bims-hylehe Biomed news
on Hypoplastic left heart syndrome
Issue of 2018‒12‒09
six papers selected by
Richard James
University of Pennsylvania


  1. Int J Cardiol Heart Vasc. 2019 Mar;22 20-25
    Larsson L, Johansson B, Sandberg C, Apers S, Kovacs AH, Luyckx K, Thomet C, Budts W, Enomoto J, Sluman MA, Wang JK, Jackson JL, Khairy P, Cook SC, Alday L, Eriksen K, Dellborg M, Berghammer M, Rempel G, Menahem S, Caruana M, Tomlin M, Soufi A, Fernandes SM, White K, Callus E, Kutty S, Moons P, , .
      Background: Physical activity is important to maintain and promote health. This is of particular interest in patients with congenital heart disease (CHD) where acquired heart disease should be prevented. The World Health Organization (WHO) recommends a minimum of 2.5 h/week of physical activity exceeding 3 metabolic equivalents (METS) to achieve positive health effects. It is unknown whether physical activity levels (PAL) in adult CHD patients differ by country of origin.Methods: 3896 adults with CHD recruited from 15 countries over 5 continents completed self-reported instruments, including the Health Behaviour Scale (HBS-CHD), within the APPROACH-IS project. For each patient, we calculated whether WHO recommendations were achieved or not. Associated factors were investigated using Generalized Linear Mixed Models.
    Results: On average, 31% reached the WHO recommendations but with a great variation between geographical areas (India: 10%-Norway: 53%). Predictors for physical activity level in line with the WHO recommendations, with country of residence as random effect, were male sex (OR 1.78, 95%CI 1.52-2.08), NYHA-class I (OR 3.10, 95%CI 1.71-5.62) and less complex disease (OR 1.46, 95%CI 1.16-1.83). In contrast, older age (OR 0.97, 95%CI 0.96-0.98), lower educational level (OR 0.41, 95%CI 0.26-0.64) and being unemployed (OR 0.57, 95%CI 0.42-0.77) were negatively associated with reaching WHO recommendations.
    Conclusions: A significant proportion of patients with CHD did not reach the WHO physical activity recommendations. There was a large variation in physical activity level by country of origin. Based on identified predictors, vulnerable patients may be identified and offered specific behavioral interventions.
    Keywords:  APPROACH-IS, assessment of patterns of patient-reported outcomes in adults with congenital heart disease — international study; Adult congenital heart disease; CHD, congenital heart disease; CI, confidence interval; HBS-CHD, health behaviour scale; Health-behaviour scale; METS, metabolic equivalents; Metabolic equivalent; NYHA, New York Heart Association (class); OR, odds ratio; PAL, physical activity level; PRO, patient-reported outcomes; Patient-reported outcome; Physical activity level; Physical activity recommendation; WHO, World Health Organization
    DOI:  https://doi.org/10.1016/j.ijcha.2018.11.004
  2. Cardiol Young. 2018 Dec 04. 1-4
    Hussain S, Al-Radi O, Yun TJ, Hua Z, Rahmat B, Rao S, Qi A, Fraser C, d'Udekem Y, Ibrahim Q, Copland I, Whitlock R, Van Arsdell G.
      BACKGROUND: A wide variety of surgical strategies are used in tetralogy of Fallot repair. We sought to describe the international contemporary practice patterns for surgical management of tetralogy of Fallot.METHODS: Surgeons from 18 international paediatric cardiac surgery centres (representing over 1800 tetralogy of Fallot cases/year) completed a Research Electronic Data Capture-based survey. Participating countries include: China (4), India (2), Nepal (1), Korea (1), Indonesia (1), Saudi Arabia (3), Japan (1), Turkey (1), Australia (1), United States of America (2), and Canada (1). Summary measures were reported as means and counts (percentages). Responses were weighted based on case volume/centre.
