bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2019‒11‒03
nine papers selected by
Richard James
University of Pennsylvania


  1. J Pediatr. 2019 Nov;pii: S0022-3476(19)30952-7. [Epub ahead of print]214 79-88
    Haxel C, Glickstein J, Parravicini E.
      OBJECTIVES: To report the outcomes of a Neonatal Palliative Care (NPC) Program at a large tertiary cardiac center caring for a subset of fetuses and neonates with life-limiting cardiac diagnoses or cardiac diagnoses with medical comorbidities leading to adverse prognoses.STUDY DESIGN: The Neonatal Comfort Care Program at New York-Presbyterian Morgan Stanley Children's Hospital/Columbia University Medical Center is an interdisciplinary team that offers the option of NPC to neonates prenatally diagnosed with life-limiting conditions, including single ventricle (SV) congenital heart disease (CHD) or less severe forms of CHD complicated by multiorgan dysfunction or genetic syndromes.
    RESULTS: From 2008 to 2017, the Neonatal Comfort Care Program cared for 75 fetuses or neonates including 29 with isolated SV CHD, 36 with CHD and multiorgan dysfunction and/or severe genetic abnormalities, and 10 neonates with a prenatal diagnosis of isolated CHD and postnatal diagnoses of severe conditions who were initially in intensive care before transitioning to NPC because of a poor prognosis.
    CONCLUSIONS: At New York-Presbyterian Morgan Stanley Children's Hospital/Columbia University Medical Center, a large tertiary cardiac center, 13.5% of parents of fetuses or neonates with isolated SV CHD opted for NPC. Twenty-six of 29 newborns with SV CHD treated with NPC died. Of the remaining, 2 neonates with mixing lesions are alive at 3 and 5 years of age, and 1 neonate was initially treated with NPC and then pursued surgical palliation. These results suggest that NPC is a reasonable choice for neonates with SV CHD.
    Keywords:  congenital heart disease; neonatal palliative care; single ventricle
    DOI:  https://doi.org/10.1016/j.jpeds.2019.07.044
  2. Heart. 2019 Oct 29. pii: heartjnl-2019-314937. [Epub ahead of print]
    Rodriguez FH, Book WM.
      Adults with congenital heart disease who underwent the Fontan operation to palliate single ventricle heart defects-by direct connection of caval venous return to the pulmonary circulation-have improved survival due to advancements in surgical and interventional techniques. However, cardiac and non-cardiac comorbidities often coexist, complicating management, and contributing to premature morbidity and mortality. Cardiac issues include heart failure symptoms related to systolic and diastolic dysfunction, atrial and ventricular arrhythmias and systemic atrioventricular valve regurgitation. Structural issues may be related to obstruction of the Fontan pathway, or to branch pulmonary artery stenosis, both of which exacerbate symptoms. Non-cardiac complications in adults involve hepatic congestion, fibrosis and cirrhosis, hepatocellular carcinoma, chronic kidney disease, stroke, venous stasis, lymphatic issues and involvement of other organ systems. 'Fontan failure' refers to circulatory dysfunction, either cardiac, non-cardiac, or both, diagnosed after exclusion or treatment of reversible contributors such as structural issues and arrhythmias. Counselling about reproductive health, mental health, perioperative management and overall wellness are paramount for patients' well-being. Fontan patients are typically managed in highly specialised adult congenital heart centres, but may present to cardiologists or other practitioners with cardiac and non-cardiac emergencies or urgencies, sometimes after being out of care. In this review, we discuss the management of the adult Fontan patient, including surveillance, cardiac and non-cardiac complications, reproductive health, and advanced therapies.
    Keywords:  Fontan physiology; complex congenital heart disease; congenital heart disease; heart failure with preserved ejection fraction; heart failure with reduced ejection fraction
    DOI:  https://doi.org/10.1136/heartjnl-2019-314937
  3. Eur J Cardiovasc Nurs. 2019 Oct 28. 1474515119881871
    Chang LY, Chiu SN, Wang CC, Weng WC, Chang HY.
      BACKGROUND: Children and adolescents with cyanotic congenital heart disease have been found to be at an increased risk of internalising problems. The underlying mechanisms, however, remain uncertain.AIMS: To examine the association between cyanotic congenital heart disease and internalising problems and assess whether parenting stress mediates this association.
    METHOD: The study sample was 699 children and adolescents (aged 2-17 years) with congenital heart disease (253 with cyanotic congenital heart disease and 446 with acyanotic congenital heart disease) in Taiwan. The Child Behavior Checklist and the Parenting Stress Index were used to assess internalising problems and parenting stress, respectively. A series of multiple regression models was conducted using the SPSS PROCESS procedure to test the association between types of congenital heart disease and internalising problems and the mediating role of parenting stress. A bootstrapping approach was applied to determine the significance of mediation.
