Background
Late complications of the Fontan operation represent a significant management challenge. In general, failing Fontan patients have two modes of presentation: those with impaired ventricular function (IVF group) and those with preserved ventricular function but with failing Fontan physiology (protein-losing enteropathy-PLE, and plastic bronchitis-PB) (PVF group). In this study we evaluated whether failing Fontan patients referred for heart transplantation had a different outcome based on the mode of presentation.
Methods
We conducted a retrospective chart review of all Fontan patients evaluated for heart transplantation at a single institution from 1994–2008. Demographic, hemodynamic, and laboratory data were collected. Patients were stratified into IVF group or PVF group based on echocardiographic criteria. Descriptive statistics and Kaplan-Meier analysis were used for hypothesis testing.
Results
Thirty four Fontan patients were evaluated for heart transplantation. Based on echo description of systolic function, 18 were categorized as IVF and 16 patients as PVF. The IVF group had significantly lower cardiac index, SvO2, and significantly higher SVR compared to the PVF group (p<0.05). PLE or PB was present in 13/16 in the PVF group and 0/18 in the IVF group. Of the 34 patients, 20 received transplantation with similar rates amongst the IVF and PVF groups. Within one year from evaluation there were 2/18 deaths in the IVF group and 7/16 deaths in the PVF group (p=0.052)
Conclusions
Failing Fontans with PVF have decreased overall survival independent of whether they were transplanted. This trend indicates a need to improve the management and/or timing for transplantation amongst this population.
Thrombotic and bleeding complications have historically been major causes of morbidity and mortality in pediatric ventricular assist device (VAD) support. Standard anticoagulation with unfractionated heparin is fraught with problems related to its heterogeneous biochemical composition and unpredictable pharmacokinetics. We sought to describe the utilization and outcomes in children with paracorporeal VAD support who are treated with direct thrombin inhibitors (DTIs) antithrombosis therapy. Retrospective multicenter review of all pediatric patients (aged <19 years) treated with a DTI (bivalirudin or argatroban) on paracorporeal VAD support, examining bleeding and thrombotic adverse events. From May 2012 to 2018, 43 children (21 females) at 10 centers in North America, median age 9.5 months (0.1–215 months) weighing 8.6 kg (2.8–150 kg), were implanted with paracorporeal VADs and treated with a DTI. Diagnoses included cardiomyopathy 40% (n = 17), congenital heart disease 37% (n = 16; single ventricle n = 5), graft vasculopathy 9% (n = 4), and other 14% (n = 6). First device implanted included Berlin Heart EXCOR 49% (n = 21), paracorporeal continuous flow device 44% (n = 19), and combination of devices in 7% (n = 3). Adverse events on DTI therapy included; major bleeding in 16% (n = 7) (2.6 events per 1,000 patient days of support on DTI), and stroke 12% (n = 5) (1.7 events per 1,000 patient days of support on DTI). Overall survival to transplantation (n = 30) or explantation (n = 8) was 88%. This is the largest multicenter experience of DTI use for anticoagulation therapy in pediatric VAD support. Outcomes are encouraging with lower major bleeding and stroke event rate than that reported in literature using other anticoagulation agents in pediatric VAD support.
Up-regulation of the soluble VEGFR-1 in pressure-loaded myocardium prevents capillary growth by trapping VEGF. Inhibition of sVEGFR-1 released sufficient VEGF to induce angiogenesis and preserved contractile function. These data suggest sVEGFR-1 as possible therapeutic targets to prevent heart failure.
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