bims-hylehe Biomed News
on Hypoplastic Left Heart Syndrome
Issue of 2018‒03‒04
six papers selected by
Richard James
University of Pennsylvania


  1. Semin Thorac Cardiovasc Surg. 2018 Feb 21. pii: S1043-0679(18)30022-4. [Epub ahead of print]
      OBJECTIVES: Although the median sternotomy has been the traditional approach for congenital heart surgery, young patients and their families often find the midline scar to be cosmetically unappealing. At our center, a right transverse axillary incision has become the standard approach for many congenital cardiac lesions due to its safety, versatility, and unsurpassed aesthetic result. We present our experience with the axillary approach for a diverse array of congenital defects.METHODS: A retrospective review of patients receiving a right transverse axillary incision for congenital cardiac surgery between 2005-2016 was conducted.
    RESULTS: The right transverse axillary incision was performed in 358 patients for 24 unique procedures. Median age was 5 years (range, 1 month to 60 years) and 225 (63%) patients were female. Median weight was 17 kg (range, 4-124 kg), with 19 (5%) patients weighing less than 6 kg. The most common lesions were atrial septal defects (n=244, 68%) and ventricular septal defects (n=72, 20%). As experience with this approach increased, other repairs included subvalvular aortic membrane resection (n=10, 3%), Tetralogy of Fallot repair (n=7, 2%), ventricular assist device placement (n=3, 1%), and mitral valve repair (n=2, 1%). There were no intra-operative deaths or conversions to sternotomy. In-hospital complications included mortality (n=1, 0.3%), reoperations for bleeding (n=5, 1%), pneumothorax or pleural effusion (n=6, 2%), and permanent pacemaker (n=4, 1%).
    CONCLUSIONS: The right axillary incision allows a safe and effective repair for a broad range of congenital heart defects and is a potential new standard of care for many patients.
    Keywords:  Cardiac surgery; Congenital heart surgery; Incision; Minimally invasive; Pediatric heart surgery
    DOI:  https://doi.org/10.1053/j.semtcvs.2018.02.011
  2. Resuscitation. 2018 Feb 21. pii: S0300-9572(18)30090-X. [Epub ahead of print]
      BACKGROUND: While therapeutic hypothermia (TH) is an effective neuroprotective therapy for neonatal hypoxic-ischemic encephalopathy, TH has not been demonstrated to improve outcome in other pediatric populations. Patients with acquired or congenital heart disease (CHD) are at high risk of both cardiac arrest and neurodevelopmental impairments, and therapies are needed to improve neurologic outcome. The primary goal of our study was to compare safety/efficacy outcomes in post-arrest CHD patients treated with TH versus controls not treated with TH.METHODS: Patients with CHD treated during the first 18 months after initiation of a post-arrest TH protocol (temperature goal: 33.5 °C) were compared to historical and contemporary post-arrest controls not treated with TH. Post-arrest data, including temperature, safety measures (e.g. arrhythmia, bleeding), neurodiagnostic data (EEG, neuroimaging), and survival were compared.
    RESULTS: Thirty arrest episodes treated with TH and 51 control arrest episodes were included. The groups did not differ in age, duration of arrest, post-arrest lactate, or use of ECMO-CPR. The TH group's post-arrest temperature was significantly lower than control's (33.6 ± 0.2 °C vs 34.7 ± 0.5 °C, p < 0.001). There was no difference between the groups in safety/efficacy measures, including arrhythmia, infections, chest-tube output, or neuroimaging abnormalities, nor in hospital survival (TH 61.5% vs control 59.1%, p = NS). Significantly more controls had seizures than TH patients (26.1% vs. 4.0%, p = 0.04). Almost all seizures were subclinical and occurred more than 24 hours post-arrest.
    CONCLUSION: Our data show that pediatric CHD patients who suffer cardiac arrest can be treated effectively and safely with TH, which may decrease the incidence of seizures.
    Keywords:  Cardiac Arrest; Congenital Heart Disease; Intensive Care; Pediatric Cardiology; Seizures; Therapeutic Hypothermia
    DOI:  https://doi.org/10.1016/j.resuscitation.2018.02.022
  3. Air Med J. 2018 Mar - Apr;37(2):pii: S1067-991X(17)30267-5. [Epub ahead of print]37(2): 120-123
      INTRODUCTION: Critically ill children who require transfer to tertiary care centers often require transport by specialized transport teams (TT). These interfacility transports require a medical control physician (MCP). Traditionally this role is assigned to fellows who are taught "on-the-job", but achieving competency in communication for those trained this way may not be optimal. We sought to close this curriculum gap by developing a MCP training program immersing emergency medicine (EM) and critical care (CC) fellows together with TT members to manage a simulated patient.METHODS: Pilot curriculum from 2014-2016 involving 1st year fellows. A case is presented initially with a referral call. By phone the fellow is to communicate with and guide the TT, who is in a separate room managing the "sick" patient using high-fidelity simulation. Each MCP and TT communication is evaluated by faculty and peers. An immediate debriefing session provided formative feedback.
