bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2019‒04‒28
ten papers selected by
Richard James
University of Pennsylvania


  1. Eur J Cardiothorac Surg. 2019 Apr 21. pii: ezz123. [Epub ahead of print]
    Goldstone AB, Baiocchi M, Wypij D, Stopp C, Andropoulos DB, Atallah J, Atz AM, Beca J, Donofrio MT, Duncan K, Ghanayem NS, Goldberg CS, Hövels-Gürich H, Ichida F, Jacobs JP, Justo R, Latal B, Li JS, Mahle WT, McQuillen PS, Menon SC, Pike NA, Pizarro C, Shekerdemian LS, Synnes A, Williams IA, Bellinger DC, Newburger J, Gaynor JW.
      OBJECTIVES: Neurodevelopmental disability is the most common complication among congenital heart surgery survivors. The Bayley scales are standardized instruments to assess neurodevelopment. The most recent edition (Bayley Scales of Infant and Toddler Development 3rd Edition, Bayley-III) yields better-than-expected scores in typically developing and high-risk infants than the second edition (Bayley Scales of Infant Development 2nd Edition, BSID-II). We compared BSID-II and Bayley-III scores in infants undergoing cardiac surgery.METHODS: We evaluated 2198 infants who underwent operations with cardiopulmonary bypass between 1996 and 2009 at 26 institutions. We used propensity score matching to limit confounding by indication in a subset of patients (n = 705).
    RESULTS: Overall, unadjusted Bayley-III motor scores were higher than BSID-II Psychomotor Development Index scores (90.7 ± 17.2 vs 77.6 ± 18.8, P < 0.001), and unadjusted Bayley-III composite cognitive and language scores were higher than BSID-II Mental Development Index scores (92.0 ± 15.4 vs 88.2 ± 16.7, P < 0.001). In the propensity-matched analysis, Bayley-III motor scores were higher than BSID-II Psychomotor Development Index scores [absolute difference 14.1, 95% confidence interval (CI) 11.7-17.6; P < 0.001] and the Bayley-III classified fewer children as having severe [odds ratio (OR) 0.24; 95% CI 0.14-0.42] or mild-to-moderate impairment (OR 0.21; 95% CI 0.14-0.32). The composite of Bayley-III cognitive and language scores was higher than BSID-II Mental Development Index scores (absolute difference 4.0, 95% CI 1.4-6.7; P = 0.003), but there was no difference between Bayley editions in the proportion of children classified as having severe cognitive and language impairment.
    CONCLUSIONS: The Bayley-III yielded higher scores than the BSID-II and classified fewer children as severely impaired. The systematic bias towards higher scores with the Bayley-III precludes valid comparisons between early and contemporary cardiac surgery cohorts.
    Keywords:  Congenital heart surgery; Neurodevelopment; Propensity score matching
    DOI:  https://doi.org/10.1093/ejcts/ezz123
  2. Front Neurol. 2019 ;10 329
    Smith JMC, Andrade JG, Human D, Field TS.
      As infant and childhood mortality has decreased in congenital heart disease, this population is increasingly reaching adulthood. Adults with congenital heart disease (ACHD) represent a group with increased risk of stroke, silent brain infarcts, and vascular cognitive impairment. Cyanotic and other complex cardiac lesions confer the greatest risk of these cerebrovascular insults. ACHD patients, in addition to having an increased risk of stroke from structural cardiac issues and associated physiological changes, may have an accelerated burden of conventional vascular risk factors, including hypertension and impaired glucose metabolism. Adult neurologists should be aware of the risks of clinically evident and subclinical cerebrovascular disease in this population. We review the existing evidence on primary and secondary stroke prevention in individuals with complex congenital heart disease, and identify knowledge gaps in need of further research, including treatment of acute stroke in this population. Multisystemic genetic syndromes are outside the scope of this review.
    Keywords:  Adults with Congenital Heart Disease (ACHD); Cardiovascular Disease; Cerebrovascular disease; Congenital Heart Disease (CHD); cognition; cyanotic; fontan; stroke
    DOI:  https://doi.org/10.3389/fneur.2019.00329
  3. Cardiol Young. 2019 Apr 23. 1-5
    Zhao QM, Niu C, Liu F, Wu L, Ma XJ, Huang GY.
      BACKGROUND: Challenges remain in the judgement of pathological murmurs in newborns at maternity hospitals, and there are still many simple major CHD patients in developing countries who are not diagnosed in a timely fashion. This study aimed to evaluate the accuracy of cardiac auscultation on neonatal CHD by general paediatricians.METHODS: We conducted a prospective study at three hospitals. All asymptomatic newborns underwent auscultation, pulse oximetry monitoring, and echocardiography. Major CHD was classified and confirmed through follow-up. We evaluated the accuracy of various degrees of murmurs for detecting major CHD to determine the most appropriate standards and time of auscultation.
