bims-hylehe Biomed news
on Hypoplastic Left Heart Syndrome
Issue of 2018‒05‒27
six papers selected by
Richard James
University of Pennsylvania


  1. Ann Thorac Surg. 2018 May 16. pii: S0003-4975(18)30678-7. [Epub ahead of print]
    Holst KA, Dearani JA, Said SM, Davies RR, Pizarro C, Knott-Craig C, Kumar TKS, Starnes V, Kumar SR, Pasquali SK, Thibault DP, Meza JM, Hill KD, Chiswell K, Jacobs JP, Jacobs ML.
      BACKGROUND: Ebstein anomaly (EA) encompasses a broad spectrum of morphology and clinical presentation. Those who are symptomatic early in infancy are generally at highest risk, but there are limited data regarding multi-centric practice patterns and outcomes. We analyzed multi-institutional data concerning operations and outcomes in neonates and infants with EA.METHODS: Index operations reported in the STS Congenital Heart Surgery Database (2010-2016) were potentially eligible for inclusion. Analysis was limited to patients with diagnosis of Ebstein anomaly and less than 1 year of age at time of surgery (neonates ≤30 days, infants 31-365 days).
    RESULTS: The study population included 255 neonates and 239 infants (at 95 centers). Among neonates, median age at operation was 7 days (IQR 4-13) and the majority required preoperative ventilation (61.6%, 157). The most common primary operation performed among neonates was Ebstein repair (39.6%, 101) followed by systemic to pulmonary shunt (20.4%, 52) and tricuspid valve closure (9.4%, 24). Overall neonatal operative mortality was 27.4% (70) with composite morbidity-mortality of 51.4% (48). For infants, median age at operation was 179 days (6 months); the most common primary operation for infants was superior cavopulmonary anastomosis (38.1%, 91) followed by Ebstein repair (15.5%, 37). Overall operative mortality for infants was 9.2% (22) with composite morbidity-mortality of 20.1% (48).
    CONCLUSIONS: Symptomatic EA in early infancy is very high risk and a variety of operative procedures were performed. A dedicated prospective study is required to more fully understand optimal selection of treatment pathways to guide a systematic approach to operative management.
    Keywords:  Ebstein anomaly; congenital heart disease; operative repair; tricuspid valve
    DOI:  https://doi.org/10.1016/j.athoracsur.2018.04.049
  2. J Thorac Cardiovasc Surg. 2018 Apr 18. pii: S0022-5223(18)31027-4. [Epub ahead of print]
    He D, Aggarwal N, Zurakowski D, Jonas RA, Berul CI, Hanumanthaiah S, Moak JP.
      OBJECTIVES: Postoperative arrhythmias are common in children undergoing congenital heart surgery. We evaluated whether intraoperative administration of magnesium was associated with a reduced occurrence of specific postoperative arrhythmias, as has been described previously, or had a broader effect on multiple arrhythmia types, and whether there existed a dose-effect of intraoperative magnesium.METHODS: We used a historical prospective observational cohort study. Propensity score matching using logistic regression was applied to establish similar populations of treatment groups balanced on 5 important covariates.
    RESULTS: Based on propensity score-matched groups, magnesium 50 mg/kg administration was associated with a reduced odds of occurrence of postoperative arrhythmias: any arrhythmia (odds ratio [OR] = 0.50, P < .001), junctional ectopic tachycardia (OR = 0.56, P = .004), accelerated junctional rhythm (OR = 0.56, P = .089), atrial tachycardia (OR = 0.48, P = .005), ventricular tachycardia (OR = 0.52, P = .04), and atrioventricular block (OR = 0.47, P = .03). The effect of magnesium on reducing the odds of occurrence of postoperative arrhythmias did not differ between 25 mg/kg versus 50 mg/kg.
    CONCLUSIONS: As many as one third of our patients undergoing congenital heart surgery had postoperative arrhythmias. Use of intraoperative magnesium was associated with a reduction in the odds of occurrence of all postoperative arrhythmias. Our results do not provide evidence that a greater dose of magnesium is associated with greater arrhythmia risk reduction. Despite the intraoperative use of magnesium, there continued to be a high residual incidence of postoperative arrhythmias, raising the question for studying new or additional agents.
    Keywords:  atrial tachycardia; atrioventricular block; congenital heart surgery; junctional ectopic tachycardia; magnesium; postoperative arrhythmias; ventricular tachycardia
    DOI:  https://doi.org/10.1016/j.jtcvs.2018.04.044
  3. Gen Thorac Cardiovasc Surg. 2018 May 19.
    Tatewaki H, Shiose A.
      In this review article, we describe pulmonary valve replacement (PVR) late after repaired Tetralogy of Fallot (TOF). Since the introduction of surgical intervention for patients with TOF in 1945, surgical management of TOF has dramatically improved early survival with mortality rates, less than 2-3%. However, the majority of these patients continue to experience residual right ventricular outflow tract pathology, most commonly pulmonary valve regurgitation (PR). The patients are generally asymptomatic during childhood and adolescence and, however, are at risk for severe PR later which can result in exercise intolerance, heart failure, arrhythmias, and sudden death. While it has been shown that PVR improves symptoms and functional status in these patients, the optimal timing and indications for PVR after repaired TOF are still debated. This article reviews the current state of management for the patient with PR after repaired TOF.
