Functional health status in children and adolescents after Fontan: comparison of generic and disease-specific assessments

Abstract

Purpose The aim of this study was to compare associations between generic versus disease-specific functional health status assessments and patient and clinical characteristics for patients with severe congenital heart disease. Methods This was a cross-sectional observational study involving 325 single ventricle patients, aged 10–18 years, after Fontan procedure. Enrolled patients underwent a medical history review, laboratory testing, and assessment of the functional health status by completion of the generic Child Report Child Health Questionnaire and the disease-specific Congenital Heart Adolescent and Teenage questionnaire. Correlated conceptually equivalent domains from both questionnaires were identified and their associations with patient and clinical variables were compared. Results From the generic assessment, patients perceived marginally lower physical functioning (p = 0.05) but greater freedom from bodily pain compared with a normal population (p < .001). The equivalent physical functioning/limitations domain of the generic instrument, compared with the disease-specific instrument, had similar associations (higher multi-variable model R2) with medical history variables (R2 = 0.14 versus R2 = 0.12, respectively) and stronger associations with exercise testing variables (R2 = 0.22 versus R2 = 0.06). Similarly, the corresponding freedom from bodily pain/symptoms domains from both questionnaires showed a greater association for the generic instrument with medical history variables (R2 = 0.15 versus R2 5 0.09, respectively) and non-cardiac conditions (R2 = 0.13 versus R2 = 0.06). The associations of each questionnaire with echocardiographic results, cardiac magnetic resonance imaging results, and serum brain natriuretic peptide levels were uniformly weak (R2 range <.01 to 0.04). Conclusions Assessment of the physical functional health status using generic and disease-specific instruments yields few differences with regard to associations between conceptually similar domains and patient and clinical characteristics for adolescents after Fontan procedure.

40-Year Follow-Up After the Fontan Operation

“…However, this is consistent with previously reported data suggesting that patients tend to perceive themselves as having a higher functional status compared with control populations (41). However, this is consistent with previously reported data suggesting that patients tend to perceive themselves as having a higher functional status compared with control populations (41).…”

Self-perception of health and social determinants in high school adolescents

“…However, nearly one-third reported to have a health problem. These data demonstrate that, as shown in previous studies 16,17 , self-perception does not necessarily depend on the actual state of health. The literature also shows that the adolescent can interpret "good health" simply as being physically well 18 .Thus, the definition of health for adolescents does not necessarily include their perceptions of general well-being or a broader health-disease process.…”

Endothelial Function and Arterial Stiffness Relate to Functional Outcomes in Adolescent and Young Adult Fontan Survivors

“…Although a majority of Fontan patients survive to adolescence and adulthood, the early onset of symptomatic functional decline is common. Multisystem organ dysfunction, or “failing Fontan physiology,” arises from chronic exposure to low cardiac output and high central venous pressure and results in impairment of critical functional outcomes including exercise capacity and quality of life (QOL) . Essential to reducing morbidities and improving Fontan outcomes is the identification of novel biomarkers that are predictive of the later development of failing Fontan physiology.…”

“…Multisystem organ dysfunction, or "failing Fontan physiology," arises from chronic exposure to low cardiac output and high central venous pressure and results in impairment of critical functional outcomes including exercise capacity and quality of life (QOL). 3,4 Essential to reducing morbidities and improving Fontan outcomes is the identification of novel biomarkers that are predictive of the later development of failing Fontan physiology. The early identification of Fontan patients who are at risk for failing Fontan physiology might allow for interventions to reduce the likelihood or to delay the rate of progression to symptomatic Fontan failure and the need for Fontan revision, conversion, or heart transplantation.…”