bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2019‒07‒14
twelve papers selected by
Richard James
University of Pennsylvania


  1. Ann Thorac Surg. 2019 Jul 09. pii: S0003-4975(19)31000-8. [Epub ahead of print]
    Chew JD, Hill KD, Jacobs ML, Jacobs JP, Killen SAS, Godown J, Wallace AS, Thibault D, Chiswell K, Bichell DP, Soslow JH.
      BACKGROUND: Turner syndrome (TS) is a genetic syndrome characterized by monosomy X (45XO) in phenotypic females and is commonly associated with congenital heart disease. We sought to describe the distribution, mortality, and morbidity of congenital heart surgery in TS and compare outcomes to subjects without genetic syndromes.METHODS: The Society of Thoracic Surgeons Congenital Heart Surgery Database was used to evaluate index cardiovascular operations performed from 2000-2017 in pediatric patients (0-18 years) with and without TS. Analyses were stratified by most common operations, including coarctation repair, aortic arch repair, partial anomalous pulmonary venous return repair, Norwood, superior cavopulmonary anastomosis (Glenn), and Fontan.
    RESULTS: Seven hundred-eighty operations in TS and 62,659 operations in controls were included. The most common TS operations included coarctation repair (n=274,35%), aortic arch repair (N=116,15%) and Norwood (N=59,8%). Compared to controls, TS subjects had lower weight-for-age Z-scores across all operations (p<0.01 for all), however, operative mortality rates did not differ significantly. Chylothorax rate was higher in TS after coarctation repair (8.8% vs 2.8%;p<0.0001) and Norwood (22% vs 8.1%;p=0.0002). Post-operative length of stay was longer in TS for coarctation repair (median 6.5,IQR[5.0,15.5] vs 5.0[4.0,9.0];p<.0001), aortic arch repair (15.0[8.0,27.5] vs 11.0[7.0,21.0];p=0.0035), and Glenn (9.0,[6.0,16.0] vs 6.0,[5.0,11.0];p=0.0132).
    CONCLUSIONS: Surgeries for left-sided obstructive lesions are the most common operations in TS. Despite increased morbidity for select operations, TS was not associated with increased operative mortality.
    DOI:  https://doi.org/10.1016/j.athoracsur.2019.05.047
  2. J Pediatr Psychol. 2019 Jul 10. pii: jsz055. [Epub ahead of print]
    Williams TS, McDonald KP, Roberts SD, Chau V, Seed M, Miller SP, Sananes R.
      Despite improved survival among children with congenital heart disease (CHD), the risk of psychosocial difficulties remains largely unchanged with an increased emphasis of improving support for parents as a mechanism to optimize outcomes.OBJECTIVE: Using qualitative and quantitative methods, the current cross-sectional study examined parents' experiences at the time of their child's diagnosis, what they thought helped their child recover, barriers to support, and identified needs for future models of care.
    METHOD: The sample included 26 parents (22 mothers, 3 fathers, and 1 mother/father pair) of children with CHD, ranging in age between 6 months and 4 years with a mean age of 2 years.
    RESULTS: Qualitative results were organized around five themes: (a) They (medical team) saved my child's life, (b) My child is going to be okay, (c) Not out of the woods, (d) Optimizing support for my child and myself, and (e) What still gets in the way. Parents uniformly expressed a need for greater mental health support for their children as well as programs to improve parents' skill and confidence, with no difference between age groups (< 2 years and > 2 years of age). Common barriers to service included distance and time off work.
    CONCLUSION: Parents' experiences informed both acute and long term implications following CHD diagnoses, and highlight current gaps in mental health care. Direction for clinical care and improved intervention opportunities are discussed.
    Keywords:  CHD; mental health; needs assessment; parent experiences; support
    DOI:  https://doi.org/10.1093/jpepsy/jsz055
  3. Cardiol Young. 2019 Jul 09. 1-6
    Holst LM, Kronborg JB, Idorn L, Bjerre JV, Vejlstrup N, Juul K, Ravn HB.
