bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2020‒03‒08
fourteen papers selected by
Richard James
University of Pennsylvania


  1. Ann Thorac Surg. 2020 Feb 27. pii: S0003-4975(20)30247-2. [Epub ahead of print]
    Hart SA, Tanel RE, Kipps AK, Hoerst AK, Graupe MA, Cassidy SC, Hlavacek AM, Clabby ML, Bush LB, Zhang W, Banerjee MM, Pasquali SK, Gaies M, Madsen N.
      BACKGROUND: Postoperative length of stay (LOS) is an important quality metric and is known to vary widely across hospitals following congenital heart surgery. Whether this variability is explained by factors associated with the intensive care unit (ICU) or acute care unit (ACU) remains unclear. We evaluated the relationship between ICU and ACU LOS and the impact of ACU characteristics on post-operative LOS.METHODS: Congenital heart surgery hospitalizations within the Pediatric Cardiac Critical Care Consortium (PC4) registry (8/2014-2/2018) were included. Models were developed for ICU, ACU, and post-operative LOS adjusting for differences in case-mix across hospitals. PC4 hospitals participating in the Pediatric Acute Care Cardiology Collaborative (PAC3) were also surveyed on ACU organizational factors and practice patterns.
    RESULTS: Overall, 19,674 hospitalizations across 27 hospitals were included. There was significant variation in ICU and ACU LOS. Post-operative LOS appeared to be most closely related to ICU LOS; 75% (6/8) of hospitals with shorter-than-expected post-operative LOS also had shorter-than-expected ICU LOS. A clear relationship between post-operative and ACU LOS was not observed. Hospitals with an ACU able to provide higher acuity care as indexed according to the PAC3 survey were more likely to have shorter post-operative LOS (p <0.01).
    CONCLUSIONS: For hospitals that achieve shorter-than-expected post-operative LOS following congenital heart surgery, ICU LOS appears to be the primary driver. Higher acuity resources in the ACU may be an important factor facilitating earlier transfer from the ICU. These data are key to informing quality improvement initiatives geared toward reducing post-operative LOS.
    Keywords:  Congenital heart disease; Pediatric; Postoperative care
    DOI:  https://doi.org/10.1016/j.athoracsur.2020.01.033
  2. Neoreviews. 2020 Mar;21(3): e179-e192
    Pettitt TW.
      Congenital heart disease is a major public health concern in the United States. Outcomes of surgery for children with congenital heart disease have dramatically improved over the last several decades with current aggregate operative mortality rates approximating 3%, inclusive of all ages and defects. However, there remains significant variability among institutions, especially for higher-risk and more complex patients. As health care moves toward the quadruple aim of improving patient experience, improving the health of populations, lowering costs, and increasing satisfaction among providers, congenital heart surgery programs must evolve to meet the growing scrutiny, demands, and expectations of numerous stakeholders. Improved outcomes and reduced interinstitutional variability are achieved through prioritization of quality assurance and improvement.
    DOI:  https://doi.org/10.1542/neo.21-3-e179
  3. Ir Med J. 2019 Dec 16. 112(10): 1019
    Lynch Á, Ng L, Lawlor P, Lavelle M, Gardner F, Breatnach C, McMahon CJ, Franklin O.
      Prenatal detection of structural congenital heart disease (CHD) optimises cardiovascular stability pre-operatively and post-operative outcomes. We compared prenatal detection rates of critical CHD in units offering universal fetal anomaly scans with those offering imaging to selected women. One hundred and thirteen infants met inclusion criteria. The overall pre-natal detection rate for critical CHD was 57% of liveborn infants. It was 71% (57/80) in hospitals who offered a universal anomaly scan and 29% (9/31) in centres offering a limited service. Postnatal diagnosis was associated with PICU admission (p=0.016) and preoperative mechanical ventilation (p=0.001). One-year mortality was 10 fold higher in the postnatally diagnosed group 15% vs 1.55% (p=0.0066). There is a significant disparity between centres offering universal anomaly versus selective screening. Prenatal detection confers advantage in terms of pre-operative stability and one year survival. Failure to deliver an equitable service exposes infants with CHD to avoidable risk.
  4. Eur J Cardiovasc Nurs. 2020 Feb 29. 1474515120909744
    Moons P.
      
    Keywords:  Heart defects; adolescents; adults; anxiety; children; congenital; depression; nursing
    DOI:  https://doi.org/10.1177/1474515120909744
  5. J Cardiovasc Med (Hagerstown). 2020 Mar 05.
    Gargiulo GD, Bassareo PP, Careddu L, Egidy-Assenza G, Angeli E, Calcaterra G.
