Ventricular assist devices as a bridge to heart transplantation in children.

UNLABELLED: The increase in time waiting for appropriate pediatric allografts for heart transplantation has mandated the use of long-term mechanical assistance in the pediatric population. Extracorporeal membrane oxygenation support has been routinely used but is limited by both its inability to provide support without life-threatening complications for longer than 2 to 3 weeks as well as the inability of patients to achieve mobility. For the past 10 years, pediatric programs have increasing experience with the use of ventricular assist devices (VADs) to bridge patients to heart transplant. This retrospective study analyzed the clinical features and outcomes of 99 pediatric patients who underwent VAD implant as a bridge to heart transplant. METHODS: Between 1993 and 2003, the Pediatric Heart Transplant Study Group enrolled 2,375 patients (age 1 day-17.9 years) listed for heart transplant from 23 participating centers. Four percent (99 patients) of those listed received VAD support as a bridge to transplantation. Seventy-seven (77%) patients survived to transplant with a mean time on support of 57 days. There were 17 deaths on support and 5 bridged to recovery. Overall incidence of adverse events was similar to the adult data with a 19% risk of stroke. There was no difference in 5-year survival after transplant for patients on VAD at time of transplant compared with those (n = 2,293) not requiring VAD (77% vs 73%, P = .8). These data suggest that despite the lack of pediatric specific devices and relatively high adverse event rate, VADs may be used as a bridge to transplant therapy in appropriate-sized children with the expectation of a successful outcome in most patients.

Physician and parent perceptions of prognosis and end-of-life experience in children with advanced heart disease.

BACKGROUND: Little is known about how physician and parent perspectives compare regarding the prognosis and end-of-life (EOL) experience of children with advanced heart disease (AHD). OBJECTIVE: The study's objective was to describe and compare parent and physician perceptions regarding prognosis and EOL experience in children with AHD. METHODS: This was a cross-sectional survey study of cardiologists and bereaved parents. Study subjects were parents and cardiologists of children with primary cardiac diagnoses who died in a tertiary care pediatric hospital between January 2007 and December 2009. Inclusion required both physician and parent to have completed surveys respective to the same patient. A total of 31 parent/physician pairs formed the analytic sample. Perceptions were measured of cardiologists and bereaved parents regarding the EOL experience of children with AHD. RESULTS: Nearly half of parents and physicians felt that patients suffered 'a great deal,' 'a lot,' or 'somewhat' at EOL, but there was no agreement between them. At diagnosis, parents more often expected complete repair and normal lifespan while the majority of physicians expected shortened lifespan without normal quality of life. Parents who expected complete repair with normal life were more likely to report 'a lot' of suffering at EOL (p=0.002). In 43% of cases, physicians reported that the parents were prepared for the way in which their child died, while the parents reported feeling unprepared. CONCLUSION: Both parents and physicians perceive suffering at EOL in patients who die of AHD. Moreover, parent expectations at diagnosis may influence perceptions of suffering at EOL. Physicians overestimate the degree of parent preparedness for their child's death.

Cognitive complexity of the medical record is a risk factor for major adverse events.

CONTEXT: Patients in tertiary care hospitals are more complex than in the past, but the implications of this are poorly understood as "patient complexity" has been difficult to quantify. OBJECTIVE: We developed a tool, the Complexity Ruler, to quantify the amount of data (as bits) in the patient's medical record. We designated the amount of data in the medical record as the cognitive complexity of the medical record (CCMR). We hypothesized that CCMR is a useful surrogate for true patient complexity and that higher CCMR correlates with risk of major adverse events. DESIGN: The Complexity Ruler was validated by comparing the measured CCMR with physician rankings of patient complexity on specific inpatient services. It was tested in a case-control model of all patients with major adverse events at a tertiary care pediatric hospital from 2005 to 2006. MAIN OUTCOME MEASURES: The main outcome measure was an externally reported major adverse event. We measured CCMR for 24 hours before the event, and we estimated lifetime CCMR. RESULTS: Above empirically derived cutoffs, 24-hour and lifetime CCMR were risk factors for major adverse events (odds ratios, 5.3 and 6.5, respectively). In a multivariate analysis, CCMR alone was essentially as predictive of risk as a model that started with 30-plus clinical factors. CONCLUSIONS: CCMR correlates with physician assessment of complexity and risk of adverse events. We hypothesize that increased CCMR increases the risk of physician cognitive overload. An automated version of the Complexity Ruler could allow identification of at-risk patients in real time.

