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Eisenmenger Syndrome Workup

  • Author: Mikhael F El-Chami, MD; Chief Editor: Park W Willis IV, MD  more...
 
Updated: Nov 23, 2014
 

Approach Considerations

Laboratory studies used in the diagnosis of Eisenmenger syndrome include complete blood count (CBC), biochemical profiles, and iron studies, in addition to blood gas assessments. Imaging studies can reveal cardiac structural defects and pulmonary changes, including irreversible alterations in the pulmonary system. Electrocardiography can also reveal signs of underlying cardiac defect and of right ventricular hypertrophy, while histologic findings can be used to determine the stage of pulmonary vascular pathology.

Prognostic assessment

If the pulmonary artery pressures do not fall with inhalation of 100% oxygen or nitric oxide, the pulmonary hypertension is considered irreversible, and the patient is not a candidate for surgical repair.[21]

Pulmonary angiography can reveal structural alterations in the pulmonary vascular bed. Irreversible changes (consistent with Heath-Edwards III severity) can be visualized and may include loss of normal arborization, as well as tortuosity, narrowing, or cut-off of small pulmonary arteries.

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Laboratory Studies

Complete blood count (CBC) findings include the following:

  • Erythrocytosis increases hematocrit and hemoglobin concentration
  • Phlebotomy-related iron deficiency decreases the mean corpuscular volume and mean corpuscular hemoglobin concentration
  • The 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 are sometimes elevated
  • Urinary biochemical analysis reveals proteinuria

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

Additional findings include the following:

  • Pulse oximetry - Cyanosis and decreased saturations may be present
  • Arterial blood gas (ABG) - Reduced resting partial pressure of carbon dioxide (PaCO 2) due to resting tachypnea and reduced partial pressure of oxygen (PaO 2) due to right-to-left shunting; mixed respiratory and metabolic acidosis
  • Brain natriuretic peptide (BNP) - BNP may be a marker for prognosis in pulmonary arterial hypertension (PAH) [22, 14]
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Chest Radiography and MRI

Radiography

In the early stages, chest radiography reveals a typical appearance of increased pulmonary flow with right ventricular or biventricular enlargement, right atrial or biatrial enlargement, pulmonary vascular plethora, and an enlarged main pulmonary artery. (See the image below.)

This radiograph reveals an enlarged right heart an 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.

Advancing pulmonary vascular disease appears as a normal cardiac silhouette with dilated main and branch pulmonary arteries without evidence of pulmonary overcirculation.

In patients with severe pulmonary vascular disease, radiography reveals a normal-sized heart, pruning of the pulmonary vasculature (ie, diminished distal/peripheral pulmonary vascularity), pulmonary infarction, and/or calcification of a patent ductus arteriosus (PDA).

Magnetic resonance imaging

Magnetic resonance imaging (MRI) can be used for the following:

  • Estimation of the magnitude of the right-to-left shunt
  • Anatomical definition (in some cases)
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Echocardiography

Two-dimensional (2-D) transthoracic imaging can reveal the particular features of the structural cardiac defect responsible for the shunt. Coexistent structural abnormalities can also be identified. (See the image below.)

Apical, 4-chamber, transthoracic view demonstratin 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.

Color-flow Doppler interrogation is useful for demonstrating the direction of intracardiac blood flow. (See the images below.)[23]

This apical, 4-chamber, transthoracic segment show This apical, 4-chamber, transthoracic 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 tricu This is a color Doppler interrogation of the tricuspid valve in a patient with 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 t 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.

Pulsed and continuous wave Doppler measurements permit quantification of the intracardiac shunt, right ventricular pressures, and estimation of pulmonary artery systolic/diastolic and mean pressures by means of the modified Bernoulli equation.[24, 25] Echocardiography can also be used to identify surgical systemic-to-pulmonary shunts. The addition of supine bicycle ergometry can demonstrate increased right-to-left shunting with exercise.

Transesophageal echocardiography

Transesophageal echocardiography is useful for imaging posterior structures, including the atria and pulmonary veins. (See the image below.)

This transesophageal image is from the midesophagu 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.
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Electrocardiography

Electrocardiographic findings are almost always abnormal in Eisenmenger syndrome. They include the following:

  • 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 V 1, deep S wave in V 6, ± ST and T wave abnormalities
  • P pulmonale

Six-minute walk test versus cardiopulmonary exercise test

The 6-minute walk test (6MWT), which requires minimal equipment and subspecialty experience, is simpler than the more formal and involved traditional cardiopulmonary exercise test (CPET).

Moreover, the 6MWT is better tolerated in younger children, who often will not comply with the multiple leads, facemask, or other equipment needed for a CPET.

The 6MWT may be effective in patients with a walk distance of less than 300 m. In patients above the 300-m threshold, however, a CPET should be considered.[26]

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Cardiac Catheterization

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

  • Severity of PAH
  • Conduit patency and pressure gradient
  • Coexisting coronary artery anomalies (rare)
  • Degree of shunting

Cardiac catheterization permits the examination of the intracardiac structure and exclusion of potentially reversible causes of pulmonary hypertension, as well as assessment of ventricular function (systolic and diastolic), examination of the intracardiac shunt, determination of pulmonary artery pressure and flow, and calculation of pulmonary vascular resistance (PVR).

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Histology

In severe pulmonary vascular disease, histologic 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.

Staging

In 1958, Heath and Edwards proposed a histologic grading of pulmonary vascular disease that corresponds to the duration and severity of injury caused by increased pressure and volume load.[9] This grading is a histopathologic classification derived from biopsies taken from isolated portions of the lung.

A biopsy of various segments of the lung could possibly be performed at the same time, yielding different histologic grades. Currently, performing lung biopsies is rarely necessary. The combination of pulmonary angiography and measurement of pulmonary vascular hemodynamics is usually sufficient to guide therapy.

Stages of pulmonary vascular pathology, according to the histopathologic criteria of Heath and Edwards, are as follows[9] :

  • Stage I - Medial hypertrophy (reversible)
  • Stage II - Cellular intimal hyperplasia in an abnormally muscular artery (reversible)
  • Stage III - Lumen occlusion from intimal hyperplasia of fibroelastic tissue (partially reversible)
  • Stage IV - Arteriolar dilation and medial thinning (irreversible)
  • Stage V - Plexiform lesion, which is an angiomatoid formation (terminal and irreversible)
  • Stage VI - Fibrinoid/necrotizing arteritis (terminal and irreversible)
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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, 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

Disclosure: Nothing to disclose.

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

Disclosure: Nothing to disclose.

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 College of Physicians, American Heart Association, and American Society of Echocardiography

Disclosure: Nothing to disclose.

Lisa A Hourigan, MBBS, FRACP Consulting Staff, Department of Cardiology, University of California, San Francisco School of Medicine

Disclosure: Nothing to disclose.

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

Disclosure: Nothing to disclose.

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

Disclosure: Nothing to disclose.

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

Disclosure: Nothing to disclose.

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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 computed tomography (CT) chest scan shows a large, unrestricted patent ductus arteriosus (PDA) in a 24-year-old woman with Eisenmenger syndrome.
This apical, 4-chamber, transthoracic 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 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 is a color Doppler interrogation of the tricuspid valve in a patient with 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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