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Surgical techniques, patient management improve Fontan operation survival rates
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Improved surgical techniques and better patient management for patients undergoing a modified Fontan operation over the past 40 years have increased long-term survival, according to data from a retrospective analysis published in the Journal of the American College of Cardiology.
“However, development of [protein-losing enteropathy] and arrhythmias and the need for reoperation during long-term follow-up pose significant management challenges,” Kavitha N. Pundi, MD, from the division of pediatric cardiology, Mayo Clinic, Rochester, Minnesota, and colleagues wrote.
Pundi and colleagues reviewed survival data among patients who had an initial Fontan operation performed at Mayo Clinic (n = 1,052; 61% men) between 1973 and 2012. The mean age was 9.4 years at the initial procedure and mean follow-up was 15 years.
Overall survival was 74% at 10 years, 61% at 20 years and 43% at 30 years. Results from multivariate analysis indicated associations between decreased overall survival rates and the following factors:
- preoperative diuretic use (HR = 1.86; 95% CI, 1.46-2.37);
- longer cardiopulmonary bypass time (HR = 1.14; 95% CI, 1.07-1.22 per 30-minute increase);
- post-bypass Fontan pressure above 20 mm Hg (HR = 2.14; 95% CI, 1.63-2.81); and
- post-bypass left atrial pressure above 13 mm Hg (HR = 1.84; 95% CI, 1.4-2.42).
Among 932 patients who survived 30 days after the Fontan operation, the survival rates were 83% at 10 years; 68% at 20 years and 48% at 30 years. Factors associated with late survival on multivariate analysis included:
- atrioventricular valve replacement at the time of the Fontan procedure (HR = 4.02; 95% CI, 2.07-7.8);
- post-bypass left atrial pressure above 13 mm Hg (HR = 2.05; 95% CI, 1.48-2.82);
- postoperative ventricular arrhythmia (HR = 1.79; 95% CI, 1.18-2.72) or renal insufficiency (HR = 2.49; 95% CI, 1.74-3.58);
- protein-losing enteropathy (HR = 1.97; 95% CI, 1.48-2.63); and
- chest tube duration of 21 days or longer (HR = 1.15; 95% CI, 1.05-1.26).
Factors associated with improved survival included undergoing surgery later than 1991 and having preoperative or intraoperative sinus rhythm, the researchers wrote.
Patients required one or more reoperations in 17% of cases. The most common types of reoperations performed were pacemaker insertion/revision (20% of patients), Fontan revision/conversion (11%) and atrioventricular valve repair/replacement (5%).
Late atrial or ventricular arrhythmias occurred in 44% of patients, and 9% developed protein-losing enteropathy. Among those with protein-losing enteropathy, the mortality rate was 72%, the researchers wrote.
“Although the Fontan operation may represent ‘definitive palliation’ for many patients with single ventricle physiology, it is by no means a ‘cure’ or ‘repair,’” Frank Cetta Jr., MD, of the Mayo Clinic, Rochester, Minnesota, told Cardiology Today. “These patients demand lifelong meticulous follow-up at centers equipped to handle their multifactorial medical problems.”
In the future, Cetta noted that use of pulmonary vasodilator medications and improved imaging of liver disease after Fontan operations may impact post-Fontan survival and decrease morbidity. Cetta added that “imaging of liver disease after Fontan [operations] is improving, but treatment for liver disease needs further refinement.” – by Trish Shea, MA
Disclosure: One researcher reports consulting for Medtronic. Cetta and the other researchers report no relevant financial disclosures.
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