BACKGROUND: After the Fontan procedure patients are at risk for reduced quality of life (QoL) and cognitive function. We aimed to assess these important factors in Danish Fontan patients and to compare the results with a group of healthy controls. METHODS: All Fontan patients living in Denmark were identified and invited to participate. QoL was evaluated using the Pediatric Quality of Life Inventory (PedsQL) version 4.0 generic core module in patients <16 years and the Short Form 36 questionnaire (SF-36) in patients ≥16 years. Cognitive function was evaluated in all patients ≥6 years using the Quick Test of Cognitive Speed. To evaluate if QoL correlated with exercise capacity, patients performed a symptom-limited bicycle test. RESULTS: 158 of 179 eligible patients (88%) consented to participate. Median age was 13.9 years (IQR: 10.2-19.3). PedsQL scores increased with age but were significantly lower among patients than among controls. SF-36 physical scores were significantly lower in patients compared to controls while psychosocial scores were similar. Cognitive speed was significantly reduced in patients at all ages compared to controls. No significant difference in PedsQL-/SF-36 scores or cognitive speed was found between hypoplastic left heart syndrome (HLHS) and non-HLHS Fontan patient. PedsQL-/SF-36 scores in patients ≥10 years correlated significantly to cognitive speed but not to peak exercise capacity. CONCLUSION: QoL is reduced in Fontan children compared to their healthy counterparts whereas in patients ≥16 years only physical, but not psychosocial QoL is reduced. Cognitive speed was significantly lower in patients at all ages compared to controls.
BACKGROUND: After the Fontan procedure patients are at risk for reduced quality of life (QoL) and cognitive function. We aimed to assess these important factors in Danish Fontan patients and to compare the results with a group of healthy controls. METHODS: All Fontan patients living in Denmark were identified and invited to participate. QoL was evaluated using the Pediatric Quality of Life Inventory (PedsQL) version 4.0 generic core module in patients <16 years and the Short Form 36 questionnaire (SF-36) in patients ≥16 years. Cognitive function was evaluated in all patients ≥6 years using the Quick Test of Cognitive Speed. To evaluate if QoL correlated with exercise capacity, patients performed a symptom-limited bicycle test. RESULTS: 158 of 179 eligible patients (88%) consented to participate. Median age was 13.9 years (IQR: 10.2-19.3). PedsQL scores increased with age but were significantly lower among patients than among controls. SF-36 physical scores were significantly lower in patients compared to controls while psychosocial scores were similar. Cognitive speed was significantly reduced in patients at all ages compared to controls. No significant difference in PedsQL-/SF-36 scores or cognitive speed was found between hypoplastic left heart syndrome (HLHS) and non-HLHS Fontan patient. PedsQL-/SF-36 scores in patients ≥10 years correlated significantly to cognitive speed but not to peak exercise capacity. CONCLUSION: QoL is reduced in Fontan children compared to their healthy counterparts whereas in patients ≥16 years only physical, but not psychosocial QoL is reduced. Cognitive speed was significantly lower in patients at all ages compared to controls.
Authors: Gali S Kolt; Barbara R Ferdman; Jessica Y Choi; Janine Henson; Van-Trang Nguyen; Emily A Farkas; Vinicius Jds Nina; Rachel Vah Nina; Renzo O CiFuentes; William F Zeman; John E Connett; Aubyn Marath Journal: Cardiol Young Date: 2020-02 Impact factor: 1.093
Authors: Karen Uzark; Victor Zak; Peter Shrader; Brian W McCrindle; Elizabeth Radojewski; James W Varni; Kaitlyn Daniels; Jill Handisides; Kevin D Hill; Linda M Lambert; Renee Margossian; Victoria L Pemberton; Wyman W Lai; Andrew M Atz Journal: J Pediatr Date: 2015-12-10 Impact factor: 4.406
Authors: Jill C Handisides; Danielle Hollenbeck-Pringle; Karen Uzark; Felicia L Trachtenberg; Victoria L Pemberton; Teresa W Atz; Timothy J Bradley; Elizabeth Cappella; Sylvia De Nobele; Georgeann Keh-Teng Groh; Michelle S Hamstra; Rosalind Korsin; Jami C Levine; Bergen Lindauer; Aimee Liou; Meghan K Mac Neal; Larry W Markham; Tonia Morrison; Kathleen A Mussatto; Aaron K Olson; Mary Ella M Pierpont; Reed E Pyeritz; Elizabeth A Radojewski; Mary J Roman; Mingfen Xu; Ronald V Lacro Journal: J Pediatr Date: 2018-09-27 Impact factor: 4.406
Authors: Andrew M Atz; Victor Zak; Lynn Mahony; Karen Uzark; Peter Shrader; Dianne Gallagher; Stephen M Paridon; Richard V Williams; Roger E Breitbart; Steven D Colan; Jonathan R Kaltman; Renee Margossian; Sara K Pasquali; Kerstin Allen; Wyman W Lai; Rosalind Korsin; Bradley S Marino; Nicole Mirarchi; Brian W McCrindle Journal: Congenit Heart Dis Date: 2014-06-17 Impact factor: 2.007
Authors: Clinton D Morgan; Michael S Wolf; Truc M Le; Chevis N Shannon; John C Wellons; Bret A Mettler Journal: Childs Nerv Syst Date: 2015-08-18 Impact factor: 1.475
Authors: Christina E Holbein; Nicholas D Fogleman; Kevin Hommel; Silke Apers; Jessica Rassart; Philip Moons; Koen Luyckx; Maayke A Sluman; Junko Enomoto; Bengt Johansson; Hsiao-Ling Yang; Mikael Dellborg; Raghavan Subramanyan; Jamie L Jackson; Werner Budts; Adrienne H Kovacs; Stacey Morrison; Martha Tomlin; Kathy Gosney; Alexandra Soufi; Katrine Eriksen; Corina Thomet; Malin Berghammer; Luis Alday; Edward Callus; Susan M Fernandes; Maryanne Caruana; Samuel Menahem; Stephen C Cook; Gwen R Rempel; Kamila White; Paul Khairy; Shelby Kutty; Gruschen Veldtman Journal: Congenit Heart Dis Date: 2018-01-03 Impact factor: 2.007