bims-hylehe Biomed news
on Hypoplastic left heart syndrome
Issue of 2019‒01‒20
seven papers selected by
Richard James
University of Pennsylvania


  1. Ann Thorac Surg. 2019 Jan 11. pii: S0003-4975(19)30069-4. [Epub ahead of print]
    Jacobs JP, Mayer JE, Pasquali SK, Hill KD, Overman DM, St Louis JD, Kumar SR, Backer CL, Tweddell JS, Dearani JA, Jacobs ML.
      The Society of Thoracic Surgeons Congenital Heart Surgery Database is a comprehensive clinical outcomes registry which captures almost all pediatric cardiac surgical operations in the United States. It is the platform for all activities of The Society of Thoracic Surgeons related to the analysis of outcomes and the improvement of quality in this subspecialty. This article summarizes current aggregate national outcomes in congenital and pediatric cardiac surgery and reviews related activities in the areas of quality measurement, performance improvement, and transparency. The reported data about aggregate national outcomes are exemplified by an analysis of ten benchmark operation groups performed from January 2014 - December 2017. This analysis documents the overall aggregate Operative Mortality for operations performed at North American participants in The Society of Thoracic Surgeons Congenital Heart Surgery Database (and the interquartile range [IQR] for participant-specific rates of Operative Mortality) for the following procedural groups: off bypass coarctation repair, 1.5% (IQR=0.0%-1.4%); ventricular septal defect (VSD) repair, 0.5% (IQR=0.0%-0.5%); tetralogy of Fallot (TOF) repair, 1.3% (IQR=0.0%-2.0%); complete atrioventricular canal repair (AVC), 2.5% (IQR=0.0%-4.1%); arterial switch operation (ASO), 2.2% (IQR=0.0%-3.0%); ASO + VSD repair, 4.6% (IQR=0.0%-7.1%); Glenn / HemiFontan, 1.8% (IQR=0.0%-2.7%); Fontan operation, 1.0% (IQR=0.0%-0.4%); truncus arteriosus repair, 9.5% (IQR=0.0%-15.4%); and Norwood (Stage 1) operation, 15.0% (IQR=8.3%-25.0%).
    DOI:  https://doi.org/10.1016/j.athoracsur.2018.12.016
  2. Pediatr Cardiol. 2019 Jan 16.
    Yi T, Fan G, Xing Y, Zhao W, Zhang L, Fan F, Jiang X, Ma Z, Yan J, Li S, Wang Q.
      OBJECTIVE: The bidirectional cavopulmonary shunt (BCPS) is an effective palliative procedure which has been widely used to boost outcome of the Fontan procedure. However, there is no standard duration time between these two procedures. Therefore, we investigated whether different time intervals between BCPS and Fontan procedure affects morbidity and mortality of Fontan patients.METHODS: Between 2004 and 2016, 210 post-BCPS patients underwent Fontan operation at Fuwai Hospital. The median interval between BCPS and Fontan procedure was 3.7 years (range 0.55-11.86 years) and this was used to divide study cohort into Group 1 (< 3.7 years; n = 124) and Group 2 (> 3.7 years; n = 86). We analyzed these patients retrospectively in terms of their preoperative characteristics and post-operative and follow-up results.
    RESULTS: Weight z-scores for age at BCPS (- 0.73 ± 1.39 vs - 1.17 ± 1.60, p < 0.05) was significantly higher in Group 2. However, saturation at room air before Fontan (76.42 ± 20.01 vs 82.85 ± 9.69, p < 0.001) was significantly higher in Group 1. The morbidity and mortality were similar between two groups. There were twelve hospital deaths (5.7%): eight (8/124, 6.5%) presented in Group 1 and four (4/86, 4.7%) in Group 2. On multi-variable analysis, risk factors for death were prolonged mechanical ventilation [hazard ratio (HR) 1.02, p = 0.004] and single right ventricle (HR 7.17, p = 0.03). After a mean follow-up of 4.95 years (range 0.74-13.62 years), one patient in Group 1 died of heart failure 13 months after Fontan procedure. The overall Fontan failure in Group 1 was similar to that in Group 2 (2.7% vs 2.6%, p = 0.985). The incidence of arrhythmias and re-intervention were not different between the two groups.