      OBJECTIVE: To describe the impact of CHD surgery in early childhood on quality of life in children aged 10-16 years with surgically corrected Ventricular Septal Defect, Transposition of the Great Arteries, and Tetralogy of Fallot.METHOD: A cross-sectional survey study of quality of life survey on 161 children and adolescents aged 10-16 years with surgically corrected Ventricular Septal Defect, Transposition of the Great Arteries, and Tetralogy of Fallot. The international Paediatric Quality of Life 4.0 quality of life questionnaires were applied and collected for assessment from patients and parents. The endpoints were total, physical, emotional, social, and school quality of life scores.
    RESULTS: The quality of life total and school scores was significantly lower in children with CHD than their healthy peers. There was no significant difference in quality of life between the three CHD groups. All three CHD groups had a significantly lower total (7.7-13.2%, p&lt;0.001) and school scores (21.1-31.6%, p&lt;0.001) than the control group. The tetralogy of Fallot group was the only group that had significantly lower scores in the physical subscale (p&lt;0.001) than the controls.
    CONCLUSION: Children and adolescents with surgically corrected CHD show losses in quality of life in total and school scores compared to healthy controls. The tetralogy of Fallot group was the only CHD group that had significantly lower physical score than the controls.
    Keywords:  CHD; Quality of life; Tetralogy of Fallot; Transposition of the Great Arteries; Ventricular Septal Defect; questionnaire
    DOI:  https://doi.org/10.1017/S1047951119001604
  4. Congenit Heart Dis. 2019 Jul 07.
    Byrne RD, Weingarten AJ, Clark DE, Huang S, Perri RE, Scanga AE, Menachem JN, Markham LW, Frischhertz BP.
      SETTING: Fontan-associated liver disease universally affects adults with single ventricle heart disease. Chronic kidney disease is also highly prevalent in adult Fontan patients. In this study, we evaluate the relationship of Fontan hemodynamics invasively and noninvasively with extra-cardiac dysfunction as measured by MELD and MELD-XI.OBJECTIVE: We hypothesize that invasive and noninvasive measures of Fontan circuit congestion and ventricular dysfunction are associated with increased MELD and MELD-XI scores.
    DESIGN: Single-center data from adults with Fontan palliation who had ongoing care, including cardiac catheterization, were retrospectively collected. Hemodynamic data from cardiac catheterization and echocardiographic assessment of ventricular and atrioventricular valve function were tested for association with serum creatinine, MELD, and MELD-XI. Linear regression was used to perform multivariable analysis in the echocardiogram cohort.
    RESULTS: Fifty-seven patients had congruent lab and catheterization data for analysis. Sixty-three and sixty-nine patients had congruent lab and echocardiogram data for MELD and MELD-XI, respectively. Of the hemodynamic variables analyzed, only decreased systemic oxygen saturation had significant correlation with elevated MELD and MELD-XI (P = .045). Patients with moderately or severely reduced ejection fraction by echocardiogram had significantly higher MELD and MELD-XI scores compared to those with normal or mildly depressed systolic ventricular function (P = .008 and P < .001 for MELD and MELD-XI, respectively). Significant differences in creatinine were also found among the ventricular dysfunction groups (P = .02).
    CONCLUSIONS: In adults following Fontan palliation, systolic ventricular dysfunction and decreased oxygen saturation were associated with hepatic and renal dysfunction as assessed by elevated serum creatinine, MELD, and MELD-XI scores.
    Keywords:  Fontan; MELD; extra-cardiac; liver; renal
    DOI:  https://doi.org/10.1111/chd.12820
  5. Cardiol Young. 2019 Jul 10. 1-6
    Brown JR, Stabler ME, Parker DM, Vricella L, Pasquali S, Leyenaar JK, Bohm AR, MacKenzie T, Parikh C, Jacobs ML, Jacobs JP, Everett AD.
      OBJECTIVE: To evaluate the association between novel pre- and post-operative biomarker levels and 30-day unplanned readmission or mortality after paediatric congenital heart surgery.METHODS: Children aged 18 years or younger undergoing congenital heart surgery (n = 162) at Johns Hopkins Hospital from 2010 to 2014 were enrolled in the prospective cohort. Collected novel pre- and post-operative biomarkers include soluble suppression of tumorgenicity 2, galectin-3, N-terminal prohormone of brain natriuretic peptide, and glial fibrillary acidic protein. A model based on clinical variables from the Society of Thoracic Surgery database was developed and evaluated against two augmented models.
