bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2020‒07‒19
ten papers selected by
Richard James
University of Pennsylvania


  1. J Spec Pediatr Nurs. 2020 Jul 14. e12303
    Erickson LA, Emerson A, Russell CL.
      PURPOSE: Single ventricle heart disease is fraught with risk for infant mortality and morbidity. During the interstage period, or the time between palliative cardiac surgeries, mobile health (mHealth) technology improves the ability of registered nurse coordinators to monitor infant symptoms through parental monitoring and reporting. The concept of parental mHealth adherence to symptom home monitoring of infants with single ventricle congenital heart disease has not been defined, despite increasing use of mHealth technology.METHODS: Rodger's concept analysis method was used to derive a unified definition of parental mHealth adherence to symptom home monitoring of infants with congenital heart disease during the single ventricle interstage period. A literature review included a search of databases for studies that addressed interstage home mHealth monitoring. Thematic analysis was applied to selected articles to derive a unified definition based on attributes, antecedents, consequences, related terms, and an illustrative case example. Sixteen publications were selected. Attributes, antecedents, and consequences of the concept were derived from the literature leading to a definition of parental mHealth adherence for infants with congenital heart disease during the single ventricle interstage period. The definition is the degree of adherence to which parents' transfer mHealth data for their infant meet healthcare providers' recommendations for symptom home monitoring. Consequences were improved infant symptom home monitoring through parental mHealth adherence measured by initiation, implementation, and discontinuation.
    CONCLUSION: The unified definition of the concept will provide a firmer ground for research in mHealth and interstage pediatric care and a guide for clinicians in developing new mHealth interventions for symptom home monitoring.
    PRACTICE IMPLICATIONS: This concept analysis hypothesizes that infants with a single ventricle during the interstage period, whose parents are mHealth symptom home monitoring adherent, will have timelier symptom identification with resultant superior outcomes compared with infants with a single ventricle during the interstage period whose parents are not mHealth adherent.
    Keywords:  cardiac; concept analysis; home nursing; interstage; pediatrics
    DOI:  https://doi.org/10.1111/jspn.12303
  2. Birth Defects Res. 2020 Jul 16.
    Fink CM, Cua CL, Nandi D, Hart SA.
      INTRODUCTION: Congenital heart defects (CHD) are common and are a frequent subject of research using large administrative databases such as the Pediatric Health Information System (PHIS) database. The capture rate of CHD within PHIS, however, has not been described.METHODS: The PHIS database includes inpatient encounters from over 52 tertiary care pediatric hospitals across the United States. We identified all patients less than 1 year of age with a cardiac defect in PHIS from 2010 to 2014 and compared these results with national prevalence estimates based on the National Birth Defects Prevention Network annual report, which served as the gold standard.
    RESULTS: The capture rate of CHD in PHIS ranged from 30 to 95% depending on the spectrum of severity and heterogeneity of the cardiac defect. The capture rate was higher for critical CHD (CCHD) at 66%, and all lesions with 70% or greater capture rate (interrupted aortic arch, truncus arteriosus, single ventricle, total anomalous pulmonary venous return, double outlet right ventricle, and hypoplastic left heart syndrome) fell within the CCHD category.
    CONCLUSIONS: Just over half of the predicted CHD patients were identified using the PHIS database. Although there is a high capture rate for CHD that require early hospitalization, there is a low capture rate for defects with a wider spectrum of disease presentation. These attributes of the PHIS database should be used to frame previous and future research using PHIS to study CHD.
    Keywords:  Pediatric Health Information System (PHIS); birth defects; congenital heart disease; critical congenital heart disease; health services research
    DOI:  https://doi.org/10.1002/bdr2.1753
  3. Health Expect. 2020 Jul 15.
    Bertaud S, Lloyd DFA, Sharland G, Razavi R, Bluebond-Langner M.
      OBJECTIVE: To explore the role of antenatal counselling in how parents make treatment decisions following an antenatal diagnosis of Hypoplastic Left Heart Syndrome (HLHS).BACKGROUND: Antenatal counselling is a critical part of patient management following a diagnosis of fetal congenital heart disease; however, there is a very limited evidence base examining how parents actually experience antenatal counselling and make decisions in this context.
    METHODS: Semi-structured interviews were conducted with women who had received an antenatal diagnosis of HLHS. Interviews were digitally recorded, anonymised and transcribed verbatim. A thematic content analysis was performed using a constant comparative approach.