    Keywords:  Adult congenital heart disease; Pulmonary regurgitation; Pulmonary valve replacement; Sudden death; Tetralogy of Fallot
    DOI:  https://doi.org/10.1007/s11748-018-0931-0
  4. Ann Thorac Surg. 2018 May 17. pii: S0003-4975(18)30697-0. [Epub ahead of print]
    Giordano R, Cantinotti M, Di Tommaso L, Comentale G, Tozzi A, Pilato E, Iannelli G, Palma G.
      BACKGROUND: The transatrial repair of the ventricular septal defect (VSD) requires an adequate exposure of its rim. We retrospectively evaluated the impact of adopting the tricuspid valve incision (TVI) technique, either with detachment or radial incision, on the postoperative outcome of children undergoing surgical VSD repair.METHODS: From January 2008 to September 2017 we retrospectively enrolled 141 patients, divided into two groups: 97 patients were subjected to TVI (68.8%) and 44 patients (31.2%) were not subjected to TVI. All patients received an echocardiogram upon discharge from the hospital and after 1 month, 3 months, 6 months and a year from the treatment.
    RESULTS: No perioperative or late deaths resulted in our dataset. TVI was associated with a slightly longer cardio-pulmonary bypass and cross-clamp time but there were no differences in the surgical outcome between the two groups. Moreover, no differences occurred concerning residual VSD, atrioventricular block or tricuspid regurgitation at discharge. Echocardiograms at follow-up were available for 134 patients (95%) with a median of 5.3 years (range 0.5-9.3) and the degree of tricuspid regurgitation did not differ between groups. No patient required reoperation for tricuspid regurgitation or residual interventricular shunt. Finally, no difference was found even when comparing the two TVI subgroups.
    CONCLUSIONS: TVI should be used whenever intraoperative exposure of VSD is compromised in order to avoid a residual shunt and atrioventricular block. Here we show that this procedure does not significantly compromise the tricuspid function although a large, multicenter, randomized controlled trial is advised to validate this hypothesis.
    Keywords:  pediatric cardiac surgery; tricuspid valve; ventricular septal defect
    DOI:  https://doi.org/10.1016/j.athoracsur.2018.04.054
  5. J Thorac Cardiovasc Surg. 2018 Apr 18. pii: S0022-5223(18)31026-2. [Epub ahead of print]
    Husain SA.
      
    DOI:  https://doi.org/10.1016/j.jtcvs.2018.04.043
  6. Eur J Med Genet. 2018 May 16. pii: S1769-7212(18)30178-2. [Epub ahead of print]
    Crowe L, Graham RH, Robson SC, Rankin J.
      Termination of pregnancy for fetal anomaly is legal in the UK with no upper limit, if two doctors, in good faith, agree "there is a substantial risk that if the child were born it would suffer from such physical or mental abnormalities as to be seriously handicapped". This is Clause E of the Human Fertlisation and Embryology Act. The most commonly sighted Clause is C, which states "the pregnancy has not exceeded its twenty-fourth week and that the continuance of the pregnancy would involve risk, greater than if the pregnancy were terminated, of injury to the physical or mental health of the pregnant woman". This study aimed to investigate health professionals' views on gestational age and acceptable termination of pregnancy for fetal anomaly (TOPFA). We undertook a questionnaire survey of UK health professionals working in fetal medicine, obstetrics and gynaecology and neonatology. A study pack consisting of a self-completion questionnaire, an invitation letter, participant information sheet, and a stamped addressed return envelope, were sent to health professionals. We used four fetal anomalies as case study examples in the questionnaire: isolated cleft lip, hypoplastic left heart, spina bifida and trisomy 21. These anomalies were chosen as they differed in terms of the type of anomaly, the type of impairment, and the perceived severity. Forty-one study packs were returned. For anomalies deemed less serious, later gestational ages were an important consideration when deciding acceptable TOPFA. Long term prognosis was considered an important factor in deciding whether a TOPFA was acceptable alongside gestational age. Clause C of the current UK legislation, which allows a legal termination prior to 24 weeks gestational age if continuing with the pregnancy would impact the mental health of the mother, was deemed a reasonable option for termination when parents are requesting a TOPFA. For each case study example, health professionals responded that TOPFA at '25 weeks and over' was acceptable (cleft lip n = 1; hypoplastic left heart n = 19; spina bifida n = 13 and Trisomy 21 n = 10). Professionals also distinguished between their personal and professional views. These findings offer new insight into how gestational age considerations influence professionals' conceptualisation of acceptable TOPFA.
    Keywords:  Gestational age; Health professionals; Termination of pregnancy; Termination of pregnancy for fetal anomaly
    DOI:  https://doi.org/10.1016/j.ejmg.2018.05.011