bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2019‒11‒17
seven papers selected by
Richard James
University of Pennsylvania


  1. Curr Probl Pediatr Adolesc Health Care. 2019 Nov 07. pii: S1538-5442(19)30126-9. [Epub ahead of print] 100685
    Howell HB, Zaccario M, Kazmi SH, Desai P, Sklamberg FE, Mally P.
      Congenital heart defects are the most common birth anomaly affecting approximately 1% of births. With improved survival in this population, there is enhanced ability to assess long-term morbidities including neurodevelopment. There is a wide range of congenital heart defects, from those with minimal physiologic consequence that do not require medical or surgical intervention, to complex structural anomalies requiring highly specialized medical management and intricate surgical repair or palliation. The impact of congenital heart disease on neurodevelopment is multifactorial. Susceptibility for adverse neurodevelopment increases with advancing severity of the defect with initial risk factors originating during gestation. Complex structural heart anomalies may pre-dispose the fetus to abnormal circulatory patterns in utero that ultimately impact delivery of oxygen rich blood to the fetal brain. Thus, the brain of a neonate born with complex congenital heart disease may be particularly vulnerable from the outset. That vulnerability is compounded during the newborn period and through childhood, as this population endures a myriad of medical and surgical interventions. For each individual patient, these factors are likely cumulative and synergistic with progression from fetal life through childhood. This review discusses the spectrum of risk factors that may impact neurodevelopment in children with congenital heart disease, describes current recommendations and practices for neurodevelopmental follow-up of children with congenital heart disease and reviews important neurodevelopmental trends in this high risk population.
    DOI:  https://doi.org/10.1016/j.cppeds.2019.100685
  2. J Card Surg. 2019 Nov 09.
    Brown MA, Rajamarthandan S, Francis B, O'Leary-Kelly MK, Sinha P.
      BACKGROUND: Congenital heart disease (CHD) continues to be among the most common birth defects, affecting an estimated 40 000 births annually in the United States. The most common complication of CHD is heart failure. With improved medical management and surgical outcomes, survival for complex congenital heart defects has dramatically improved, but consequentially there are more adults with CHD than children with CHD. Due to longer-term sequelae of CHD, surgical and medical treatment previously thought to be curative is now realized at best to be palliative, and there is a considerable burden of CHD-related heart failure. Stem cell therapy as an adjunct to current surgical and medical strategies is being explored in an effort to ameliorate CHD-related heart failure. This review aims to explore the current literature with regard to stem cell therapy for CHD as well as ongoing trials.METHODS: A MEDLINE (Ovid), MEDLINE (Pubmed), and clinicaltrials.gov search were performed using the medical subject headings congenital heart defects combined with hematopoietic stem cells, stem cell transplantation, mesenchymal stem cells (MSC), cell- or tissue-based therapy, or MSC transplantation. Articles must have been published after 2010.
    RESULTS: Twenty three articles and 9 ongoing trials met all inclusion criteria.
    CONCLUSIONS: Areas of interest include myocardiocyte regeneration, tissue graft development to minimize reoperations, and methods of stem cell delivery. While several small trials are showing promise, it is too soon to make definitive statements about the future of stem cell therapies in this field.
    Keywords:  Norwood procedure; congenital heart disease; hypoplastic left heart syndrome; pediatric cardiac surgery; stem cell therapy
    DOI:  https://doi.org/10.1111/jocs.14312
  3. Eur J Cardiovasc Nurs. 2019 Nov 13. 1474515119885858
    Leslie CE, Schofield K, Vannatta K, Jackson JL.
      BACKGROUND: Congenital heart disease places survivors at increased risk for cardiovascular complications as they age and requires long-term medical management. Perceived health competence, or how capable one feels in managing one's health, is linked to emotional adjustment in various disease populations, but has not been investigated among congenital heart disease survivors.AIM: The purpose of this study was to examine the relationship of perceived health competence at baseline (T1) as a predictor of anxiety and depressive symptoms three years later (T2).
    METHODS: Congenital heart disease survivors (n=125; MT1age=27; 58.6% female; severity of cardiac lesion: 25% simple, 44% moderate, 31% complex) were recruited from a pediatric and an adult hospital as part of a larger study. Participants completed the Perceived Health Competence Scale and the Youth or Adult Self-Report at T1 for anxiety and affective/depressive symptoms. At T2, participants completed the Hospital Anxiety and Depression Scale. New York Heart Association functional class, a measure of functional impairment, was abstracted from medical charts at T2.
    RESULTS: Lower T1 perceived health competence was significantly associated with greater emotional distress at T1 (depression r=-0.47; anxiety: r=-0.45), as well as greater T2 functional impairment (r=-0.41). T1 perceived health competence also predicted T2 anxiety and depressive symptoms, which remained significant when including T1 anxiety and depressive symptoms and T2 functional impairment.
