bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2020‒01‒19
six papers selected by
Richard James
University of Pennsylvania


  1. N Z Med J. 2020 Jan 17. 133(1508): 111-117
    Cloete E, Gentles TL, Bloomfield FH.
      The mortality risk for infants with critical congenital heart disease (CCHD) unrecognised at the time of birth is high. Pulse oximetry has been utilised as a screening tool for the detection of these anomalies in the newborn as the majority will have a degree of hypoxaemia. This screening strategy has a moderate sensitivity and excellent specificity for the detection of CCHD, and a low false-positive rate. Respiratory and infective diseases are responsible for a large number of positive test results. The early recognition of these diseases can also improve health outcomes. Different approaches have been taken to introduce screening, ranging from hospital-led initiatives to mandatory state-wide policies. A study conducted in New Zealand demonstrated that sector-led screening initiatives are unlikely to result in equitable outcomes. In this midwifery-led maternity setting a nationwide pulse oximetry screening programme with adequate human and material resources should be introduced.
  2. JAMA Pediatr. 2020 Jan 13. e195316
    Wu Y, Kapse K, Jacobs M, Niforatos-Andescavage N, Donofrio MT, Krishnan A, Vezina G, Wessel D, du Plessis A, Limperopoulos C.
      Importance: Prenatal maternal psychological distress can result in detrimental mother and child outcomes. Maternal stress increases with receipt of a prenatal diagnosis of fetal congenital heart disease (CHD); however, the association between maternal stress and the developing brain in fetuses with CHD is unknown.Objective: To determine the association of maternal psychological distress with brain development in fetuses with CHD.
    Design, Setting, and Participants: This longitudinal, prospective, case-control study consecutively recruited 48 pregnant women carrying fetuses with CHD and 92 healthy volunteers with low-risk pregnancies from the Children's National Health System between January 2016 and September 2018. Data were analyzed between January 2016 and June 2019.
    Exposures: Fetal CHD and maternal stress, anxiety, and depression.
    Main Outcomes and Measures: Maternal stress, anxiety, and depression were measured using the Perceived Stress Scale, Spielberger State-Trait Anxiety Inventory, and Edinburgh Postnatal Depression Scale, respectively. Volumes of fetal total brain, cerebrum, left and right hippocampus, cerebellum, and brainstem were determined from 3-dimensionally reconstructed T2-weighted magnetic resonance imaging (MRI) scans.
    Results: This study included 223 MRI scans from 140 fetuses (74 MRIs from 48 fetuses with CHD and 149 MRIs from 92 healthy fetuses) between 21 and 40 weeks' gestation. Among 48 women carrying fetuses with CHD, 31 (65%) tested positive for stress, 21 (44%) for anxiety, and 14 (29%) for depression. Among 92 pregnant women carrying healthy fetuses, 25 (27%) tested positive for stress, 24 (26%) for anxiety, and 8 (9%) for depression. Depression scores were higher among 17 women carrying fetuses with single-ventricle CHD vs 31 women carrying fetuses with 2-ventricle CHD (3.8; 95% CI, 0.3 to 7.3). Maternal stress and anxiety were associated with smaller left hippocampal (stress: -0.003 cm3; 95% CI, -0.005 to -0.001 cm3), right hippocampal (stress: -0.004; 95% CI, -0.007 to -0.002; trait anxiety: -0.003; 95% CI, -0.005 to -0.001), and cerebellar (stress: -0.06; 95% CI, -0.09 to -0.02) volumes only among women with fetal CHD. Impaired hippocampal regions were noted in the medial aspect of left hippocampal head and inferior aspect of right hippocampal head and body. Impaired cerebellar regions were noted in the anterior superior aspect of vermal and paravermal regions and the left cerebellar lobe.
    Conclusions and Relevance: These findings suggested that psychological distress among women carrying fetuses with CHD is prevalent and is associated with impaired fetal cerebellar and hippocampal development. These data underscore the importance of universal screening for maternal psychological distress, integrated prenatal mental health support, and targeted early cognitive-behavioral interventions given that stress is a potentially modifiable risk factor in this high-risk population.
    DOI:  https://doi.org/10.1001/jamapediatrics.2019.5316
  3. Hellenic J Cardiol. 2020 Jan 14. pii: S1109-9666(20)30002-6. [Epub ahead of print]
    Sfyridis PG, Mylonas KS, Avramidis D, Kontzoglou K, Kouraklis G, Rokkas CK, Sarris GE.
      
    Keywords:  Cardiopulmonary Exercise Test; Fontan
    DOI:  https://doi.org/10.1016/j.hjc.2019.12.008
  4. J Thorac Cardiovasc Surg. 2019 Nov 30. pii: S0022-5223(19)33962-5. [Epub ahead of print]
    Weixler VHM, Zurakowski D, Kheir J, Guariento A, Kaza AK, Baird CW, Del Nido PJ, Emani SM.
      OBJECTIVES: The study aim was to compare Fontan patients undergoing lateral tunnel (LT) versus extracardiac conduit (ECC) technique.METHODS: Fontan patients (LT vs ECC) from January 2000 to December 2017 were analyzed retrospectively. Baseline characteristics were analyzed as covariates. Primary outcomes (ie, mortality, Fontan failure, thrombosis, and pacemaker implantation) were compared using time-to-event models. Subgroup analysis including only initially fenestrated cases and propensity score matching were performed.