    RESULTS: Unplanned readmission or mortality within 30 days of cardiac surgery occurred among 21 (13%) children. The clinical model augmented with pre-operative biomarkers demonstrated a statistically significant improvement over the clinical model alone with a receiver-operating characteristics curve of 0.754 (95% confidence interval: 0.65-0.86) compared to 0.617 (95% confidence interval: 0.47-0.76; p-value: 0.012). The clinical model augmented with pre- and post-operative biomarkers demonstrated a significant improvement over the clinical model alone, with a receiver-operating characteristics curve of 0.802 (95% confidence interval: 0.72-0.89; p-value: 0.003).
    CONCLUSIONS: Novel biomarkers add significant predictive value when assessing the likelihood of unplanned readmission or mortality after paediatric congenital heart surgery. Further exploration of the utility of these novel biomarkers during the pre- or post-operative period to identify early risk of mortality or readmission will aid in determining the clinical utility and application of these biomarkers into routine risk assessment.
    Keywords:  Paediatric CHD; biomarkers; prediction; readmission
    DOI:  https://doi.org/10.1017/S1047951119001471
  6. Ann Thorac Surg. 2019 Jul 04. pii: S0003-4975(19)30930-0. [Epub ahead of print]
    Mery CM, De León LE, Trujillo-Diaz D, Ocampo EC, Dickerson HA, Zhu H, Adachi I, Heinle JS, Fraser CD, Ermis PR.
      BACKGROUND: The Fontan procedure is the accepted standard for single-ventricle palliation. The goal of this study was to determine short- and mid-term outcomes of patients undergoing a Fontan operation at a single institution and to identify contemporary risk factors for acute and chronic failure.METHODS: All patients undergoing a Fontan operation between 1995-2016 were included. Failure was defined as death, transplantation, Fontan takedown or revision, fenestration creation or enlargement, plastic bronchitis, protein-losing enteropathy, or major perioperative reintervention. Multivariable logistic and Cox regression models were used to identify risk factors for acute (perioperative) and chronic (after hospital discharge and/or 30 days postoperatively) failure.
    RESULTS: The cohort included 610 patients. Median age at surgery was 4 years. Median follow-up was 6.8 years. Trends showed increasing use of extracardiac conduits, non-fenestrated Fontan, and extubation in the operating room. Perioperative mortality was 0.5% (n=3). Transplant-free survival at 5, 10, and 15 years was 97%, 94%, and 92%, respectively; freedom-from-failure was 91%, 89%, and 87%, respectively. Extubation in the operating room was associated with lower risk of acute failure (OR=0.30,0.11-0.87). Independent risk factors for chronic failure included genetic syndrome (HR=2.54,1.11-5.83), ventricular dysfunction (HR=3.86,1.81-8.24), cardiopulmonary bypass time in 30-minute intervals (HR=1.242,1.100-1.402), and persistent pleural effusions (HR=4.26,2.25-8.07). Moderate or severe atrioventricular valve regurgitation (HR=2.61,1.13-6.02) and cardiopulmonary bypass time (HR=1.22,1.03-1.45) were associated with reduced long-term transplant-free survival.
    CONCLUSIONS: Contemporary mid-term outcomes for Fontan patients are reassuring. Lifelong follow-up is mandatory to determine long-term outcomes and need for additional surgery as patients reach adulthood.
    Keywords:  Fontan failure; Fontan operation; Single ventricle; congenital heart surgery; survival
    DOI:  https://doi.org/10.1016/j.athoracsur.2019.05.039
  7. Ann Thorac Surg. 2019 Jul 06. pii: S0003-4975(19)30994-4. [Epub ahead of print]
    du Plessis K, d'Udekem Y.
      BACKGROUND: Moderate to severe neurodevelopmental impairments are recognized as a significant comorbidity in a quarter to one third of single ventricle patients. This article reviews the nature of neurodevelopment across the lifespan, contributing factors and interventions.METHODS: A focused review was performed of all English articles that address the topic from 1980 to 2018.
    RESULTS: Innate patient factors, rather than intraoperative factors, impact on the development of neurodevelopmental outcomes, yet a large portion of causality factors remain unexplained. There are also limits to how we currently measure neurodevelopmental outcomes. Limited studies in adulthood, and a lack of longitudinal studies hampers our full understanding of how neurodevelopment progresses across time, but estimates are that adults with neurodevelopmental impairments will be affected by abnormal brain aging and early onset dementia. Intervention services and its efficacy have not been researched in depth, and it has not been researched across the lifespan.
