bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2020‒02‒09
six papers selected by
Richard James
University of Pennsylvania


  1. Eur J Cardiothorac Surg. 2020 Feb 08. pii: ezz382. [Epub ahead of print]
    Kelly TJ, Zannino D, Brink J, Konstantinov IE, Cheung MM, d'Udekem Y, Brizard CP.
      OBJECTIVES: The aim of this study was to study the impact of a decision-making protocol for shunt type in the Norwood procedure for hypoplastic left heart syndrome. Our cohort extends from 2004 to 2016. In era 1 (pre-2008), there was no policy for the choice of Norwood shunt. In era 2 (post-2008), a standard protocol was implemented. The right ventricle (RV)-to-pulmonary artery conduit was utilized for low-birth weight patients (<2.5 kg). The right modified Blalock-Taussig Shunt (RBTS) was constructed for normal birth weight patients.METHODS: The records of 133 consecutive operative patients with hypoplastic left heart syndrome anatomy between 2004 and 2016 were retrospectively reviewed. Survival risk factors were analysed using the Cox proportional hazards risk model.
    RESULTS: The Norwood procedure was performed at a mean age of 2.9 ± 1.9 days. Bidirectional cavopulmonary shunt was performed at a median age of 99 days (interquartile range 91-107). In era 1, 38.6% (22/57) of patients received the RBTS and 61.4% (35/57) of patients received the RV-to-pulmonary artery conduit. In era 2, 86.8% (66/76) of patients received the RBTS and 13.2% (10/76) of patients received the RV-to-pulmonary artery conduit. The actuarial survival to Fontan was 72.2% (96/133). Era 1 patients were more likely to die within the 1st year (hazard ratio = 2.310, P = 0.025).
    CONCLUSIONS: The shunt protocol may improve outcomes in high-risk patients, and we have demonstrated the reliability of the RBTS in low-risk patients. The short- and mid-term outcomes of our Norwood population justify the continued efforts to improve surgical and perioperative management.
    Keywords:  Blalock-Taussig shunt; Hypoplastic left heart syndrome; Long-term survival; Norwood operation; Right ventricle-to-pulmonary artery conduit; Survival risk factors
    DOI:  https://doi.org/10.1093/ejcts/ezz382
  2. Cardiol Young. 2020 Feb 03. 1-6
    Chaves AH, Baker-Smith CM, Rosenthal GL.
      INTRODUCTION: Infants undergoing stage 1 palliation for hypoplastic left heart syndrome may have post-operative feeding difficulties. Although the cause of feeding difficulties in these patients is multi-factorial, residual arch obstruction may affect gut perfusion, contributing to feeding intolerance. We hypothesised that undergoing arch reintervention following stage 1 palliation would be associated with post-operative feeding difficulties.METHODS: This was a retrospective cohort study. We analysed data from the National Pediatric Cardiology Quality Improvement Collaborative, which maintains a multicentre registry for infants with hypoplastic left heart syndrome discharged home following stage 1 palliation. Patients who underwent arch reintervention (percutaneous or surgical) prior to discharge following stage 1 palliation were compared with those who underwent non-aortic arch interventions after stage 1 palliation and those who underwent no intervention. Median post-operative days to full enteral feeds and weight for age z-scores were compared. Predictors of post-operative days to full feeds were identified.
    RESULTS: Among patients who underwent arch reintervention, post-operative days to full enteral feeds were greater than for those who underwent non-aortic arch interventions (25 versus 16, p = 0.003) or no intervention (median days 25 versus 12, p < 0.001). Arch intervention, multiple interventions, gestational age, and the presence of a gastrointestinal anomaly were predictors of days to full feeds.
    CONCLUSIONS: Repeat arch intervention is associated with a longer time to achieve full enteral feeding in patients with hypoplastic left heart syndrome after stage 1 palliation. Further investigation of this association is needed to understand the role of arch obstruction in feeding problems in these patients.
