows a large, unrestricted patent ductus
arteriosus (PDA) in a 24-year-old woman with Eisenmenger syndrome.
Examples of congenital heart disease subtypes that may cause pulmonary vascular
disease and proceed to Eisenmenger syndrome include the following:
Increased pulmonary arterial flow - Atrial septal defect (ASD), systemic
arteriovenous fistulae, total anomalous pulmonary venous return
Increased pulmonary arterial pressure and flow - Large VSD, large PDA,
truncus arteriosus, single ventricle with unobstructed pulmonary blood flow
Elevated pulmonary venous pressure - Mitral stenosis, cor triatriatum,
obstructed pulmonary venous return
Epidemiology
Eisenmenger syndrome usually develops before puberty but may develop in

adolescence and early adulthood.
Patients in underdeveloped countries are more likely to present late with

uncorrected congenital cardiac lesions and a markedly elevated pulmonary vascular
resistance (PVR). They are more likely to be inoperable secondary to Eisenmenger
physiology.
Etiology
Eisenmenger syndrome occurs in patients with large, congenital cardiac or surgically

created extracardiac left-to-right shunts. These shunts initially cause increased
pulmonary blood flow.
If left unchecked, increased pulmonary blood flow and/or elevated pulmonary arterial
pressure can result in remodeling of the pulmonary microvasculature, with
subsequent obstruction to pulmonary blood flow. This is commonly referred to as
pulmonary vascular obstructive disease (PVOD).
According to Ohms law, flow (Q) is inversely related to resistance (R) and is directly
proportional to pressure (P), as represented by the equation Q = P/R. Any increase
in flow, as is observed in patients with intracardiac defects and initial left-to-right
shunts, results in increased pulmonary artery pressures. Additionally, any increase
in resistance, as occurs in PVOD, results in a decrease in effective flow at the same
pressure.
The progression to Eisenmenger physiology is represented by a spectrum of
morphologic changes in the capillary bed that progress from reversible lesions to
irreversible ones. Endothelial dysfunction and smooth muscle proliferation result
from the changes in flow and pressure, increasing the PVR.[7]
The cellular and molecular mechanisms remain fully uncharacterized, representing

pathways of inflammation, cell proliferation, vasoconstriction, and fibrosis.[8] The
mechanism of pulmonary hypertension in congenital heart disease may share
characteristics with other mechanisms of pulmonary hypertension, but the pathways
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remain complex.
In 1958, Heath and Edwards proposed a histologic classification to describe the

changes in Eisenmenger syndrome.[9] Stages I and II represent disease that is most
likely reversible. Stage III disease may still be reversible, but in progressing to
stages IV-VI, the disease is thought to become irreversible. Pulmonary biopsies are
rarely performed today for this condition.
Natural history
Failure to reduce pulmonary pressures in the first 2 years of life may result in the
failure of the normal regression of the intimal smooth muscle. This is followed by the
progressive changes described by Heath and Edwards.[9] The condition then
advances to irreversible pulmonary hypertension, defined as unresponsiveness to
inhalation of 100% oxygen or nitric oxide. This point usually correlates to a PVR of
more than 12 Woods units.
Clinically, patients gradually develop the following complications of advanced
pulmonary vascular disease:
Dyspnea upon exertion
Syncope
Chest pain
Stroke
Brain abscess
Cyanosis
Congestive heart failure
Dysrhythmia
Hyperviscosity complications
Pulmonary hemorrhage/hemoptysis
Endocarditis
The time frame for this process depends on the anatomic nature of the lesion and
whether conditions, such as trisomy 21 (Down syndrome), that are known to
accelerate the development of PVOD are present.[10] Without intervention, reversal
of flow may happen in early childhood or around puberty, and progression of
symptoms may lead to death by the second or third decade of life.[11, 12]
Interestingly, adult patients with Eisenmenger syndrome may have a better
prognosis compared with those with other causes of pulmonary hypertension.[13]
Causes
Causes of Eisenmenger syndrome include the following:
Large, uncorrected cardiac shunt or palliative, surgically created systemicto-pulmonary shunt for congenital heart disease
Large, nonrestrictive VSD
Nonrestrictive PDA
Atrioventricular septal defect, including a large ostium primum ASD without a
ventricular component
Aortopulmonary window
Palliative, surgically created systemic-to-pulmonary anastomosis for
treatment of congenital heart disease
Prognosis
Eisenmenger syndrome is uniformly fatal; however, some patients survive into the
sixth decade of life. The usual life expectancy of a patient with Eisenmenger
syndrome is 20-50 years if the syndrome is diagnosed promptly and treated with
vigilance. The onset of pulmonary hemorrhage is usually the hallmark of a rapid
progression of the disease.[14]
The complications of chronic cyanotic heart disease affect multiple organ systems,
including the hematologic, skeletal, renal, and neurologic systems, causing
significant morbidity and mortality.
The quality of life is poor in patients with Eisenmenger syndrome because exercise
tolerance is extremely limited (due to limited oxygen uptake resulting from an
inability to increase pulmonary blood flow) and complications are profound. Poor
prognosis is predicted by syncope, elevated-right sided pressures, and hypoxemia.
A study by Salehian et al reported that left ventricular dysfunction (defined as left

