bims-hylehe Biomed news
on Hypoplastic left heart syndrome
Issue of 2019‒03‒24
nine papers selected by
Richard James
University of Pennsylvania


  1. Curr Cardiol Rep. 2019 Mar 18. 21(5): 29
    Misra A, Sriram C, Gupta P, Humes R.
      PURPOSE OF REVIEW: This article attempts to review some of the commonly seen repaired congenital heart defects in the adult population (ACHD), with a focus on important echocardiographic findings that may assist a practitioner in recognizing and managing this group of patients.RECENT FINDINGS: The prevalence of ACHD population is increasing, and currently, there are over 1 million adults with congenital heart disease. At the current time, the total number of ACHD patients exceeds the total number of pediatric patients with CHD. The recently released 2018 American College of Cardiology/American Heart Association guidelines for the management of adults with congenital heart disease (ACHD) recommends transthoracic echocardiography for all ACHD patients for initial assessment and serial assessment as needed (class of recommendation: I) and echocardiography remains the mainstay for diagnosing and managing these patients in adjunct with other available imaging modalities. It is imperative for all cardiologists to be familiar with the echocardiographic features of the commonly seen repaired CHD in adult population.
    Keywords:  Adult congenital heart disease; Echocardiography
    DOI:  https://doi.org/10.1007/s11886-019-1116-x
  2. Congenit Heart Dis. 2019 Mar 18.
    Alenius Dahlqvist J, Sunnegårdh J, Hanséus K, Strömvall Larsson E, Nygren A, Dalén M, Berggren H, Johansson Ramgren J, Wiklund U, Rydberg A.
      OBJECTIVE: Fontan surgery is performed in children with univentricular heart defects. Previous data regarding permanent pacemaker implantation frequency and indications in Fontan patients are limited and conflicting. We examined the prevalence of and risk factors for pacemaker treatment in a consecutive national cohort of patients after Fontan surgery in Sweden.METHODS: We retrospectively reviewed all Swedish patients who underwent Fontan surgery from 1982 to 2017 (n = 599).
    RESULTS: After a mean follow-up of 12.2 years, 13% (78/599) of the patients with Fontan circulation had received pacemakers. Patients operated with the extracardiac conduit (EC) type of total cavopulmonary connection had a significantly lower prevalence of pacemaker implantation (6%) than patients with lateral tunnel (LT; 17%). Mortality did not differ between patients with (8%) and without pacemaker (5%). The most common pacemaker indication was sinus node dysfunction (SND) (64%). Pacemaker implantation due to SND was less common among patients with EC. Pacemaker implantation was significantly more common in patients with mitral atresia (MA; 44%), double outlet right ventricle (DORV; 24%) and double inlet left ventricle (DILV; 20%). In contrast, patients with pulmonary atresia with intact ventricular septum and hypoplastic left heart syndrome were significantly less likely to receive a pacemaker (3% and 6%, respectively).
    CONCLUSIONS: Thirteen percent of Fontan patients received a permanent pacemaker, most frequently due to SND. EC was associated with a significantly lower prevalence of pacemaker than LT. Permanent pacemaker was more common in patients with MA, DORV, and DILV.
    Keywords:  Fontan circulation; congenital heart disease; pacemaker; sinus node dysfunction
    DOI:  https://doi.org/10.1111/chd.12766
  3. Interact Cardiovasc Thorac Surg. 2019 Mar 15. pii: ivz040. [Epub ahead of print]
    Asada S, Yamagishi M, Itatani K, Maeda Y, Taniguchi S, Fujita S, Hongu H, Yaku H.
      OBJECTIVES: The ideal configuration of a reconstructed aortic arch in the Norwood procedure for hypoplastic left heart syndrome is still a matter of debate. Chimney reconstruction was developed to avoid postoperative complications and turbulent flow in the aortic arch. This study sought to clarify early outcomes of the procedure and verify its haemodynamic advantages using computational fluid dynamics (CFD).METHODS: Fourteen consecutive patients with hypoplastic left heart syndrome or a variant who underwent chimney reconstruction in the Norwood procedure between January 2013 and March 2018 were enrolled. Median age and body weight at the time of operation were 2.5 months and 4.1 kg, respectively. Thirteen patients (93.9%) had been palliated with previous bilateral pulmonary artery (PA) banding. In addition, patient-specific CFD models of neoarches based on postoperative computed tomograms from 6 patients were created and the flow profiles analysed.
