bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2019‒12‒22
seven papers selected by
Richard James
University of Pennsylvania


  1. Eur J Gastroenterol Hepatol. 2019 Dec 16.
    Keung CY, Zentner D, Gibson RN, Phan DH, Grigg LE, Sood S, Nicoll AJ.
      Cardiac hepatopathy is the liver injury resulting from congestion and ischaemia associated with acute or chronic heart failure. The improved longevity of adults with operated congenital heart disease who develop heart failure as an increasingly late event makes this form of liver injury increasingly clinically relevant. Patients with congenital heart disease with a single ventricle anomaly, who require creation of a Fontan circulation, are particularly vulnerable as they have elevated venous filling pressures with chronic liver congestion. Progression to liver fibrosis and eventually cirrhosis may occur, with its associated risks of liver failure and hepatocellular carcinoma. This risk likely increases over the patient's lifetime, related to the duration post-surgical repair and reflects the chronicity of congestion. Liver biopsy is rarely performed due to a higher risk of complications in the setting of elevated venous pressures, and the frequent use of anticoagulation. Non-invasive methods of liver assessment are poorly validated and different factors require consideration compared to other chronic liver diseases. This review discusses the current understanding of cardiac hepatopathy in congenital heart disease patients with a Fontan circulation. This entity has recently been called Fontan Associated Liver Disease in the literature, with the term useful in recognising that the pathophysiology is incompletely understood, and that long-standing venous pressure elevation and hypoxaemia are presumed to play an additional significant role in the pathogenesis of the liver injury.
    DOI:  https://doi.org/10.1097/MEG.0000000000001641
  2. J Am Heart Assoc. 2020 Jan 07. 9(1): e013807
    Nicholson GT, Glatz AC, Qureshi AM, Petit CJ, Meadows JJ, McCracken C, Kelleman M, Bauser-Heaton H, Gartenberg AJ, Ligon RA, Aggarwal V, Kwakye DB, Goldstein BH.
      Background In infants with ductal-dependent pulmonary blood flow, the impact of palliation strategy on interstage growth and feeding regimen is unknown. Methods and Results This was a retrospective multicenter study of infants with ductal-dependent pulmonary blood flow palliated with patent ductus arteriosus (PDA) stent or Blalock-Taussig shunt (BTS) from 2008 to 2015. Subjects with a defined interstage, the time between initial palliation and subsequent palliation or repair, were included. Primary outcome was change in weight-for-age Z-score. Secondary outcomes included % of patients on: all oral feeds, feeding-related medications, higher calorie feeds, and feeding-related readmission. Propensity score was used to account for baseline differences. Subgroup analysis was performed in 1- (1V) and 2-ventricle (2V) groups. The cohort included 66 PDA stent (43.9% 1V) and 195 BTS (54.4% 1V) subjects. Prematurity was more common in the PDA stent group (P=0.051). After adjustment, change in weight-for-age Z-score did not differ between groups over the entire interstage. However, change in weight-for-age Z-score favored PDA stent during the inpatient interstage (P=0.005) and BTS during the outpatient interstage (P=0.032). At initial hospital discharge, PDA stent treatment was associated with all oral feeds (P<0.001) and absence of feeding-related medications (P=0.002). Subgroup analysis revealed that 2V but not 1V patients demonstrated significant increase in weight-for-age Z-score. In the 2V cohort, feeding-related readmissions were more common in the BTS group (P=0.008). Conclusions In infants with ductal-dependent pulmonary blood flow who underwent palliation with PDA stent or BTS, there was no difference in interstage growth. PDA stent was associated with a simpler feeding regimen and fewer feeding-related readmissions.
    Keywords:  congenital heart disease; outcomes research; surgery
    DOI:  https://doi.org/10.1161/JAHA.119.013807
  3. Curr Pediatr Rev. 2019 Dec 17.
    Mantler T, Jackson KT, Baer J, White J, Ache B, Shillington K, Ncube N.
      BACKGROUND: Children with medical complexity (CMC) and their parents are affected physically and mentally during transitions in care. Coordinated models of care show promise in improving health outcomes.OBJECTIVE: The purpose of this scoping review was to examine research related to CMC and their parents and transitions in care. The aim was 3-fold: (1) to examine the extent, range, and nature of research activity related to the impact of transitions on physical and mental health for CMC and their parents; (2) to summarize and disseminate research findings for key knowledge users; and (3) to identify research gaps in the existing literature to inform future studies.
    METHODS: Twenty-three sources were identified through database searches and five articles met the inclusion criteria of CMC (multi-organ involvement or technology- dependent) (or parents of CMC) transitioning from hospital to alternate levels of care where outcome measures were physical or mental health related.
    RESULTS: Numerical analysis revealed substantial variation in methodological approaches and outcome measures. Content analysis revealed two themes for parents of CMC during this transition: (1) emotional distress, and (2) high expectations; and three themes for CMC: (1) improved health, (2) changes in emotion, and (3) disrupted relationships.
