Pediatric Eisenmenger Syndrome and Pulmonary Hypertension Follow-up

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

Further Inpatient Care

  • Patients do not require hospitalization for therapeutic erythropheresis but may need attention for infectious disease complications and/or other hematologic concerns.
  • Initiation of vasodilator therapy may require inpatient hospitalization and observation.
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Further Outpatient Care

  • Good dental hygiene is required to prevent infectious concerns, such as infective endocarditis or brain abscess.
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Inpatient & Outpatient Medications

  • A partial list of medications includes aspirin to prevent thrombotic complications, allopurinol for gout, iron supplementation for microcytosis, and digitalis and diuretics for symptoms of heart failure.
  • Supplemental oxygen is a powerful pulmonary arterial vasodilator and remains a critical component of the treatment for pulmonary hypertension.
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Transfer

  • Nonurgent transfers are made to a tertiary facility because this disease, by nature, is a chronic and terminal condition.
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Deterrence/Prevention

  • Prevention is critical. When recognized in a timely fashion, congenital cardiac defects can be effectively treated with minimal morbidity and mortality. Eisenmenger syndrome is, by definition, an untreated lesion that has progressed to the point of inoperability.
  • Many congenital heart defects can be identified in utero when families receive appropriate prenatal care. Continued perinatal care and routine follow-up with a qualified pediatrician leads to identification of most lesions not identified prenatally.
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Complications

  • Syncope and sudden death
  • Polycythemia and hyperviscosity syndrome
  • Congestive heart failure
  • Dysrhythmia
  • Surgical complications
  • Brain abscess and stroke
  • Pulmonary infarction and hemorrhage
  • Hyperuricemia
  • Infective endocarditis
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Prognosis

  • This disease 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.
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Patient Education

  • Inform patients that diet and weight control are essential.
  • Educate patients to avoid smoking.
  • Provide an exercise prescription.
  • Advise abstinence from or moderate intake of alcohol.
  • Educate patients about contraception options and pregnancy risk.[41]
    • The mortality rate in pregnant patients with Eisenmenger syndrome is approximately 50%.
    • Contraception by means of tubal ligation (with subacute bacterial endocarditis [SBE] prophylaxis) may be recommended. Oral or implantable contraceptives might promote pulmonary infarction through activation of the coagulation cascade.
  • Educate patients about the signs and symptoms of polycythemia and hyperviscosity.
  • Inform patients of the importance of dental hygiene.
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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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