    RESULTS: Survival rates at 1, 3 and 5 years were 76.6%, 67.3% and 67.3%, respectively. No patient developed left PA compression by neoaorta, neoaortic dilation or neoaortic insufficiency. Only 2 patients (14.3%) required surgical intervention for recoarctation. Fontan completion was performed on 5 patients. On CFD analysis, all reconstructed aortic arches showed low energy loss (9.16-14.4 mW/m2) and low wall shear stresses.
    CONCLUSIONS: Chimney reconstruction was a feasible technique when homografts were not readily available. CFD analyses underscored the fact that this technique produced excellent flow profiles. Larger studies should be conducted to clarify long-term outcomes.
    Keywords:  Chimney reconstruction; Low energy loss; Norwood procedure; Recoarctation
    DOI:  https://doi.org/10.1093/icvts/ivz040
  4. Br Dent J. 2019 Mar;226(6): 447-452
    Hughes S, Balmer R, Moffat M, Willcoxson F.
      The Paediatric Congenital Heart Disease Standards and Specifications (PCHDSS) were published in May 2016 by NHS England. The standards describe in detail the cardiac care patients should expect in England. They are also the first cardiology standards to include an oral health section. The dental standards outline what oral health care patients should receive from both cardiology and dental healthcare professionals, with immediate effect. Children with congenital heart disease (CHD) are at increased risk of infective endocarditis and often have poorer oral health compared to healthy children. Children with cardiac disease can be complex to manage appropriately due to their increased dental anxiety and reduced access to dental care. The PCHDSS dental section highlights the importance of collaborative working between cardiology, primary care and paediatric dentistry. This should ensure preventive advice is delivered regularly, oral disease diagnosed early and patients managed or referred appropriately. This article will summarise CHD, the PCHDSS, its implications and discuss the oral health of children with a cardiac defect. The importance of treatment planning and dental management for this high risk group, in addition to informing readers when to refer to specialist care will also be described.
    DOI:  https://doi.org/10.1038/s41415-019-0094-0
  5. J Pediatr Surg. 2019 Feb 28. pii: S0022-3468(19)30132-0. [Epub ahead of print]
    Kelley-Quon LI, Kenney BD, Bartman T, Thomas R, Robinson V, Nwomeh BC, Bapat R.
      BACKGROUND: Skin-to-skin care (SSC) for infants improves physiologic stability, pain perception, brain development, parental bonding, and overall survival. Using quality improvement (QI) methodology, this project aimed to increase SSC for surgical infants in the neonatal intensive care unit (NICU).METHODS: A multidisciplinary working group composed of key NICU stakeholders instituted a needs assessment querying perceptions and concerns about SSC. Based on survey results, multiple system level interventions were implemented. Data for surgical infants receiving SSC during hospitalization were tracked over time using the electronic health record.
    RESULTS: Overall, 315 infants requiring a surgical consult were admitted to the NICU in the first 12 months of the project. After six months, SSC rates in this group increased from 51% to 60.5% (p < 0.01) and were sustained for 12 months. After one year, nursing staff reporting that they were somewhat to very comfortable providing SSC for surgical infants increased from 44% to 75% (p = 0.001) and the percent of nurses providing SSC for a surgical infant increased from 12% to 37% (p = 0.001). Inadvertent extubation did not significantly increase after implementation of the QI project.
    CONCLUSIONS: Using QI methodology and multidisciplinary engagement, SSC was integrated safely into the routine care of surgical infants in the NICU.
    LEVEL OF EVIDENCE: Level V.
    Keywords:  Quality improvement; Skin-to-skin care; Surgery
    DOI:  https://doi.org/10.1016/j.jpedsurg.2019.02.016
  6. Eur Heart J Cardiovasc Pharmacother. 2019 Mar 23. pii: pvz014. [Epub ahead of print]
    Woudstra OI, Kuijpers JM, Meijboom FJ, Post MC, Jongbloed MRM, Duijnhouwer AL, van Dijk APJ, van Melle JP, Konings TC, Zwinderman AH, Mulder BJM, Bouma BJ.
