title: Quality of Life of Pediatric Patients With Long QT Syndrome.
abstract: Children with long QT syndrome (LQTS) live with the risk of sudden death, activity restrictions, and the need for daily medications. We sought to evaluate the quality of life (QOL), self-perception, and behavior of patients with LQTS as perceived by both patients and their parents and identify predictors of lower QOL. QOL (Pediatric QOL Inventory [PedsQL] and Pediatric Cardiac Quality of Life Inventory [PCQLI]), self-perception, and behavioral inventories were completed by patients with LQTS and their parents. Comparison of PedsQL scores was made to published data for healthy children using t tests, and PCQLI scores were compared with those of patients with differing complexity of congenital heart disease. Mixed modeling was used for multivariable analysis. Sixty-one patients with LQTS were evaluated (age 13.6 ± 3.0 years; male 49%). Compared with healthy children, the PedsQL Total, Psychosocial, and Physical Health Summary scores were significantly lower for patients with LQTS and parent proxy reports (p ≤0.001). In general, PCQLI scores of patients with LQTS and parents were similar to those of patients with tetralogy of Fallot (p ≥0.2), lower than those of patients with bicuspid aortic valve (p ≤0.02), and higher than those of patients with single ventricle (p ≤0.03). Lower patient and parent PCQLI scores were associated with internalizing problems. For parents, the presence of a cardiac device and medication side effects were additionally associated with lower PCQLI scores. In conclusion, patients with LQTS and their parents report lower QOL than normal children secondary to physical and psychosocial factors. Increasing focus on the psychological well-being of these patients is needed in an effort to improve their QOL.
authors.affiliation: Division of Pediatric Cardiology, Department of Pediatrics, The Heart Institute at Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio. Electronic address: Richard.czosek@cchmc.org.
authors.forename: Richard J
authors.initials: RJ
authors.lastname: Czosek
authors.affiliation: Division of Pediatric Cardiology, Department of Pediatrics, Children's National Medical Center, Washington, DC.
authors.forename: Jonathan R
authors.initials: JR
authors.lastname: Kaltman
authors.affiliation: Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio; Division of Biostatistics, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.
authors.forename: Amy E
authors.initials: AE
authors.lastname: Cassedy
authors.affiliation: Division of Cardiology, Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
authors.forename: Maully J
authors.initials: MJ
authors.lastname: Shah
authors.affiliation: Division of Cardiology, Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
authors.forename: Victoria L
authors.initials: VL
authors.lastname: Vetter
authors.affiliation: Division of Pediatric Cardiology, Department of Pediatrics, the UCSF Benioff Children's Hospital, San Francisco, California.
authors.forename: Ronn E
authors.initials: RE
authors.lastname: Tanel
authors.affiliation: Department of Pediatrics, The Heart Program at Nicklaus Children's Hospital, Florida International University Herbert Wertheim College of Medicine, Miami, Florida.
authors.forename: Gil
authors.initials: G
authors.lastname: Wernovksy
authors.affiliation: Cardiorespiratory Department, Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom; Department of Pediatric Cardiology, Royal Brompton and Harefield NHS Foundation Trust, London, United Kingdom.
authors.forename: Jo
authors.initials: J
authors.lastname: Wray
authors.affiliation: Division of Cardiology, Department of Pediatrics, Ann & Robert F. Lurie Children's Hospital of Chicago, Chicago, Illinois.
authors.forename: Bradley S
authors.initials: BS
authors.lastname: Marino
mesh_terms: D000293:Adolescent; D002648:Child; D003430:Cross-Sectional Studies; D004562:Electrocardiography; D004644:Emotions / Q000502:physiology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008133:Long QT Syndrome / Q000503:physiopathology / Q000523:psychology*; D008297:Male; D011788:Quality of Life; D012649:Self Concept; D011795:Surveys and Questionnaires