    CONCLUSIONS: Fontan procedure could be performed safely in patient who stayed in long duration between Fontan procedure and BCPS without affecting the operative and long-term follow-up results. However, for post-BCPS patients with severe hypoxemia, earlier age at Fontan might be a good choice.
    Keywords:  Complex congenital heart disease; Fontan physiology; Glenn procedure; Long-term survival; Morbidity and mortality
    DOI:  https://doi.org/10.1007/s00246-018-2049-7
  3. Ann Thorac Surg. 2019 Jan 09. pii: S0003-4975(19)30063-3. [Epub ahead of print]
    Reich B, Heye KN, Tuura ROG, Beck I, Wetterling K, Hahn A, Aktintürk H, Schranz D, Jux C, Kretschmar O, Hübler M, Latal B, Knirsch W.
      BACKGROUND: The long-term impact of altered hemodynamics after Stage II in children with hypoplastic left heart syndrome (HLHS) and hypoplastic left heart complex (HLHC) on cerebral growth and neurodevelopmental outcome is unknown. We aimed to investigate whether elevated central venous and atrial filling pressures before Fontan procedure may be associated with smaller brain volumes and poorer neurodevelopmental outcome after initial Hybrid procedure.METHODS: In a two-center cohort study semi-automated segmentation of cerebral MRI scans was conducted in 25 children with HLHS/HLHC (25 Hybrid) before Fontan procedure (27.6 ± 4.3 months) and in 8 healthy controls (29.7 ± 9.5 months). Study patients were evaluated with the Bayley Scales of Infant and Toddler Development III (Bayley-III) and a neurological examination. Hemodynamic measures after Stage II were assessed with cardiac catheterization at two years of age before Fontan completion. Children with known genetic comorbidities were excluded.
    RESULTS: In HLHS/HLHC patients, higher atrial filling pressures (6 ± 3 mmHg, range 2-14 mmHg) were correlated with reduced brain volumes and lower language composite score, while higher Glenn pressures (10 ± 3 mmHg, range 6-16 mmHg) were related to higher cerebrospinal fluid, reduced brain volumes and lower cognitive, language, and motoric composite scores in the Bayley-III. Compared to controls, white matter volumes were reduced, and cerebrospinal fluid volumes were increased in study patients.
    CONCLUSIONS: These data suggest that altered cardiovascular hemodynamics after stage II influence brain growth and neurodevelopmental outcome in infants with HLHS/HLHC.
    DOI:  https://doi.org/10.1016/j.athoracsur.2018.12.012
  4. Health Psychol. 2019 Feb;38(2): 151-161
    Tesson S, Butow PN, Sholler GF, Sharpe L, Kovacs AH, Kasparian NA.
      OBJECTIVE: Guidelines recommend psychological intervention for children, adolescents, and adults with childhood-onset heart disease and their families, yet a comprehensive review of interventions is lacking. We aimed to determine the efficacy of psychological interventions for this population.METHOD: We searched 6 electronic databases until August, 2017 for English-language, controlled trials of psychological interventions for children, adolescents, or adults with congenital heart disease, inherited arrhythmias, or cardiomyopathies, or their family members. Outcomes of interest included: anxiety, depression, psychological stress and distress, health-related quality of life, coping and adjustment, developmental outcomes, physical health, and parent and family outcomes.
    RESULTS: Of 7,660 identified articles, 11 articles reporting on 9 unique interventions met inclusion criteria. Four interventions included adolescents or adults with congenital heart disease, 2 of which also included individuals with cardiomyopathies, valvular heart conditions, or inherited arrhythmias. Five interventions targeted parents, predominantly mothers, of children with congenital heart disease. Clinical and methodological diversity was observed across trials. Parent-focused interventions demonstrated some improvements in maternal mental health, including anxiety and worry, coping, and family functioning. Evidence for the efficacy of interventions for adolescents and adults was limited. Most trials (8/9) were at "high" or "serious" risk of bias.
    CONCLUSIONS: Despite an established evidence-base for psychological interventions in other chronic illness populations, evidence of efficacy for children and adults with childhood-onset heart disease and their families was limited. Interventions using conceptual frameworks tested in methodologically robust trials are needed to enhance the provision of mental health care for people of all ages with childhood-onset heart disease. (PsycINFO Database Record (c) 2019 APA, all rights reserved).