    RESULTS: 11 fellows participated and 10 completed a post-simulation survey (91%). The fellows and TT members rated the curriculum as "highly important" and positively viewed the interprofessional collaboration. Respondents were neutral when asked if communication skills improved.
    CONCLUSION: The MCP training curriculum was viewed favorably and participants reported that this formalized training is needed.
    DOI:  https://doi.org/10.1016/j.amj.2017.12.003
  4. J Artif Organs. 2018 Feb 24.
      The aim of this work was to analyze a shrouded impeller pediatric ventricular assist device (SIP-VAD). This device has distinctive design characteristics and parameter optimizations for minimization of recirculation flow and reduction in high-stress regions that cause blood damage. Computational Fluid Dynamics (CFD) simulations were performed to analyze the optimized design. The bench-top prototype of SIP-VAD was manufactured with biocompatible stainless steel. A study on the hydrodynamic and hemodynamic performance of the SIP-VAD was conducted with predictions from CFD and actual experimentation values, and these results were compared. The CFD analysis yielded a pressure range of 29-90 mmHg corresponding to flow rates of 0.5-3 L/min over 9000-11000 rpm. The predicted value of the normalized index of hemolysis (NIH) was 0.0048 g/100 L. The experimental results with the bench-top prototype showed a pressure rise of 30-105 mmHg for the flow speed of 8000-12000 rpm and flow rate of 0.5-3.5 L/min. The maximum difference between CFD and experimental results was 4 mmHg pressure. In addition, the blood test showed the average NIH level of 0.00674 g/100 L. The results show the feasibility of shrouded impeller design of axial-flow pump for manufacturing the prototype for further animal trials.
    Keywords:  Computational fluid dynamics; Hydraulic performance; Pediatric ventricular assist device; Shrouded impeller
    DOI:  https://doi.org/10.1007/s10047-018-1028-3
  5. J Pediatr Surg. 2018 Feb 07. pii: S0022-3468(18)30057-5. [Epub ahead of print]
      Pediatric surgeons treat a variety of conditions that are distinguished by their low occurrence rate, complexity, and need for integrated multidisciplinary care. Although randomized controlled trials (RCTs) are considered the gold standard for generating evidence to inform best practice, they are poorly suited to rare diseases based on the variability of illness severity, unpredictability in clinical course, and the impact limitations of studying a single intervention at a time. An alternative to RCTs for comparative effectiveness research for rare diseases in pediatric surgery is the patient registry, which collects detailed and condition-specific patient level data related to illness severity, treatment, and outcome, and allows a large, disease-specific database to be created for the dual purposes of collaborative research and quality improvement across participating sites. This review discusses the various functions of a patient registry in fulfilling its mandate of evidence-based practice and outcome improvement using examples from a variety of existing pediatric surgical registries. The value proposition of patient registries as sources of knowledge, facilitators of practice standardization, and enablers of continuous quality improvement is discussed.
    Keywords:  Evidence-based medicine; Patient registry; Randomized controlled trials; Rare disease
    DOI:  https://doi.org/10.1016/j.jpedsurg.2018.02.003
  6. J Crit Care. 2018 Feb 16. pii: S0883-9441(17)31280-7. [Epub ahead of print]45 149-155
      OBJECTIVE: To study the role of parental resilience, emotions accessed during admission and perceived stress in predicting the degree of parental posttraumatic stress disorder (PTSD), anxiety and depression symptoms after a child's treatment in intensive care.METHODS: This was prospective longitudinal cohort study. A total of 196 parents of pediatric intensive care survivors completed questionnaires assessing resilience, perceived stress, emotions experienced during admission, 48h post-discharge (T0). Sociodemographic and medical data were also collected. Main outcomes were anxiety, depression and PTSD, three (T1) and six (T2) months later.
    RESULTS: At T2, 23% of parents reported clinically significant levels of symptoms of PTSD, 21% reported moderate-severe anxiety, and 9% reported moderate-severe depression. These rates were not statistically different to rates at T1. Path analyses indicated that 47% of the variance in psychopathology symptoms at T2 could be predicted from the variables assessed at T0. Resilience was a strong negative predictor of psychopathology symptoms, but this effect was mostly indirect, mediated by the stress that parents perceive during their child's critical hospitalization.
    CONCLUSIONS: Mobilizing coping in order to maintain resilience and to decrease their perceived stress levels could improve parents' mental health outcomes following their child's intensive care treatment.
    Keywords:  Anxiety; Depression; Longitudinal; Parents; Pediatric intensive care; Posttraumatic stress
    DOI:  https://doi.org/10.1016/j.jcrc.2018.02.006