bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2019‒09‒15
eleven papers selected by
Richard James
University of Pennsylvania


  1. Ann Pediatr Cardiol. 2019 Sep-Dec;12(3):12(3): 254-286
    Saxena A, Relan J, Agarwal R, Awasthy N, Azad S, Chakrabarty M, Dagar KS, Devagourou V, Dharan BS, Gupta SK, Iyer KS, Jayranganath M, Joshi R, Kannan B, Katewa A, Kohli V, Kothari SS, Krishnamoorthy KM, Kulkarni S, Kumar RM, Kumar RK, Maheshwari S, Manohar K, Marwah A, Mishra S, Mohanty SR, Murthy KS, Rao KN, Suresh PV, Radhakrishnan S, Rajashekar P, Ramakrishnan S, Rao N, Rao SG, Chinnaswamy Reddy HM, Sharma R, Shivaprakash K, Subramanyan R, Kumar RS, Talwar S, Tomar M, Verma S, Vijaykumar R.
      A number of guidelines are available for the management of congenital heart diseases (CHD) from infancy to adult life. However, these guidelines are for patients living in high-income countries. Separate guidelines, applicable to Indian children, are required when recommending an intervention for CHD, as often these patients present late in the course of the disease and may have coexisting morbidities and malnutrition. Guidelines emerged following expert deliberations at the National Consensus Meeting on Management of Congenital Heart Diseases in India, held on August 10 and 11, 2018, at the All India Institute of Medical Sciences. The meeting was supported by Children's HeartLink, a nongovernmental organization based in Minnesota, USA. The aim of the study was to frame evidence-based guidelines for (i) indications and optimal timing of intervention in common CHD; (ii) follow-up protocols for patients who have undergone cardiac surgery/catheter interventions for CHD; and (iii) indications for use of pacemakers in children. Evidence-based recommendations are provided for indications and timing of intervention in common CHD, including left-to-right shunts (atrial septal defect, ventricular septal defect, atrioventricular septal defect, patent ductus arteriosus, and others), obstructive lesions (pulmonary stenosis, aortic stenosis, and coarctation of aorta), and cyanotic CHD (tetralogy of Fallot, transposition of great arteries, univentricular hearts, total anomalous pulmonary venous connection, Ebstein's anomaly, and others). In addition, protocols for follow-up of postsurgical patients are also described, disease wise. Guidelines are also given on indications for implantation of permanent pacemakers in children.
    Keywords:  Congenital heart disease; intervention; surgery
    DOI:  https://doi.org/10.4103/apc.APC_32_19
  2. Semin Thorac Cardiovasc Surg. 2019 Sep 10. pii: S1043-0679(19)30274-6. [Epub ahead of print]
    Welke KF, Pasquali SK, Lin P, Backer CL, Overman DM, Romano JC, Karamlou T.
      The objective of this study is to simulate regionalization of congenital heart surgery (CHS) in the United States and assess the impact of such a system on travel distance and mortality. Patients ≤18 years of age that underwent CHS were identified in 2012 State Inpatient Databases. Operations were stratified by the Risk Adjustment for Congenital Heart Surgery, version 1 (RACHS-1) method, with high risk defined as RACHS-1 levels 4-6. Regionalization was simulated by progressive closure of hospitals, beginning with the lowest volume hospital. Patients were moved to the next closest hospital. Analyses were conducted 1) maintaining original hospital mortality rates and 2) estimating mortality rates based on predicted surgical volumes after absorbing moved patients. 153 hospitals from 36 states performed one or more operation (19,064 operations). With regionalization wherein, all hospitals performed > 310 operations, 37 hospitals remained, from 12.5 to 17.4% fewer deaths occurred (83-116/666), and median patient travel distance increased from 38.5 to 69.6 miles (p<0.01). When only high-risk operations were regionalized, 3.9 to 5.9% fewer deaths occurred (26-39/666) and the overall mortality rate did not change significantly. Regionalization of CHS in the United States to higher volume centers may reduce mortality with minimal increase in patient travel distance. Much of the mortality reduction may be missed if solely high-risk patients are regionalized.