    RESULTS: A total of 6750 newborns were included. The median age of auscultation was 43 hours. Cardiac murmurs were identified in 6.6% of newborns. For all CHD, 44.4% had varying degrees of murmurs. A murmur of grade ≥2 used as a reference standard for major CHD had a sensitivity of 89.58%. The false positive rate of murmurs of grade ≥2 for detecting major CHD was significantly negatively related to auscultation time, with 84.4% of false positives requiring follow-up for non-major CHD cardiac issues. Auscultation after 27 hours of life could reduce the false positive rate of major CHD from 2.7 to 0.9%.
    CONCLUSIONS: With appropriate training, maternity hospital's paediatricians can detect major CHD with high detection rates with an acceptable false positive rate.
    Keywords:  Congenital heart disease; cardiac auscultation; echocardiography; newborn screening
    DOI:  https://doi.org/10.1017/S1047951119000799
  4. Int J Cardiol. 2019 Apr 11. pii: S0167-5273(18)36764-0. [Epub ahead of print]
    Yamamura K, Sakamoto I, Morihana E, Hirata Y, Nagata H, Yamasaki Y, Okumura Y, Kohashi K, Koto K, Tsutsui H, Ohga S.
      BACKGROUND: Congestive hepatopathy and hepatocellular carcinoma is a serious complication after Fontan procedure. Liver fibrosis due to hepatic congestion could occur also in adult patients after repair of tetralogy of Fallot (rTOF). However, the incidence and severity remain unclear.METHODS: A total of 111 patients with adult congenital heart disease between 2009 and 2016 were enrolled. Liver fibrosis markers and hemodynamic parameters assessed by cardiac magnetic resonance imaging and catheterization were analyzed in 50 rTOF patients having significant pulmonary regurgitation and/or stenosis, 50 Fontan patients and 11 controls.
    RESULTS: Liver fibrosis markers in patients with rTOF were significantly higher than controls, and tended to be lower than Fontan patients (median, hyaluronic acid: 25.8 vs. 15.9 vs. 40.8, type IV collagen: 129 vs. 113 vs. 166, ng/mL, p < 0.05, respectively). Patients with rTOF showed abnormal hyaluronic acid levels more frequently than controls, and less frequently than Fontan patients (22% vs. 0% vs. 38%, respectively, p < 0.05). Multivariate analyses indicated a positive association of right atrial pressure with type IV-collagen or hyaluronic acid levels (each, p < 0.001, p = 0.003). Abdominal ultrasonography revealed hepatic congestion in 50% of rTOF patients tested. Liver biopsy of the two rTOF patients with highest hyaluronic acid levels showed pathological evidence of moderate and severe (F2 and F3) liver fibrosis and one had combined hepatocellular and cholangiocarcinoma.
    CONCLUSIONS: We first demonstrated elevated liver fibrosis markers in adult patients with rTOF. These levels may help to predict the progressive liver disease as well as consider the timing of pulmonary valve replacement.
    Keywords:  Biomarker; Liver cancer; Liver fibrosis; Pulmonary valve replacement; Tetralogy of Fallot
    DOI:  https://doi.org/10.1016/j.ijcard.2019.04.032
  5. Dev Med Child Neurol. 2019 Apr 25.
    Wotherspoon JM, Eagleson KJ, Gilmore L, Auld B, Hirst A, Johnson S, Stocker C, Heussler H, Justo RN.
      AIM: To assess outcomes in adolescence after surgery for congenital heart disease (CHD) in infancy. Domains analysed included cognition and executive function, social and emotional well-being, adaptive behaviour, academic achievement, and health-related quality of life (HRQoL).METHOD: Twenty-one participants (10 males, 11 females) ranged in age from 14 to 17 years (mean 15y 4.8mo, SD 8.4mo). Twenty had biventricular repairs. All were classified as New York Heart Association class I. Measures included: Wechsler Intelligence and Achievement scales; Wide Range Assessment of Memory and Learning, Second Edition; California Verbal Learning Test - Children's Version; Behaviour Rating Inventory of Executive Function; Conners, Third Edition; Adaptive Behavior Assessment System, Second Edition; Behavior Assessment System for Children, Second Edition; Rey-Osterrieth Complex Figure; and Pediatric Quality of Life Inventory.
    RESULTS: Outcomes were significantly lower (p≤0.01) than population norms for processing speed, mathematical achievement, attention, and visual-spatial ability. Participants reported more frequent learning problems but more positive family relations. HRQoL was significantly lower across most domains by self- and parent-proxy report.
    INTERPRETATION: Individuals with CHD may experience difficulties across a range of domains. These findings emphasize the importance of comprehensive screening, early intervention, and long-term follow-up, as deficits may extend into young adulthood.
    WHAT THIS PAPER ADDS: Identified cognitive, learning, and attentional impairments in adolescents after congenital heart disease surgery in infancy. Combined self-report, caregiver report, and laboratory tasks in a comprehensive neurodevelopmental assessment protocol. Health-related quality of life was lower across most domains.
    DOI:  https://doi.org/10.1111/dmcn.14251
  6. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2019 ;pii: S1092-9126(18)30014-0. [Epub ahead of print]22 61-65
    Connolly HM, Miranda WR, Egbe AC, Warnes CA.
