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CLINICAL RESEARCH: CONGENITAL HEART DISEASE

Diagnostic assessment before Fontan operation in patients with bidirectional cavopulmonary anastomosis

Are noninvasive methods sufficient?

Pamela S. Ro, MD, Jack Rychik, MD, FACC^*, Meryl S. Cohen, MD, FACC^*, William T. Mahle, MD, FACC and Jonathan J. Rome, MD, FACC^*,*

^* Division of Cardiology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
Dr. Ro is currently affiliated with the Division of Cardiology, Columbus Children's Hospital, Columbus, Ohio, USA
Dr. Mahle is currently affiliated with Sibley Heart Center, Children's Health Care of Atlanta, Atlanta, Georgia, USA

Manuscript received March 18, 2003; revised manuscript received January 7, 2004, accepted February 24, 2004.

^* Reprint requests and correspondence: Dr. Jonathan J. Rome, Division of Cardiology, The Children's Hospital, of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, Pennsylvania 19104, USA.
rome{at}email.chop.edu

Presented at the 11th Annual Scientific Sessions of the American Society of Echocardiography, June 11 to 14, 2000, Chicago, Illinois.

OBJECTIVES: This study was designed to determine if a subset of patients who have undergone bidirectional cavopulmonary anastomosis could be identified in which catheterization was of little benefit before completion of the Fontan procedure.

BACKGROUND: Diagnostic evaluation before Fontan procedure has typically included cardiac catheterization. However, the overall management strategy for patients with functional single ventricle has evolved to include staging bidirectional cavopulmonary anastomosis in most, and it has become uncommon to exclude patients from Fontan based on catheterization data.

METHODS: Patients who underwent bidirectional cavopulmonary anastomosis and had complete echocardiograms and catheterizations within three months of each other between January 1992 and October 1997 were evaluated with a series of clinical and echocardiographic characteristics to identify a subset in whom catheterization was predicted to be of little added value ("no-cath" group). The predictive value and sensitivity of these criteria in excluding patients who required additional intervention, were excluded from Fontan, or died within 30 days of Fontan was determined.

RESULTS: A total of 99 patients who underwent bidirectional cavopulmonary anastomosis at 6.7 months (range 2.9 months to 14 years) were studied; 46 met criteria for the "no-cath" group. Noninvasive criteria stratified all patients who died (n = 5) or did not proceed to Fontan (n = 1) and 9 of 11 who required additional interventions to the "cath" group. Thus, the negative predictive value of these criteria was 93%.

CONCLUSIONS: Our data suggest that catheterization before Fontan could be avoided in a large percentage of patients without adversely affecting outcome; prospective evaluation of this strategy is warranted.

Abbreviations and Acronyms   AV = atrioventricular   NPV = negative predictive value   PPV = positive predictive value