Demonstrating responsiveness of the pediatric cardiac quality of life inventory in children and adolescents undergoing arrhythmia ablation, heart transplantation, and valve surgery.

PURPOSE: Pediatric Cardiac Quality of Life Inventory (PCQLI) is a disease-specific pediatric cardiac health-related quality of life (HRQOL) instrument that is reliable, valid, and generalizable. We aim to demonstrate PCQLI responsiveness in children undergoing arrhythmia ablation, heart transplantation, and valve surgery before and after cardiac intervention. METHODS: Pediatric cardiac patients 8-18 years of age from 11 centers undergoing arrhythmia ablation, heart transplantation, or valve surgery were enrolled. Patient and parent-proxy PCQLI Total, Disease Impact and Psychosocial Impact subscale scores were assessed pre- and 3-12 months follow-up. Patient clinical status was assessed by a clinician post-procedure and dichotomized into markedly improved/improved and no change/worse/much worse. Paired t-tests examined change over time. RESULTS: We included 195 patient/parent-proxies: 12.6 ± 3.0 years of age; median follow-up time 6.7 (IQR = 5.3-8.2) months; procedural groups - 79 (41%) ablation, 28 (14%) heart transplantation, 88 (45%) valve surgery; clinical status - 164 (84%) markedly improved/improved, 31 (16%) no change/worse/much worse. PCQLI patient and parent-proxies Total scores increased (p ≤ 0.013) in each intervention group. All PCQLI scores were higher (p < 0.001) in the markedly improved/improved group and there were no clinically significant differences in the PCQLI scores in the no difference/worse/much worse group. CONCLUSION: The PCQLI is responsive in the pediatric cardiac population. Patients with improved clinical status and their parent-proxies reported increased HRQOL after the procedure. Patients with no improvement in clinical status and their parent-proxies reported no change in HRQOL. PCQLI may be used as a patient-reported outcome measure for longitudinal follow-up and interventional trials to assess HRQOL impact from patient and parent-proxy perspectives.

It is important to have quality of life (QOL) measures that are sensitive to change in QOL before and after procedures and to be sensitive to change over time. The Pediatric Cardiac Quality of Life Inventory (PCQLI) is a QOL measure specifically developed for children with cardiac disease. This study assessed the responsiveness of the PCQLI to detect change in QOL over time. QOL in Children and adolescents who were being treated for abnormal heart rhythms, heart transplantation, and aortic, pulmonary, or mitral valve surgery were assessed before and after their procedure. Children and adolescents with improved clinical status post-procedure, and their parents, reported better QOL after the procedure. Patients with no improvement from a cardiac standpoint and their parents reported no change in QOL after their procedure. The PCQLI may be used to assess QOL before and after cardiac procedures or medical treatment and follow QOL over time.

Analysis of clinical and candidate genetic risk factors for postoperative atrial tachycardia after congenital heart surgery in infants.

BACKGROUND: Atrial tachycardia (AT) after infant congenital heart disease (CHD) surgery is associated with increased mortality. Polymorphisms in PITX2 (rs2200733) and IL6 (rs1800795) are associated with postoperative atrial fibrillation in adults but have not been studied in CHD. The objective was to test the hypothesis that clinical factors and variants in PITX2 and IL6 are associated with postoperative AT in infants with CHD. METHODS: Infants (<1 year of age) undergoing CHD surgery between September 2007 and May 2016 were included. Subjects had daily assessment of telemetry and were genotyped for the 2 variants. Univariate and multivariate analyses were performed to test for factors independently associated with AT. RESULTS: Of 1,067 enrolled infants, 164 had postoperative AT (15.4%); 95 required treatment (8.9%). AT was associated with risk for extracorporeal membrane oxygenation, operative mortality, and longer duration of ventilation, as well as intensive care unit and hospital stays. PITX2 and IL6 genotypes were not associated with AT or AT requiring treatment. In multivariate analysis, use of 2 or more inotropes, age ≤ 28 days; Risk Adjusted classification for Congenital Heart Surgery, Version 1, score ≥ 3; and bypass time were all independently associated with AT. Factors independently associated with treated AT include use of 2 or more inotropes; age ≤ 28 days; and Risk Adjusted classification for Congenital Heart Surgery, Version 1, score ≥ 3. CONCLUSION: AT occurs in 15% of infants after CHD surgery and is associated with increased morbidity and mortality. Risk factors include use of 2 or more inotropes, neonatal age, and higher surgical complexity score. We observed no association between common genetic variants in PITX2 and IL6 and AT in infants after CHD surgery.

Impact of Surgical Complexity on Health-Related Quality of Life in Congenital Heart Disease Surgical Survivors.

