bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2020‒03‒22
eleven papers selected by
Richard James
University of Pennsylvania


  1. J Am Heart Assoc. 2020 Mar 17. 9(6): e014172
    Marshall KH, D'Udekem Y, Sholler GF, Opotowsky AR, Costa DSJ, Sharpe L, Celermajer DS, Winlaw DS, Newburger JW, Kasparian NA.
      Background People with a Fontan circulation experience a range of physical, psychosocial and neurodevelopmental challenges alongside, or caused by, their cardiac condition, with significant consequences for health-related quality of life (HRQOL). We meta-analyzed HRQOL outcomes reported by people with a Fontan circulation or their proxies and evaluated predictors of poorer HRQOL. Methods and Results Six electronic databases were searched for peer-reviewed, English-language articles published before March 2019. Standardized mean differences (SMD) were calculated using fixed and random-effects models. Fifty articles reporting on 29 unique studies capturing HRQOL outcomes for 2793 people with a Fontan circulation and 1437 parent-proxies were analyzed. HRQOL was lower in individuals with a Fontan circulation compared with healthy referents or normative samples (SMD, -0.92; 95% CI, -1.36 to -0.48; P<0.001). Lower scores were reported across all HRQOL domains, with the largest differences found for physical (SMD, -0.90; 95% CI, -1.13 to -0.67; P<0.001) and school/work functioning (SMD, -0.71; 95% CI, -0.90 to -0.52; P<0.001). Meta-regression analyses found no significant predictors of self-reported physical functioning, but older age at Fontan operation was associated with poorer emotional functioning (β=-0.124; P=0.004), and diagnosis of hypoplastic left heart was associated with poorer social functioning (β=-0.007; P=0.048). Sensitivity analyses showed use of the PedsQL Core Module was associated with lower HRQOL scores compared with the Short-Form Health Survey-36. Conclusions HRQOL outcomes for people with a Fontan circulation are lower than the general population. Optimal care acknowledges the lifelong impact of the Fontan circulation on HRQOL and offers targeted strategies to improve outcomes for this growing population.
    Keywords:  Fontan circulation; chronic illness; congenital heart disease; health‐related quality of life; mental health; psychological stress
    DOI:  https://doi.org/10.1161/JAHA.119.014172
  2. J Clin Med. 2020 Mar 13. pii: E779. [Epub ahead of print]9(3):
    Andonian C, Beckmann J, Ewert P, Freilinger S, Kaemmerer H, Oberhoffer-Fritz R, Sack M, Neidenbach R.
      BACKGROUND: Due to advances in the diagnosis and treatment of congenital heart disease (CHD), the number of adults who are surviving with congenital heart disease (ACHD) is constantly growing. Until recently, the psychological effects of CHD had been widely neglected. Current research provides evidence for an increased risk of emotional distress in ACHD. The concept of illness identity attempts to explain how patients experience and integrate their CHD into their identities. The present study investigated illness identity in relation to clinical parameters and psychological functioning. Psychometric properties of the German version of the Illness Identity Questionnaire (IIQD) were examined.METHODS: Self-reported measures on illness identity and psychological functioning (HADS-D) were assessed in a representative sample of 229 ACHD (38 ± 12.5 (18-73) years; 45% female) at the German Heart Center Munich. Descriptive analyses and multiple regression models were conducted. Confirmatory factor analysis was performed to validate the IIQD.
    RESULTS: The IIQD demonstrated good reliability. The originally-postulated four-factor structure could not be replicated. Anatomic disease complexity and functional status significantly influenced illness identity. Illness identity accounted for unique variances in depression and anxiety: Maladaptive illness identity states (i.e., , engulfment and rejection) were associated with higher emotional distress, whereas adaptive illness (i.e., , acceptance and enrichment) identity states were linked to lower emotional distress.
    CONCLUSIONS: Illness Identity emerged as a predictor of emotional distress in ACHD. Findings raise the possibility that interventions designed to target a patient's illness identity may improve psychological well-being and cardiac outcomes in ACHD.
    Keywords:  adults with congenital heart disease; anxiety; depression; illness identity; prevention; psychological situation
    DOI:  https://doi.org/10.3390/jcm9030779
  3. J Am Heart Assoc. 2020 Apr 07. 9(7): e015871
    Reddy S, Handler SS, Wu S, Rabinovitch M, Wright G.