ventricular ejection fraction [LVEF] < 50%), right ventricular hypertrophy, and signs
and symptoms of heart failure predict mortality in patients with Eisenmenger
syndrome.[15] A 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 complex congenital heart
disease.[16]
Uncorrected congenital heart disease with development of Eisenmenger complex
portends an insidious progression to near complete physical disability.
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Occurrence
The frequency of pulmonary hypertension and the subsequent development of
reversed shunting vary depending on the specific heart defect and operative
intervention. Such variations include the following:
Large, nonrestrictive VSD or PDA - Approximately 50% of infants with one of

these defects develop pulmonary hypertension by early childhood
VSD or PDA and transposition of the great arteries - Forty percent of patients
develop pulmonary hypertension within the first year of life
Large secundum ASD - The natural history of a large secundum ASD differs
in that the 10% of cases that progress to pulmonary hypertension do so more
slowly and usually not until after the third decade of life
Persistent truncus arteriosus and unrestricted pulmonary blood flow - All
patients develop severe pulmonary hypertension by the second year of life
Common atrioventricular canal - Almost all patients develop severe
pulmonary hypertension by the second year of life
Surgically created systemic-to-pulmonary shunt - The frequency of
pulmonary hypertension varies depending on size and anatomy
Blalock-Taussig anastomosis (subclavian artery to pulmonary artery) - Ten
percent of patients develop pulmonary hypertension
Waterston (ascending aorta to pulmonary artery) or Potts (descending aorta
to pulmonary artery) shunt - Thirty percent of patients develop pulmonary
hypertension.
Morbidity
Complications in Eisenmenger syndrome include the following:
Hematologic complications - These include hyperviscosity syndromes related

to secondary erythrocytosis and bleeding diatheses
Nervous system complications These include brain abscess, transient
cerebral ischemia, thrombotic stroke, and intracerebral hemorrhage
Hyperbilirubinemia - Increases the risk of gallstones
Hyperuricemia - Can cause nephrolithiasis and secondary gout
Hypertrophic osteoarthropathy - Causes bone pain and tenderness
Vision loss - Reports document transient vision loss related to peripheral
retinal microvascular abnormalities
Congestive heart failure
Dysrhythmia
Pulmonary infarction and hemorrhage
Infective endocarditis
Syncope - The systemic vascular bed is prone to vasodilation and
subsequent systemic arterial hypotension, which can cause syncope
Sudden death
Mortality
Patients with Eisenmenger syndrome usually do not survive beyond the second or
third decade. Long-term survival depends on the patients age at the onset of
pulmonary hypertension and the coexistence of additional adverse features, such as
Down syndrome. Survival predominantly depends on right ventricular function. The
mortality rate in pregnant patients with Eisenmenger syndrome is reported to be
approximately 50%, although it may be higher.
The most frequent terminal event in this syndrome is a combination of hypoxemia

and arrhythmia in the setting of rapid increases in pulmonary vascular resistance or
decreases in systemic vascular resistance (SVR). Death also commonly results from
congestive heart failure, massive hemoptysis, or thromboembolism.[11]
A study by Diller et al indicated that survival rates for untreated patients with
Eisenmenger syndrome may have been overestimated in previous studies and that
these rates have not improved since the 1970s. The report involved a literature
review of 12 studies published between 1971 and 2013 (1131 patients total), along
with an analysis of 219 contemporary, treatment-nave patients at the investigators
own institution.[17]
The investigators stated that almost none of the studies appropriately accounted for
immortal time bias and therefore overestimated patients survival chances by as
much as 20 years. When Diller and colleagues took immortal time bias into account,
they determined that the 10-year mortality rate among untreated patients
approached 30-40%. They also found no indication that the chances of survival for
these patients were better than they would have been in the 1970s, 1980s, or
1990s, although survival prospects were found to be better than for patients in the
1950s and 1960s.[17]
Patient Education
The following points should be considered in patient education:
Inform patients that diet and weight control are essential
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Educate patients to avoid smoking