      Congenitally corrected transposition (ccTGA) is a rare form of congenital heart disease characterized by atrioventricular and ventriculoarterial discordance. Patients with ccTGA usually have associated congenital cardiovascular conditions; less than 1% have no associated lesions. Generally, ccTGA is identified during infancy or childhood with features of heart failure or cyanosis when there are associated lesions such as ventricular septal defect and/or pulmonic stenosis. Presentation later in life generally occurs when there are either mild or no associated lesions. Presentation during adulthood may be prompted by symptoms or signs of cardiovascular disease or due to abnormal findings on cardiac testing. Management of patients with ccTGA depends on presentation, symptoms, and associated defects. In this review, we will focus on the management of adult patients with ccTGA.
    Keywords:  Congenitally corrected transposition of the great arteries management in adult
    DOI:  https://doi.org/10.1053/j.pcsu.2019.02.006
  7. J Clin Nurs. 2019 Apr 23.
    Skelton H, Dahlen HG, Psaila K, Schmied V.
      AIMS AND OBJECTIVES: This qualitative study explored the experiences of neonatal nurses with facilitating closeness between parents and babies with congenital abnormalities in the Neonatal Intensive Care Unit (NICU).BACKGROUND: Babies with congenital abnormalities often require admission to the Neonatal Intensive Care Unit. Parents may experience emotional distancing from their baby, due to their response to their baby's congenital abnormality, as well as physical separation due to their baby's hospitalisation in the NICU. NICU nurses can help facilitate the development of closeness between babies and parents in the NICU.
    METHOD: This qualitative interpretive description study involved face-to-face, semi-structured interviews with twelve neonatal nurses following which the data collected were thematically analysed. The consolidated criteria for reporting qualitative research (COREQ) checklist was used in preparing this paper.
    RESULTS: Three major themes emerged from the analysis- 'Everyone copes differently,' 'You have to focus on what is the normal thing' and 'It's very much an individualised approach.' Participants often felt unprepared to care for babies with congenital abnormalities, and implemented coping mechanisms to overcome the emotional labour they experienced. Skin-to-skin cuddles were considered the most beneficial strategy for parents to develop closeness with their baby. Participants recognised that they sometimes 'pushed' parents into engaging with their baby. Participants highlighted the importance of individualised, supportive care for these parents.
    CONCLUSIONS: Neonatal nurses require increased education about congenital abnormalities and individualised care to support them in caring for babies with congenital abnormalities.
    RELEVANCE TO CLINICAL PRACTICE: Further research is needed on parental experience of developing closeness with a baby with a congenital abnormality in the NICU. This article is protected by copyright. All rights reserved.
    Keywords:   NICU ; closeness; congenital abnormality; parent
    DOI:  https://doi.org/10.1111/jocn.14894
  8. J Heart Lung Transplant. 2019 Apr 04. pii: S1053-2498(19)31458-5. [Epub ahead of print]
    Kulkarni A, Patel N, Singh TP, Mossialos E, Mehra MR.
      BACKGROUND: In this study we sought to evaluate risk factors (RFs) for death or heart transplantation (D-HT) in single-ventricle (SV) physiology due to tricuspid atresia (TA), pulmonary atresia‒intact ventricular septum (PA-IVS), and heterotaxy with SV (HX), clinical conditions for which outcome data are limited.METHODS: To conduct a systematic review, we included citations that evaluated occurrence of D-HT in SV physiology of TA, PA-IVS, and HX in English articles published between January 1998 and December 2017 based on inclusion and exclusion criteria, following the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) guidelines. The Cochrane Risk of Bias in Non-Randomized Studies-Interventions (ROBINS-I) tool for non-randomized studies was used to assess the risk of bias. Meta-analysis was performed if RF data were available in more than 3 studies.
    RESULTS: Of 11,629 citations reviewed, 30 met inclusion criteria. All 30 were observational, retrospective studies. In all, 1,770 patients were included, 481 died and 21 underwent HT (63 lost to follow-up); 723 patients reached Fontan completion. We found that systemic ventricular dysfunction (odds ratio [OR] 20.7, confidence interval [CI] 10.0-42.5, I2 = 0%) and atrioventricular valve regurgitation (AVR) were associated with risk of D-HT (OR 3.7, CI 1.9-6.9, I2 = 14%). RF associations with D-HT could not be derived for right ventricle‒dependent coronary circulation, pulmonary arteriovenous malformations, total anomalous pulmonary venous return, arrhythmias, and pulmonary atresia.
    CONCLUSIONS: This systematic review and meta-analysis has identified a high mortality rate in children born with non-HLHS SV heart disease and points to potential under-utilization of HT. Systemic ventricular dysfunction and AVR were identified as RFs for D-HT in this subset of patients SV with TA, PA-IVS, and HX.
    Keywords:  congenital heart disease; death; heart transplantation; heterotaxy syndrome; pulmonary atresia‒intact ventricular septum; tricuspid atresia
    DOI:  https://doi.org/10.1016/j.healun.2019.04.001