    Keywords:  Hypoplastic left heart syndrome; Norwood; coarctation; feeding problems; stage 1 palliation
    DOI:  https://doi.org/10.1017/S1047951120000177
  3. Pediatr Cardiol. 2020 Feb 01.
    Hamzah M, Othman HF, Elsamny E, Agarwal H, Aly H.
      The objective of this study was to identify patient and hospitalization characteristics associated with in-hospital mortality in infants with hypoplastic left heart syndrome (HLHS). We conducted a retrospective analysis of a large administrative database, the National Inpatient Sample dataset of the Healthcare Cost and Utilization Project for the years 2002-2016. Neonates with HLHS were identified by ICD-9 and ICD-10 codes. Hospital and patient factors associated with inpatient mortality were analyzed. Overall, 18,867 neonates met the criteria of inclusion; a total of 3813 patients died during the hospitalization (20.2%). In-hospital mortality decreased over the years of the study (27.0% in 2002 vs. 18.3% in 2016). Extracorporeal membrane oxygenation utilization was 8.1%. Univariate and multivariate logistic regression analyses were used to identify risk factors for in-hospital mortality in infants with hypoplastic left heart syndrome. Independent non-modifiable risk factors for mortality were birth weight < 2500 g (Adjusted odds ratio (aOR) 2.16 [1.74-2.69]), gestational age < 37 weeks (aOR 1.73 [1.42-2.10]), chromosomal abnormalities (aOR 3.07 [2.60-3.64]) and renal anomalies (aOR 1.34 [1.10-1.61]). Independent modifiable risk factors for mortality were being transferred-in from another hospital (aOR 1.15 [1.03-1.29]), use of extracorporeal membrane oxygenation (aOR 12.74 [10.91-14.88]). Receiving care in a teaching hospital is a modifiable variable, and it decreased the odds of mortality (aOR 0. 78 [0.64-0.95]). In conclusion, chromosomal anomalies, Extra Corporeal Membrane Oxygenation, gestational age < 37 weeks or birth weight < 2500 g were associated with increased odds of mortality. Modifiable variables as receiving care at birth center and in a hospital designated as a teaching hospital decreased the odds of mortality.
    Keywords:  Chromosomal abnormality; ECMO; Hypoplastic left heart syndrome; Mortality
    DOI:  https://doi.org/10.1007/s00246-020-02312-3
  4. JAMA Cardiol. 2020 Feb 05.
    Budts W, Ravekes WJ, Danford DA, Kutty S.
      The Fontan circulation, accomplished by direct surgical connection of the vena cavae to the pulmonary arteries, can be an effective palliation for patients with a single ventricle. However, failure of the Fontan circulation can result from mechanical obstruction, cardiac arrhythmias, increasing pulmonary vascular resistance, or deteriorating ventricular performance. Although systolic ventricular failure can usually be identified by a combination of clinical signs, symptoms, and imaging findings, diastolic ventricular dysfunction is likely an underrecognized cause of Fontan failure. Methods for detection of impaired diastolic function in a single ventricle are evolving, and established techniques appropriate in the biventricular heart lack validation in single ventricle. Association of biomarkers, cardiac magnetic resonance, and echocardiographic findings with invasively acquired pressure-volume loop data in humans may offer a way forward to accurate, noninvasive diagnosis. Today, therapy for severe diastolic ventricular dysfunction in the Fontan circulation is often disappointing and may require consideration of a ventricular assist device or even cardiac transplant. Progress toward improved outcomes of the Fontan palliation likely depends on successful innovation in primary prevention strategies and the development of more effective pharmacotherapy.
    DOI:  https://doi.org/10.1001/jamacardio.2019.5459
  5. Child Care Health Dev. 2020 Feb 03.
    Blais AZ, Lougheed J, Yaraskavitch J, Adamo KB, Longmuir P.
      BACKGROUND: Participation in physical activity is essential to the long-term health and development of all children. However, children living with cardiac conditions are typically not active enough to sustain positive health outcomes. Understanding the experiences of children living with congenital heart disease in community-based settings could help inform the physical activity counselling practices of clinicians. The current study explored the perceptions of 7- to 10-year-old children with moderate or complex congenital heart disease as they participated in a 10-week, multi-sport programme.METHODS: Detailed field notes recorded the discussions and behaviours of 11 participants (45% female) each week during the programme sessions. Among those, 4 participants (50% female) were purposively selected to participate in pre- and post-programme focus groups to gather more detailed accounts of their experiences.
