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Clinical outcome of the Fontan operation in patients with impaired ventricular function

^a Department of Cardiovascular Surgery, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, 2-5-1 Shikata, Okayama City, Okayama 700-8558, Japan
^b Department of Pediatrics, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, 2-5-1 Shikata, Okayama City, Okayama 700-8558, Japan
^c Cardiac Care Unit, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, 2-5-1 Shikata, Okayama City, Okayama 700-8558, Japan

Received 15 September 2008; received in revised form 14 April 2009; accepted 17 April 2009.

^_* Corresponding author. Tel.: +81 86 235 7359; fax: +81 86 235 7431. (Email: s_sano{at}cc.okayama-u.ac.jp).

Objective: Although a staged Fontan strategy allows for an excellent outcome in high-risk patients, an impaired ventricular function remains a significant factor of early/late mortality and morbidity. This study evaluated the clinical outcome of the Fontan operation in patients with impaired ventricular function. Methods: A retrospective review was performed on 217 patients who had undergone the Fontan operation between 1991 and 2007. Results: Twenty-nine (13%) of the 217 patients had an impaired ventricular function (ejection fraction (EF) <50%). The median age at the time of the operation was 3 (range: 1–31 years) years. There were five adult patients. The ventricular morphology was right in 20 patients (including five hypoplastic left heart syndrome (HLHS)) and others (left and two-ventricle) in nine patients. Heterotaxy syndrome was present in eight patients. Previous surgical interventions included bidirectional Glenn anastomoses in 24, modified Blalock–Taussig shunts in two and pulmonary artery banding in two. The preoperative EF was 43 ± 6%. Significant (moderate or severe) atrioventricular valve regurgitation was noted in four patients. The percutaneous oxygen saturation (SpO₂) was 82 ± 5%. The pulmonary artery pressure and pulmonary artery index were 11 ± 3 mmHg and 296 ± 102 mm² m^–2, respectively. All 29 patients underwent the Fontan operation without any early mortality. There were two late mortalities and two re-operations. EF was maintained at 59 ± 15% at a median follow-up of 7.5 (range: 1–19) years. The percent normal systemic ventricular end-diastolic volume decreased from 174 ± 95% to 124 ± 39% (p < 0.05). The SpO₂ increased to 92 ± 2%. The mean cardiothoracic ratio in chest X-ray and B-type natriuretic peptide were 51% (range: 35–68%) and 22 pg ml^–1 (range: 9–382 pg ml^–1), respectively. Three patients developed congestive heart failure, seven had arrhythmia and two developed protein-losing enteropathy. The New York Heart Association (NYHA) class functional class is I in 21 patients, II in five and III in one. Conclusions: Acceptable clinical outcomes were observed at an intermediate follow-up of the Fontan operation in patients with an impaired ventricular function.

Key Words: Fontan operation • Single ventricle • Ventricular function • Clinical outcome