Eisenmenger Syndrome 

  • Author: Mikhael F El-Chami, MD; Chief Editor: Park W Willis IV, MD   more...
 
Updated: Dec 12, 2011
 

Background

In 1897, Eisenmenger reported the case of a 32-year-old man who had showed exercise intolerance, cyanosis, heart failure, and hemoptysis prior to death. Autopsy showed a large ventricular septal defect (VSD) and overriding aorta. This was the first description of a link between a large congenital cardiac shunt defect and the development of pulmonary hypertension.

An example is shown in the image below.

This radiograph reveals an enlarged right heart anThis 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.
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Pathophysiology

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. Subsequently, usually before puberty, pulmonary vascular disease causes pulmonary hypertension, ultimately resulting in reversed or bidirectional shunt flow with variable degrees of cyanosis.

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Epidemiology

Frequency

International

The frequency of pulmonary hypertension and the subsequent development of reversed shunting vary depending on the specific heart defect and operative interventions.

Approximately 50% of infants with a large, nonrestrictive VSD or patent ductus arteriosus (PDA) develop pulmonary hypertension by early childhood.

Forty percent of patients with VSD or PDA and transposition of the great arteries develop pulmonary hypertension within the first year of life.

The natural history of a large secundum atrial septal defect (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.

All patients with persistent truncus arteriosus and unrestricted pulmonary blood flow, and almost all patients with common atrioventricular canal, develop severe pulmonary hypertension by the second year of life.

The frequency of pulmonary hypertension in surgically created systemic-to-pulmonary shunts varies depending on size and anatomy.

Ten percent of those with a Blalock-Taussig anastomosis (subclavian artery to pulmonary artery) develop pulmonary hypertension compared to 30% of those with a Waterston (ascending aorta to pulmonary artery) or a Potts (descending aorta to pulmonary artery) shunt.

Mortality/Morbidity

Exercise tolerance is severely impaired due to an inability to increase pulmonary blood flow, thereby limiting oxygen uptake. The systemic vascular bed is prone to vasodilation and subsequent systemic arterial hypotension, which can cause syncope.

Long-term survival depends on the age at onset of pulmonary hypertension and the coexistence of additional adverse features, such as Down syndrome. Survival predominantly depends on right ventricular function. These patients usually do not survive beyond the second or third decade. The most frequent terminal event is a combination of hypoxemia and arrhythmia in the setting of rapid increases in pulmonary vascular resistance or decreases in systemic vascular resistance (SVR).

The complications of chronic cyanotic heart disease affect multiple organ systems, including hematologic, skeletal, renal, and neurologic, causing significant morbidity and mortality.

Sex

No overall sex predilection has been reported.

Age

Eisenmenger syndrome usually develops before puberty but may develop in adolescence and early adulthood.

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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, and Heart Rhythm Society

Disclosure: Medtronic Inc Grant/research funds 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 and Sigma Xi

Disclosure: Nothing to disclose.

Specialty Editor Board

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

Marschall S Runge, MD, PhD  Charles and Anne Sanders Distinguished Professor of Medicine, Chairman, Department of Medicine, Vice Dean for Clinical Affairs, University of North Carolina at Chapel Hill School of Medicine

Marschall S Runge, MD, PhD is a member of the following medical societies: American Association for the Advancement of Science, American College of Cardiology, American College of Physicians-American Society of Internal Medicine, American Federation for Clinical Research, American Federation for Medical Research, American Heart Association, American Physiological Society, American Society for Clinical Investigation, American Society for Investigative Pathology, Association of American Physicians, Association of Professors of Cardiology, Association of Professors of Medicine, Southern Society for Clinical Investigation, and Texas Medical Association

Disclosure: Pfizer Honoraria Speaking and teaching; Merck Honoraria Speaking and teaching; Orthoclinica Diagnostica Consulting fee Consulting

Amer Suleman, MD  Private Practice

Amer Suleman, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Institute of Stress, American Society of Hypertension, Federation of American Societies for Experimental Biology, Royal Society of Medicine, and Society of Cardiac Angiography and Interventions

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

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous authors Lisa A Hourigan, MBBS and Elyse Foster, MD to the development and writing of this article.

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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.
Apical 4-chamber transthoracic view demonstrating an ostium primum atrial septal defect (ASD) with enlarged right-side chambers. RA = right atrium, RV = right ventricle, LA = left atrium, LV = left ventricle.
This CT chest scan shows a large, unrestricted patent ductus arteriosus (PDA) in a 24-year-old woman with Eisenmenger syndrome. Asc Ao = ascending aorta.
This transthoracic apical 4-chamber segment shows color Doppler flow across the interatrial septum at the site of a large ostium primum atrial septal defect (ASD). RA = right atrium, LA = left atrium.
This transesophageal image is from the mid esophagus of a patient with Eisenmenger syndrome secondary to an unrestricted patent ductus arteriosus (PDA). It shows a severely dilated pulmonary artery. Pulm a = pulmonary artery, Asc Ao = ascending aorta.
This is a color Doppler interrogation of the tricuspid valve in a case of Eisenmenger syndrome. It demonstrates an elevated estimated right ventricular systolic pressure of 106 mm Hg + right atrial pressure, reflecting pulmonary hypertension. TR = tricuspid regurgitation.
This is the transthoracic Doppler examination of the pulmonic valve in a 24-year-old woman with Eisenmenger syndrome secondary to an uncorrected ostium primum atrial septal defect (ASD). This reveals an elevated estimated pulmonary artery diastolic pressure of 51 mm Hg + right atrial pressure. PR = pulmonic regurgitation.
 
 
 
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