Predictors of long-term adverse outcomes in patients with congenital coronary artery fistulae

Anne Marie Valente, James E. Lock, Kimberlee Gauvreau, Elizabeth Rodriguez-Huertas, Caitlyn Joyce, Laurie Armsby, Emile A. Bacha, Michael J. Landzberg

Research output: Contribution to journalArticlepeer-review

93 Scopus citations


Background:Significant morbidities, including angina, symptomatic heart failure, and myocardial infarction, have been reported after coronary artery fistula (CAF) closure; however, predictors that may be associated with adverse outcomes have not been established. The goal of this investigation is to describe the long-term outcomes witnessed in patients with either treated or untreated CAF at our institution and to investigate whether certain features predicted adverse outcomes. Methods and Results:The records and angiograms of patients with CAF who underwent a diagnostic cardiac catheterization at Children's Hospital Boston from 1959 through 2008 were reviewed. Of 76 patients identified, 20% were associated with additional congenital heart disease. Forty-four underwent transcatheter closure, 20 underwent surgical repair, and no intervention was performed in the remaining 12 subjects. Three patients who had initially undergone surgical closure had a second intervention, 1 underwent repeat surgery, and 2 underwent transcatheter closure. One patient who had undergone transcatheter closure underwent a second transcatheter closure for residual fistula. Major complications, including myocardial infarction, angina with coronary thrombosis, and symptomatic cardiomyopathy, occurred in 11 (15%) patients. The sole angiographic feature that was predictive of adverse outcome was drainage of the CAF into the coronary sinus (P<0.001). Clinical predictors associated with adverse outcomes included older age at diagnosis (P<0.001), tobacco use (P=0.006), diabetes (P=0.05), systemic hypertension (P<0.001), and hyperlipidemia (P<0.001). Conclusions:Long-term complications of CAF closure may include coronary thrombosis, myocardial infarction, and cardiomyopathy. CAF that drain into the coronary sinus are at particularly high-risk of long-term morbidities after closure, and strategies including long-term anticoagulation should be considered.

Original languageEnglish (US)
Pages (from-to)134-139
Number of pages6
JournalCirculation: Cardiovascular Interventions
Issue number2
StatePublished - Apr 2010


  • Complication
  • Congenital
  • Coronary disease
  • Fistula

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine


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