    Keywords:  CHSD; Congenital heart surgery; Risk adjustment; Zone improvement plan
    DOI:  https://doi.org/10.1053/j.semtcvs.2019.09.005
  3. J Clin Psychol Med Settings. 2019 Sep 10.
    Clancy T, Jordan B, de Weerth C, Muscara F.
      The experiences of diagnosis of congenital heart disease (CHD), cardiac surgery and hospitalisation(s) are distressing and represent a significant stressor for a child and family, which may impact psychosocial development. This systematic review provides a synthesis of psychosocial outcomes of infants and young children with congenital heart disease who had cardiac surgery early in life. Twenty-eight studies related to infant and young children's psychosocial development, specifically emotional, social and behavioural functioning were identified. Variability was related to methodological factors including differences in study design, varying measurement tools and heterogeneous samples. Despite these limitations, the majority of studies were of high quality. The most common finding was a high prevalence of low-severity emotional and behavioural dysregulation. Young children with severe CHD or comorbid conditions experienced greater impairment, with higher rates of externalising behaviour problems, although internalising behaviour problems were also evident. This review integrates findings from literature in the past 28 years on the psychosocial well-being of infants and young children with CHD and demonstrates a risk for emotional, social and behavioural development difficulty, and, importantly, that symptoms of psychosocial impairment are detectable very early in infancy. We advocate for assessment and monitoring of emotional and behavioural regulation and social development to be routinely conducted from infancy to enable prevention and early intervention.
    Keywords:  Congenital heart disease; Development; Infants/young children; Psychosocial
    DOI:  https://doi.org/10.1007/s10880-019-09651-1
  4. F1000Res. 2019 ;pii: F1000 Faculty Rev-1530. [Epub ahead of print]8
    van der Ven JPG, van den Bosch E, Bogers AJCC, Helbing WA.
      Tetralogy of Fallot (ToF) is the most common type of cyanotic congenital heart disease. Since the first surgical repair in 1954, treatment has continuously improved. The treatment strategies currently used in the treatment of ToF result in excellent long-term survival (30 year survival ranges from 68.5% to 90.5%). However, residual problems such as right ventricular outflow tract obstruction, pulmonary regurgitation, and (ventricular) arrhythmia are common and often require re-interventions. Right ventricular dysfunction can be seen following longstanding pulmonary regurgitation and/or stenosis. Performing pulmonary valve replacement or relief of pulmonary stenosis before irreversible right ventricular dysfunction occurs is important, but determining the optimal timing of pulmonary valve replacement is challenging for several reasons. The biological mechanisms underlying dysfunction of the right ventricle as seen in longstanding pulmonary regurgitation are poorly understood. Different methods of assessing the right ventricle are used to predict impending dysfunction. The atrioventricular, ventriculo-arterial and interventricular interactions of the right ventricle play an important role in right ventricle performance, but are not fully elucidated. In this review we present a brief overview of the history of ToF, describe the treatment strategies currently used, and outline the long-term survival, residual lesions, and re-interventions following repair. We discuss important remaining challenges and present the current state of the art regarding these challenges.
    Keywords:  Congenital Heart Disease; Fallot; Outcomes; Survival; Tetralogy
    DOI:  https://doi.org/10.12688/f1000research.17174.1
  5. J Cardiothorac Vasc Anesth. 2019 Aug 09. pii: S1053-0770(19)30811-0. [Epub ahead of print]
    Goeddel LA, Jung YH, Patel P, Upchurch P, Fernando RJ, Ramakrishna H.
      
    Keywords:  Adult Congenital Heart Disease; Guideline Based Management; atrial septal defect
    DOI:  https://doi.org/10.1053/j.jvca.2019.08.004
  6. Cardiol Young. 2019 Sep 10. 1-5
    Bokma JP, Daily JA, Kovacs AH, Oechslin EN, Baumgartner H, Khairy P, Mulder BJM, Veldtman GR.
