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Eur J Cardiothorac Surg 2005;28:529-535
© 2005 Elsevier Science NL

Original articles

Intermediate-term outcome following the fontan operation: a survival, functional and risk-factor analysis

Department of Congenital Heart Disease, Guy's Hospital, Guy's and St Thomas Hospitals NHS Trust, St Thomas Street, London SE1 9RT, UK

Received 10 December 2004; received in revised form 10 May 2005; accepted 28 June 2005.

^* Corresponding author. Address: UCSF Department of Surgery, Division of Pediatric Cardiothoracic Surgery, 513 Parnassus Avenue, Room S-549, Box 0117, San Francisco, CA 94143-0117, USA. Tel.: +1 415 476 3437; fax: +1 415 476 9678. (Email: nelson.a{at}bigfoot.com).

Abstract

Objective: To investigate survival and risk factors influencing intermediate outcome after the Fontan procedure. Methods: Retrospective analysis of 122 patients operated between April 1991 and September 2002. Poor outcome was defined as late death or poor functional status (intractable supraventricular arrhythmias/NYHA 3–4) necessitating revision surgery. Results: 64(52%) patients had an intermediate bi-directional cavo-pulmonary shunt (BCPS). 91 (76%) patients had a lateral tunnel total cavo-pulmonary connection, 21(17%) patients had an atrio-pulmonary connection and 10 (8%)patients had a Kawashima connection. There were 6 (5%)early deaths. Over a median follow up of 54 months (1–133), 12 (10%)patients have had surgical revision for poor functional status. There were 7 (6%) late deaths, 5 of which occurred after revision surgery. Univariate analysis identified older age at operation (>4 years) (P=0.04), higher postoperative pulmonary artery pressure at 24h (P=0.012), arrhythmia postoperatively (P=0.03) or during follow-up (P=0.01) and the requirement for anticoagulation during follow-up (P=0.03) as significant predictors of poorer outcome. Patients who had an intermediate BCPS (P=0.002) or Norwood Stage 1 (P=0.05) had a better outcome. Multivariate analysis identified an intermediate Glenn shunt and lower postoperative pulmonary artery pressure as significant predictors of better outcome. Actuarial freedom from death or revision is 93% (±2), 88% (±3), 86% (±4) and 69% (±7) at 1, 5, 7 and 9 years respectively. Actuarial freedom from death or revision for the lateral tunnel group is 92% (±2), 89% (±3), 85% (±5) and 66% (±10) respectively. Conclusion: Atrio-pulmonary connection results in a higher incidence of arrhythmias and failure than the lateral tunnel Fontan. Even in patients with a lateral tunnel Fontan there is a continuing hazard phase in the intermediate term. Mid-term outcome appears to be favorably influenced by an intermediate BCPS.

Key Words: Fontan • Lateral tunnel • Mid-term

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