    RESULTS: Eight mothers of surviving children with HLHS were interviewed. Eight key themes emerged including new perspectives on how women receive antenatal counselling and how it affects their decision making. Three themes in particular are new to the literature: (a) Mothers of children with HLHS reported feelings of intense guilt that arose in the antenatal period around potentially causing the condition in their child. (b) For this group of women, recollections of perceived pessimism during antenatal counselling had a lasting impact. (c) Despite support from partners or extended family, women nevertheless experienced a strong sense that antenatal decision making was largely a 'maternal' responsibility.
    CONCLUSIONS: When recounting their experiences of antenatal counselling, mothers of surviving children with HLHS offer new perspectives that can guide fetal cardiologists in how best to support their individual patients. Further research is needed to comprehensively understand the experience of prospective parents counselled for severe forms of fetal congenital heart disease.
    Keywords:  counselling; decision making; hypoplastic left heart syndrome; prenatal diagnosis
    DOI:  https://doi.org/10.1111/hex.13103
  4. Methodist Debakey Cardiovasc J. 2020 Apr-Jun;16(2):16(2): 130-137
    Vukicevic M, Filippini S, Little SH.
      Structural heart interventions (SHIs) are increasingly applicable in a wide range of heart defects, but the intricate and dynamic nature of cardiac structures can make SHIs challenging to perform. Three-dimensional (3D) printed modeling integrates advanced clinical imaging and 3D printing technology to replicate patient-specific anatomy for comprehensive planning and simulation of SHIs. This review discusses the basic principles of patient-specific 3D print model development, print material selection, and model fabrication and highlights how cardiovascular 3D printing can be used in preprocedural planning, device sizing, enhanced communication, and procedure simulation.
    Keywords:  3D printing; congenital heart defects; heart valves; patient-specific models; structural heart interventions
    DOI:  https://doi.org/10.14797/mdcj-16-2-130
  5. World J Pediatr Congenit Heart Surg. 2020 Jul 14. 2150135120934741
    Dearani JA, Stephens EH, Guleserian KJ, Overman DM, Backer CL, Romano JC, St Louis JD, Sarris GE, Bacha E, Tweddell JS.
      As recovery of congenital heart surgery programs begins during this COVID-19 pandemic, we review key considerations such as screening, protection of patients and health care workers (HCWs), case prioritization, barriers to reactivation, redesign of patient care teams, contribution of telemedicine, modification of trainees' experiences, preparation for potential resurgence, and strategies to maintain HCW wellness. COVID-19 has tested the resolve and grit of our specialty and we have an opportunity to emerge more refined.
    Keywords:  COVID-19; congenital cardiac surgery; crisis management
    DOI:  https://doi.org/10.1177/2150135120934741
  6. Ann Thorac Surg. 2020 Jul 08. pii: S0003-4975(20)31098-5. [Epub ahead of print]
    Hoffman GM, Niebler RA, Scott JP, Bertrandt RA, Wakeham MK, Thompson NE, Ghanayem NS, Stuth EA, Mitchell ME, Woods RK, Hraska V, Mussatto KA, Tweddell JS.
      BACKGROUND: Mortality following stage one palliation of hypoplastic left heart syndrome remains significant. Both cardiac output (CO) and systemic vascular resistance (SVR) contribute to hemodynamic vulnerability. Simultaneous measures of mean arterial pressure and somatic regional NIRS saturation can classify complex hemodynamics into four distinct states, with a low-CO state of higher risk. We sought to identify interventions associated with low-CO state occupancy and transition.METHODS: Perioperative data were prospectively collected in an IRB approved database. Hemodynamic state was classified as high CO, high SVR, low SVR, and low CO using bivariate analysis. Associations of static and dynamic support levels and state classifications over 48 postoperative hours were tested between states and across transitions using mixed regression methods in a quasi-experimental design.
    RESULTS: Data from 10272 hours in 214 patients were analyzed. A low-CO state was observed in 142 patients for 1107 hours. Both low-CO and ECMO had increased mortality risk. The low-CO state was characterized by lower milrinone but higher catecholamine dose. Successful transition out of low-CO was associated with increased milrinone dose and hemoglobin concentration. Increasing milrinone and hemoglobin levels predicted reduced risk of low-CO in future states.
    CONCLUSIONS: Bivariate classification objectively defines hemodynamic states and transitions with distinct support profiles. Maintaining or increasing inodilator and hemoglobin levels were associated with improved hemodynamic conditions and were predictive of successful future transitions from the low-CO state.
    Keywords:  CHD (hypoplastic left heart syndrome; Circulatory hemodynamics; Inotropic/vasoactive drugs; Modeling (computer; Monitoring (Near-infrared spectroscopy); Neonate; Norwood operation); mathematical; statistical)
    DOI:  https://doi.org/10.1016/j.athoracsur.2020.05.068
  7. Can J Cardiol. 2020 Jul 13. pii: S0828-282X(20)30595-X. [Epub ahead of print]
    Caterini JE, Campisi E, Cifra B.