BACKGROUND: Surgical complexity and related morbidities may affect long-term patient quality of life (QOL). Aristotle Basic Complexity (ABC) score and Risk Adjustment in Congenital Heart Surgery (RACHS-1) category stratify the complexity of pediatric cardiac operations. The purpose of this study was to examine the relationship between surgical complexity and QOL and to investigate other demographic and clinical variables that might explain variation in QOL in pediatric cardiac surgical survivors. METHODS AND RESULTS: Pediatric Cardiac Quality of Life (PCQLI) study participants who had undergone cardiac surgery were included. The PCQLI database provided sample characteristics and QOL scores. Surgical complexity was defined by the highest ABC raw score or RACHS-1 category. Relationships among surgical complexity and demographic, clinical, and QOL variables were assessed using ordinary least squares regression. A total of 1416 patient-parent pairs were included. Although higher ABC scores and RACHS-1 categories were associated with lower QOL scores (P<0.005), correlation with QOL scores was poor to fair (r=-0.10 to -0.29) for all groups. Ordinary least squares regression showed weak association with R(2)=0.06 to R(2)=0.28. After accounting for single-ventricle anatomy, number of doctor visits, and time since last hospitalization, surgical complexity scores added no additional explanation to the variance in QOL scores. CONCLUSIONS: ABC scores and RACHS-1 categories are useful tools for morbidity and mortality predictions prior to cardiac surgery and quality of care initiatives but are minimally helpful in predicting a child's or adolescent's long-term QOL scores. Further studies are warranted to determine other predictors of QOL variation.

NutriSTEP® is Reliable for Internet and Onscreen Use.

PURPOSE: NutriSTEP(®) screens for nutritional risk in preschoolers (3-5 years of age). Availability has been limited to paper versions. The objective is to test reliability for Internet and Onscreen use. METHODS: Two studies were conducted with parents in several Ontario Early Years Centres (Internet (n = 63)) and in the community and schools in Timmons, Guelph, and Ottawa, Ontario (Onscreen (n = 64)). Parents completed NutriSTEP(®) either on paper or using Internet or Onscreen versions. Two weeks later, the alternate mode was completed. Reliability was assessed using Intraclass Correlations (ICC) and Pearson Correlations (PC) on total and attribute scores, Kappa coefficients (κ) for risk, and Wilcoxon Signed Rank Test for responses on individual questions. RESULTS: For total scores, Internet and Onscreen ICCs were 0.94 and 0.91, respectively, with PCs of 0.89 and 0.84, respectively. Attribute scores were 0.69-0.91 (ICC) and 0.70-0.84 (PC) for Internet, and 0.81-0.92 (ICC) and 0.68-0.85 (PC) for Onscreen. κ amongst risk categories was 0.58 (P = 0.000) for Internet and 0.50 (P = 0.000) for Onscreen. For individual dichotomized questions, 5 of 17 (Onscreen and Internet) were excellent (κ > 0.75); 11 of 17 (Internet) and 9 of 17 (Onscreen) were adequate (0.40 < κ > 0.75); 0 of 17 (Internet) and 2 of 17 (Onscreen) questions were poor (κ < 0.4) in agreement between modes. CONCLUSIONS: Internet and Onscreen versions of NutriSTEP(®) are reliable.

Risk of hearing impairment in pediatric liver transplant recipients: a single center study.

As survival rates following liver transplantation have increased, health care providers must assess the impact of transplantation on dimensions other than traditional medical measures. Hearing impairment may adversely impact social, emotional, cognitive, academic, and speech and language development. We hypothesized that children who undergo liver transplantation are at risk for hearing impairment due to exposure to ototoxic drugs. We conducted a review of 74 children who had undergone liver transplantation between December 1996 and September 2000 at Cincinnati Children's Hospital Medical Center. Hearing was assessed at discharge by an audiologist using age and developmentally appropriate techniques. The principal outcome measure was sensorineural hearing impairment. Independent variables were age at transplantation, United Network for Organ Sharing (UNOS) status at transplantation, primary diagnosis, post-transplant length of hospital stay, days of treatment with aminoglycosides, and days of treatment with loop diuretics. Eleven of 74 children (15%) had sensorineural hearing loss, of whom four had severe to profound hearing loss. Multivariate analyses showed that the adjusted relative risk for hearing loss in patients with hepatoblastoma was 66 and that there was a 5% increase risk for hearing loss for each additional day of hospitalization. Age at transplantation, UNOS status, and days of treatment with loop diuretics or aminoglycosides did not achieve significance in the model. Sensorineural hearing impairment occurs in a subset of pediatric patients following liver transplantation. Patients with hepatoblastoma or those who experience prolonged hospitalization after transplantation are at increased risk. Our observations are of particular importance for pediatric liver transplant recipients since the median age at transplantation is 12-18 months, a critical period for language acquisition.