      AIMS: To assess medication use in adult congenital heart disease(ACHD) patients compared to the age- and sex-matched general population, identify patterns of pharmacotherapy, and analyze associations between pharmacotherapy and adverse outcomes in ACHD.METHODS AND RESULTS: Data of 14,138 ACHD patients from the CONCOR-registry(35[24-48]years, 49% male) and age- and sex-matched referents(1:10-ratio) were extracted from the Dutch Dispensed Drug Register for the years 2006-2014. ACHD patients had more cardiovascular and non-cardiovascular drugs than referents(median 3 vs 1, p < 0.001). Polypharmacy, defined as ≥ 5 dispensed drug types yearly, was present in 30% of ACHD and 15% of referents(OR = 2.47[95%CI 2.39-2.54]). Polypharmacy was independently associated with female sex(OR = 1.92[95%CI 1.88-1.96]), older age(for men:OR=2.3/10years[95%CI 2.2-2.4], for women:OR=1.6/10years[95%CI 1.5-1.6];Pinteraction<0.001), and ACHD severity(mild:OR=2.51[95%CI 2.40-2.61], moderate:OR=3.22[95%CI 3.06-3.40], severe:OR=4.87[95%CI 4.41-5.38]). Cluster analysis identified three subgroups with distinct medication patterns; a "low medication use" group(8-year cumulative survival:98%), and a "cardiovascular" and "comorbidity" group with lower survival(92% and 95%, respectively). Cox regression revealed a strong association between polypharmacy and mortality(HR = 3.94[95%CI 3.22-4.81]), corrected for age, sex and defect severity. Polypharmacy also increased the risk of hospitalization for adverse drug events(HR = 4.58[95%CI 2.04-10.29]).
    CONCLUSION: Both cardiovascular and non-cardiovascular medication use is high in ACHD with twice as much polypharmacy compared to the matched general population. Patients with polypharmacy had a 4-fold increased risk of mortality and adverse drug events. Recognition of distinct medication patterns can help identify patients at highest risk. Drug regimens need repeating evaluation to assess the appropriateness of all prescriptions. More high-quality studies are needed to improve ACHD care with more evidence-based pharmacotherapy.
    Keywords:  Adult congenital heart disease; adverse drug events; cluster analysis; dispensed drugs; multiple medications; phenomapping; polypharmacy
    DOI:  https://doi.org/10.1093/ehjcvp/pvz014
  7. Arch Dis Child. 2019 Mar 20. pii: archdischild-2018-316441. [Epub ahead of print]
    Mazurak M, Kusa J.
      
    Keywords:  alfred blalock; congenital heart defect; helen taussig; tetralogy of fallot history of cardiac surgery; walton lillehei
    DOI:  https://doi.org/10.1136/archdischild-2018-316441
  8. JPEN J Parenter Enteral Nutr. 2019 Mar 22.
    Scheeffer VA, Ricachinevsky CP, Freitas AT, Salamon F, Rodrigues FFN, Brondani TG, Sutil AT, Ferreira CHT, Matte UDS, da Silveira TR.
      BACKGROUND: Undernutrition is a common problem among children with congenital heart disease (CHD) and may lead to poorer surgical outcomes. A higher intake of energy during the postoperative period of CHD surgery seems to be associated with better outcomes. This study aimed to investigate the effect of the use of energy-enriched formula (EE-formula) compared with normocaloric formula during 30 days after CHD surgery.METHODS: A randomized controlled trial with patients undergoing heart surgery in a tertiary hospital in southern Brazil from March 2017 to December 2017 was performed. The intervention group received EE-formula (1 kcal/mL), and the control group received normocaloric formula (0.67 kcal/mL). The researcher in charge of anthropometric evaluation was blinded to the randomization.
    RESULTS: Fifty-nine patients were included; 30 in control group and 29 in intervention group. There were no statistically significant differences between groups regarding age, gender, anthropometry, and surgical risk classification after randomization. A statistically significant difference in z-score of weight for age and in weight gain variation rate between groups after intervention was observed. Antibiotic use was less frequent in the intervention group, and hospital length of stay was shorter. General gastrointestinal side effects were similar between groups, whereas diarrhea was more frequent in the intervention group. However, this side effect was limited and had spontaneous resolution in 4 out of 6 cases.
    CONCLUSION: This study demonstrates that EE-formula use after heart surgery of patients with CHD is well tolerated and may improve short-term nutrition outcome, decrease hospital stay, and reduce antibiotic use.
    Keywords:  heart diseases; infant formula; infant nutrition disorders
    DOI:  https://doi.org/10.1002/jpen.1530