    CONCLUSION: The findings from this scoping review reveal for parents, transitions in care are fraught with emotional distress and high expectations; and for CMC there are improvements in quality of life and emotional health post- hospital to home transitions when collaborative models of care are available. This review serves as an early attempt to summarize the literature and demonstrate a need for further research.
    Keywords:  CMC; Caregivers; Children with medical complexity; Mental Health; Physical Health; Transitions in Care
    DOI:  https://doi.org/10.2174/1573396316666191218102734
  4. J Thorac Cardiovasc Surg. 2019 Oct 31. pii: S0022-5223(19)32375-X. [Epub ahead of print]
    Riggs KW, Zafar F, Lorts A, Chin C, Bryant R, Tweddell JS, Morales DLS.
      OBJECTIVE: Improvements in surgical technique, critical care, and early repair for congenital heart disease (CHD) have led to improved outcomes with heart transplantation, often used as a salvage procedure after failed palliation, especially in infants. These patients, however, often have several risk factors for poor posttransplant survival. We aimed to identify the reality of survival after heart transplantation in patients "limping to transplant" with common risk factors.METHODS: All heart transplant recipients younger than 18 years were identified from the UNOS data set from 2000 to 2017. Modifiable risk factors (MRFs) of mechanical ventilation, renal dysfunction, and liver dysfunction at transplant and nonmodifiable risk factors of infancy at listing or CHD were examined. One-year posttransplant survival was analyzed with logistic regression.
    RESULTS: Of 4101 transplants, 1459 patients (36%) had 1 or more MRFs. There was a decrease in 1-year survival with additional MRFs up to a 9.1-times increased risk of death in an infant with CHD. A noninfant without CHD and no MRFs had a 95% 1-year survival, in contrast to an intubated patient with CHD without other end-organ dysfunction, who had 1-year survival of 76%, which decreased to 58% if they were an infant and also had renal dysfunction.
    CONCLUSIONS: Patients "limping to transplant" with multiple risk factors demonstrates decreasing early survival relative to those without other end-organ dysfunction. It is imperative that we have transparent discussions about expected outcomes with these families and identify ways to optimize patients' conditions through other supportive avenues to improve posttransplant outcomes.
    Keywords:  heart transplantation; mortality; pediatric; risk factors
    DOI:  https://doi.org/10.1016/j.jtcvs.2019.10.077
  5. Front Pediatr. 2019 ;7 494
    Schiller RM, Tibboel D.
      Over the years, it has become clear that children growing up after neonatal critical illness are at high risk of long-term neurocognitive deficits that impact their school performance and daily life activities. Although the pathophysiological mechanisms remain largely unknown, emerging evidence seems to suggest that long-term neuropsychological deficits following neonatal critical illness are not associated with the type of treatment, such as extracorporeal membrane oxygenation (ECMO), but rather with underlying disease processes. In this review, neurocognitive outcome and brain pathology following neonatal critical respiratory and cardiac illness, either treated with or without ECMO, are described and compared in order to gain insight into potential underlying pathophysiological mechanisms. Putting these findings together, it becomes apparent that both children with complex congenital heart disease and children who survived severe respiratory failure are at risk of neurocognitive deficits later in life. Neurorehabilitation strategies, such as Cogmed working-memory training, are discussed. While prevention of neurocognitive deficits altogether should be strived for in the future, this is not realistic at this moment. It is therefore of great importance that children growing up after neonatal critical illness receive long-term care that includes psychoeducation and personalized practical tools that can be used to improve their daily life activities.
    Keywords:  ECMO (extracorporeal membrane oxygenation); Newborn; circulatory failure; hippocampus; respiratory failure
    DOI:  https://doi.org/10.3389/fped.2019.00494
  6. J Am Heart Assoc. 2020 Jan 07. 9(1): e013691
    Kutty S, Jacobs ML, Thompson WR, Danford DA.
      
    Keywords:  Fontan procedure; heart failure; outcome and process assessment; outcomes research
    DOI:  https://doi.org/10.1161/JAHA.119.013691
  7. Per Med. 2020 Jan;17(1): 33-41
    Huang J, Gou B, Rong F, Wang W.
      Aim: Explore if dexmedetomidine (DEX) improves neurodevelopment and cognitive impairment in infants with congenital heart disease. Materials & methods: We retrospectively analyzed 256 pediatric patients aged less than 2 years with heart disease undergoing thoracic surgery. The intelligence quotient and neurodevelopment were tested. Mortality, incidence of postoperation adverse events, duration of mechanical ventilation, and length of stay were recorded and compared. Results: Compared with those not administered DEX, intelligence quotient scores and neurodevelopment evaluation scores increased in patients receiving perioperative DEX. There were no significant differences in mortality, duration of mechanical ventilation or length of stay. Conclusion: The administration of DEX might improve neural development and reduce the adverse effects of general anesthesia in infants with congenital heart disease undergoing surgery and extracorporeal circulation.
    Keywords:  anesthesia complication; cardiopulmonary bypass; children anesthesia; cognitive impairment; congenital heart disease; dexmedetomidine; infants; neurodevelopment
    DOI:  https://doi.org/10.2217/pme-2019-0003