      OBJECTIVE: Subspecialisation is increasingly a fundamental part of the contemporary practice of medicine. However, little is known about how medical trainees learn in the modern era, and particularly in growing and relatively new subspecialties, such as adult CHD. The purpose of this study was to assess institutional-led and self-directed learning strategies of adult CHD fellows.METHODS: This international, cross-sectional online survey was conducted by the International Society for Adult Congenital Heart Disease and consisted primarily of categorical questions and Likert rating scales. All current or recent (i.e., those within 2 years of training) fellows who reported training in adult CHD (within adult/paediatric cardiology training or within subspecialty fellowships) were eligible.
    RESULTS: A total of 75 fellows participated in the survey: mean age: 34 ± 5; 35 (47%) female. Most adult CHD subspecialty fellows considered case-based teaching (58%) as "very helpful", while topic-based teaching was considered "helpful" (67%); p = 0.003 (favouring case-based). When facing a non-urgent clinical dilemma, fellows reported that they were more likely to search for information online (58%) than consult a faculty member (29%) or textbook (3%). Many (69%) fellows use their smartphones at least once daily to search for information during regular clinical work.
    CONCLUSIONS: Fellows receiving adult CHD training reported a preference for case-based learning and frequent use of online material and smartphones. These findings may be incorporated into the design and enhancement of fellowships and development of online training resources.
    Keywords:  Adult CHD; fellowship training; medical education
    DOI:  https://doi.org/10.1017/S1047951119002063
  7. Clin J Am Soc Nephrol. 2019 Sep 09. pii: CJN.00690119. [Epub ahead of print]
    Parikh CR, Greenberg JH, McArthur E, Thiessen-Philbrook H, Everett AD, Wald R, Zappitelli M, Chanchlani R, Garg AX.
      BACKGROUND AND OBJECTIVES: Survival after surgical repair for congenital heart disease has markedly improved; however, there are limited data on long-term ESKD and mortality during childhood.DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: We conducted an observational, population-based cohort study of children who had their first surgery for congenital heart disease within 10 years of birth. The study was conducted in Ontario, Canada, where residents have universal access to health care services. Each child who underwent surgical repair was matched to ten children from the general population who were similar in age, sex, index date, rurality, and neighborhood income. Primary outcomes of all-cause mortality and ESKD were reported until March 2015.
    RESULTS: We followed 3600 children with congenital heart disease for a median of 5.9 (interquartile range, 2.9-9.0) years after their surgical repair. Median age at first surgery was 150 (interquartile range, 40-252) days and 22% were low birth weight (<2500 g). During follow-up, 140 (4%) children who had surgery for congenital heart disease died and 52 (1%) reached ESKD. The cumulative incidence of death and ESKD at 1, 5, and 10 years was higher in children with surgical repair of congenital heart disease (death: 3%, 4%, and 5%, respectively; ESKD: 1%, 2%, and 2%, respectively) compared with the matched control population without any congenital heart disease (death: 0.06%, 0.10%, and 0.13%, respectively; ESKD: 0.00%, 0.02%, and 0.02%, respectively). The risk of ESKD and death increased with severity of congenital heart disease, with the highest risk in children with hypoplastic left heart syndrome and increased in children who had surgical repair of congenital heart disease compared with those without surgical repair.
    CONCLUSIONS: The risk of mortality and ESKD is high in children who undergo surgical repair for congenital heart disease compared to the general population.
    Keywords:  ESRD; Ontario; cardiac surgical procedures; cardiovascular; child; children; chronic kidney disease; chronic kidney failure; clinical epidemiology; congenital heart defects; death; follow-up studies; humans; hypoplastic left heart syndrome; incidence; low birth weight infant; pediatric nephrology; pediatrics; renal function; renal function decline; risk
    DOI:  https://doi.org/10.2215/CJN.00690119
  8. J Am Heart Assoc. 2019 Sep 17. 8(18): e013608
    Farr SL, Downing KF, Ailes EC, Gurvitz M, Koontz G, Tran EL, Alverson CJ, Oster ME.
