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Andersons pediatric cardiology 1763

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implantation in an apex-forming left ventricle Patients with systemic morphologic right ventricles present challenges in the implantation of the ventricular cannula, owing to the different anatomy of the right compared with the left ventricle (e.g., muscle bundles) and the fact that the right ventricle does not form an apex Adjustments also need to be made to the orientation of the pump because the systemic ventricle may be anterior (D-transposition of the great arteries) or more posterior and with dextrorotation (L-transposition of the great arteries), and the great vessels situated differently than in normal anatomy Both pulsatile85–89 and CF pumps have been used for this application.90,91 Many of these authors offer nuanced descriptions of the implantation technique in their reports Until recently, most publications on MCS in ACHD were in the form of case reports,92–95 single center reviews,96,97 and a few database analyses,83,98 all with a very limited number of patients Then, in 2017, a review of INTERMACS data identified 126 of 16,182 patients receiving MCS as having ACHD.99 The study found that ACHD patients with LVADs have survival similar to non-ACHD patients Of those patients, 63 had systemic morphologic left ventricle and 17 were SVH Overall, the ACHD patients were younger and significantly more likely to undergo implantation as a bridge to transplant BiVAD and TAH were more common in ACHD patients (21% vs 7%), which may have been a reflection of them more commonly being INTERMACS profile 1 compared with their LVAD counterparts Once again, BiVAD/TAH support was the only variable independently associated with mortality (early phase hazard ratio of 4.4) Surprisingly, single ventricle was not seen to be a risk factor, but as the authors noted, this may have been a type II error secondary to small numbers In line with adult practices, 82% of the ACHD patients underwent CF device implantation Notably, ventricular morphology was not associated with mortality This study demonstrated a promising role for VAD support in ACHD populations with good bridge to transplant outcomes and encouraged more timely and frequent VAD support of ACHD patients in severe heart failure Perioperative Management The perioperative management of VAD patients can be challenging, and there are a number of important considerations Patient condition at the time of VAD implant is critically important, and as much as possible the patient condition should be optimized However, as noted previously, many children are in cardiogenic shock at the time of placement of a durable VAD Whether a period of MCS with ECMO or a temporary VAD prior to placement of a durable VAD improves long-term outcomes remains an important outstanding question.100–102 In the immediate postoperative period, control of bleeding and timing of anticoagulation are important considerations In the Berlin Heart EXCOR trial, major bleeding was reported in 40% to 50% of patients.103 This is important because anticoagulation is needed to prevent embolic disease including stroke, which is also common in the early postoperative period The stroke rate was nearly 30% in the Berlin Heart EXCOR trial, with most events happening in the early postoperative period.103,104 This highlights the need for early control of bleeding to allow for safe early initiation of anticoagulation therapy Major bleeding is also common among children supported with CF VADs, although the incidence of stroke appears to be less.105 It is not clear the contribution of VADspecific versus patient-specific factors in the development of neurologic and other post-VAD adverse events The type of anticoagulation used, timing of anticoagulation initiation, and ongoing surveillance and adjustment of anticoagulation vary greatly among different devices and different centers.106,107 Much of the early medical therapy is aimed at supporting the right ventricle Most patients are supported with a LVAD alone, and in the absence of SVH disease, are at risk for right ventricular failure in the immediate postoperative period Although most children, especially those with DCM, can be successfully managed without mechanical right ventricular support, BiVAD support is occasionally needed Unfortunately, there are no clear criteria that identify patients that would benefit from a BiVAD or TAH.108–110 In a review of patients supported with the Berlin Heart EXCOR VAD, no factor was identified that predicted a clinical benefit from BiVAD support, including small patient size, ECMO, and high bilirubin levels.110 This is not to suggest that right ventricular failure does not happen in the immediate postoperative period or that it is not associated with major morbidity However, it can be successfully managed medically with the early use of pulmonary vasodilators such as inhaled nitric oxide for afterload reduction, inotropic support (e.g., milrinone, dopamine, epinephrine), and optimizing pulmonary mechanics with a strategy of early extubation Overall, biventricular support with the use of a BiVAD or TAH occurs in less than 20% of patients.4 There are many other potential complications of VADs in children, and major adverse events are relatively common in children as in adults.16,111 Overall, the freedom from any major adverse events of device malfunction, infection, major bleeding, or neurologic dysfunction is less than 25% by 6 months after VAD placement.105 Some of these adverse events are higher in paracorporeal pulsatile flow devices as compared with intracorporeal CF devices, although the contribution of device versus patients factors to these adverse events needs further study.16,105 Most of the complications can be managed and do not lead to permanent disability or death, and many of these patients are successfully supported on their VAD to transplant.16,104,112 After the immediate recovery period, much attention is paid to ongoing rehabilitation, maximizing nutrition, and reducing morbidities associated with prolonged intensive care unit and hospital care, including hospital-acquired infections As noted previously, many patients go to the operating room for VAD placement with multiorgan system dysfunction, and improving this state is crucial for a successful VAD outcome, which for most children is heart transplantation Patients on VAD support who go into heart transplantation with severe multiorgan system dysfunction have a hospital mortality of nearly 20% compared with only 2% among VAD-supported patients who have no organ dysfunction at the time of transplant.13 This has led to practice of many centers to delay active listing for heart transplantation until the patient has recovered well and is free from organ dysfunction after VAD placement.11,113 Outpatient Management Increased use of intracorporeal VADs in pediatric heart failure patients has allowed for discharge to home Successful transition to the outpatient setting requires a significant amount of effort and mobilization of resources from a range of providers involved in the patient's day-to-day care A pediatric VAD program includes providers across multiple disciplines with additional training in device management A team is generally a multidisciplinary team of specialists who in most instances have undergone additional device training, ... well and is free from organ dysfunction after VAD placement.11,113 Outpatient Management Increased use of intracorporeal VADs in pediatric heart failure patients has allowed for discharge to home Successful transition to the outpatient setting... requires a significant amount of effort and mobilization of resources from a range of providers involved in the patient's day-to-day care A pediatric VAD program includes providers across multiple disciplines with additional training in device management

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    Section 5 Heart Failure and Transplantation

    66 Chronic Mechanical Circulatory Support

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