    CONCLUSIONS: Further research into neurodevelopmental testing, which accurately predicts future functioning, is needed. The complexity of implementing systematic and effective neurodevelopmental interventions in the busy lives of patients and families, who often have multiple other clinical demands, need a complex and thoughtful solution within the context of a multidisciplinary care team approach. The role of targeted psycho-education interventions, which decrease maternal worry and improve family functioning, should also continue to be explored in patients with a Fontan circulation.
    Keywords:  Fontan; Single ventricle; neurodevelopment
    DOI:  https://doi.org/10.1016/j.athoracsur.2019.05.043
  8. Arch Dis Child. 2019 Jul 11. pii: archdischild-2019-317859. [Epub ahead of print]
    Ewer AK, Deshpande SA, Gale C, Stenson BJ, Upton M, Evans C, Oddie SJ.
      
    Keywords:  congenital heart defect; newborn; pulse oximetry; screening
    DOI:  https://doi.org/10.1136/archdischild-2019-317859
  9. Cardiol Young. 2019 Jul 10. 1-4
    Egbe AC, Crestanello J, Dearani JA, Osman K, Banala K, Angirekula M, Najam M, Ammash NM.
      BACKGROUND: There are limited outcome data in adults with tetralogy of Fallot and pulmonary atresia. The purpose of this study was to describe re-operations and all-cause mortality in adults with tetralogy of Fallot and pulmonary atresia.METHODS: Retrospective review of adults with repaired tetralogy of Fallot and pulmonary atresia who received care at the Mayo Adult Congenital Heart Disease Clinic, 1990-2016. All-cause mortality was calculated as events per 100 patient-years from the time of first presentation to the Adult Congenital Heart Disease Clinic.
    RESULTS: Of the 221 patients, the age at initial tetralogy of Fallot repair was 6 (5-13) years, and the age at first presentation to the clinic was 27 - 8 years. All patients had at least one right ventricular to pulmonary artery conduit re-operation. There were 31 deaths (14%) at mean age of 41 - 14 years. The causes of death were end-stage heart failure (n = 17), sudden cardiac death (n=9), post-operative death after cardiac surgery (n = 2), sepsis with multi-system organ failure (n = 2), and unknown (n = 1). All-cause mortality rate was 1.7 per 100 patient-years. The risk factors for all-cause mortality were older age (&gt;12 years) at the time of repair (hazard ratio 1.41, 95 confidence interval 1.06-2.02, p = 0.033), non-sustained ventricular tachycardia (hazard ratio 1.36, 95 confidence interval 1.17-2.47, p = 0.015), and left ventricular ejection fraction &lt;50% (hazard ratio 1.39, 95 confidence interval 1.08-2.31, p = 0.031).
    CONCLUSION: Based on a review of 221 adults with repaired tetralogy of Fallot and pulmonary atresia, all patients had re-operations and all-cause mortality rate was 1.7 events per 100 patient-years. The current study provides important outcomes data for risk stratification in adults with tetralogy of Fallot and pulmonary atresia.
    Keywords:  Tetralogy of Fallot; mortality; pulmonary atresia; re-operation
    DOI:  https://doi.org/10.1017/S1047951119001598
  10. Eur J Cardiothorac Surg. 2019 Jul 10. pii: ezz203. [Epub ahead of print]
    Raatz A, Schöber M, Zant R, Cesnjevar R, Rüffer A, Purbojo A, Dittrich S, Alkassar M.
      OBJECTIVES: This study evaluated the various risk factors for chylothorax and persistent serous effusions (>7 days) after congenital heart surgery and developed equations to calculate the probability of their occurrence.METHODS: We performed a retrospective review of different medical databases at the University Hospital of Erlangen between January 2014 and December 2016. Full model regression analysis was used to identify risk factors, and prediction algorithms were set up to calculate probabilities. Discriminative power of the models was checked with the help of C-statistics.
