Original article
Pediatric cardiac
Ventricular Performance in Long-Term Survivors After Fontan Operation

https://doi.org/10.1016/j.athoracsur.2010.07.055Get rights and content

Background

Ventricular function and arrhythmia in patients with Fontan circulation in long-term follow-up are still unknown.

Methods

We retrospectively reviewed 48 patients who survived and were followed up for more than 15 years, among 110 patients who underwent Fontan operation in our institute from 1979 to 1992. Atriopulmonary connection was performed in 26 patients and total cavopulmonary connection in 22. The patients were categorized into right ventricle, left ventricle, and biventricle groups. Follow-up cardiac catheterization and exercise test were performed routinely every 5 years post surgery. Median age at Fontan operation was 5 years.

Results

Mean follow-up was 18.5 years. Cardiac index in the total cavopulmonary connection group was higher than in the atriopulmonary connection group at 10 and 15 years post surgery (p < 0.05). Ejection fraction in the left-ventricle group was higher than in the right-ventricle group. End-diastolic volume at 5, 10, and 15 years was significantly lower than at 1 year (p < 0.05). End-diastolic pressure at 10 years was significantly higher than at 1 and 5 years (p < 0.05). Beyond 15 years, 6 patients developed ventricular tachycardia. The only significant risk factors for the onset of ventricular tachycardia in a multivariate analysis were age at Fontan operation and absolute age (p < 0.05).

Conclusions

Long-term follow-up of patients demonstrated that postoperative ventricular systolic performance seemed to become steady. Ventricular tachycardia was detected 15 years post surgery, especially in older patients with older age at Fontan operation, possibly revealing a risk factor in the long-term postoperative period, thereby meriting further consideration.

Section snippets

Patients

Between October 1979 and May 1992, 110 patients underwent Fontan operations at our institution. There were 26 hospital deaths and 18 late deaths. Causes of hospital deaths were Fontan circulation failure due to ventricular dysfunction or pulmonary condition in 19 patients and noncardiac event in 7 patients. Causes of late deaths were Fontan circulation failure due to ventricular dysfunction in 4 patients, Fontan circulation failure due to pathway obstruction in 6 patients, intractable atrial

Results

Mean follow-up period after Fontan operation was 18.5 ± 3.2 years. Mean age at last follow-up was 24.8 ± 5.4 years.

A total of 4 patients, 1 in the APC group and 3 in the TCPC group, suffered from protein losing enteropathy. The patient in the APC group underwent Fontan conversion 14 years after APC-type Fontan operation, but protein losing enteropathy PLE persisted. As for thromboembolic events, 3 patients in the APC group suffered from brain infarction.

Of the 26 patients in the APC group, 15

Comment

In this study we analyzed the serial changes in parameters at cardiac catheterization and exercise test every 5 years in Fontan patients post surgery. We admit that the parameters measured did not reflect actual functional class because cardiac catheterization was performed with the patient under sedation [9]. Nonetheless, we could find the trend of cardiopulmonary parameters during the 15-year follow-up.

Cardiac index in the APC group began to decrease after 10 years post surgery. It was lower

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