      The Fontan procedure is often the only definitive palliative surgical option for patients with a variety of complex CHD sharing in common, a single, dominant ventricle. In recent decades, imaging and therapeutic improvement have played a crucial role in those patients in whom many complications can hamper their life. After 50 years from the first procedure, heart transplantation remains the only definitive treatment for those with a failing Fontan circulation.
    DOI:  https://doi.org/10.2459/JCM.0000000000000951
  6. Arch Cardiovasc Dis. 2020 Feb 26. pii: S1875-2136(19)30138-X. [Epub ahead of print]
    Friedman KG, Tworetzky W.
      Fetal cardiac intervention (FCI) is a novel and evolving technique that allows for in utero treatment of a subset of congenital heart disease. This review describes the rationale, selection criteria, technical features, and current outcomes for the three most commonly performed FCI: fetal aortic stenosis with evolving hypoplastic left heart syndrome (HLHS); HLHS with intact or restrictive atrial septum; and pulmonary atresia with intact ventricular septum, with concern for worsening right ventricular (RV) hypoplasia.
    Keywords:  Aortic stenosis; Cardiologie fœtale; Cathétérisme interventionnel; Fetal cardiology; Hypoplasie du ventricule gauche; Hypoplastic left heart syndrome; Sténose aortique; Transcatheter intervention
    DOI:  https://doi.org/10.1016/j.acvd.2019.06.007
  7. Thorac Cardiovasc Surg. 2020 Mar 01.
    Vervoort D.
      Six billion people worldwide lack access to safe, timely, and affordable cardiac surgical care when needed, despite cardiovascular diseases remaining the world's leading cause of mortality. The large surgical backlog of rheumatic heart disease, stable and high incidence of congenital heart disease, and growing burden of ischemic heart disease around the world calls for urgent scaling of cardiovascular services beyond mere prevention. National Surgical, Obstetric, and Anesthesia Plans are being developed by countries as holistic health systems interventions to increase access to surgical care, but to date, limited to no attention has been given to the inclusion of cardiovascular care.
    DOI:  https://doi.org/10.1055/s-0039-1700969
  8. Ann Thorac Surg. 2020 Feb 27. pii: S0003-4975(20)30244-7. [Epub ahead of print]
    Huang L, Dalziel KM, du Plessis K, Winlaw DS, Cordina R, Pflaumer A, Justo RN, Wheaton GR, Bullock A, Verrall CE, d'Udekem Y.
      BACKGROUND: Surgery is the cornerstone of treatment for single-ventricle patients, but life just begins for most when the staged procedures are completed. A package of care including primary care, out-of-hospital specialist visits and medication is required for patients living with Fontan. This study quantified the current state of out-of-hospital health care use across childhood for Fontan patients using evidence from Australia.METHODS: Patients recruited from the Fontan Registry were linked with the administrative Medicare (universal health insurance) data. Frequency of medical and pharmaceutical care and costs were estimated.
    RESULTS: Data for 115 patients with 12,726 medical and 8,336 pharmaceutical claims were obtained. It was estimated that from 0-20 years of age, patients on average visited a general practitioner 6.4 times per year (95% CI 5.9, 7.0), and specialists including cardiologists 2.8 times per year (95% CI 2.5, 3.0). Average use of allied health professional care peaked at age 6-9 years (1.0 visits per year, 95% CI 0.7, 1.4) with psychological services being most prominent. For pharmaceuticals, an average of 13.3 prescriptions filled per patient per year throughout childhood were observed (95% CI 12.4, 14.2). Overall, out-of-hospital doctor visits of all types totaled an average of 11 visits per year from birth to 20 years. A decline in care was observed when patients reached 18 years across services.
    CONCLUSIONS: Patterns of out-of-hospital health care use were observed, however current guidelines are silent as to whether this is optimal. Further research is needed to better understand the comprehensive needs of this population.
    DOI:  https://doi.org/10.1016/j.athoracsur.2020.01.030
  9. Am J Obstet Gynecol. 2020 Feb 27. pii: S0002-9378(20)30228-3. [Epub ahead of print]
    , Combs CA, Hameed AB, Friedman AM, Hoskins IA.
