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Applying heart failure guidelines to adult congenital heart disease patients

Robert E Shaddy1, Gary Webb

  • 1Division of Cardiology, The Children's Hospital of Philadelphia, and University of Pennsylvania School of Medicine, 34th Street and Civic Center Blvd, Philadelphia, PA 19104-4399, USA. shaddyr@email.chop.edu

Expert Review of Cardiovascular Therapy
|February 6, 2008
PubMed

Insights

Adults with repaired congenital heart defects often have heart dysfunction and exercise intolerance. Current heart failure guidelines may apply to those with a systemic left ventricle, but not those without.

Area of Science:

  • Cardiology
  • Adult Congenital Heart Disease
  • Heart Failure

Background:

  • A growing population of adults with congenital heart defects (CHDs) requires ongoing cardiovascular care.
  • Many adults with repaired CHDs experience myocardial dysfunction, exercise intolerance, and heart failure.
  • Neurohormonal activation in these patients mirrors that seen in acquired heart failure.

Purpose of the Study:

  • To review the current state of heart failure management in adults with CHDs.
  • To identify patient groups at highest risk for heart failure progression.
  • To highlight the need for evidence-based therapeutic strategies for specific CHD populations.

Main Methods:

  • Review of existing literature and clinical guidelines for heart failure management.
  • Analysis of patient populations with CHDs, categorizing by ventricular support (systemic left vs. non-left ventricle).
  • Assessment of exercise intolerance as a predictor of adverse outcomes.

Main Results:

  • Patients without a systemic left ventricle (e.g., Mustard/Senning repairs for transposition of the great arteries, Fontan procedure) are at the highest risk for heart failure.
  • Exercise intolerance is a significant predictor of hospitalization and mortality in these high-risk CHD populations.
  • Established heart failure guidelines for acquired heart disease may be applicable to CHD patients with a systemic left ventricle.

Conclusions:

  • Evidence-based therapeutic guidelines are lacking for adult CHD patients without a systemic left ventricle.
  • Further research is needed to develop tailored treatment strategies for patients with single ventricle physiology or systemic right ventricles.
  • Optimizing management for adults with CHDs requires addressing both ventricular function and systemic circulatory challenges.

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