bims-hylehe Biomed news
on Hypoplastic left heart syndrome
Issue of 2018‒11‒11
five papers selected by
Richard James
University of Pennsylvania


  1. J Heart Lung Transplant. 2018 Sep 25. pii: S1053-2498(18)31662-0. [Epub ahead of print]
    VanderPluym CJ, Adachi I, Niebler R, Griffiths E, Fynn-Thompson F, Chen S, O'Connor MJ, Machado D, Hawkins B, Bleiweis MS, Koehl DA, Cantor RS, Morales D, Lorts A.
      BACKGROUND: Since 2012, there has been growing use of the HeartWare (Medtronic, Mounds View, MN) intracorporeal continuous flow (CF) ventricular assist device (VAD) in children, despite it not being labeled for use in pediatric patients. We sought to describe the use and outcomes of children with HeartWare VADs.METHODS: We identified all patients aged < 19 years and young adults aged 19 to 30 years supported with HeartWare who were entered into the pediatric portion (Pedimacs) of the Interagency Registry for Mechanically Assisted Circulatory Support (Intermacs) and the Intermacs registries, respectively, between September 2012 and June 2017. Adverse events and outcomes were analyzed and compared.
    RESULTS: We identified 192 children and 247 young adult HeartWare patients. Baseline characteristics of children differed from young adults, with lower median weight of 51.5 kg (range, 13.1-162) vs 75.8 kg (range, 29.8-191; p ≤ 0.0001) and body surface area of 1.5 m2 (range, 0.6-2.9 m2) vs 1.9 m2 (range, 1.1-3.2 m2; p ≤ 0.0001) . At the time of implant, 12 children weighed < 20 kg, and 58.3% of these children had congenital heart disease compared with 11.7% in children who weighed ≥ 20 kg and 6.1% in young adults (p ≤ 0.0001). Median duration of support was 2.8 months (IQR, 1.3-6.0 months) in children and 9.7 months (IQR 4.0-19.2 months) in young adults (p ≤ 0.0001). Serious adverse events in children and young adults included infection in 27% and 44% of patients, respectively (p=0.0002), major bleeding in 23% and 23%, respectively (p = 0.9), device malfunction/pump thrombosis in 11% and 19.0%, respectively (p = 0.04), and stroke in 10% and 12%, respectively (p = 0.5). Of the children who weighed < 20 kg at time of implant, 0% had major bleeding, 16.7% had infections, and 8.3% had stroke. Overall survival was not statistically different between children and young adults, and there was no increased mortality in children who weighed < 20 kg. Rate of discharge on HeartWare was 80% in young adults vs 48% in children who weighed ≥ 20 kg and only 33% in children who weighed < 20 kg.
    CONCLUSIONS: Survival in children supported with HeartWare is encouraging and comparable to young adults; however, adverse events are not uncommon in children. Ongoing evaluation of the HeartWare use in children is necessary to further decrease the rate of adverse events and understand obstacles to discharge.
    Keywords:  HeartWare; Pedimacs; continuous-flow; outcomes; ventricular assist device
    DOI:  https://doi.org/10.1016/j.healun.2018.09.015
  2. Ann Thorac Surg. 2018 Oct 31. pii: S0003-4975(18)31539-X. [Epub ahead of print]
    Gaies M, Pasquali SK, Nicolson SC, Shekerdemian L, Witte M, Wolf M, Zhang W, Donohue JE, Mahle WT, .
      BACKGROUND: The Pediatric Heart Network Collaborative Learning Study (PHN CLS) successfully changed practice at 4 hospitals to increase the rate of early extubation within 6 hours after infant heart surgery. It is unknown whether this practice continued after study completion.METHODS: We linked the PHN CLS dataset to the Pediatric Cardiac Critical Care Consortium registry to compare outcomes at 4 active hospitals between the study period (post-CPG) and the first year after study completion (follow-up) after a 3-month washout. Inclusion/exclusion criteria were the same across eras. Primary outcome was early extubation rate after tetralogy of Fallot or aortic coarctation repair. Secondary outcomes included time to first extubation, intensive care, and hospital lengths of stay.
    RESULTS: There were 121 patients post-CPG and 139 in follow-up with no difference in patient characteristics or operation subtypes. Post-CPG early extubation rate declined from 67% to 30% in follow-up (p<0.0001); time to first extubation increased (4.5 vs. 13.5 hours, p<0.0001). One hospital maintained the rate of early extubation (72% vs. 67%), whereas the other three had significantly lower rates in follow-up (p<0.02 for each). Intensive care (2.8 vs. 2.9 days) and post-operative hospital stay (6 vs. 5 days) did not differ between eras (p>0.05 for both). Findings were consistent across operation subtypes.
