Pediatric Eisenmenger Syndrome and Pulmonary Hypertension Workup

  • Author: Brian M Cummings, MD; Chief Editor: Stuart Berger, MD   more...
 
Updated: Mar 25, 2011
 

Laboratory Studies

The following studies are indicated in patients with Eisenmenger syndrome:

  • CBC count
    • RBCs - Polycythemia (ie, an increased number of RBCs), especially in iron deficient states (microcytic, hypochromic)
    • Hemoglobin and hematocrit - Increase in response to worsening cyanosis
    • Mean corpuscular hemoglobin and mean corpuscular volume
  • Uric acid and bilirubin levels: These are increased with polycythemia.
  • Pulse oximetry: Cyanosis and decreased saturations may be present.
  • ABG: Both the PaO2 and PCO2 may be decreased secondary to right-to-left shunting and resting tachypnea, respectively.
  • Brain natriuretic peptide (BNP): BNP may be a marker for prognosis in pulmonary artery hypertension (PAH).[14]
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Imaging Studies

  • Chest 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.
    • 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).
  • Echocardiography
    • Two-dimensional transthoracic imaging can reveal the particular features of the structural cardiac defect.
    • Color-flow Doppler interrogation is useful in demonstrating the direction of intracardiac blood flow.[15]
    • Pulsed and continuous wave Doppler measurements enable quantification of intracardiac shunt, right ventricular pressures, and estimation of pulmonary artery systolic/diastolic and mean pressures by means of the modified Bernoulli equation.[16, 17]
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Other Tests

Electrocardiogram reveals signs of underlying cardiac defect and of right ventricular hypertrophy:

  • Right axis deviation, Tall R wave in V1, deep S wave in V6 ± ST and T-wave abnormalities
  • May see P pulmonale, atrial and ventricular arrhythmias.

The 6-minute walk test (6MWT) versus cardiopulmonary exercise testing (CPET):

  • 6MWT is simpler than the more formal and involved traditional CPET.
  • 6MWT is better tolerated in younger children who often won't comply with the multiple leads, facemask, or other equipment needed for a CPET.
  • 6MWT requires minimal equipment and subspecialty experience.
  • The 6MWT may be effective in patients with a walk distance less than 300 m. In patients above the 300 m threshold, a cardiopulmonary exercise test should be considered.[18]
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Procedures

Cardiac catheterization permits the following:

  • Examination of the intracardiac structure and exclusion of potentially reversible causes of pulmonary hypertension
  • Assessment of ventricular function (systolic and diastolic)
  • Examination of the intracardiac shunt
  • Determination of pulmonary artery pressure and flow
  • Calculation of pulmonary vascular resistance (PVR)
  • Pulmonary angiography: Pulmonary angiography can reveal structural alterations in the pulmonary vascular bed. Irreversible changes (consistent with Heath-Edwards III severity) can be visualized and can include a loss of normal arborization, as well as tortuosity, narrowing, or cut-off of small pulmonary arteries.
  • Determination of the reversibility of the pulmonary hypertension: 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.[19]
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Histologic Findings

  • 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.[7] This grading is a histopathologic classification 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.
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Staging

Stages of pulmonary vascular pathology are presented here with respect to the histopathologic criteria presented by Heath and Edwards.[7]

  • 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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Proceed to Treatment & Management
 
 
Contributor Information and Disclosures
Author

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.

Coauthor(s)

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.

Specialty Editor Board

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

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.

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.

Gilbert Z Herzberg, MD  Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College; Consulting Staff, Department of Pediatrics, Sound Shore Medical Center

Gilbert Z Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

Chief Editor

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.

Additional Contributors

The author would like to thank Dr. Jeff Myers for his work on earlier versions of this topic and Dr. Lahoud-Rahme for her critical review.

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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.
 
 
 
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