      Low levels of habitual physical activity in children and adolescents with congenital heart disease (CHD) leads to various negative health outcomes, yet review of the literature indicates that many physicians, caregivers and patients restrict physical activity due to safety concerns. There is no evidence supporting absolute restriction of physical activity in pediatric patients with CHD, in fact, physically active lifestyles are important for pediatric patients living with CHD as much as for the general population. To encourage long-term maintenance of physically active lifestyles, physical activity counseling and exercise prescription should be started early in childhood and be a core component of every patient encounter. Physical activity counseling should include clear messaging from physicians about recommended physical activities, and not just restrictions, and a personalized written exercise prescription for the patient and family. Regular follow-up is essential to ensure adherence to recommendations, monitor patient responses to the increased physical activity level, and for long-term surveillance. This review discusses the importance of a physically active lifestyle in children and adolescents with and without surgically repaired CHD and hypertrophic cardiomyopathy, excluding those with electrical abnormalities and channelopathies. It outlines gaps in knowledge regarding best practices for physical activity promotion in these patient populations, and provides recommendations on how to include physical activity promotion and exercise prescription in clinical practice based on existing literature. An important role for clinicians is identified, as the type of messaging they provide regarding physical activity will have a major impact on patients' and families' decisions to adopt an active lifestyle.
    Keywords:  congenital heart disease; exercise prescription; pediatrics; physical activity
    DOI:  https://doi.org/10.1016/j.cjca.2020.07.003
  8. Clin Exp Pediatr. 2020 Jul 15.
    Yoon YM, Bae SP, Kim YJ, Kwak JG, Kim WH, Song MK, Shin SH, Kim EK, Kim HS.
      Background: Despite advances in neonatal intensive care and surgical procedures, perinatal mortality rates for premature infants with congenital heart disease (CHD) remain relatively high.Purpose: We aimed to describe the outcomes of premature infants with critical CHD and identify the risk factors including the new modified version of the Risk Adjustment for Congenital Heart Surgery (M-RACHS) category associated with in-hospital mortality in a Korean tertiary center.
    Methods: This was a retrospective cohort study of premature infants with critical CHD admitted to the neonatal intensive care unit from January 2005 to December 2016.
    Results: A total of 78 premature infants were enrolled. The median gestational age (GA) at birth was 34.9 weeks (range, 26.7-36.9 weeks), and the median birth weight was 1.91 kg (range, 0.53-4.38 kg). Surgical or percutaneous intervention was performed in 68 patients with a median GA at birth of 34.7 weeks (range, 26.7-36.8 weeks) and a median birth weight of 1.92 kg (range, 0.53-4.38 kg). The in-hospital survival rate was 76.9% among all enrolled preterm infants and 86.8% among patients who received an intervention. Very low birth weight (VLBW), persistent pulmonary hypertension of the newborn (PPHN), bronchopulmonary dysplasia (BPD), and M-RACHS category 5 or higher (more complex CHD) were independently associated with in-hospital mortality. For the 68 premature infants undergoing cardiac interventions, independent risk factors for mortality were VLBW, BPD, and CHD complexity. Late preterm infant and age at intervention were not associated with patient survival.
    Conclusion: For premature infants with critical CHD, VLBW, PPHN, BPD, and M-RACHS category ≥5 were risk factors for mortality. A careful approach to surgical intervention and prenatal care should be taken according to CHD type and neonatal condition.
    Keywords:  Congenital heart disease; Mortality; Outcome; Premature
    DOI:  https://doi.org/10.3345/cep.2019.01522
  9. J Am Coll Cardiol. 2020 Jul 21. pii: S0735-1097(20)35397-3. [Epub ahead of print]76(3): 334-341
    Zafar F, Lubert AM, Katz DA, Hill GD, Opotowsky AR, Alten JA, Goldstein SL, Alsaied T.
      The Fontan procedure has improved survival and quality of life for patients with single ventricle physiology. Along with extended life expectancy comes a growing population that is experiencing long-term multiorgan adverse effects. Whereas cardiorenal interactions have been extensively studied in patients with a structurally normal heart, these are less well understood in patients with a single ventricle. Several studies have investigated the prevalence of reduced glomerular filtration rate and albuminuria in the Fontan population; however, the long-term implication of renal dysfunction is not well established in this population. This paper provides a concise review of the published reports on the pathophysiology and spectrum of Fontan-associated renal disease. It also identifies gaps in currently available evidence that can guide ongoing and future research.
    Keywords:  Fontan; chronic kidney disease; cystatin C; renal resistive index
    DOI:  https://doi.org/10.1016/j.jacc.2020.05.042