Aims With the continued success of treating children and adolescents with congenital (CHD) or acquired heart disease, we now need to assess quality of life (QoL) outcomes rather than simple mortality. The Pediatric Cardiac Quality of Life Inventory (PCQLI) is a new self-administered disease-specific QoL measure, comprising both patient and parent–proxy reporting and recently shown to be reliable and valid in the USA. The purpose of the current trial was to assess reliability and validity of the PCQLI scores in the UK.

Methods Three UK tertiary paediatric cardiac centres recruited patients aged 8–18 years with congenital or acquired heart disease. Patient–parent pairs completed the PCQLI, a generic QoL measure (PedsQL4.0) and non-QoL instruments (Self Perception Profile for Children/Adolescents (SPPC/A); Achenbach Youth Self Report Form and Child Behavior Checklist). Test-retest reliability was performed by administering the PCQLI at two time points and assessed by correlation coefficients. Validity was assessed by correlation of PCQLI scores between patients and parents and with severity of CHD, medical care utilisation, and PedsQL, SPPC/A and Achenbach scores.

Results This preliminary study was completed by 125 child-parent pairs and 125 adolescent-parent pairs. Diagnostic categories were biventricular repair (40%), Fontan palliation (20%), unrepaired CHD (20%), heart transplantation (2.8%) and acquired heart disease (17.8%). Correlations evaluating test-retest reliability ranged from 0.64–0.9. Correlations for patient and parent scores were moderate for both child and adolescent groups (0.49–0.72). Lower PCQLI scores were associated with Fontan palliation (p<0.001) and increasing number of hospitalisations (p<.005) and cardiac catheterisations (p<.001). Scores for the PCQLI and generic QoL measure were highly correlated (0.66–0.77). Lower PCQLI total scores were associated with lower global self-worth on the SPPC/A (p<.001) and lower total competency scores on the Achenbach measures (p<.001).

Conclusions Preliminary analysis of PCQLI data collected in the UK suggests that it is a reliable and valid disease-specific QoL measure for children and adolescents with congenital or acquired heart disease. This novel tool will allow for outcomes assessment in the paediatric cardiac population.