    RESULTS: Primary repair is the prevalent strategy (83%) with variation in age at elective repair (range). Approximately 47% of sites use patient age as a factor in determining the strategy, with age 90% of all trans-annular repairs.
    CONCLUSIONS: In this cohort representing 11 countries, there is variation in tetralogy of Fallot surgical management with no consensus on standard of practice. A large international prospective cohort study would allow analysis of impact of underlying anatomy and repair strategy on early and late outcomes.
    Keywords:  CHD; survey; tetralogy of Fallot
    DOI:  https://doi.org/10.1017/S1047951118001932
  3. Congenit Heart Dis. 2018 Dec 02.
    Hormaza VM, Conaway M, Schneider DS, Vergales JE.
      OBJECTIVE: Limited information is known on how right ventricular function affects outcomes after stage 2 palliation. We evaluated the impact of different right ventricular indices prior to stage 2 palliation on morbidity and mortality.DESIGN: Retrospective study design.
    SETTING: Pediatric Heart Network Single Ventricle Reconstruction Trial Public Data Set.
    PATIENT: Any variant of stage 1 palliation and all anatomic hypoplastic left heart syndrome variants in the trial were evaluated. Echocardiograms prior to stage 2 palliation were analyzed and compared between those who failed and those who survived.
    INTERVENTION: None.
    OUTCOME MEASURES: Mortality was defined as death, listed for transplant, or transplanted after stage 2 palliation. Morbidity was evaluated as hospital length of stay and duration of intubation.
    RESULTS: A total of 283 patients met criteria for analysis. Of those, only 18 patients failed stage 2. Right ventricular fractional area change was less in those who failed (30% vs 34%, P = .039) and right ventricular indexed end-diastolic volume and end-systolic volume were larger in those who failed (142.74 mL/ BSA1.3 vs 111.29 mL/BSA1.3 , P = .023, 88.45 mL/ BSA1.3 vs 62.75 mL/ BSA1.3 , P = .025, respectively). Larger right ventricular indexed end-diastolic and systolic volumes were associated with failure (OR 1.17 [1.01-1.35] P = .021, OR 1.25 [1.03-1.52] P = .021, respectively). Every 10% increase in RV ejection fraction had a 63% decrease in length of stay and a 68% decrease in duration of intubation (P = .014, and P = .039, respectively).
    CONCLUSION: Patients with decreased right ventricular fractional area change and larger right ventricular indexed end-diastolic and systolic volumes were more likely to fail stage 2 palliation. Those with preserved right ventricular function had a shorter hospital length of stay and duration of intubation. Echocardiographic measurements of right ventricular indices during the interstage period can be utilized to determine the prognosis following stage 2 palliation.
    Keywords:  SVR; echocardiography; hypoplastic left heart syndrome; right ventricular function; stage 2 palliation
    DOI:  https://doi.org/10.1111/chd.12722
  4. Circ Arrhythm Electrophysiol. 2018 Nov;11(11): e006305
    Saarel EV, Law I, Berul CI, Ackerman MJ, Kanter RJ, Sanatani S, Cohen MI, Berger S, Fischbach PS, Burton DA, Dziura J, Brandt C, Simone L, Li F, Olshansky B, Cannom DS, Lampert RJ.
      BACKGROUND: Despite safety concerns, many young patients with implantable cardioverter-defibrillators (ICDs) participate in sports. We undertook a prospective, multinational registry to determine the incidence of serious adverse events because of sports participation. The primary end points were death or resuscitated arrest during sports or injury during sports because of arrhythmia or shock. Secondary end points included system malfunction and incidence of ventricular arrhythmias requiring multiple shocks for termination.METHODS: Athletes with ICDs aged ≤21 years were included in this post hoc subanalysis of the ICD Sports Registry. Data on sports and clinical outcomes were obtained by phone interview and medical records review. ICD shocks and clinical details of lead malfunction were classified by 2 electrophysiologists.