      Congenital heart defects (CHD) are a leading cause of neonatal morbidity and mortality. Accurate prenatal diagnosis of CHD can reduce morbidity and mortality by improving prenatal care, facilitating predelivery pediatric cardiology consultation, and directing delivery to facilities with resources to manage the complex medical and surgical needs of newborns with CHD. Unfortunately, less than one-half of CHD cases are detected prenatally, resulting in lost opportunities for counseling, shared decision-making, and delivery at an appropriate facility. Quality improvement initiatives to improve prenatal CHD detection depend on the ability to measure the rate of detection at the level of providers, facilities, or populations, but no standard metric exists for measuring the detection of CHD at any level. The need for such a metric was recognized at a Cooperative Workshop held at the 2016 Annual Meeting of the Society for Maternal-Fetal Medicine, which recommended the development of a quality metric to assess the rate of prenatal detection of clinically significant CHD. In this paper, we propose potential quality metrics to measure prenatal detection of "critical" CHD, defined as defects with a high rate of morbidity or mortality or that require surgery or tertiary follow-up. One metric is based on a retrospective approach, assessing whether postnatally diagnosed CHD had been identified prenatally. Other metrics are based on a prospective approach, assessing the sensitivity and specificity of prenatal diagnosis of CHD by comparing prenatal ultrasound findings with newborn findings. Potential applications, limitations, challenges, barriers, and value for both approaches are discussed. We conclude that future development of these metrics will depend on an expansion of the International Classification of Diseases (ICD) system to include specific codes that distinguish fetal CHD from newborn CHD and on the development of record systems that facilitate the linkage of fetal records (in the maternal chart) with newborn records.
    DOI:  https://doi.org/10.1016/j.ajog.2020.02.040
  10. Pediatr Cardiol. 2020 Mar 03.
    Evans WN, Acherman RJ, Mayman GA, Galindo A, Rothman A, Winn BJ, Yumiaco NS, Restrepo H.
      This investigation analyzed the rate of hepatic fibrosis progression in post-Fontan patients that underwent hepatic biopsy. The study cohort comprised post-Fontan patients that underwent cardiac catheterization and transvenous liver biopsy between March 2012 and September 2019. We identified 126 patients that met inclusion criteria. Of the 126, 27 (21%) had a lateral tunnel Fontan, and 99 (79%) had an extracardiac Fontan. For the 27 lateral tunnel Fontan patients, age at Fontan was 4 ± 2 years, and for the 99 extracardiac Fontan patients age at Fontan was 4 ± 2 years (p = 0.98). For the 27 lateral tunnel Fontan patients, the average total fibrosis score was 3.0 ± 1.5; and for the 99 extracardiac Fontan patients, the average total fibrosis was 2.7 ± 1.7 (p = 0.48). For the lateral tunnel Fontan patients, the average Fontan duration was 20 ± 6 years; and for the 99 extracardiac Fontan patients, the average Fontan duration was 11 ± 5 years (p < 0.001). For the 27 lateral tunnel Fontan patients, the average rate of fibrosis progression was 0.16 ± 0.10 total fibrosis score/year; and for the 99 extracardiac Fontan patients, the average rate of fibrosis progression was 0.30 ± 0.23 total fibrosis score/year (p < 0.001). In conclusion, our findings suggest that those with extracardiac Fontans have a faster rate of hepatic fibrosis progression than those with lateral tunnel Fontans. More extensive or multi-institutional studies will be needed to confirm these findings and define the clinical significance of discrepant rates of hepatic fibrosis in post-Fontan patients.
    Keywords:  Cardiac catheterization; Fontan; Hepatic biopsy; Hepatic fibrosis
    DOI:  https://doi.org/10.1007/s00246-020-02331-0
  11. Int J Cardiol. 2020 Feb 24. pii: S0167-5273(19)34253-6. [Epub ahead of print]
    Kessler N, Feldmann M, Schlosser L, Rometsch S, Brugger P, Kottke R, Knirsch W, Oxenius A, Greutmann M, Latal B.
      BACKGROUND: Little is known about the prevalence of structural brain abnormalities and cognitive functioning in the growing population of patients with adult congenital heart disease (ACHD). Thus, our aim was to assess structural abnormalities on brain magnetic resonance imaging (MRI) and their association with intelligence quotient (IQ) in ACHD patients.METHODS: Cross-sectional study in ACHD patients and healthy controls as comparison group. Brain MRI was performed on a 3 T MR scanner, and inspection of structural abnormalities was performed blinded to ACHD or control status. IQ was estimated using the vocabulary and matrix reasoning subtests from the Wechsler Adult Intelligence Scale, Fourth Edition.
