Assessment of parental decision making in congenital heart disease, cardiomyopathy and heart transplantation: an observational study analysing decisional characteristics and preferences.

OBJECTIVE: We explore shared decision making (DM) in guardians of children with heart disease by assessing the desired weight of influence on DM and factors that may alter the relative weight of parent or medical team influence. METHODS: Guardians of patients <21 years and admitted >1 week in the paediatric cardiac intensive care unit (PCICU) were recruited. Twelve vignettes were designed including technical (antibiotic selection, intubation, peripherally inserted central catheter placement, ventricular assist device placement, heart transplant, organ rejection, heart rhythm abnormalities and resuscitation effort) and non-technical vignettes (cessation of life-sustaining therapies, depression treatment, obesity and palliative care referral). Participants responded to questions on DM characteristics and one question querying preference for relative weight of parent or medical team influence on DM. RESULTS: Of 209 participants approached, 183 were included. Most responded with equal desire of medical team and parental influence on DM in all vignettes (range 41.0%-66.7%). Technical scenarios formed one cluster based on DM characteristics, compared with non-technical scenarios. Factors that increase the relative weight of parental influence on DM include desired input and involvement in big-picture goals (OR 0.274, CI [0.217 to 0.346]; OR 0.794, CI [0.640 to 0.986]). Factors that increase the relative weight of medical team influence on DM include perception of medical expertise needed (OR 1.949 [1.630 to 2.330]), urgency (OR 1.373 [1.138 to 1.658]), benefit (OR 1.415 [1.172 to 1.710]), number of PCICU admissions (OR 1.134 [1.024 to 1.256]) and private insurance (OR 1.921 [1.144 to 3.226]). CONCLUSION: Although factors may alter the weight of influence on DM, most parents desire equal parental and medical team influence on DM.

Risk factors associated with post-transplant superior caval vein stenosis in paediatric heart transplantation.

Superior caval vein stenosis is a known complication following paediatric heart transplantation. Herein, we sought to assess the incidence of superior caval vein stenosis and need for intervention in a single centre paediatric heart transplantation programme. A retrospective review was performed to identify variables associated with superior caval vein stenosis and need for intervention. Patients were identified based on angiographic and echocardiographic signs of superior caval vein stenosis. Of 204 paediatric heart transplantation recipients, 49 (24.0%) had evidence of superior caval vein stenosis with no need for catheter intervention and 12 (5.9%) had superior caval vein stenosis requiring catheter intervention. Overall, patients with superior caval vein stenosis with and without intervention had more cavopulmonary anastomosis (41.7%; 20.4%), pre-transplant superior caval vein procedures (41.7%; 28.6%), and bicaval approach (100.0%; 98.0%), compared to the group with no stenosis (11.9% and p = 0.015, 12.6% and p = 0.004, 73.4% and p < 0.001, respectively). Smaller recipients and donors were more likely to need intervention. Intervention was also seen more frequently in recipients who were younger at diagnosis (4.7 years) compared to non-intervention (13.3 years; p = 0.040). Re-intervention was required in 16.7% patients (n = 2) and was not associated with any complications.

Family functioning in pediatric heart transplantation: Variables associated with poor outcomes.

Family functioning is integral in a child's life and is linked to quality of life in health as well as disease. This has been scarcely studied in pediatric orthotopic heart transplantation (pOHT). In this study, we evaluate demographic and clinical factors associated with family functioning in this patient population. Pediatric post-transplant families were recruited in an outpatient setting (n = 71). The PedsQL Family Impact Module was administered, along with the Parent and Adolescent Medication Barriers Scales (PMBS; AMBS) and the McArthur socioeconomic scale. Associations between clinical and demographic variables and scaled scores were evaluated. In our sample, patients with congenital heart disease, developmental delay, and enteral feeding had lower total impact (P = .026; P = .011; P = .008) and parent self-reported HRQL scores (P = .018; P = .012; P = .005). Patients with developmental delay and enteral feeding also had lower family functioning summary scores (P = .025; P = .031). Higher parent educational status was associated with lower total impact scores (P = .043). Higher PMBS scores demonstrated negative correlation with total impact (P < .001), parent self-reported HRQL (P < .001), and family functioning summary scores (P = .003). Multiple linear regression analysis identified developmental delay, parental education, and PMBS as independent variables associated with family functioning. Our study highlights important factors impacting family functioning in pOHT. Developmental delay, higher parental education, and PMBS were associated with poorer family functioning. Our findings emphasize the need for a multi-disciplinary approach including serial psychological assessment and interventions in the management of pOHT patients in order to optimize family functioning.

