Tacy E Downing1, Kiona Y Allen1, Andrew C Glatz1, Lindsay S Rogers2, Chitra Ravishankar1, Jack Rychik1, Jennifer A Faerber3, Stephanie Fuller4, Lisa M Montenegro5, James M Steven5, Thomas L Spray4, Susan C Nicolson5, J William Gaynor4, David J Goldberg6. 1. Division of Pediatric Cardiology, The Cardiac Center at The Children's Hospital of Philadelphia, Philadelphia, Pa. 2. Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio. 3. Department of Pediatrics, The Cardiac Center at The Children's Hospital of Philadelphia, Philadelphia, Pa. 4. Department of Surgery, The Cardiac Center at The Children's Hospital of Philadelphia, Philadelphia, Pa. 5. Department of Anesthesiology and Critical Care, The Cardiac Center at The Children's Hospital of Philadelphia, Philadelphia, Pa. 6. Division of Pediatric Cardiology, The Cardiac Center at The Children's Hospital of Philadelphia, Philadelphia, Pa. Electronic address: goldbergda@email.chop.edu.
Abstract
OBJECTIVE: Existing studies of patients palliated with the Fontan operation are limited by heterogeneous patient populations and incomplete follow-up. This study aimed to describe long-term post-Fontan survival in a modern patient cohort. METHODS: All 773 patients who underwent a first Fontan operation at our institution between 1992 and 2009 were reviewed. The primary outcome was the composite endpoint of Fontan takedown, heart transplantation, or death before 2013. RESULTS: Follow-up rate was 99.2%. Survival with intact Fontan circulation was 94% at 1 year (95% confidence interval [95% CI], 92%-95%), 90% at 10 years (95% CI, 88%-92%), 85% at 15 years (95% CI, 82%-88%), and 74% at 20 years (95% CI, 67%-80%). Distinct risk factors were identified for early (≤1 year) and late composite outcomes. Independent risk factors for early outcome included prolonged pleural drainage (hazard ratio [HR], 4.4; P < .001), intensive care unit stay >1 week (HR, 2.4; P < .001), Fontan before 1997 (HR, 3.3; P < .001), preoperative atrioventricular valve regurgitation (HR, 2.0; P < .001), and longer crossclamp time (HR, 1.3 per 10 minutes; P < .001). Late outcome was predicted by atrioventricular valve regurgitation prior to Fontan (HR, 2.0; P ≤ .001), and post-Fontan ICU stay >1 week (HR, 2.4; P < .001). CONCLUSIONS: Long-term mortality after Fontan operation remains substantial. Risk factors for death or loss of Fontan circulation differ between the early and late postoperative periods. Long-term survival has not improved appreciably over the last decade, suggesting that alternatives to the Fontan are warranted.
OBJECTIVE: Existing studies of patients palliated with the Fontan operation are limited by heterogeneous patient populations and incomplete follow-up. This study aimed to describe long-term post-Fontan survival in a modern patient cohort. METHODS: All 773 patients who underwent a first Fontan operation at our institution between 1992 and 2009 were reviewed. The primary outcome was the composite endpoint of Fontan takedown, heart transplantation, or death before 2013. RESULTS: Follow-up rate was 99.2%. Survival with intact Fontan circulation was 94% at 1 year (95% confidence interval [95% CI], 92%-95%), 90% at 10 years (95% CI, 88%-92%), 85% at 15 years (95% CI, 82%-88%), and 74% at 20 years (95% CI, 67%-80%). Distinct risk factors were identified for early (≤1 year) and late composite outcomes. Independent risk factors for early outcome included prolonged pleural drainage (hazard ratio [HR], 4.4; P < .001), intensive care unit stay >1 week (HR, 2.4; P < .001), Fontan before 1997 (HR, 3.3; P < .001), preoperative atrioventricular valve regurgitation (HR, 2.0; P < .001), and longer crossclamp time (HR, 1.3 per 10 minutes; P < .001). Late outcome was predicted by atrioventricular valve regurgitation prior to Fontan (HR, 2.0; P ≤ .001), and post-Fontan ICU stay >1 week (HR, 2.4; P < .001). CONCLUSIONS: Long-term mortality after Fontan operation remains substantial. Risk factors for death or loss of Fontan circulation differ between the early and late postoperative periods. Long-term survival has not improved appreciably over the last decade, suggesting that alternatives to the Fontan are warranted.
Authors: Jiyong Moon; Li Shen; Donald S Likosky; Vikram Sood; Reilly D Hobbs; Peter Sassalos; Jennifer C Romano; Richard G Ohye; Edward L Bove; Ming-Sing Si Journal: J Am Coll Cardiol Date: 2020-07-28 Impact factor: 24.094
Authors: David J Goldberg; Victor Zak; Bryan H Goldstein; Brian W McCrindle; Shaji C Menon; Kurt R Schumacher; R Mark Payne; Jonathan Rhodes; Kimberly E McHugh; Daniel J Penny; Felicia Trachtenberg; Michelle S Hamstra; Marc E Richmond; Peter C Frommelt; Matthew D Files; James L Yeager; Victoria L Pemberton; Mario P Stylianou; Gail D Pearson; Stephen M Paridon Journal: Am Heart J Date: 2018-04-03 Impact factor: 4.749
Authors: David J Goldberg; Victor Zak; Bryan H Goldstein; Kurt R Schumacher; Jonathan Rhodes; Daniel J Penny; Christopher J Petit; Salil Ginde; Shaji C Menon; Seong-Ho Kim; Gi Beom Kim; Todd T Nowlen; Michael V DiMaria; Benjamin P Frischhertz; Jonathan B Wagner; Kimberly E McHugh; Brian W McCrindle; Amanda J Shillingford; Arash A Sabati; Anji T Yetman; Anitha S John; Marc E Richmond; Matthew D Files; R Mark Payne; Andrew S Mackie; Christopher K Davis; Shabana Shahanavaz; Kevin D Hill; Ruchira Garg; Jeffrey P Jacobs; Michelle S Hamstra; Stacy Woyciechowski; Kathleen A Rathge; Michael G McBride; Peter C Frommelt; Mark W Russell; Elaine M Urbina; James L Yeager; Victoria L Pemberton; Mario P Stylianou; Gail D Pearson; Stephen M Paridon Journal: Circulation Date: 2019-11-17 Impact factor: 29.690
Authors: David J Goldberg; Victor Zak; Brian W McCrindle; Hua Ni; Russell Gongwer; Jonathan Rhodes; Robert P Garofano; Jonathan R Kaltman; Linda M Lambert; Lynn Mahony; Renee Margossian; Zebulon Z Spector; Richard V Williams; Andrew M Atz; Stephen M Paridon Journal: Pediatr Cardiol Date: 2020-09-25 Impact factor: 1.655
Authors: C G Stevens; J Kay; K Pickett; K Campbell; A D Khanna; E Yeung; S D Miyamoto; R M Jacobsen Journal: Pediatr Cardiol Date: 2021-06-18 Impact factor: 1.655