    RESULTS: Eight hundred one Fontan patients: LT (n = 638) versus ECC (n = 163) were included. Median follow-up time was 4.8 years (range, 1.1-10.8 years). Baseline characteristics were similar except for age: LT versus ECC: 2.6 years (range, 2.2-3.2 years) versus 3.1 years (range, 2.6-4.7 years) (P < .01) and mean pulmonary artery pressure: LT versus ECC: 12 mm Hg (11-15 mm Hg) versus 11 mm Hg (10-13 mm Hg) (P < .05). Early mortality was significantly higher in ECC versus LT group (3.1%; vs 0.5%; P < .05). Freedom from death, heart transplantation and Fontan failure were significantly longer in LT vs ECC (P < .01). After correcting for age, diagnosis, surgical technique, surgeon, mean pulmonary artery pressure, and fenestration, the ECC group showed worse freedom from death (hazard ratio, 2.8; P < .01) and Fontan failure (hazard ratio, 3.0; P < .01). No difference in pacemaker implantation rate was demonstrated (P = .25). Early fenestration closure was associated with higher risk of early (hazard ratio, 30.5) and late mortality (hazard ratio, 3.5). After matching, log-rank tests showed significant differences between the 2 groups for Fontan failure at 5 and 10 years (P < .01) and mortality at 5 years (P = .02).
    CONCLUSIONS: When compared with ECC, LT Fontan is associated with better short and midterm outcomes. Spontaneous fenestration closure is an independent risk factor for early/late mortality.
    Keywords:  Fontan procedure; Lateral tunnel; extracardiac conduit; univentricular palliation
    DOI:  https://doi.org/10.1016/j.jtcvs.2019.11.048
  5. J Card Surg. 2020 Jan 16.
    Al Absi HS, Kiraly L, Mora B, Al Zoebie L, Al Shehhi S, Talo H.
      Fontan procedure is the preferred palliation for patients with single ventricles.OBJECTIVES: To evaluate early morbidity and mortality after Fontan operation in 87 consecutive patients, between 2007 and 2017.
    METHODS: Early survival, duration of intensive care unit (ICU), and hospital stays were the main outcomes evaluated. Potential influencing factors evaluated included preoperative and intraoperative variables.
    RESULTS: Fontan procedure was performed at a median age of 4.2 years (range, 17 months-26 years), and a median weight of 15.5 kg (range, 8-72 kg). Extracardiac Fontan was the procedure of choice. The median cardiopulmonary bypass time was 122  minutes (range, 58-550 minutes). The majority had a fenestration (75 out of 87). Postoperatively, the median duration of ICU stay and total hospital stay were (4, 1-76 days) and (16, 1-85 days), respectively. Fontan failure occurred in one patient (1%). Overall early survival was 94%, resulting in a mortality rate of 6%. Univariate analysis showed that heterotaxy (odds ratio [OR], 2.222; confidence interval [CI], 1.345-6.250; P = .003) and decreased ventricular function (OR, 2.207; CI, 1.348-6.061; P = .002) significantly decreased survival. The same analysis failed to identify any statistically significant risk factors for prolonged hospital and ICU stays.
    CONCLUSION: Our reported mortality and morbidity rates compared favorably with the reported rates. Therefore, Fontan operation can be performed in a tertiary care center in the United Arab Emirates with favorable early postoperative outcomes.
    Keywords:  Fontan-Kreutzer procedure; univentricular heart disease
    DOI:  https://doi.org/10.1111/jocs.14425
  6. Dev Neurorehabil. 2020 Jan 12. 1-7
    Fourdain S, Simard MN, Dagenais L, Materassi M, Doussau A, Goulet J, Gagnon K, Prud'Homme J, Vinay MC, Dehaes M, Birca A, Poirier NC, Carmant L, Gallagher A.
      Purpose: This retrospective study aims to describe the gross motor development of children aged 4 to 24 months with congenital heart disease (CHD) enrolled in a systematic developmental follow-up program and to describe the frequency of physical therapy sessions they received between 4 and 8 months of age.Methods: Twenty-nine infants with CHD underwent motor evaluations using the AIMS at 4 months, and the Bayley-III at 12 and 24 months.Results: Based on AIMS, 79% of 4-month-old infants had a gross motor delay and required physical therapy. Among these, 56.5% received one to two physical therapy sessions, and 43.5% received three to six sessions. Infants who benefited from regular interventions tended to show a better improvement in motor scores from 12 to 24 months.Conclusion: This study highlights the importance of early motor screening in infants with CHD and suggests a potential benefit of early physical therapy in at-risk children.Abbreviations: CHD: Congenital heart disease; AIMS: Alberta Infant Motor Scales; Bayley-III: Bayley Scales of Infant and Toddler Development, Third edition; Bayley-III/GM: Gross Motor section of the Bayley Scales of Infant and Toddler Development, Third edition.
    Keywords:  Alberta Infant Motor Scales (AIMS); Bayley Scales of Infant and Toddler Development; Congenital heart disease (CHD); Third edition (Bayley-III); early intervention; gross motor development
    DOI:  https://doi.org/10.1080/17518423.2020.1711541