Prognostic value of exercise testing during heart transplant evaluation in children.

BACKGROUND: Maximum oxygen consumption (peak VO2) <50% predicted on exercise testing is a class I indication for heart transplant (HT) listing in children. This recommendation is based on exercise data in adults. We assessed the association of peak VO2<50% predicted during HT evaluation with freedom from death or deterioration in children. METHODS AND RESULTS: We analyzed all children who underwent exercise testing during HT evaluation at our center between 2002 and 2011. Patients with peak VO2<50% predicted were compared with those with peak VO2 ≥ 50% predicted for the composite outcome of death before HT, initiation of mechanical circulatory support, and HT at highest urgency status, using time-to-event analyses. There were 50 children in the study (median age, 15 years; interquartile range, 13-17 years; 24 girls; 18 with palliated single ventricle). Overall, 24 children reached the composite end point. Peak VO2<50% predicted was associated with outcome in children with biventricular circulation (hazard ratio, 4.7; 95% confidence interval, 1.8-12.3; P<0.001) but not in those with a palliated single ventricle (hazard ratio, 1.3; 95% confidence interval, 0.1-12.0; P=0.80). Similarly, VE/VCO2 slope ≥ 34 was associated with outcome in children with biventricular circulation (hazard ratio, 2.7; 95% confidence interval, 1.1-7.1; P<0.001) but not in children with a palliated single ventricle. CONCLUSIONS: Exercise testing during HT evaluation in children with biventricular circulation identified those at higher risk of death or deterioration in this small study. Larger studies are needed to assess the role of exercise testing during HT evaluation in children with a palliated single ventricle.

Safety and early outcomes using a corticosteroid-avoidance immunosuppression protocol in pediatric heart transplant recipients.

BACKGROUND: Long-term oral corticosteroids have been a mainstay of maintenance immunosuppression in pediatric heart transplantation. In this study, we report early clinical outcomes in a cohort of pediatric heart transplant recipients managed using a steroid-avoidance protocol. METHODS: Of the 70 patients who underwent heart transplantation during the study period, 55 eligible recipients, including 49 non-sensitized and 6 sensitized (all 55 with negative crossmatch) patients, entered a steroid-avoidance immunosuppression protocol consisting of thymoglobin induction followed by a 2-drug, tacrolimus-based, corticosteroid-free regimen. The primary outcome variable was freedom from moderate rejection (International Society for Heart and Lung Transplantation [ISHLT] Grade 2R/3A or antibody-mediated rejection). RESULTS: The median age at transplant was 7.1 years (range 2 weeks to 22 years) and median follow-up was 19 months (range 2 to 46 months). Fifty patients survived to discharge after transplantation. Of these patients, 2 (4%) were discharged on steroids and 8 (16%) started on maintenance steroids at follow-up. Rejection was diagnosed in 8 patients (Grade 2R cellular rejection in 3 and antibody-mediated rejection in 5). Freedom from rejection was 92% at 6 months (95% confidence interval [CI] 80% to 97%) and 87% at 1 year (CI 73% to 94%). Post-transplant survival was 91% at 6 months (CI 79% to 96%) and 88% at 12 and 24 months (CI 75% to 95%). There was 1 death due to rejection (antibody-mediated) 8 months after transplantation. CONCLUSIONS: An immunosuppression protocol consisting of induction followed by corticosteroid avoidance appears to achieve acceptable rejection rates during the first year post-transplant in pediatric heart transplant recipients.