Andersons pediatric cardiology 1762

Weinstein et al.54 Valeske et al.56 Halaweish et al.72 Jabbar et al.73 Hoganson et al.74 Arnaoutakis et al.60 2014 — EXCOR 2014 19, male EXCOR BiVAD 3/5 transplanted at 1, 3, and 229 days; other 2 died at >360 days of support Transplanted at 23 days 2015 14, male EXCOR Transplanted at 179 days 2015 23, male HeartMate II Transplanted >3 years of support 2015 4, male EXCOR Transplanted at 26 days HVAD, TAH, EXCOR, Thoratec pVAD 3/4 transplanted Poh et al.75 2017 ROTAFLOW, EXCOR Woods et al.76 2017 HVAD 2/5 transplanted; 1 discharged home in vegetative state, 2 died from multiorgan failure and embolic event 3/3 transplanted at 148, 272, and 271 d SynCardia databasea — SynCardia TAH 3/5 transplanted CCHMCb — HeartMate II, CentriMag, HeartWare 2/3 transplanted Total 2017 35 18, male 14, male 5, male 23, male — 12, male 13, male 17, male 31, male 12, male 5, male 18, male 24, female 15, male 25, male 37, male 23/35 (66%) positive outcomec aCommunication with lead researcher at SynCardia bCommunication with Cincinnati Children's Hospital Medical Center cNumber does not account for all patients during this time frame, as not all were reported BiVAD, Biventricular assist device; pVAD, peripheral VAD; RVAD, right ventricular assist device; TAH, total artificial heart; VAD, ventricular assist device Patients with late end-stage Fontan failure in which there is significant end- organ dysfunction (e.g., protein-losing enteropathy, plastic bronchitis, cachexia) are known to be poor transplant candidates with significant peritransplant issues, as well as inferior long-term outcomes One of these late complications in isolation does not preclude transplantation, but as a patient accumulates multiple comorbidities, one must question what we are affording them with a transplant The goal is not to limp these patients to transplant knowing their chance of being alive in 5 years is not great; rather, we should attempt to make them better candidates for transplant Perhaps in these patients, a TAH can help to resuscitate them not only because of the supraphysiologic cardiac index (4 L/min per m2) but, more importantly, a low central venous pressure of 3 to 5 mm Hg It is the latter that a VAD nor even a fresh transplant can afford the patient We believe this unique hemodynamic effect allows for the resuscitation of kidneys and livers thought to have failed secondary to congestion, as well as allowing proteinlosing enteropathy to resolve In addition, the ability to nutritionally and physically rehabilitate prior to transplant should not be underestimated The TAH has been used five times (personal communication with Syncardia), with a 60% survival in failing Fontan procedures, including the one reported by Rossano et al.77 Along with smaller future TAH sizes (50 cc), the use of virtual surgical implantation can help to establish fit in smaller patients with unique palliated congenital anatomy,39 expanding eligibility for future TAH candidates In all, the experience with VADs following stage I palliation has been dismal and requires extreme caution when contemplating their use over ECMO In patients with SVP and after Glenn or Fontan palliations, MCS has proven to be a meaningful way of bridging patients to transplantation with good posttransplant survival Even still, thoughtful patient and device selection, as well as timing of support, is essential for good outcomes Elevated Pulmonary Vascular Resistance Many patients with long-standing heart failure, especially from CHD or restrictive physiology, present with markedly elevated pulmonary vascular resistance and may be considered “untransplantable.” A period of LVAD support and maximal medical manipulation of the pulmonary vascular resistance often lowers this resistance significantly, as seen by repeat catheterizations These patients then are eligible for transplant, which is the expected course for the vast majority of such patients as has been documented by multiple series.78,79 In addition, now that pediatric patients greater than 25 kg are almost exclusively supported by an intracorporeal CF VAD, there is less concern over whether the pulmonary vascular resistance changes Regardless, LVAD support in these patients can allow them to be discharged with their devices and resume almost a normal lifestyle Adult Congenital Heart Disease The number of adults with congenital heart disease (ACHD) has long since surpassed the number of children with CHD and represents an increasing proportion of heart transplant recipients These patients face a higher early mortality rate and higher risk of retransplantation These patients are also likely to have longer mean waiting times and significantly higher pulmonary vascular resistances.80–82 It is therefore likely that increasing numbers of these patients will require VAD implantation prior to heart transplantation; however, Gelow et al confirmed that VAD use is less common in ACHD patients listed for transplant compared with non-ACHD patients (3% vs 17%).83 This represents significant hesitation to support these patients mechanically, which we can surmise is secondary to their complexity and presentation at adult programs who may not be as familiar with some of the more complex anatomy and physiology The authors concluded this is clinically relevant because reduced VAD use appears to contribute to lower listing status and impacts organ allocation to ACHD patients, as well as waitlist and transplant survival They found that the difference has grown increasingly disparate over time and suggested that separate organ allocation for CHD patients may be justified In response to this issue, the Organ Procurement and Transplantation Network Board of Directors reviewed organ allocation policy and issued a statement to Regional Review Boards to give higher consideration to increasing the listing status of ACHD patients, although it did not ultimately change the current policy.84 Many ACHD patients have SVHs or transposition of the great arteries with either a systemic morphologic left ventricle or a systemic morphologic right ventricle Both adult and pediatric patients with SVH have been supported with VADs, as noted previously Consideration may be given to either common atrial or ventricular cannulation, depending on residual ventricular function and the risk of thromboembolism Patients with both L-transposition of the great arteries and D-transposition corrected by an atrial switch procedure (Senning or Mustard) are susceptible to failure of the systemic morphologic right ventricle, necessitating heart transplantation VADs have generally been designed for ... majority of such patients as has been documented by multiple series.78,79 In addition, now that pediatric patients greater than 25 kg are almost exclusively supported by an intracorporeal CF VAD, there is less concern over whether the... either a systemic morphologic left ventricle or a systemic morphologic right ventricle Both adult and pediatric patients with SVH have been supported with VADs, as noted previously Consideration may be given to either common atrial