bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2019‒08‒11
five papers selected by
Richard James
University of Pennsylvania

  1. Ann Thorac Surg. 2019 Aug 06. pii: S0003-4975(19)31078-1. [Epub ahead of print]
    Pasquali SK, Gaies M, Banerjee M, Zhang W, Donohue J, Russell M, Gaynor JW.
      BACKGROUND: Emerging data across many fields suggest unique patient characteristics can impact disease manifestation and response to therapy, supporting "precision medicine" approaches and more individualized and targeted therapeutic strategies. In children undergoing congenital heart surgery (CHS), current risk models primarily focus on the population-level, and their utility in understanding precise characteristics that place individual patients at risk for poor outcome remains unclear.METHODS: We analyzed index surgeries in the Pediatric Cardiac Critical Care Consortium (PC4) registry (8/14-5/16) and utilized a previously constructed model containing patient factors typically included in in-hospital mortality risk models (age, weight, prematurity, chromosomal anomalies/syndromes, pre-operative factors, STAT score). Partitioned variances based on a hierarchical generalized linear model were used to estimate the proportion of variation in mortality explained by these factors.
    RESULTS: A total of 8406 operations (22 hospitals) were included. We found that only 30% of the total between-patient variation in mortality in our cohort was explained by the patient factors included in our model. Age, STAT score, and pre-operative mechanical ventilation explained the greatest proportion of variation. Of the variation that remained unexplained, 95% was attributable to unmeasured patient factors. In stratified analyses, these results were consistent across patient subgroups.
    CONCLUSIONS: Patient factors typically included in CHS risk models explain only a small portion of total variation in mortality. A better understanding of other under-recognized factors is critical in further defining risk profiles and in developing more individualized and tailored therapeutic strategies.
    Keywords:  congenital heart disease; outcomes
  2. Cardiol Young. 2019 Aug 05. 1-11
    van der Mheen M, Meentken MG, van Beynum IM, van der Ende J, van Galen E, Zirar A, Aendekerk EWC, van den Adel TPL, Bogers AJJC, McCusker CG, Hillegers MHJ, Helbing WA, Utens EMWJ.
      OBJECTIVE: Children with congenital heart disease and their families are at risk of psychosocial problems. Emotional and behavioural problems, impaired school functioning, and reduced exercise capacity often occur. To prevent and decrease these problems, we modified and extended the previously established Congenital Heart Disease Intervention Program (CHIP)-School, thereby creating CHIP-Family. CHIP-Family is the first psychosocial intervention with a module for children with congenital heart disease. Through a randomised controlled trial, we examined the effectiveness of CHIP-Family.METHODS: Ninety-three children with congenital heart disease (age M = 5.34 years, SD = 1.27) were randomised to CHIP-Family (n = 49) or care as usual (no psychosocial care; n = 44). CHIP-Family consisted of a 1-day group workshop for parents, children, and siblings and an individual follow-up session for parents. CHIP-Family was delivered by psychologists, paediatric cardiologists, and physiotherapists. At baseline and 6-month follow-up, mothers, fathers, teachers, and the child completed questionnaires to assess psychosocial problems, school functioning, and sports enjoyment. Moreover, at 6-month follow-up, parents completed program satisfaction assessments.
    RESULTS: Although small improvements in child outcomes were observed in the CHIP-Family group, no statistically significant differences were found between outcomes of the CHIP-Family and care-as-usual group. Mean parent satisfaction ratings ranged from 7.4 to 8.1 (range 0-10).
    CONCLUSIONS: CHIP-Family yielded high program acceptability ratings. However, compared to care as usual, CHIP-Family did not find the same extent of statistically significant outcomes as CHIP-School. Replication of promising psychological interventions, and examination of when different outcomes are found, is recommended for refining interventions in the future.Trial registryDutch Trial Registry number NTR6063,
    Keywords:  Congenital heart defects; children; families; intervention; psychosocial well-being
  3. Heart Lung Circ. 2019 Jul 22. pii: S1443-9506(19)31351-4. [Epub ahead of print]
    Menahem S.
  4. Methodist Debakey Cardiovasc J. 2019 Apr-Jun;15(2):15(2): 105-110
    Backer CL, Mavroudis C.
      Patients with a functionally univentricular heart who have had an atriopulmonary Fontan are at risk for atrial dilatation, atrial arrhythmias, and progressive circulatory failure. Between 1994 and 2018, we performed 149 Fontan conversions with arrhythmia surgery and epicardial pacemaker placement at Ann & Robert H. Lurie Children's Hospital of Chicago. This operation converts the atriopulmonary Fontan to an extracardiac Fontan that improves hemodynamics and controls the atrial arrhythmias. Operative mortality during that time was 2%, and freedom from death or heart transplant at 10 years is 84%. For properly selected patients, Fontan conversion improves both survival and quality of life. Patients with an atriopulmonary Fontan constitute an eroding population, as they face many comorbidities and have a decreased life expectancy without treatment; therefore, all patients with an atriopulmonary Fontan should be evaluated for this procedure.
    Keywords:  Fontan conversion; arrhythmia; atrial fibrillation; atriopulmonary Fontan; congenital heart surgery; cryoablation; epicardial pacemaker; protein-losing enteropathy; tricuspid atresia