      Background Our objective was to estimate receipt of preconception health care among women with congenital heart defects (CHD), according to 2017 American Heart Association recommendations, as a baseline for evaluating recommendation implementation. Methods and Results Using 2007 to 2013 IBM MarketScan Commercial Databases, we identified women with CHD diagnosis codes ages 15 to 44 years who became pregnant and were enrolled in health insurance for ≥11 months in the year before estimated conception. We assessed documentation of complete blood count, electrolytes, thyroid-stimulating hormone, liver function, ECG, comprehensive echocardiogram, and exercise stress test, using procedural codes, and outpatient prescription claims for US Food and Drug Administration category D and X cardiac-related medications. Differences were examined according to CHD severity, age, region of residence, year of conception, and documented encounters at obstetric and cardiology practices. We found 2524 pregnancies among 2003 women with CHD (14.4% severe CHD). In the 98.3% of women with a healthcare encounter in the year before conception, <1% received all and 22.6% received no American Heart Association-recommended tests or assessments (range: 54.4% for complete blood count to 3.1% for exercise stress test). Women with the highest prevalence of receipt of recommended care were 35 to 44 years old, pregnant in 2012 to 2013, or had a documented obstetric or cardiology encounter in the year before conception (P<0.05 for all). In 9.0% of pregnancies, ≥1 prescriptions for US Food and Drug Administration category D or X cardiac-related medications were filled in the year before conception. Conclusions A low percentage of women with CHD received American Heart Association-recommended preconception health care in the year before conception.
    Keywords:  congenital cardiac defect; preconception; pregnancy
    DOI:  https://doi.org/10.1161/JAHA.119.013608
  9. Pediatr Res. 2019 Sep 09.
    Cabrera-Mino C, Roy B, Woo MA, Singh S, Moye S, Halnon NJ, Lewis AB, Kumar R, Pike NA.
      BACKGROUND: Adolescents with single ventricle heart disease (SVHD) who have undergone the Fontan procedure show cognitive/memory deficits. Mammillary bodies are key brain sites that regulate memory; however, their integrity in SVHD is unclear. We evaluated mammillary body (MB) volumes and their associations with cognitive/memory scores in SVHD and controls.METHODS: Brain MRI data were collected from 63 adolescents (25 SVHD; 38 controls) using a 3.0-Tesla MRI scanner. Cognition and memory were assessed using Montreal Cognitive Assessment (MoCA) and Wide Range Assessment of Memory and Learning 2. MB volumes were calculated and compared between groups (ANCOVA, covariates: age, sex, and total brain volume [TBV]). Partial correlations and linear regression were performed to examine associations between volumes and cognitive scores (covariates: age, sex, and TBV).
    RESULTS: SVHD group showed significantly lower MoCA and WRAML2 scores over controls. MB volumes were significantly reduced in SVHD over controls. After controlling for age, sex, and TBV, MB volumes correlated with MoCA and delayed memory recall scores in SVHD and controls.
    CONCLUSION: Adolescents with SVHD show reduced MB volumes associated with cognitive/memory deficits. Potential mechanisms of volume losses may include developmental and/or hypoxic/ischemic-induced processes. Providers should screen for cognitive deficits and explore possible interventions to improve memory.
    DOI:  https://doi.org/10.1038/s41390-019-0569-3
  10. BMC Pediatr. 2019 Sep 10. 19(1): 326
    Ribera I, Ruiz A, Sánchez O, Eixarch E, Antolín E, Gómez-Montes E, Pérez-Cruz M, Cruz-Lemini M, Sanz-Cortés M, Arévalo S, Ferrer Q, Vázquez E, Vega L, Dolader P, Montoliu A, Boix H, Simões RV, Masoller N, Sánchez-de-Toledo J, Comas M, Bartha JM, Galindo A, Martínez JM, Gómez-Roig L, Crispi F, Gómez O, Carreras E, Cabero L, Gratacós E, Llurba E.