    RESULTS: Compared with acyanotic congenital heart disease, cyanotic congenital heart disease was associated with increased levels of internalising problems (B=2.52, P<0.01), and this association was mediated by parenting stress (B=0.97, 95% bias-corrected bootstrap confidence interval 0.24, 1.75). In particular, parents of children with cyanotic congenital heart disease reported significantly more parenting stress compared with parents of children with acyanotic congenital heart disease (B=4.63, P<0.01), which contributed to elevated levels of internalising problems in the offspring (B=0.21, P<0.001).
    CONCLUSION: Cyanotic congenital heart disease conferred risks for internalising problems in children and adolescents, and this association was mediated by parenting stress. Interventions to decrease internalising problems in children and adolescents with cyanotic congenital heart disease may be more effective by targeting parenting stress.
    Keywords:  Congenital heart disease; cyanotic; internalising problems; mediation; parenting stress
    DOI:  https://doi.org/10.1177/1474515119881871
  4. Congenit Heart Dis. 2019 Oct 27.
    Golfenshtein N, Hanlon AL, Deatrick JA, Medoff-Cooper B.
      OBJECTIVE: Parents of infants with congenital heart disease (CHD) experience increased parenting stress levels, potentially interfering with parenting practices and bear adverse family outcomes. Condition severity has been linked to parenting stress. The current study aimed to explore parenting stress trajectories over infancy in parents of infants with complex CHD, and to compare them by post-operative cardiac physiology.DESIGN: Data from a larger prospective cohort study was analyzed using longitudinal mixed-effects regression modeling.
    SETTING: Cardiac intensive care unit and outpatient clinic of a 480-bed children's hospital in the American North-Atlantic region.
    PARTICIPANTS: Parents of infants with complex CHD (n = 90).
    MEASURES: Parenting stress was measured via the parenting stress index-long form over four time points during infancy.
    RESULTS: Parents of infants with a single-ventricle heart experienced a decrease in total stress over time. Parents of infants with a biventricular heart experienced a decrease in attachment-related stress, and an increase in stress related to infant temperament over time. Parenting stress trajectories over time significantly differed between groups on infant temperamental subscales.
    CONCLUSIONS: Findings highlight stressful and potentially risky periods for parents of infants with complex CHD, and introduce additional illness-related and psychosocial/familial aspects to the parenting stress concept. Early intervention may promote parental adaptive coping and productive parenting practices in this population.
    Keywords:  congenital heart disease; infancy; longitudinal design; parenting stress; uni-ventricular heart
    DOI:  https://doi.org/10.1111/chd.12858
  5. Child Neuropsychol. 2019 Oct 31. 1-27
    Phillips JM, Longoria JN.
      Congenital heart disease (CHD) is among the most prevalent birth defects in the United States. Given that children with CHD are at risk for differences with development, learning, and psychosocial functioning, effective intervention becomes a central tenant of recommendations following neuropsychological consultation and evaluation. The primary focus of this review is to summarize available interventions for children and adolescents with CHD. The existing CHD literature has concentrated on early developmental services, psychopharmacological treatment, and need for academic supports. The literature is limited with regard to intervention studies that target cognitive deficits and psychosocial difficulties. To address this discrepancy, efficacious interventions that have been used to mitigate these concerns within other medical groups are also discussed in an effort to provide options for alternative recommendations and services. The current paper is intended to facilitate comprehensive care for cardiac patients by providing clinicians with a review of the available intervention literature, as well as potential interventions that may serve as supplemental strategies in the interim.
    Keywords:  CHD; Congenital heart disease; birth defect; intervention; pediatric
    DOI:  https://doi.org/10.1080/09297049.2019.1682131
  6. Pediatr Cardiol. 2019 Nov 01.
    Davis JAM, Bass A, Humphrey L, Texter K, Garee A.
      Children with single ventricle congenital heart defects (SVCHD) experience a significant risk of early mortality throughout their lifespan, particularly during their first year of life. Due to the intense care needed for these children and families, pediatric palliative care (PPC) team consults should be routine; however, medical staff are often reluctant to broach the idea of PPC to families. The involvement of PPC for many carries with it an association to end-of-life (EOL) care. Setting the standard of PPC involvement from the time of admission for the first palliative surgery led to increased family support, decreased days to consult, improved acceptance and communication. The purpose of this article is to describe a quality improvement project of early integration of PPC with families of children with SVCHD. Lessons learned will be presented, including the resources needed and the barriers encountered in assimilating PPC into the standard of care for all patients with SVCHD. The single ventricle (SV) and PPC teams collaborated to enhance the support given to SV families. Education was initiated with cardiology and PPC providers to understand the goal of consistent PPC consults beginning after birth for patients with SVCHD. Parents were educated during fetal consultation regarding the involvement of the PPC team. The SV team ensured compliance with the PPC initiative by identifying eligible patients and requesting consult orders from the primary providers. PPC consultation increased significantly over the 40 month study period to nearly 100% compliance for children with SVCHD who are undergoing pre-Fontan surgery. In addition, mean days to consult decreased dramatically during the study to a current average of 3 days into the patient's hospitalization; the data likely suggest that more PPC consults were routinely ordered versus urgently placed for unexpected complications. Data indicate that patients are being followed by the PPC team at an earlier age and stage in their SV journey which allows for more opportunity to provide meaningful support to these patients and families. The early involvement of the PPC team for children with SV physiology was operationally feasible and was accepted by families, thus allowing PPC providers to establish a therapeutic relationship early in the disease trajectory with the family. It allowed more continuity throughout the SV journey in a proactive fashion rather than a reactive manner.