    CONCLUSIONS: Extubation practice in the first year of follow-up after the PHN CLS reverted toward pre-study levels. One of four hospitals maintained its early extubation strategy, suggesting that specific implementation and maintenance approaches may effectively sustain impact from quality initiatives.
    DOI:  https://doi.org/10.1016/j.athoracsur.2018.09.024
  3. Anesthesiol Clin. 2018 Dec;pii: S1932-2275(18)30070-3. [Epub ahead of print]36(4): 509-521
    Tateosian VS, Richman DC.
      Cardiac risk stratification before surgery informs consent, may advise optimization interventions, and guides intraoperative and postoperative management and monitoring. Published guidelines provide an outline for risk stratification but are only updated every 5 to 10 years; hence, cardiology expert opinion is often needed. Preoperative cardiovascular evaluation starts with an excellent history and physical examination. Accurate assessment of exercise tolerance is paramount in defining risk and determining the need for further testing. Risk/benefit ratio needs to be assessed and reviewed with all stakeholders, which pertains to deciding on cardiac intervention before surgery and bleeding versus thrombosis risk when managing medications.
    Keywords:  CAD (coronary artery disease); Cardiac risk assessment; Congenital heart disease; Congestive heart failure; PCI (percutaneous coronary interventions); RCRI (revised cardiac risk index); Surgical risk; Valvular heart disease
    DOI:  https://doi.org/10.1016/j.anclin.2018.07.003
  4. J Heart Lung Transplant. 2018 Oct 02. pii: S1053-2498(18)31689-9. [Epub ahead of print]
    Schumacher KR, SunkyungYu , Butts R, Castleberry C, Chen S, Edens E, Godown J, Johnson J, Kemna M, Lin K, Lowery R, Simpson K, West S, Wilmot I, Gossett JG.
      BACKGROUND: The influence of Fontan-associated protein-losing enteropathy's (PLE) severity, duration, and treatment on heart transplant (HTx) outcomes is unknown. We hypothesized that long-standing PLE and PLE requiring more intensive therapy are associated with increased post-HTx mortality.METHODS: This 12-center, retrospective cohort study of post-Fontan patients with PLE referred for HTx from 2003 to 2015 involved collection of demographic, medical, surgical, and catheterization data, as well as PLE-specific data, including duration of disease, intensity/details of treatment, hospitalizations, and complications. Factors associated with waitlist and post-HTx outcomes and PLE resolution were sought.
    RESULTS: Eighty patients (median of 5 per center) were referred for HTx evaluation. Of 68 patients listed for HTx, 8 were removed due to deterioration, 4 died waiting, and 4 remain listed. In 52 patients undergoing HTx, post-HTx 1-month survival was 92% and 1-year survival was 83%. PLE-specific factors, including duration of PLE pre-HTx, pre-HTx hospitalizations, need for/frequency of albumin replacement, PLE therapies, and growth parameters had no association with post-HTx mortality. Immunosuppressant regimen was associated with mortality; standard mycophenolate mofetil immunotherapy was used in 95% of survivors compared with only 44% of non-survivors (p = 0.03). Rejection (53%) and infection (42%) post-HTx were common, but not associated with PLE-specific factors. PLE resolved completely in all but 1 HTx survivor at a median of 1 month (interquartile range 1 to 3 months); resolution was not affected by PLE-specific factors.
    CONCLUSIONS: PLE severity, duration, and treatment do not influence post-HTx outcome, but immunosuppressive regimen may have an impact on survival. PLE resolves in nearly all survivors.
    Keywords:  Fontan; congenital heart disease, protein-losing enteropathy; heart transplant; pediatric transplant; protein-losing enteropathy
    DOI:  https://doi.org/10.1016/j.healun.2018.09.024
  5. Anesthesiol Clin. 2018 Dec;pii: S1932-2275(18)30083-1. [Epub ahead of print]36(4): 689-700
    Basel A, Bajic D.
      The article reviews frequently encountered preoperative concerns with a goal of minimizing complications during administration of pediatric anesthesia. It is written with general anesthesiologists in mind and provides a helpful overview of concerns for pediatric patient preparation for routine and nonemergent procedures or interventions. It covers unique topics for the pediatric population, including gestational age, respiratory and cardiovascular concerns, fasting guidelines, and management of preoperative anxiety, as well as the current hot topic of the potential neurotoxic effects of anesthetics on the developing brain.
    Keywords:  Congenital heart disease; Pediatric; Premature; Preoperative anxiety; Preoperative evaluation; Respiratory infection
    DOI:  https://doi.org/10.1016/j.anclin.2018.07.016