Eisenmenger Syndrome Workup

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

Laboratory Studies

CBC count findings include the following:

  • Erythrocytosis increases hematocrit and hemoglobin concentration.
  • Phlebotomy-related iron deficiency decreases the mean corpuscular volume and mean corpuscular hemoglobin concentration.

Red cell mass is increased with erythrocytosis.

Bleeding time is prolonged by platelet dysfunction.

Biochemical profile findings include the following:

  • Increased conjugated bilirubin
  • Increased uric acid
  • Urea and creatinine sometimes elevated

Erythrocytic hypoglycemia is an artifactually low blood glucose level caused by increased in vitro glycolysis in the setting of increased red cell mass.

Iron study findings include the following:

  • Reduced serum ferritin due to phlebotomy-related iron store reduction
  • Increased total iron binding capacity

Urine biochemical analysis reveals proteinuria.

Arterial blood gas findings include the following:

  • Reduced resting PaCO2 due to resting tachypnea and reduced PaO2 due to right-to-left shunting
  • Mixed respiratory and metabolic acidosis
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Imaging Studies

Chest radiograph findings include the following:

  • Right ventricular and right atrial enlargement
  • Features of pulmonary hypertension, including dilated main pulmonary artery, increased hilar vascular markings, and pruned peripheral vessels

Transthoracic echocardiogram findings include the following:

  • The structural cardiac defect responsible for the shunt can be defined by the 2-dimensional imaging.
  • The location of cardiac shunt can usually be demonstrated by color Doppler.
  • The pressure gradient across the defect can be estimated.
  • Estimated pulmonary artery systolic and diastolic pressures
  • Identification of coexistent structural abnormalities
  • Left and right ventricular size and function: A study by Salehian et al reported that left ventricular dysfunction (defined as LVEF < 50%), right ventricular hypertrophy, and signs and symptoms of heart failure predict mortality in patients with Eisenmenger syndrome.[3]
  • Identification of surgical systemic-to-pulmonary shunts
  • The addition of supine bicycle ergometry can demonstrate increased right-to-left shunting with exercise.

Transesophageal echocardiogram is useful for imaging posterior structures, including the atria and pulmonary veins.

MRI findings include the following:

  • Can estimate magnitude of the right-to-left shunt
  • Useful for anatomical definition in some cases

Examples are shown in the images 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. This CT chest scan shows a large, unrestricted patThis CT chest scan shows a large, unrestricted patent ductus arteriosus (PDA) in a 24-year-old woman with Eisenmenger syndrome. Asc Ao = ascending aorta. Apical 4-chamber transthoracic view demonstrating 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 transthoracic apical 4-chamber segment shows 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 is a color Doppler interrogation of the tricuThis 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 tThis 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. This transesophageal image is from the mid esophagThis 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.
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Other Tests

Electrocardiogram findings include the following:

  • Almost always abnormal results and includes signs of right heart hypertrophy in addition to abnormalities associated with the underlying defect
  • Frontal plane QRS right axis deviation
  • Tall monophasic R wave in V1, deep S wave in V6, ± ST and T wave abnormalities
  • P pulmonale
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Procedures

Cardiac catheterization can be of value, after collecting clinical and noninvasive data, to confirm and/or demonstrate the following:

  • Severity of pulmonary arterial hypertension
  • Conduit patency and pressure gradient
  • Coexisting coronary artery anomalies (rare)
  • Degree of shunting
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Histologic Findings

In severe pulmonary vascular disease, histological analysis reveals abnormal extension of muscle into small peripheral arteries, severe medial smooth muscle hypertrophy of existing muscular arteries, plexiform lesions and increased intercellular material, and a reduction in the overall concentration and size of arteries.

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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.

References

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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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