    CONCLUSION: Congenital heart disease survivors who feel more competent in managing their health may be less likely to experience future anxiety and depressive symptoms. Perceived health competence may be a worthwhile target for psychosocial intervention to promote emotional wellbeing among congenital heart disease survivors and ensure the best outcomes.
    Keywords:  Congenital heart disease; New York Heart Association; anxiety; depression; perceived health competence
    DOI:  https://doi.org/10.1177/1474515119885858
  4. Transl Pediatr. 2019 Oct;8(4): 290-301
    McCormick AD, Schumacher KR.
      Half a century has passed since the original Fontan palliation. In the interim, surgical and medical advancements have allowed more single ventricle patients to reach Fontan and long-term survival for Fontan patients to improve significantly. However, the risk for Fontan failure and need for heart transplantation remains. In this article we discuss mechanisms of Fontan failure, risk factors and special considerations for Fontan patients in both medical and surgical management around heart transplantation and provide an updated review of survival for Fontan patients after heart transplantation.
    Keywords:  Heart defects; congenital; heart transplantation; risk factors
    DOI:  https://doi.org/10.21037/tp.2019.06.03
  5. Pediatr Cardiol. 2019 Nov 11.
    Asagai S, Inai K, Shimada E, Harada G, Sugiyama H.
      Various late complications are associated with the Fontan procedure. In patients who undergo the Fontan procedure, the central venous pressure (CVP) tends to be higher than normal. However, the relationship between CVP (at rest and during exercise) and late complications associated with the Fontan procedure is unknown. Thirty-four patients who underwent the Fontan procedure were enrolled in this study. The median age was 19.3 years, and the median time after the Fontan procedure was 12.7 years. With exercise, the CVP increased from a median of 11 to 19 mmHg, and the cardiac index increased from a median of 2.1 to 4.4 l/min/m2. In 38% of the patients, CVP measured at the resting condition and during exercise differed. Laboratory results indicated no significant difference between the patients in the high-CVP group and low-CVP group at rest. In contrast, during exercise, brain natriuretic peptide (67 ± 38 vs. 147 ± 122 pg/ml, p < 0.05), gamma-glutamyl transpeptidase (63 ± 33 vs. 114 ± 30 IU/l, p < 0.05), type IV collagen 7S (6.7 ± 1.3 vs. 8.1 ± 1.3 ng/ml, p < 0.05), and creatinine (0.72 ± 3.14 vs. 0.83 ± 3.16 mg/dl, p < 0.05) levels were significantly higher in the high-CVP group than in the low-CVP group. Elevated CVP during exercise may be associated with long-term complications after the Fontan procedure; hence, CVP should be measured during exercise in patients who underwent the Fontan procedure to accurately predict the risk of developing such complications.
    Keywords:  Central venous pressure; Exercise; Fontan procedure; Long-term complication
    DOI:  https://doi.org/10.1007/s00246-019-02249-2
  6. Clin Liver Dis (Hoboken). 2019 Oct;14(4): 138-141
    Hilscher MB, Kamath PS.
      
    DOI:  https://doi.org/10.1002/cld.828
  7. J Am Heart Assoc. 2019 Nov 19. 8(22): e014088
    Wong K, Tecson K, Cedars A.
      Background The prevalence of adult congenital heart disease (ACHD) is increasing in the United States because of improved survival into adulthood. The unique physiology of ACHD commonly leads to multiorgan dysfunction, prompting interest in outcomes after multiorgan (heart+X) transplantation. Methods and Results We queried the SRTR (Scientific Registry of Transplant Recipients) database to examine 5-year outcomes in ACHD patients (aged ≥18 years) who underwent dual organ (heart+kidney/liver/lung) transplantation between 2000 and 2016. Cox proportional hazards models were constructed to look at survival of dual organ transplant recipients versus heart-only recipients in the ACHD population and heart+lung recipients versus heart-only recipients in the ACHD populations and versus non-ACHD recipients of heart+lung transplant. We then constructed a multivariable model to investigate independent risk factors for 5-year mortality after multiorgan transplant. Overall, 5-year mortality was greater for multiorgan (heart+kidney/liver/lung) transplant compared with heart-only transplant. On further analysis, only heart+lung transplant was associated with increased mortality. Outcomes after heart+lung transplant were no different between the ACHD and non-ACHD population. Risk factors for increased risk of 5-year mortality in ACHD patients after multiorgan transplant included heart+lung transplant, previous cardiac surgery, and severe functional limitation. Conclusions The mortality risk associated with multiorgan heart transplant in ACHD patients is attributable primarily to heart+lung transplants. Multiorgan transplant in ACHD does not convey increased risk compared with the non-ACHD population. Need for multiorgan transplant should not be an impediment to listing ACHD patients needing a heart transplant.
    Keywords:  adult congenital heart disease; heart failure; mortality; multiorgan transplant; transplantation
    DOI:  https://doi.org/10.1161/JAHA.119.014088