eMedicine Specialties > Cardiology > Congenital Heart Disease in the Adult

Eisenmenger Syndrome: Follow-up

Author: Mikhael F El-Chami, MD, Fellow, Department of Cardiology, Emory University School of Medicine
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
Contributor Information and Disclosures

Updated: Jun 5, 2008

Follow-up

Further Inpatient Care

  • Transfer patient to a specialized cardiology unit.
  • Monitor patient in an intensive care unit.

Further Outpatient Care

  • Patient should follow up at a specialized cardiology clinic within 2 weeks of discharge, and every 3 months when stable.

Deterrence/Prevention

  • Educate patients regarding infective endocarditis risk reduction.
  • Avoid situations that exacerbate vasodilation.
  • Avoid abrupt, strenuous, or isometric exercise.
  • Restrict activities that may result in further oxygen desaturation, symptomatic arrhythmias, or right ventricular dysfunction.
  • Stop steady-state exercise at onset of symptoms.

Complications

  • Hematological complications include hyperviscosity syndromes related to secondary erythrocytosis and bleeding diatheses.
  • Nervous system complications include brain abscess, transient cerebral ischaemia, thrombotic stroke, and intracerebral hemorrhage.
  • Hyperbilirubinemia increases the risk of gallstones.
  • Hyperuricemia can cause nephrolithiasis and secondary gout.
  • Hypertrophic osteoarthropathy causes bone pain and tenderness.
  • Reports document transient visual loss related to peripheral retinal microvascular abnormalities.

Prognosis

  • Survival is limited by complications and deteriorating ventricular function.
  • Sudden death always is a threat.

Miscellaneous

Medicolegal Pitfalls

  • Avoid significant reductions in SVR.
    • Lowering systemic arterial blood pressure can cause syncope.
    • A reduced SVR increases right-to-left shunting and worsens systemic arterial oxygen saturation.
  • Remember to administer antibiotic prophylaxis for infective endocarditis according to the recommendations of the American Heart Association.
  • Advise unequivocally against pregnancy; provide adequate contraceptive advice and genetic counseling.
    • Pregnancy carries a maternal mortality rate of approximately 50%.
    • Intrauterine devices are not recommended due to the risk of infection and bleeding.
    • Congenital cardiac abnormalities occur in approximately 10% of offspring.

Special Concerns

  • Maternal considerations with pregnancy
    • Despite the fact that more women with congenital heart disease than ever before are reaching reproductive age, maternal mortality rates in patients with congenital heart disease have not improved in the last 50 years and pregnancy is absolutely contraindicated in the Eisenmenger syndrome.
    • Although the maternal mortality rate is reported to range from 23-52% in different series, most experienced physicians estimate that the mortality rate is in excess of 50%.
    • The most critical time is postpartum, and the majority of deaths occur in the first week.
    • Factors that increase the risk of a peripartum death include congestive heart failure, sudden increases in pulmonary vascular resistance or decreases in SVR, bleeding/anemia, hematocrit greater than 60%, oxygen saturation less than 80%, and syncope.
    • Excessive straining should be avoided during the second stage of labor. Therefore, assisted delivery usually is recommended.
    • Caesarian delivery carries a higher mortality rate and should be reserved for obstetric indications, such as cephalopelvic disproportion.
    • The use of anticoagulants is controversial. The rationale for anticoagulation is that the risk of clotting during pregnancy is increased when associated with preexisting cyanosis. However, reports indicate that anticoagulation has contributed to mortality in several patients.
    • If anticoagulants are used, a suggested protocol is heparin until 12 hours predelivery, then warfarin from 48 hours postdelivery to the end of the puerperium. At the minimum, ensure proper leg care, use of elastic bandages, sufficient hydration, and early mobilization to prevent deep venous thrombosis.
  • Fetal consideration with pregnancy
    • The main risks to the fetus include arterial oxygen desaturation, hypoxemia, and polycythemia
    • The fetal mortality rate ranges from 7.8-28.0%.
    • Only 15% of babies are born at term.
  • Flying on commercial airplanes
    • Flying in patients with Eisenmenger syndrome carries the following risk:
      • Deep venous thrombosis, especially since this group of patients is predisposed to thrombotic events
      • Compromised oxygen delivery at high altitude
    • A study comparing air travel history over the past decade in 53 patients with Eisenmenger syndrome 48 patients with acyanotic found no major adverse events in either group. One patient in the Eisenmenger group had a probable transient ischemic attack (TIA) and one required supplemental oxygen after exposure to ambient cigarette smoke in flight.19
    • This study revealed that patients with Eisenmenger syndrome fly frequently and safely.
    • Nevertheless, it is recommended that patients avoid dehydration and inactivity during travel.
 
Acknowledgments

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.



More on Eisenmenger Syndrome

Overview: Eisenmenger Syndrome
Differential Diagnoses & Workup: Eisenmenger Syndrome
Treatment & Medication: Eisenmenger Syndrome
Follow-up: Eisenmenger Syndrome
Multimedia: Eisenmenger Syndrome
References
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Further Reading

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Keywords

Eisenmenger complex, Eisenmenger defect, Eisenmenger disease, Eisenmenger tetralogy, exercise intolerance, cyanosis, heart failure, hemoptysis, ventricular septal defect, VSD, overriding aorta, congenital cardiac shunt defect, pulmonary hypertension, patent ductus arteriosus, PDA, large congenital cardiac left-to-right shunts, surgically created extracardiac left-to-right shunts, increased pulmonary blood flow, transposition of the great arteries, atrial septal defect, persistent truncus arteriosus, unrestricted pulmonary blood flow, common atrioventricular canal, Blalock-Taussig anastomosis, Waterston shunt, Potts shunt, chronic cyanotic heart disease, large nonrestrictive ventricular septal defect, nonrestrictive patent ductus arteriosus

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Contributor Information and Disclosures

Author

Mikhael F El-Chami, MD, Fellow, Department of Cardiology, Emory University School of Medicine
Mikhael F El-Chami, MD is a member of the following medical societies: Alpha Omega Alpha and American College of Cardiology
Disclosure: Nothing to disclose.

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.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Marschall S Runge, MD, PhD, Charles and Anne Sanders Distinguished Professor of Medicine, Chairman of Medicine, Vice Dean for Clinical Affairs, Chairman, Department of Medicine, 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

CME Editor

Amer Suleman, MD, Consultant in Electrophysiology and Cardiovascular Medicine, Department of Internal Medicine, Division of Cardiology, Medical City Dallas Hospital
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.

 
 
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