    Keywords:  Cardiac; Collaboration; End-of-life; Palliative; Pediatric; Single ventricle
    DOI:  https://doi.org/10.1007/s00246-019-02231-y
  7. Heart. 2019 Oct 29. pii: heartjnl-2019-315430. [Epub ahead of print]
    Daley M, du Plessis K, Zannino D, Hornung T, Disney P, Cordina R, Grigg L, Radford DJ, Bullock A, d'Udekem Y.
      OBJECTIVE: Patients undergoing single-ventricle palliation have experienced significant improvement in survival in the recent era. However, a substantial proportion of these patients undergo reoperations. We performed a review of the Australia and New Zealand (ANZ) Fontan Registry to determine the overall reintervention and reoperative burden in these patients.METHODS: A retrospective longitudinal cohort study was performed using data from patients who underwent a Fontan operation between 1975 and 2016 from the ANZ Fontan Registry. The data obtained included Fontan operation, reinterventions and most recent follow-up status. We examined the type and timing of reinterventions and survival.
    RESULTS: Of the 1428 patients identified, 435 (30%) underwent at least one reintervention after the Fontan operation: 110 patients underwent early reintervention and 413 underwent late reinterventions. Excluding Fontan conversion and transplantation, 220 patients underwent at least one interventional procedure and 209 patients underwent at least one reoperation. Fenestration closure and pacemaker-related procedures were the most common catheter and surgical interventions, respectively. The cumulative incidence of reintervention following Fontan was 23%, 37% and 55% at 10, 20 and 30 years, respectively. Survival and freedom from failure were worse in patients requiring later reintervention after Fontan surgery (51% vs 83% and 42% vs 69%, respectively at 30 years, p<0.001). This difference persisted after excluding pacemaker-related procedures (p<0.001). Operative mortality for non-pacemaker late reoperations after Fontan was 6%.
    CONCLUSIONS: A substantial proportion of Fontan patients require further intervention to maintain effective single-ventricle circulation. Patients undergoing reoperation after Fontan have higher rates of mortality and failure, despite intervention.
    Keywords:  complex congenital heart disease; congenital heart disease; congenital heart disease surgery; fontan physiology
    DOI:  https://doi.org/10.1136/heartjnl-2019-315430
  8. World J Pediatr Congenit Heart Surg. 2019 Oct 28. 2150135119878702
    Ryan KR, Jones MB, Allen KY, Marino BS, Casey F, Wernovsky G, Lisanti AJ.
      As survivable outcomes among patients with complex congenital heart disease (cCHD) have continued to improve over the last several decades, more attention is being dedicated to interventions that impact not just survival but quality of life among patients with cCHD. In particular, patients with cCHD are at risk for impaired neurodevelopmental outcomes. In this review summarizing select presentations given at the 14th Annual Pediatric Cardiac Intensive Care Society's Annual Meeting in 2019, we discuss the neurodevelopmental phenotype of patients with cCHD, patients at greatest risk of impaired development, and three specific modifiable risk factors impacting development.
    Keywords:  congenital heart disease; congenital heart surgery; developmental phenotype; long term outcomes; neurodevelopment
    DOI:  https://doi.org/10.1177/2150135119878702
  9. J Am Coll Cardiol. 2019 Nov 05. pii: S0735-1097(19)37529-1. [Epub ahead of print]74(18): 2295-2312
    Givertz MM, DeFilippis EM, Landzberg MJ, Pinney SP, Woods RK, Valente AM.
      In the contemporary era, nearly 85% of children with congenital heart disease will reach adulthood. Despite optimal medical and surgical treatment, many will experience a progressive decline in cardiopulmonary function leading to advanced heart failure. These patients present unique anatomic and physiological challenges to the care team, and unlike adults with acquired heart disease who progress to severe heart failure, advanced treatment options such as mechanical circulatory support and cardiac transplant may be limited. Severe ventricular dysfunction and/or pulmonary hypertension may not be amenable to corrective repair. Heart transplantation with or without mechanical circulatory support may be the only option for highly selected patients. The aim of this review is to describe advanced heart failure therapies for adults with congenital heart disease, including the general approach to evaluation and management, pre- and post-operative care, anticipated short- and long-term outcomes, and future directions for clinical care and research.
    Keywords:  adult congenital heart disease; heart failure; heart transplant; mechanical circulatory support
    DOI:  https://doi.org/10.1016/j.jacc.2019.09.004