Organ availability and acceptability limit pediatric HTx. What characteristics define an unacceptable or high-risk pediatric donor remains unclear. The purpose of this study was to characterize a large cohort of pediatric donors and determine the donor risk factors, including cumulative risk, that affect recipient survival. Data from the PHTS, a prospective multicenter study, were used to examine the impact of donor factors on the outcomes of patients listed <18 yr of age who received a HTx between 1993 and 2009. Donor data were available for 3149 of 3156 HTx (99.8%). Donor cause of death, need for inotropes, or CPR did not affect survival outcomes (p = 0.05). Ischemic time also did not have an impact on overall recipient survival; however, longer ischemic times negatively impacted one-yr post-transplant survival (p < 0.0001). There was no impact of cumulative risk factors on survival (p = 0.8). Although used in a minority of cases, hormonal therapy in the donor positively impacted survival (p = 0.03). In multivariate analysis, the only donor factor associated with decreased survival was smaller donor BSA, the other factors being related to the recipient characteristics. When analyzed by recipient age, there were no donor-related factors that affected survival for those who received a transplant at <6 months of age. Longer ischemic time (p < 0.0001) and greater age difference between the recipient and donor (p = 0.0098) were donor-related factors impacting early-phase survival for recipients who received a graft at ≥10 yr of age. Factors perceived to define a marginal or high-risk pediatric heart donor including inotrope use, CPR and donor cause of death may have less impact on outcomes than previously thought. Longer ischemic times did impact one yr, but not overall survival, and this impact was much greater with older donors. Parameters for accepting a donor heart can potentially be expanded, especially in the infant age group, but strong consideration should always be given to the interaction between ischemic time and donor age.
BACKGROUND: The reported ventricular assist device (VAD) experience in the pediatric congenital heart disease (CHD) population is limited. We sought to describe contemporary use and outcomes of VADs in children with CHD and compare these outcomes to those of non-CHD children. METHODS: Patients enrolled in the Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs) between September 19, 2012 through June 30, 2017 were included. CHD was classified as biventricular vs single ventricle (Stages 1, 2, or 3). Outcomes were compared between groups and multivariable analysis was used to identify factors associated with mortality on the device. RESULTS: Among the 471 patients enrolled, 108 (24%) had CHD (45 biventricular and 63 single ventricle). CHD patients were younger (5.7 § 5.7 years vs 9.8 § 6.5 years; p < 0.0001) and smaller (0.8 § 0.5 m 2 vs 1.2 § 0.7 m 2 ; p < 0.0001) compared with non-CHD patients. CHD patients were more likely to receive a paracorporeal continuous-flow VAD (36.1% vs 12.9%; p < 0.0001) and less likely to receive an implantable continuous-flow VAD (27.8% vs 55.0%; p < 0.0001) compared with non-CHD patients. After 6 months on a VAD, CHD patients had higher mortality (36.4% vs 12.1%) and a lower transplantation rate (29.1% vs 59.9%) than non-CHD patients (p < 0.0001). In the multivariable analysis, CHD was the factor most strongly associated with mortality on VAD (hazard ratio [HR] = 2.9; p < 0.0001), whereas the factors implantable continuous-flow device and high-volume center were protective (HR = 0.3, p < 0.0001, and HR = 0.6, respectively; p = 0.02). CONCLUSIONS: VAD use in children with CHD is associated with increased mortality and decreased transplant rates compared to children without CHD. For the subgroup of children with
There are minimal data regarding chronic management of single-ventricle ventricular assist device (VAD) patients. This study aims to describe our center's multidisciplinary team management of single-ventricle patients supported long term with the Berlin Heart EXCOR Pediatric VAD. Patient #1 was a 4-year-old with double-outlet right ventricle with aortic atresia, L-looped ventricles, and heart block who developed heart failure 1 year after Fontan. She initially required extracorporeal membrane oxygenation support and was transitioned to Berlin Heart systemic VAD. She was supported for 363 days (cardiac intensive care unit [CICU] 335 days, floor 28 days). The postoperative course was complicated by intermittent infection including methicillin-resistant Staphylococcus aureus, intermittent hepatic and renal insufficiencies, and transient antithrombin, protein C, and protein S deficiencies resulting in multiple thrombi. She had a total of five pump changes over 10 months. Long-term medical management included anticoagulation with enoxaparin, platelet inhibition with aspirin and dipyridamole, and antibiotic prophylaxis using trimethoprim/sulfamethoxazole. She developed sepsis of unknown etiology and subsequently died from multiorgan failure. Patient #2 was a 4-year-old with hypoplastic left heart syndrome who developed heart failure 2 years after bidirectional Glenn shunt. At systemic VAD implantation, he was intubated with renal insufficiency. Post-VAD implantation, his renal insufficiency resolved, and he was successfully extubated to daytime nasal cannula and biphasic positive airway pressure at night. He was supported for 270 days (CICU 143 days, floor 127 days). The pump was upsized to a 50-mL pump in May 2011 for increased central venous pressures (29 mm Hg). Long-term medical management included anticoagulation with warfarin and single-agent platelet inhibition using dipyridamole due to aspirin resistance. He developed increased work of breathing requiring intubation, significant anasarca, and bleeding from the endotracheal tube. The family elected to withdraw support. Although both patients died prior to heart transplantation, a consistent specialized multidisciplinary team approach to the medical care of our VAD patients, consisting of cardiothoracic surgeons, heart transplant team, hematologists, pharmacists, infectious disease physicians, psychiatrists, specialty trained bedside nursing, and nurse practitioners, allowed us to manage these patients long term while awaiting heart transplantation.
Survival of Fontan patients after HT has significantly improved in the current era. Currently, expected post-HT survival for Fontan patients is on par with other CHD patients. Fontan patients should not be excluded from consideration for HT solely on a history of Fontan.
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