      BACKGROUND: Congenital heart disease (CHD) is the most prevalent congenital malformation affecting 1 in 100 newborns. While advances in early diagnosis and postnatal management have increased survival in CHD children, worrying long-term outcomes, particularly neurodevelopmental disability, have emerged as a key prognostic factor in the counseling of these pregnancies.METHODS: Eligible participants are women presenting at 20 to < 37 weeks of gestation carrying a fetus with CHD. Maternal/neonatal recordings are performed at regular intervals, from the fetal period to 24 months of age, and include: placental and fetal hemodynamics, fetal brain magnetic resonance imaging (MRI), functional echocardiography, cerebral oxymetry, electroencephalography and serum neurological and cardiac biomarkers. Neurodevelopmental assessment is planned at 12 months of age using the ages and stages questionnaire (ASQ) and at 24 months of age with the Bayley-III test. Target recruitment is at least 150 cases classified in three groups according to three main severe CHD groups: transposition of great arteries (TGA), Tetralogy of Fallot (TOF) and Left Ventricular Outflow Tract Obstruction (LVOTO).
    DISCUSSION: The results of NEURO-HEART study will provide the most comprehensive knowledge until date of children's neurologic prognosis in CHD and will have the potential for developing future clinical decisive tools and improving preventive strategies in CHD.
    TRIAL REGISTRATION: NCT02996630 , on 4th December 2016 (retrospectively registered).
    Keywords:  Cardiac function and fetal brain MR; Congenital heart disease; Neurodevelopment; Predictive markers
    DOI:  https://doi.org/10.1186/s12887-019-1689-y
  11. J Chin Med Assoc. 2019 Sep 06.
    Chen YC, Weng KP, Chien KJ, Chen BH, Hsieh KS, Ta IH, Huang SH, Peng HH, Huang JS, Wu MT.
      BACKGROUND: Hepatic dysfunction is an important long-term complication in Fontan patients. The purpose of this study was to evaluate the hepatic computed tomography (CT) findings after Fontan surgery and identify their association with clinical parameters.METHODS: This study recruited 43 patients (20 female patients aged 15.3±6.8 years) who underwent Fontan surgery. Medical records were reviewed to collect their age, sex, congenital heart disease type, date of Fontan surgery, laboratory data, and hepatic CT findings. The relationship between hepatic findings and clinical parameters was analyzed.
    RESULTS: The follow-up duration was 6.8 ± 4.1 years. Abnormal hepatic parenchymal enhancement was observed in 77% of the patients, with mild degree in 18, moderate degree in 10, and severe degree in 5 patients. According to the univariate analysis, risk factors for hepatic parenchymal enhancement were follow-up duration {odds ratio (OR): 1.354 (95% confidence interval (CI): 1.024-2.078), p = 0.042}, hypoplastic left ventricle syndrome {OR: 3.262 (95% CI: 1.145-5.628), p = 0.002}, mean pulmonary artery pressure {OR: 1.598 (95% CI: 1.089-2.132), p = 0.026}, pulmonary vascular resistance index {OR: 1.263 (95% CI: 1.068-1.245), p = 0.032}, and brain natriuretic peptide {OR: 1.956 (95% CI: 1.085-2.673), p = 0.045}. According to the multivariate analysis, only hypoplastic left ventricle syndrome {OR: 3.856 (95% CI: 1.389-5.863), p = 0.001}, mean pulmonary artery pressure {OR: 1.846 (95% CI: 1.362-2.549), p = 0.015}, and pulmonary vascular resistance index {OR: 1.185 (95% CI: 1.042-1.736), p = 0.047} were significant risk factors for abnormal parenchymal enhancement.
    CONCLUSION: Abnormal hepatic parenchymal enhancement detected through CT is common in Fontan patients. Regular liver function test in conjunction with imaging studies may be considered when following up Fontan patients.
    DOI:  https://doi.org/10.1097/JCMA.0000000000000185