Postoperative vasoactive inotropic score is predictive of outcomes in pediatric heart transplantation.

Vasoactive inotrope score (VIS) is scarcely studied in pediatric orthotopic heart transplantation (pOHT). We conducted a retrospective review of pOHT (<21 years) recipients. Max VIS and mean VIS were calculated at 0-24 and 24-48 hours post-pOHT. Patients were divided into groups based on ISHLT guidelines: high (>10) and low (≤10). In our group (n = 104), patients with high max and mean VIS groups at 0-24 and 24-48 hours had longer bypass times (high: >130 minutes; low: <108 minutes; P < .05) and high max and mean VIS groups at 0-24 hours had longer ischemic times (high: >215 minutes; low: <192 minutes; P < .05). Patients with high max and mean VIS at 0-24 and 24-48 hours had longer hospital stay, ventilation, inotrope duration, more cardiac events, and acute kidney injury postoperatively (P < .05). High max VIS at 24-48 hours and high mean VIS at 24-48 hours had higher 3-year mortality (P = .04; P = .02). Multivariate analysis confirmed the association of VIS with short-term outcomes. However, VIS was not identified as an independent predictor of mortality. The ROC curve exhibits 10 as the ideal cutoff with area under the curve >0.8 for primary graft dysfunction (PGD).

Decline in rejection in the first year after pediatric cardiac transplantation: a multi-institutional study.

BACKGROUND: Rejection is a major cause of morbidity and mortality after pediatric heart transplantation (HTx). Survival after pediatric HTx has improved over time, but whether there has been an era-related improvement in the occurrence of allograft rejection is unknown. METHODS: The Pediatric Heart Transplant Study (PHTS) database was queried for patients who underwent HTx from January 1993 to December 2005 to determine the incidence of rejection and identify factors associated with the first episode of rejection in the first year after HTx. RESULTS: Data were reviewed in 1,852 patients from 36 centers. The incidence of rejection declined over 13 years at a rate of -2.58 +/- 0.41 (p < 0.001) from approximately 60% to 40% (p < 0.001). The mean number of episodes of rejection also significantly fell at a rate of -0.05 +/- 0.01 per patient/year from 1.19 to 0.66 (p < 0.001). The incidence of rejection with hemodynamic compromise and death from rejection did not change. Multivariate analysis for the risk of a first rejection episode demonstrated decreased risk of rejection with later year of HTx (odds ratio [OR], 0.88; 95% confidence interval [CI], 0.85-0.91; p < 0.001) and use of mechanical support (OR, 0.65; 95% CI, 0.42-0.99; p = 0.046). Increased risk of rejection was associated with positive donor-specific crossmatch (OR, 1.85; 95% CI, 1.18-2.88; p = 0.007) and older recipient age (OR, 1.05; 95% CI, 1.02-1.07; p < 0.001). CONCLUSIONS: Although the overall incidence and prevalence of rejection has substantially decreased over time in pediatric HTx recipients in the first year after HTx, the rate of rejection with hemodynamic compromise or death from rejection remains unchanged.

Ventricular assist device implantation in the pediatric population: does pump size selection and associated hemodynamics impact outcomes?

The use of pediatric ventricular assist devices (VADs) continues to evolve with the availability of smaller blood pumps. We examine the correlation of implanting appropriate sized blood pumps with a lower incidence of VAD related complications (VADRC). A 7-year retrospective review was undertaken for all pediatric VAD patients. Optimal VAD hemodynamics were defined as cardiac index of 2.7 L/m2 and rate of 80 beats per minute (bpm) with complete fill/empty of the blood pump. Patient/blood pump size match, VAD rate and fill/empty ratios were calculated (optimum = 1.0) and then correlated with incidence of VADRC. The study included 22 patients, mean age 9.77 years (6 mo-18 yrs) and mean body surface area (BSA) of 1.14 m2 (0.14 m2-2.32 m2), who underwent VAD implantation. VADRC included death while on support (n = 5), bleeding requiring reoperation (n = 8), hemolysis (n = 2), neurologic events (n = 2), thrombus formation (n = 3), and infection (n = 3). Six patients were bridged to transplant without any VADRC. This subset of patients had a mean blood pump size match ratio of 0.98, VAD rate ratio of 0.92 and fill/empty ratio of 1.00. Patients with VADRC (n = 16) were found to have a mean blood pump size match ratio of 0.72, VAD rate ratio of 0.72 and fill/empty ratio of 0.78. We report a series of pediatric patients with wide ranging BSA receiving VAD implantation. Selection of appropriate sized blood pumps can be correlated with decreased VADRC.