      Abstracts Because of remarkable advances in survival over the past 40 years, the worldwide population of individuals with single ventricle heart disease living with Fontan circulation has grown to ≈70 000, with nearly half aged >18 years. Survival to at least 30 years of age is now achievable for 75% of Fontan patients. On the other hand, single ventricle patients account for the largest group of the 6000 to 8000 children hospitalized with circulation failure, with or without heart failure annually in the United States, with the highest in-hospital mortality. Because there is little understanding of the underlying mechanisms of heart failure, arrhythmias, pulmonary and lymphatic vascular abnormalities, and other morbidities, there are no specific treatments to maintain long-term myocardial performance or to optimize overall patient outcomes.
    Keywords:  atrial tachycardia; congenital cardiac defect; pulmonary vascular changes; single ventricle; stroke in children
    DOI:  https://doi.org/10.1161/JAHA.119.015871
  4. Front Pediatr. 2020 ;8 26
    Kovacevic A, Simmelbauer A, Starystach S, Elsässer M, Müller A, Bär S, Gorenflo M.
      Objectives: Empirical assessment of parental needs and affecting factors for counseling success after prenatal diagnosis of congenital heart disease (CHD). Methods:Counseling success after fetal diagnosis of CHD was assessed by a validated standardized questionnaire. The dependent variable "Effective Counseling" was measured in five created analytical dimensions (1. "Transfer of Medical Knowledge-ToMK"; 2. "Trust in Medical Staff-TiMS"; 3. "Transparency Regarding the Treatment Process-TrtTP"; 4. "Coping Resources-CR"; 5. "Perceived Situational Control-PSC"). Analyses were conducted with regard to influencing factors and correlations. Results: Sixty-one individuals (n = 40 females, n = 21 males) were interviewed in a tertiary medical care center. Median gestational age at first parental counseling was 28 + 6 weeks. Parental counseling was performed four times (median), mostly by pediatric cardiologists (83.6%). Overall counseling was successful in 46.3%, satisfying in 51.9%, and unsuccessful in 1.9%. Analyses of the analytical dimensions show that counseling was less successful for TOMK (38.3%) and PSC (39%); success rates were higher if additional written information or links to web sources were provided (60 and 70%, respectively). Length of consultation was positively correlated to counseling success for ToMK (r = 0.458), TrtTP (r = 0.636), PSC (r = 0.341), and TiMS (r = 0.501). Interruptions were negatively correlated to the dimensions TiMS (r = -0.263), and TrtTP (r = -0.210). In the presence of high-risk CHD (37.5%) overall counseling success was lower (26.1%). By cross table analysis and to a low degree of positive correlation in one dimension (ToMK; r = 0.202), counseling tends to be less successful for ToMK, TrtTP, and TiMS if parents have not been counseled by cardiologists. Analyses regarding premises show a parental need for a separate counseling room, which significantly impacts ToMK (r = -0,390) and overall counseling success (r = -0.333). A language barrier was associated with lower success rates for ToMK, TiMS, and CR (21.4, 42.9, and 30.8%). Conclusions: Data from this multidisciplinary study indicate that parents after fetal diagnosis of CHD need uninterrupted counseling of adequate duration and quality in a separate counseling room. Providing additional written information or links to adequate web sources after initial counseling seems necessary. High-risk CHD needs more attention for counseling. There is a trend towards more counseling success if provided by cardiologists.
    Keywords:  Congenital heart disease (CHD); counseling; empirical assessment; fetal cardiology; parental needs
    DOI:  https://doi.org/10.3389/fped.2020.00026
  5. Pediatr Cardiol. 2020 Mar 20.
    Galantowicz M.
      Many forces are once again bringing the congenital heart surgeon and interventional cardiologist, and the teams that support them, ever closer together in what has been deemed Hybrid Approaches to congenital heart disease. The goal of these hybrid approaches is to improve the quantity and quality of life for the patients we serve.
    Keywords:  Congenital heart surgery; Hybrid procedures
    DOI:  https://doi.org/10.1007/s00246-020-02299-x
  6. J Pediatr Nurs. 2020 Mar 13. pii: S0882-5963(19)30547-0. [Epub ahead of print]
    Hwang JH, Chae SM.
