Tacy E Downing1, Kiona Y Allen2, David J Goldberg2, Lindsay S Rogers2, Chitra Ravishankar2, Jack Rychik2, Stephanie Fuller2, Lisa M Montenegro2, James M Steven2, Matthew J Gillespie2, Jonathan J Rome2, Thomas L Spray2, Susan C Nicolson2, J William Gaynor2, Andrew C Glatz1. 1. From the Division of Pediatric Cardiology (T.E.D., D.J.G., C.R., J.R., M.J.G., J.J.R., A.C.G.), Department of Surgery (S.F., T.L.S., J.W.G.), and Department of Anesthesiology and Critical Care (L.M.M., J.M.S., S.C.N.), The Perelman School of Medicine at the University of Pennsylvania, and Cardiac Center at The Children's Hospital of Philadelphia; Division of Cardiology, University of Texas, Houston (L.S.R.); and Division of Critical Care, Lurie Children's Hospital, Chicago, IL (K.Y.A.). tacy.downing@mch.com glatz@email.chop.edu. 2. From the Division of Pediatric Cardiology (T.E.D., D.J.G., C.R., J.R., M.J.G., J.J.R., A.C.G.), Department of Surgery (S.F., T.L.S., J.W.G.), and Department of Anesthesiology and Critical Care (L.M.M., J.M.S., S.C.N.), The Perelman School of Medicine at the University of Pennsylvania, and Cardiac Center at The Children's Hospital of Philadelphia; Division of Cardiology, University of Texas, Houston (L.S.R.); and Division of Critical Care, Lurie Children's Hospital, Chicago, IL (K.Y.A.).
Abstract
BACKGROUND: There are limited follow-up studies examining surgical and catheter-based reinterventions in long-term survivors of the Fontan operation. METHODS AND RESULTS: All 773 patients who underwent Fontan at our institution between 1992 and 2009 were retrospectively reviewed. Current information regarding post-Fontan intervention was available for 70%. By 20 years after Fontan, 65% of patients had experienced either surgical or transcatheter intervention. The median time to first reintervention was 9.8 years. Freedom from reoperation was 69% at 15 years and 63% at 20 years. The most common operations were pacemaker placement and Fontan revision. Risk factors for pacemaker placement included systemic left ventricle (hazard ratio [HR], 2.2; P=0.006) and lateral tunnel Fontan (HR, 4.3; P=0.001). Freedom from interventional catheterization was 53% at 15 years and 50% at 20 years. The most common procedures performed were fenestration closure and pulmonary artery intervention. Catheter intervention for anatomic indications was associated with Fontan after 2002 (HR, 2.1; P=0.007), Norwood operation (HR, 2.3; P=0.001), and longer cardiopulmonary bypass time (HR, 1.1 per 10 minutes; P=0.001). Catheter intervention for physiological indications was associated with prolonged post-Fontan pleural drainage (HR, 4.0; P<0.001) and hypoplastic left heart syndrome (HR, 2.0; P=0.01). CONCLUSIONS: In this study of Fontan survivors, two thirds of patients required surgical or catheter-based reintervention by 20 years. Families should be counseled that the Fontan is typically not the final stage of single-ventricle palliation.
BACKGROUND: There are limited follow-up studies examining surgical and catheter-based reinterventions in long-term survivors of the Fontan operation. METHODS AND RESULTS: All 773 patients who underwent Fontan at our institution between 1992 and 2009 were retrospectively reviewed. Current information regarding post-Fontan intervention was available for 70%. By 20 years after Fontan, 65% of patients had experienced either surgical or transcatheter intervention. The median time to first reintervention was 9.8 years. Freedom from reoperation was 69% at 15 years and 63% at 20 years. The most common operations were pacemaker placement and Fontan revision. Risk factors for pacemaker placement included systemic left ventricle (hazard ratio [HR], 2.2; P=0.006) and lateral tunnel Fontan (HR, 4.3; P=0.001). Freedom from interventional catheterization was 53% at 15 years and 50% at 20 years. The most common procedures performed were fenestration closure and pulmonary artery intervention. Catheter intervention for anatomic indications was associated with Fontan after 2002 (HR, 2.1; P=0.007), Norwood operation (HR, 2.3; P=0.001), and longer cardiopulmonary bypass time (HR, 1.1 per 10 minutes; P=0.001). Catheter intervention for physiological indications was associated with prolonged post-Fontan pleural drainage (HR, 4.0; P<0.001) and hypoplastic left heart syndrome (HR, 2.0; P=0.01). CONCLUSIONS: In this study of Fontan survivors, two thirds of patients required surgical or catheter-based reintervention by 20 years. Families should be counseled that the Fontan is typically not the final stage of single-ventricle palliation.
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