Provide an exercise prescription
Advise abstinence from or only moderate intake of alcohol
Educate patients about contraception options and pregnancy risk (the
mortality rate in pregnant patients with Eisenmenger syndrome is
approximately 50%) [18]
Contraception by means of tubal ligation (with subacute bacterial endocarditis
[SBE] prophylaxis) may be recommended
Oral or implantable contraceptives may promote pulmonary infarction through
activation of the coagulation cascade
Educate patients about the signs and symptoms of polycythemia and
hyperviscosity
Inform patients about the importance of dental hygiene
Additional resources for patients with pulmonary hypertension can be found at the
Pulmonary Hypertension Association Web site.
Clinical Presentation
Contributor Information and Disclosures
Author
Mikhael F El-Chami, MD Assistant Professor, Department of Cardiology, Division of Electrophysiology, Emory
University School of Medicine
Mikhael F El-Chami, MD is a member of the following medical societies: Alpha Omega Alpha, American College
of Cardiology, Heart Rhythm Society
Disclosure: Received grant/research funds from Medtronic Inc for principle investigator.
Coauthor(s)
Charles D Searles, Jr, MD Assistant Professor of Medicine, Division of Cardiology, Emory University School of
Medicine; Consulting Staff, Division of Cardiology, Director of Stress Echo Laboratory, Grady Memorial Hospital
Charles D Searles, Jr, MD is a member of the following medical societies: American Heart Association, Sigma Xi
Disclosure: Nothing to disclose.
Chief Editor
Park W Willis IV, MD Sarah Graham Distinguished Professor of Medicine and Pediatrics, University of North
Carolina at Chapel Hill School of Medicine
Park W Willis IV, MD is a member of the following medical societies: American Society of Echocardiography
Acknowledgements
Stuart Berger, MD Professor of Pediatrics, Division of Cardiology, Medical College of Wisconsin; Chief of
Pediatric Cardiology, Medical Director of Pediatric Heart Transplant Program, Medical Director of The Heart
Center, Children's Hospital of Wisconsin
Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American
College of Cardiology, American College of Chest Physicians, American Heart Association, and Society for
Cardiac Angiography and Interventions
Disclosure: Nothing to disclose
Brian M Cummings, MD Pediatric Critical Care; Director Pediatric Transport, Medical Director PALS,
MassGeneral Hospital for Children, Instructor in Pediatrics, Harvard Medical School
Brian M Cummings, MD is a member of the following medical societies: American Academy of Pediatrics
Elyse Foster, MD Director of Adult Echocardiography Laboratory and Adult Congenital Heart Disease Service,
Department of Internal Medicine, Division of Cardiology, Moffitt Hospital; Assistant Professor of Cardiology,
University of California, San Francisco, School of Medicine
Elyse Foster, MD is a member of the following medical societies American College of Cardiology, American
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College of Physicians, American Heart Association, and American Society of Echocardiography

Lisa A Hourigan, MBBS, FRACP Consulting Staff, Department of Cardiology, University of California, San
Francisco School of Medicine
Christopher Johnsrude, MD, MS Chief, Division of Pediatric Cardiology, University of Louisville School of
Medicine; Director, Congenital Heart Center, Kosair Children's Hospital
Christopher Johnsrude, MD, MS is a member of the following medical societies: American Academy of Pediatrics
and American College of Cardiology
Disclosure: St Jude Medical Honoraria Speaking and teaching
John W Moore, MD, MPH Professor of Clinical Pediatrics, Section of Pediatric Cardiology, Department of
Pediatrics, University of California San Diego School of Medicine; Director of Cardiology, Rady Children's Hospital
John W Moore, MD, MPH is a member of the following medical societies: American Academy of Pediatrics,
American College of Cardiology, and Society for Cardiac Angiography and Interventions
Jeff L Myers, MD, PhD Chief, Pediatric and Congenital Cardiac Surgery, Department of Surgery, Massachusetts
General Hospital; Associate Professor of Surgery, Harvard Medical School
Jeff L Myers, MD, PhD is a member of the following medical societies: American College of Surgeons, American
Heart Association, and International Society for Heart and Lung Transplantation
Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College
of Pharmacy; Editor-in-Chief, Medscape Drug Reference
Disclosure: Medscape Salary Employment
Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of
Pharmacy; Editor-in-Chief, Medscape Drug Reference
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Eisenmenger Syndrome: Background

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Eisenmenger Syndrome: Background

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Eisenmenger Syndrome: Background, Epidemiology, Etiology

Eisenmenger syndrome refers to any untreated congenital cardiac defect with

Lesions in Eisenmenger syndrome, such as large septal defects, are characterized

Eisenmenger syndrome was initially described in 1897, when Victor Eisenmenger

Pulmonary hypertension is defined as a mean pulmonary artery pressure of more

An intracardiac communication allows high pulmonary artery pressures to develop

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