    RESULTS: Four main themes surrounding physical activity were identified: 1) motivation, 2) self-efficacy, 3) peer influences and 4) family influences. Although feelings of excitement and enjoyment towards physical activity were prevalent throughout the data ("I'm really excited … because I really like those sports"), participants also often felt frustrated, nervous, and fatigued ("I'm not very good at the skills"). Social inclusion with peers and family influences were meaningful reasons to engage in physical activity ("I really like playing games together"). Following the completion of the programme, participants emphasized their enjoyment of physical activity as a primary source of motivation, demonstrating an important shift from recognizing positive health outcomes (" … it's good for you") towards more intrinsic sources of motivation ("… because it's fun").
    CONCLUSION: Opportunities for physical activity which enhance positive experiences and build intrinsic motivation should be identified and promoted to children with congenital heart disease. Community-based programmes may also be an appropriate context for children with cardiac conditions to engage and maintain participation in physical activity through adolescence.
    Keywords:  chronic disease; community-based intervention; perceived physical competence; physical activity counselling; social inclusion; sport
    DOI:  https://doi.org/10.1111/cch.12754
  6. Eur J Obstet Gynecol Reprod Biol. 2020 Jan 29. pii: S0301-2115(20)30047-6. [Epub ahead of print]246 145-150
    Chilikov A, Wainstock T, Sheiner E, Pariente G.
      OBJECTIVE: Due to advances in medical care available for patients with congenital heart disease, these patients now have a longer life span. As a result, the number of pregnancies of mothers with congenital heart disease has increased. The purpose of the current study was to evaluate perinatal outcomes of women with congenital heart disease and to examine long-term cardiovascular morbidity of offspring to mothers with congenital heart disease.MATERIALS AND METHODS: A population-based cohort analysis was performed including all singleton deliveries occurring between years 1991-2014 at Soroka University Medical Center. The primary exposure was defined as maternal congenital heart disease. Short-term perinatal outcomes as well as long-term cardiovascular related hospitalizations of the offspring up to the age of 18 years were compared between women with and without congenital heart disease. To compare perinatal outcomes between the two study groups, we conducted a Generalized Estimation Equation (GEE) model accounting for maternal age and multiple occurrence in the cohort, parity, fertility treatment, smoking and induction of labor. To compare cumulative long-term cardiovascular morbidity, we compared cardiovascular hospitalization incidence of offspring of mothers with and without congenital heart disease using Kaplan-Meier survival curve and Weibull regression analysis to control for maternal age, diabetes, smoking, hypertension, birth weight, and cesarean delivery.
    RESULTS: During the study period 243,313 deliveries met the inclusion criteria, of which 369 were of mothers with congenital heart disease. Maternal congenital heart disease was found to be an independent risk factor for low birth weight (adjusted OR = 1.5 CI 95 % 1.06-2.32, p=0.022) and cesarean delivery (adjusted OR = 1.4 CI 95 % 1.09-1.86, p = 0.008). Mothers with congenital heart disease also had higher risk for placental abruption, but it was not statistically significant (adjusted OR = 2.3 CI 95 % 0.87-6.16, p = 0.09). Long-term cardiovascular morbidity was comparable between the groups (Log rank p = 0.251; adjusted HR = 1.6 CI 95 % 0.62-4.42, p = 0.313).
    CONCLUSION: In conclusion, pregnancies of women with congenital heart disease are at an increased risk for adverse short-term perinatal outcomes, such as low birth weight and cesarean delivery. However, we did not find an increase in long-term cardiovascular morbidity among offspring of these women; still this association cannot be ruled out.
    Keywords:  Long-term cardiovascular morbidity; Maternal congenital heart disease; Offspring; Pediatric
    DOI:  https://doi.org/10.1016/j.ejogrb.2020.01.038