    RESULTS: A total number of 67 (55% males) ACHD patients and 55 (51% males) controls were included (mean age 26.9 and 26.0 years respectively). Abnormalities on brain MRI were detected in 29 of 46 (63%) ACHD patients and in none of the controls. Abnormalities consisted of focal infarction or atrophy, white matter lesions, microhemorrhages, and global atrophy. Mean estimated IQ was significantly lower in ACHD patients than in controls (98.51 versus 104.38; 95% CI: -10.09 to -1.66; P value = 0.007). Comparison between patients with and without cerebral abnormalities revealed no significant difference in estimated IQ.
    CONCLUSION: Our findings indicate a high prevalence and wide spectrum of structural brain abnormalities in ACHD patients. Furthermore, this population is at a higher risk of impaired intellectual functioning than healthy controls. However, the present study could not establish a statistically significant association between MRI findings and estimated IQ.
    CLINICAL TRIAL REGISTRATION: ClinicalTrials.gov ID: NCT04041557; URL: https://clinicaltrials.gov/ct2/show/NCT04041557?term=NCT04041557&rank=1.
    Keywords:  ACHD; Adult congenital heart disease; IQ; Neuroimaging; Structural brain abnormalities
    DOI:  https://doi.org/10.1016/j.ijcard.2020.02.061
  12. J Cardiothorac Vasc Anesth. 2020 Feb 01. pii: S1053-0770(20)30113-0. [Epub ahead of print]
    Kim J, Wu A, Grogan T, Wingert T, Scovotti J, Kratzert W, Neelankavil JP.
      OBJECTIVES: To assess whether lactate levels are associated with clinical outcomes in adult congenital heart disease patients who undergo cardiac surgery.DESIGN: Retrospective study.
    SETTING: Single quaternary academic referral center.
    PARTICIPANTS: Adult congenital heart disease patients (≥18 y old) with congenital heart disease undergoing cardiac surgery with cardiopulmonary bypass.
    INTERVENTIONS: Participants were classified into 3 groups according to their peak arterial lactate level within the first 48 hours of surgery.
    MEASUREMENTS AND MAIN RESULTS: In-hospital 30-day mortality, hospital and intensive care unit length of stay, duration of mechanical ventilation after surgery, acute kidney injury defined by Acute Kidney Injury Network criteria, and intensive care unit and hospital readmission within 30 days of surgery were examined. There was no significant difference among different lactate level groups in acute kidney injury, hospital length of stay, intensive care unit length of stay, hours of mechanical ventilation, need for redo surgery, or rates of hospital or intensive care unit readmission. In multivariable analysis, which included cardiopulmonary bypass time, redo surgery, nonelective case, and the adult congenital heart disease complexity score, lactate levels were not a significant predictor of either acute kidney injury or hospital length of stay.
    CONCLUSIONS: The appeal of using lactate levels to risk stratify-patients or to develop a model to predict mortality and morbidity has potential merit, but currently there is insufficient evidence to use lactate levels as a predictor of outcomes in adult patients with congenital heart disease undergoing cardiac surgery.
    Keywords:  adult congenital heart disease; congenital heart disease; perioperative lactate
    DOI:  https://doi.org/10.1053/j.jvca.2020.01.051
  13. Prenat Diagn. 2020 Mar 05.
    Sokołowski Ł, Fendler W, Tobota Z, Kordjalik P, Krekora M, Słodki M, Respondek-Liberska M.
      OBJECTIVES: To evaluate the incidence of hypoplastic left heart syndrome (HLHS) and the efficiency of the screening program using data from the Polish National Registry for Fetal Cardiac Anomalies. To investigate whether HLHS incident rates in Poland are seasonally variable.METHODS: Data on 791 cases of HLHS from the Registry collected between 2004 and 2016 was analyzed.
    RESULTS: The median gestational age for the 734 cases of HLHS detected was 23 weeks. Comparing the age at time of HLHS detection between 2004 and 2016, a decrease from 26 to 20.8 weeks was observed. We noted a rapid increase in HLHS incidence during the initial years of the Registry data, the annual percentage change during that period was 22.0% and this trend lasted until 2010. In the following years, the Registry became representative of the general population which has an estimated incidence of HLHS of 20.93 cases per 100 000 live births. We observed no clear seasonal patterns of HLHS incidence in our population.
    CONCLUSION: The Registry reached a plateau state in terms of diagnosing new cases of HLHS. No evidence of seasonality has been noted. The average gestational age of patients identified as having HLHS decreased significantly during the study period. This article is protected by copyright. All rights reserved.
    DOI:  https://doi.org/10.1002/pd.5677