bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2020‒05‒24
seven papers selected by
Richard James
University of Pennsylvania


  1. Pediatr Cardiol. 2020 May 18.
    Swanson TM, Patel A, Baxter AJ, Morris SA, Maskatia SA, Lantos JD.
      In order to evaluate physicians' willingness to seek legal action to mandate surgery when parents refuse surgery for various congenital heart lesions, we surveyed pediatric cardiologists and cardiovascular surgeons at 4 children's hospitals. We asked whether physicians would support parental refusal of surgery for specific heart defects and, if not, whether they would seek legal action to mandate surgery. We then analyzed associations between physicians' willingness to mandate surgery and national operative mortality rates for each lesion. We surveyed 126 cardiologists and 9 cardiac surgeons at four tertiary referral centers. Overall response rate was 77%. Greater than 70% of physicians would seek legal action and mandate surgery for the following lesions: ventricular septal defect, coarctation of the aorta, complete atrioventricular canal, transposition of the great arteries, tetralogy of Fallot, and unobstructed total anomalous pulmonary venous return. Surgery for all of these lesions has reported mortality rates of < 5%. Physicians were less likely to seek legal action when parents refused surgery for Shone complex, any single ventricle lesion, or any congenital heart disease accompanied by Trisomy 13 or Trisomy 18. Among experts in pediatric cardiology, there is widespread agreement about the appropriate response to parental refusal of surgery for most congenital heart lesions, and these lesions tended to be heart defects with lower surgical mortality rates. Lesions for which there was greater consensus among experts were those with the best outcomes. There was less consensus for lesions with higher mortality rates. Such surveys, revealing disagreement among expert professionals, can provide an operational definition of the current professional "gray zone" in which parental preferences should determine treatment.
    Keywords:  Comfort care; Congenital heart disease; Ethics
    DOI:  https://doi.org/10.1007/s00246-020-02367-2
  2. J Pediatr. 2020 May 13. pii: S0022-3476(20)30337-1. [Epub ahead of print]
    Hill GD, Bingler M, McCoy AB, Oster ME, Uzark K, Bates KE.
      OBJECTIVE: Within the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC), a learning health network developed to improve outcomes for patients with hypoplastic left heart syndrome and variants, we assessed which centers contributed to reductions in mortality and growth failure.STUDY DESIGN: Centers within the NPC-QIC were divided into tertiles based on early performance for mortality and separately for growth failure. These groups were evaluated for improvement from the early to late time period and compared with the other groups in the late time period.
    RESULTS: Mortality was 3.8% for the high-performing, 7.6% for the medium-performing, and 14.4% for the low-performing groups in the early time period. Only the low-performing group had a significant change (P < .001) from the early to late period. In the late period, there was no difference in mortality between the high- (5.7%), medium- (7%), and low- (4.6%) performing centers (P = .5). Growth failure occurred in 13.9% for the high-performing, 21.9% for the medium-performing, and 32.8% for the low-performing groups in the early time period. Only the low-performing group had a significant change (P < .001) over time. In the late period, there was no significant difference in growth failure between the high- (19.8%), medium- (21.5%), and low- (13.5%) performing groups (P = .054).
    CONCLUSIONS: Improvements in the NPC-QIC mortality and growth measures are primarily driven by improvement in those performing the worst in these areas initially without compromising the success of high-performing centers. Focus for improvement may vary by center based on performance.
    Keywords:  quality improvement; single ventricle
    DOI:  https://doi.org/10.1016/j.jpeds.2020.03.014
  3. J Am Heart Assoc. 2020 May 22. e017224
    Alsaied T, Aboulhosn JA, Cotts TB, Daniels CJ, Etheridge SP, Feltes TF, Gurvitz MZ, Lewin MB, Oster ME, Saidi A.
      The corona virus disease -2019 (COVID-19) is a recently described infectious disease caused by the severe acute respiratory syndrome corona virus 2 with significant cardiovascular implications. Given the increased risk for severe COVID-19 observed in adults with underlying cardiac involvement, there is concern that patients with pediatric and congenital heart disease (CHD) may likewise be at increased risk for severe infection. The cardiac manifestations of COVID-19 include myocarditis, arrhythmia and myocardial infarction. Importantly, the pandemic has stretched health care systems and many care team members are at risk for contracting and possibly transmitting the disease which may further impact the care of patients with cardiovascular disease. In this review, we describe the effects of COVID-19 in the pediatric and young adult population and review the cardiovascular involvement in COVID-19 focusing on implications for patients with congenital heart disease in particular.
    Keywords:  COVID‐19; adult congenital heart disease; congenital heart disease; pediatric cardiology
    DOI:  https://doi.org/10.1161/JAHA.120.017224
  4. J Card Surg. 2020 May 22.
    Do-Nguyen CC, Kilcoyne MF, Gray P, Jonas RA.
