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Outcome and assessment after the modified Fontan procedure for hypoplastic left heart syndrome

P E Farrell1, A C Chang, K A Murdison

  • 1Division of Cardiology, Children's Hospital of Philadelphia, PA 19104.

Circulation
|January 11, 1992
PubMed

Insights

Survival rates after the modified Fontan procedure for hypoplastic left heart syndrome (HLHS) were comparable to other complex heart conditions. The right ventricle as a systemic ventricle showed excellent intermediate results in HLHS survivors.

Area of Science:

  • Pediatric Cardiology
  • Congenital Heart Surgery
  • Cardiac Physiology

Background:

  • Study reviews outcomes of 76 patients with hypoplastic left heart syndrome (HLHS) undergoing modified Fontan procedures.
  • Patients aged 5 months to 6 years received palliative surgery before the Fontan procedure between 1984 and 1989.

Purpose of the Study:

  • To evaluate the outcomes of modified Fontan procedures in HLHS patients.
  • To assess the intermediate results of using the right ventricle as the systemic ventricle in HLHS survivors.

Main Methods:

  • Modified Fontan procedures included transatrial baffle (n=10) or inferior vena cava baffle with pulmonary artery augmentation (n=66).
  • Actuarial survival rates were calculated at 1 month, 1, 2, and 4 years.
  • Postoperative cardiac catheterization was performed on survivors to assess hemodynamic values.

Main Results:

  • Actuarial survival rates were 74% (1 month), 58% (12 months), 56% (2 years), and 52% (4 years).
  • Hemodynamic values in 25 catheterized survivors showed a cardiac index of 2.8 +/- 0.6 l/min/m2 and right arterial pressure of 11 +/- 2 mm Hg.
  • No significant tricuspid or native pulmonary valve insufficiency was observed.

Conclusions:

  • Survival after the Fontan procedure for HLHS is comparable to other complex congenital heart lesions.
  • The right ventricle as a systemic ventricle yielded excellent intermediate results in HLHS patients who survived reconstructive and Fontan surgery.
Abstract

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