    RESULTS: A total of 129 young athletes participating in competitive (n=117) or dangerous (n=12) sports were enrolled. The mean age was 16 years (range, 10-21; 40% female; 92% white). The most common diagnoses were long QT syndrome (n=49), hypertrophic cardiomyopathy (n=30), and congenital heart disease (n=16). The most common sports were basketball and soccer, including 79 varsity/junior varsity high school and college athletes. During a median follow-up of 42 months, 35 athletes (27%) received 38 shocks. There were no occurrences of death, arrest, or injury related to arrhythmia, during sports. There was 1 ventricular tachycardia/ventricular fibrillation storm during competition. Freedom from lead malfunction was 92.3% at 5 years and 79.6% at 10 years.
    CONCLUSIONS: Although shocks related to competition/practice are not uncommon, there were no serious adverse sequelae. Lead malfunction rates were similar to previously reported in unselected pediatric ICD populations.
    CLINICAL TRIAL REGISTRATION: URL: https://www.clinicaltrials.gov . Unique identifier: NCT00637754.
    Keywords:  arrhythmias, cardiac; death, sudden, cardiac; defibrillators, implantable; shock; sports
    DOI:  https://doi.org/10.1161/CIRCEP.118.006305
  5. Cardiol Young. 2018 Dec 04. 1-4
    Reddy S, Polito A, Staveski S, Dalton H.
      There are substantial knowledge gaps, practice variation, and paucity of controlled trials owing to the relatively small number of patients with critical heart disease. The Pediatric Cardiac Intensive Care Society has recognised this knowledge gap as an area needing a more comprehensive and evidence-based approach to the management of the critically ill child with heart disease. To address this, the Pediatric Cardiac Intensive Care Society created a scientific statements and white papers committee. Scientific statements and white papers will present the current state-of-the-art in areas where controversy exists, providing clinicians with guidance in diagnostic and therapeutic strategies, particularly where evidence-based data are lacking. This paper provides a template for other societies and organisations faced with the task of developing scientific statements and white papers. We describe the methods used to perform a systematic literature search and evidence rating that will be used by all scientific statements and white papers emerging from the Pediatric Cardiac Intensive Care Society. The Pediatric Cardiac Intensive Care Society aims to revolutionise the care of children with heart disease by shifting our efforts from individual institution-based practices to national standardised protocols and to lay the ground work for multicentre high-impact research directions.
    Keywords:  Hydatid cyst; children; echocardiography and scans; pericardial tumour
    DOI:  https://doi.org/10.1017/S1047951118002019
  6. Congenit Heart Dis. 2018 Dec 02.
    Dasgupta S, Dave I, McCracken CE, Mohl L, Sachdeva R, Border W.
      BACKGROUND: Physicians are exposed to workplace factors that may result in acute or chronic stress resulting in burnout. This may impact the productivity and result in suboptimal patient care practices.METHODS: We surveyed pediatric cardiology attending physicians at our institution to assess their perception of burnout and work-life balance using the Maslach Burnout Inventory and the Areas of Work-Life Survey.
    RESULTS: Forty-five out of the 50 pediatric cardiology attendings responded to the survey. They were divided into 4 groups: Interventional/Electrophysiology [n = 3], Cardiac Intensive Care/Inpatient [n = 8], Non-Invasive Imaging [n = 6], and Outpatient [n = 28]. The Maslach Burnout Inventory demonstrated group-specific scores in the areas of emotional exhaustion, depersonalization, and personal accomplishment that were all significantly better than the general population. However, group-specific Areas of Work-Life Survey results demonstrated concerning findings with respect to the perception of work-life balance.
    CONCLUSIONS: Although the Maslach Burnout Inventory did not demonstrate significant burnout among the attending physicians, the Areas of Work-Life Survey results demonstrated reduced work engagement, which can impact patient care and lead to burnout in the future. Based on these results, we plan to implement strategies to help increase work engagement and improve overall organizational effectiveness.
    Keywords:  balance; pediatric cardiology; worklife
    DOI:  https://doi.org/10.1111/chd.12723