      PURPOSE: This qualitative descriptive study sought to explore the experiences of fathers of children with severe congenital heart defects (CHDs) in Korea.METHODS: The participants were nine fathers of children under 5 years of age who had undergone open heart surgery within the last 5 years. Data were collected using in-depth individual interviews from February to March 2015. Qualitative data were analyzed using the thematic analysis method.
    RESULTS: Three themes and nine sub-themes emerged from the data. The three themes were "heartbreaking suffering," "self-control during a great struggle," and "being a father of a child with CHD." This study described coping strategies that fathers used to balance their life, including self-control, redefinition of the situation, and seeking family support resources, which enabled them to practice normalization and live an ordinary life despite limitations.
    CONCLUSION: The findings of this study indicate that considerable psychological distress of fathers was similar to that of mothers for weeks to months after the sudden diagnosis of CHD and heart operations. The results also depict the coping strategies, contemporary fathering experience and the parenting role of fathers in raising children with severe CHDs.
    PRACTICE IMPLICATIONS: Healthcare professionals should understand the experience of fathers of children with severe CHDs and their needs for social support. It is also necessary to develop nursing interventions that focus on fathers' needs.
    Keywords:  Congenital heart defects; Coping behavior; Father-child relations; Psychological stress; Self-control
    DOI:  https://doi.org/10.1016/j.pedn.2020.02.040
  7. J Pediatr Health Care. 2020 Mar 11. pii: S0891-5245(19)30660-1. [Epub ahead of print]
    Zheng K, Abraham C, Bruzzese JM, Smaldone A.
      INTRODUCTION: Depression is prevalent among adolescents with chronic illness. However, little is known about how depression affects chronic illness over time. This review aimed to synthesize longitudinal relationships between depression and disease control, self-management behaviors, illness-related morbidity, and quality of life.METHOD: Four databases were searched, including PubMed, Cumulative Index of Nursing and Allied Health Literature, PsycINFO, and EMBASE. Inclusion criteria were cohort studies examining depression among adolescents aged 10-21 years with a chronic illness and studies published in English. Study quality was appraised using the Newcastle-Ottawa scale and data was synthesized by the outcome.
    RESULTS: Of the 3,463 articles identified, 11 were included in the review. For adolescents with diabetes, increased depressive symptoms predicted decreased metabolic control and monitoring, medication adherence, quality of life, and increased hospitalization. Studies on cystic fibrosis, congenital heart disease, sickle cell disease, and juvenile idiopathic arthritis were limited but demonstrated that depressive symptoms affected the quality of life, disability, pain, and hospitalization rates/costs.
    DISCUSSION: Evidence supports the need for mental health care strategies suitable for adolescents with chronic illness. Future research is needed to examine the effects of depressive symptoms across diversified chronic illness populations.
    Keywords:  Adolescent health; chronic disease; comorbidity; depression; mental health
    DOI:  https://doi.org/10.1016/j.pedhc.2020.01.008
  8. Pediatr Crit Care Med. 2020 Mar 13.
    Chao BK, Claessens NHP, Lim JM, Gorodetsky C, Au-Young SH, Guerguerian AM, Marini D, Blaser S, Shroff M, Saini AK, Seed M, Chau V, Miller SP.
      OBJECTIVES: The aims of this study were to: i) determine the spectrum of brain injury and ii) compare brain volumes between pre- and postoperative brain MRI in the infants receiving extracorporeal membrane oxygenation compared with those who did not require extracorporeal membrane oxygenation.DESIGN: Cohort study of infants with D-transposition of the great arteries or single ventricle physiology. Brain volume (cm) was measured using a segmentation of a volumetric T1-weighted gradient echo sequence. Brain imaging findings (intraventricular hemorrhage, white matter injuries, and stroke) were analyzed with respect to known clinical risk factors for brain injury and adverse neurodevelopmental outcomes. Clinical factors were collected by retrospective chart review. The association between brain volume and extracorporeal membrane oxygenation was evaluated using generalized estimating equations to account for repeated measures.
    SETTING: Prospective and single-centered study.
    PATIENTS: One hundred nine infants (median gestational age, 39.1 wk) with D-transposition of the great arteries (n = 77) or single ventricle physiology (n = 32) were studied pre- and postoperatively with MRI as per clinical protocol.
    INTERVENTIONS: None.