    RESULTS: Of 745 operations on 667 patients, 68 chylothoraxes (9.1%) and 125 persistent pleural effusions (16.8%) were diagnosed. Lowest temperature [P = 0.043; odds ratio (OR) 0.899], trisomy 21 (P = 0.001; OR 5.548), a higher vasoactive inotropic score on the day of surgery (P = 0.001; OR 1.070) and use of an assist device (P = 0.001; OR 5.779) were significantly associated with chylothorax. Risk factors for persistent serous effusions were a given or possible involvement of the aortic arch during the operation (P = 0.000; OR 3.982 and 2.905), univentricular hearts (P = 0.019; OR 2.644), a higher number of previous heart operations (P = 0.014; OR 1.436), a higher vasoactive inotropic score 72 h after surgery (P = 0.019; OR 1.091), a higher central venous pressure directly after surgery (P = 0.046; OR 1.076) and an aortic cross-clamp time >86 min (P = 0.023; OR 2.223), as well as use of an assist device (P = 0.002; OR 10.281). The prediction models for both types of effusions proved to have excellent discriminative power.
    CONCLUSIONS: Persistent serous effusion is associated with a higher vasoactive inotropic score 72 h after surgery, an aortic cross-clamp time >86 min and elevated central venous pressure directly after surgery, which, in combination, potentially indicate cardiac stress. The developed logistic algorithm helps to estimate future likelihood.
    Keywords:  Cardiac insufficiency; Cardiac surgery; Chylothorax; Congenital heart disease; Persistent effusions; Risk factor
    DOI:  https://doi.org/10.1093/ejcts/ezz203
  11. Congenit Heart Dis. 2019 Jul 10.
    Delaney AE, Dadlez NM, Marshall AC.
      BACKGROUND: In pediatric cardiac care, many centers participate in multiple, national, domain-specific registries, as a major component of their quality assessment and improvement efforts. Small cardiac programs, whose clinical activities and scale may not be well-suited to this approach, need alternative methods to assess and track quality.METHODS: We conceived of and piloted a rapid-approach cardiac quality assessment, intended to encompass multiple aspects of the service line, in a low-volume program. The assessment incorporated previously identified measures, drawn from multiple sources, and ultimately relied on retrospective chart review.
    RESULTS: A collaborative, multidisciplinary team formed and came to consensus on quality metrics pertaining to 3 chosen areas of clinical activity in the program. Despite the use of multiple different data sources and the need for manual chart review in data collection, a rich assessment of these program components was completed for presentation in 6 weeks.
    CONCLUSIONS: While small programs may not participate in the spectrum of cardiac care registries available, these same centers can benefit from them by adapting some of their validated metrics for use in internal, self-maintained quality reports. Our pilot of this alternative approach revealed opportunities for improved quality assessment practices; the product can serve as a baseline for future prospective assessment and reporting, as well as longitudinal internal benchmarking.
    Keywords:  congenital heart disease; health care quality assessment; outcomes assessment; pediatric hospitals
    DOI:  https://doi.org/10.1111/chd.12821
  12. Acta Paediatr. 2019 Jul 12.
    Cloete E, Gentles TL, Webster DR, Davidkova S, Dixon LA, Alsweiler JM, Bloomfield FH, .
      AIM: To assess local and individual factors that should be consideration in the design of a pulse oximetry screening strategy in New Zealand's midwifery-led maternity setting.METHODS: An intervention study was conducted over 2 years. Three hospitals and 4 primary maternity units participated in the study. Post-ductal saturation levels were measured on well infants with a gestation of ≥35 weeks. Infant activity and age (hours) at the time of the test were recorded.
    RESULTS: Screening was performed on 16,644 of 27,172 (61%) eligible infants. The age at which the screening algorithm was initiated varied significantly among centres. The probability of achieving a pass result (saturations ≥95%) in the context of no underlying pathology ranged from 0.94 for an unsettled infant screened <4 hours of age to 0.99 (p <0.001) when the test was performed after 24 hours on a settled infant. Forty-eight (0.3%) infants failed to reach saturation targets: 37 had significant pathology of which 3 had cardiac disease.
    CONCLUSION: Screening practices were influenced by the setting in which it was undertaken. Infant activity and age at the time of testing can influence saturation levels. Screening is associated with the identification of significant non-cardiac pathology. This article is protected by copyright. All rights reserved.
    Keywords:  congenital heart disease; midwifery-led maternity setting; screening strategy
    DOI:  https://doi.org/10.1111/apa.14934