    DOI:  https://doi.org/10.1037/hea0000704
  5. Am Heart J. 2018 Dec 05. pii: S0002-8703(18)30333-8. [Epub ahead of print]209 9-19
    Burstein DS, Shamszad P, Dai D, Almond CS, Price JF, Lin KY, O'Connor MJ, Shaddy RE, Mascio CE, Rossano JW.
      BACKGROUND: Children with congenital heart disease (CHD) are at risk for advanced heart failure (AHF). We sought to define the mortality and resource utilization in CHD-related AHF in children and young adults.METHODS: All hospitalizations in the Pediatric Health Information System database involving patients ≤21 years old with a CHD diagnosis and heart failure requiring at least 7 days of continuous inotropic support between 2004 and 2015 were included. Hospitalizations including CHD surgery were excluded.
    RESULTS: Of 465,482 CHD hospitalizations, AHF was present in 2,712 (0.6%) [58% infant, 55% male, 30% single ventricle]. AHF therapies frequently used included extracorporeal membrane oxygenation (ECMO) (15%) and cardiac transplant (16%). Ventricular assist device (VAD) support was rare (3%), although VAD use significantly increased from 2004 to 2015 (P < .0010). Hospital mortality in CHD with AHF was 26%, with higher mortality associated with single ventricle heart disease (OR 1.64, 95% CI 1.23-2.19; P = .0009), infancy (OR 1.71, 95% CI 1.17-2.5; P = .0057), non-white race (OR 1.28, 95% CI 1.04-1.59; p=0.0234), and chronic complex comorbidities (OR 1.76, 95% CI 1.34-2.30; P < .0001). Over the 11-year study period, despite the significant increase in CHD-related AHF hospitalizations (P < .0001), hospital mortality improved (P = .0011). Median hospital costs were $252,000, a 6-fold increase above those without AHF, and was primarily driven by hospital length of stay (P < .0001).
    CONCLUSION: AHF in children with CHD in uncommon but increasing and is associated with significant morbidity, mortality and resource utilization. Approximately 1 in 5 children do not survive to hospital discharge. Many risk factors for mortality may not be modifiable, and further study is needed to identify modifiable risk factors and improve care for this complex population.
    DOI:  https://doi.org/10.1016/j.ahj.2018.11.010
  6. J Thorac Cardiovasc Surg. 2018 Dec 04. pii: S0022-5223(18)33215-X. [Epub ahead of print]
    Lamari-Fisher A, Mery CM.
      
    DOI:  https://doi.org/10.1016/j.jtcvs.2018.11.086
  7. J Matern Fetal Neonatal Med. 2019 Jan 15. 1-9
    Pavlicek J, Gruszka T, Polanska S, Dolezalkova E, Matura D, Spacek R, Simetka O, Salounova D, Kacerovsky M.
      OBJECTIVES: This study aimed to evaluate the prenatal rate of congenital heart defects (CHDs) and the frequency of termination of pregnancy (TOP) due to a CHD, depending on the severity of the defect and concomitant diseases of the fetus.METHODS: The data were assessed retrospectively between 2002 and 2017. Ultrasound examination was performed mostly in the second trimester. For analysis, the CHDs were divided into three groups of severity and three groups of fetus impairment.
    RESULTS: A total of 40,885 fetuses underwent echocardiography. The CHDs were detected in 1.0% (398/40,885) and were an isolated anomaly in 69% (275/398). Forty-nine percent (197/398) of families decided to TOP. In all groups of severity, the rate of TOP rose linearly when comparing isolated defects and cases with associated morphological and genetic impairments. The TOP was significantly dependent on the associated anomalies in patients with the most correctable defects (p < .001) and the severity of CHDs in isolated cases without any other impairment (p < .001).
    CONCLUSION: The parents' decision to terminate increased with the severity of the defect and the associated anomalies of the fetus. The parents were mostly influenced by the associated anomalies when the CHD was correctable, and genetic factors played a more important role than morphological ones.
    Keywords:  Congenital heart defect; fetal echocardiography; screening; termination of pregnancy
    DOI:  https://doi.org/10.1080/14767058.2018.1564029