    MEASUREMENTS AND MAIN RESULTS: Of the 28 infants (26%) receiving extracorporeal membrane oxygenation, 19 (68%) were supported with extracorporeal membrane oxygenation once, and nine (32%) were supported 2-4 times. On postoperative MRI, new white matter injury was found in only five (17%) of the extracorporeal membrane oxygenation infants versus 40 (49%) in the non-extracorporeal membrane oxygenation group (p = 0.073). The rate of stroke (9% vs 10%), intraventricular hemorrhage (24% vs 29%), and hypoxic ischemia (3% vs 14%) did not differ between the non-extracorporeal membrane oxygenation and extracorporeal membrane oxygenation groups (all p > 0.5). Accounting for D-transposition of the great arteries or single ventricle physiology diagnosis, infants requiring extracorporeal membrane oxygenation had slower brain volume with single (β = -1.67) or multiple extracorporeal membrane oxygenation runs ([β = -6.54]; overall interaction p = 0.012).
    CONCLUSIONS: Patients with d-transposition of the great arteries or single ventricle physiology undergoing extracorporeal membrane oxygenation at our center have a similar incidence of brain injury but more significant impairment of perioperative brain volumes than those not requiring extracorporeal membrane oxygenation.
    DOI:  https://doi.org/10.1097/PCC.0000000000002336
  9. Pediatr Cardiol. 2020 Mar 20.
    Holzer RJ, Dayton JD.
      Small study sizes are a limiting factor in assessing outcome measures in pediatric cardiology. It is even more difficult to assess the outcomes of congenital catheterizations where the sample sizes are even smaller, particularly on a individual institutional level. The creation of multicenter registries is a method by which investigators can pool data to better assess quality and outcome measures of these procedures. No registry is perfect with several being available today, each with its own strengths and weaknesses. In addition, there are a multitude of methods currently used to assess quality and outcomes from the data contained in these registries, each having its own limitations as well. Nonetheless, multicenter registrities remain one of the best available options to improve the quality of care for pediatric interventional cardiac catheterization. Below, we provide an overview of the current state of quality assessment/improvement in pediatric interventional cardiology including a review of the available registrities and the metrics used to measure quality of care and outcomes.
    Keywords:  Catheterization; Outcomes; Pediatric interventional cardiology; Quality improvement
    DOI:  https://doi.org/10.1007/s00246-020-02300-7
  10. Pediatr Cardiol. 2020 Mar 20.
    Ibrahim N, Gillette N, Patel H, Peiris V.
      Pediatric medical device approvals lag behind adult approvals. Historically, medical devices have rarely been designed specifically for children, but use in children has most often borrowed from adult or general use applications. While a variety of social, economic, and clinical factors have contributed to this phenomenon, the regulatory process remains a fundamental aspect of pediatric device development and commercialization. FDA's Center for Devices and Radiological Health (CDRH) has established programmatic and technological areas of advancement to support innovation that serves the public health needs of children and special populations. We highlight four regulatory areas that have the potential to shape the future of pediatric cardiology: the CDRH Early Feasibility Study Program, advancements in 3D printing or additive manufacturing, computational modeling and simulation, and the use of real-world evidence for regulatory applications. These programs have the potential to impact all stages of device development, from early conception, design, and prototyping to clinical evidence generation, regulatory review, and finally commercialization. The success of these programs relies on a collaborative community of stakeholders, including government, regulators, device manufacturers, patients, payers, and the academic and professional community societies.
    Keywords:  3D printing; Device regulation; Innovation; Pediatric device development; Personalized medicine; Real world evidence; Technology
    DOI:  https://doi.org/10.1007/s00246-020-02296-0
  11. Pediatr Cardiol. 2020 Mar 20.
    Kang SL, Jaeggi E, Ryan G, Chaturvedi RR.
      Fetal cardiac interventions (FCI) offer the opportunity to rescue a fetus at risk of intrauterine death, or more ambitiously to alter disease progression. Most of these fetuses require multiple additional postnatal procedures, and it is difficult to disentangle the effect of the fetal procedure from that of the postnatal management sequence. The true clinical impact of FCI may only be discernible in large-volume institutions that can commit to a standardized postnatal approach and have sufficient case volume to overcome their FCI learning curve.
    Keywords:  Atrial stent; Fetal cardiac intervention; HLHS
    DOI:  https://doi.org/10.1007/s00246-020-02294-2