      
    Keywords:  Fontan procedure; congenital heart disease; fenestration
    DOI:  https://doi.org/10.1111/jocs.14617
  5. Can J Cardiol. 2020 May 10. pii: S0828-282X(20)30443-8. [Epub ahead of print]
    Wan A, Weingarten K, Rapoport A.
      The field of pediatric cardiology has witnessed major changes over the past few decades that have considerably altered patient outcomes, including decreasing mortality rates for many previously untreatable conditions. Despite this, some pediatric cardiology programs are increasingly choosing to partner with their institutional palliative care teams. Why is this? The field of palliative care has also experienced significant shifts over a similar period of time. Today's palliative care is focused on improving quality of life, for any patient with a serious or life-threatening condition, regardless of where they might be on their disease trajectory. Research has clearly demonstrated that improved outcomes can be achieved for a variety of patient cohorts through early integration of palliative care; recent evidence suggests that the same may be true in pediatric cardiology. All pediatric cardiologists need to be aware of what pediatric palliative care has to offer their patients, especially those who are not actively dying. This manuscript reviews the evolution of palliative care and provides a rationale for its integration into the care of children with advanced heart disease. Readers will gain a sense of how and when to introduce palliative care to their families, as well as insight into what pediatric palliative care teams have to offer. Additional research is required to better delineate optimal partnership between palliative care and pediatric cardiology so that we may promote maximal quality of life of patients concurrently with continued efforts to push the boundaries of quantity of life.
    DOI:  https://doi.org/10.1016/j.cjca.2020.04.041
  6. J Cardiothorac Vasc Anesth. 2020 Mar 07. pii: S1053-0770(20)30215-9. [Epub ahead of print]
    Finucane E, Jooste E, Machovec KA.
      Recent decades have witnessed incredible developments in the care of children with congenital heart disease (CHD), such that survival into adulthood is the expected outcome. Improved survival has shifted the focus from improvements in mortality to improvements in morbidity, with long-term neurologic sequelae among the most important. Children with CHD who undergo corrective procedures in infancy and early childhood have a high rate of neurodevelopmental disability later in childhood. Impaired neurocognition is a result of many factors, including prenatal brain injury; preoperative hemodynamic derangements; exposure to anesthetic drugs; and the abnormal physiological states associated with cardiopulmonary bypass, low-flow perfusion, and deep hypothermic circulatory arrest. The intraoperative period presents a challenge to the anesthesiologist because this is a vulnerable period for the neurologic system. Transcranial Doppler ultrasound, electroencephalography, near-infrared spectroscopy, and processed electroencephalography are the neuromonitoring modalities that may be used intraoperatively. Even though each modality has merits, no single modality is able to reliably guide changes to management that improve neurologic outcomes. The best strategy is likely a multimodal neurologic monitoring strategy, although the combination of monitoring may depend on local resources and patient risk factors. This review provides a brief overview of the current knowledge regarding neurodevelopmental outcomes in children with CHD and summarizes the evidence for the use of the following 4 neuromonitoring modalities: transcranial Doppler, cerebral near-infrared spectroscopy, standard electroencephalography, and processed electroencephalography.
    Keywords:  cardiac anesthesia; congenital heart defects; intraoperative monitoring; neurodevelopment; neurodevelopmental outcomes
    DOI:  https://doi.org/10.1053/j.jvca.2020.02.054
  7. Pediatr Cardiol. 2020 May 18.
    Hughes A, Carter K, Cyrus J, Karam O.
      Patients with congenital heart disease (CHD) are surviving longer thanks to improved surgical techniques and increasing knowledge of natural history. Pleural effusions continue to be a complication that affect many surgical patients and are associated with increased morbidity, many times requiring readmission and additional invasive procedures. The risks for development of pleural effusion after hospital discharge are ill-defined, which leads to uncertainty related to strategies for prevention. Our primary objective was to determine, in patients with CHD requiring cardiopulmonary bypass, the prevalence of post-surgical pleural effusions leading to readmission. The secondary objective was to identify risk factors associated with post-surgical pleural effusions requiring readmission. We identified 4417 citations; 10 full-text articles were included in the final review. Of the included studies, eight focused on single-ventricle palliation, one looked at Tetralogy of Fallot patients, and another on pleural effusion in the setting of post-pericardiotomy syndrome. Using a random-effect model, the overall prevalence of pleural effusion requiring readmission was 10.2% (95% CI 4.6; 17.6). Heterogeneity was high (I2 = 91%). In a subpopulation of patients after single-ventricle palliation, the prevalence was 13.0% (95% CI 6.0;21.0), whereas it was 3.0% (95% CI 0.4;6.75) in patients mostly with biventricular physiology. We were unable to accurately assess risk factors. A better understanding of this complication with a focus on single-ventricle physiology will allow for improved risk stratification, family counseling, and earlier recognition of pleural effusion in this patient population.
    Keywords:  Cardiopulmonary bypass; Child; Congenital heart defects; Pericardial effusion; Postpericardiotomy syndrome; Thoracic surgery
    DOI:  https://doi.org/10.1007/s00246-020-02365-4