bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2019‒07‒21
eleven papers selected by
Richard James
University of Pennsylvania


  1. Am J Cardiol. 2019 Jun 11. pii: S0002-9149(19)30590-9. [Epub ahead of print]
    Khanna AD, Duca LM, Kay JD, Shore J, Kelly SL, Crume T.
      The aim of this study was to estimate the prevalence of the full spectrum of mental illness in adolescents (aged 11 to 17) and adults (aged 18 to 64) with congenital heart defects (CHDs) in the population-level Colorado Congenital Heart Disease Surveillance System. Further we sought to investigate whether severity of the defect, frequency of recent cardiac procedures or underlying genetic disorders influence these estimates. The cohort included patients in clinical care for CHDs between January 1, 2011 and December 31, 2013, identified across multiple healthcare systems and insurance claims. Of 2,192 adolescents with CHDs, 20% were diagnosed with a mental illness with the most prevalent categories being developmental disorders (8%), anxiety disorders (6%), attention, conduct, behavior, impulse control disorders (6%), and mood disorders (5%). Of 6,924 adults with CHDs, 33% were diagnosed with a mental illness with the most prevalent categories being mood disorders (13%), anxiety disorders (13%), and substance-related disorders (6%). Greater lesion complexity was associated with a higher likelihood of anxiety and developmental disorders in both adolescents and adults. Adolescents and adults who had ≥2 cardiac procedures in the 3-year surveillance period had a 3- and 4.5-fold higher likelihood of a mental illness diagnosis, respectively, compared with those who had fewer than 2 cardiac procedures. Finally, patients with a genetic syndrome were more likely to have a mental illness diagnosis. In conclusion, mental illness is a prevalent co-morbidity in the adolescent and adult population with CHDs, thus comprehensive care should include mental health care.
    DOI:  https://doi.org/10.1016/j.amjcard.2019.05.023
  2. Semin Thorac Cardiovasc Surg. 2019 Jul 12. pii: S1043-0679(19)30188-1. [Epub ahead of print]
    Hiraiwa A, Kawasaki Y, Ibuki K, Hirono K, Matsui M, Yoshimura N, Origasa H, Oishi K, Ichida F.
      OBJECTIVE: To define the correlation between neuroanatomic and developmental outcomes of children with single ventricle (SV) or transposition of the great arteries (TGA), a prospective longitudinal study was performed in preschool and school-age children.METHOD: Twenty-seven children with congenital heart disease (CHD) (9, TGA; 18, SV) were included. Participants underwent 3-dimensional magnetic resonance imaging (MRI) and neurodevelopmental assessment at around 3 years (preschool age) and at 9 years (school age), and 48 healthy controls underwent MRI, and their data were used to derive best-fit models for normal brain volumes for comparisons with CHD patients.
    RESULTS: Total brain volume (TBV) and regional brain volumes remained significantly smaller in SV children than in TGA children at both time points, though the growth slope of TBV was not significantly different between the SV and TGA groups. Although the psychomotor developmental index (PDI) at preschool was significantly lower in SV patients, the full-scale IQ (FSIQ) at school age was not significantly lower in SV patients. There was a strong correlation between FSIQ and TBV (r = 0.49, P = 0.005).
    CONCLUSION: Despite the current best practices, persistently lower TBV was seen in SV patients until 9 years of age. For both the SV and TGA groups, TBV at 3 years was a strong predictor of TBV at 9 years. Since there was a correlation between TBV and IQ at 9 years, identification of factors that affect brain growth until 3 years will be imperative to improve patients' cognitive function at school age.
    Keywords:  Brain development; Congenital heart disease; Neurodevelopment; Single ventricle; Transposition of the great arteries
    DOI:  https://doi.org/10.1053/j.semtcvs.2019.06.013
  3. World J Pediatr Congenit Heart Surg. 2019 Jul;10(4): 475-484
    Salve GG, Datar GM, Perumal G, Singh AAV, Ayer JG, Roberts P, Sholler GF, Cole AD, Pigott N, Loughran-Fowlds A, Weatherall A, Alahakoon TI, Orr Y, Nicholson IA, Winlaw DS.
      BACKGROUND: Management of hypoplastic left heart syndrome (HLHS) presents many challenges. We describe our institutional outcomes for management of patients with HLHS over the past 12 years and highlight our strategy for those with highly restrictive/intact interatrial septum (R/I-IAS).METHODS: Eighty-eight neonates with HLHS underwent surgical treatment, divided equally into Era-I (n = 44, April 2006 to February 2013) and Era-II (n = 44, March 2013 to June 2018). Up to 2013, all patients with R/I-IAS were delivered at an adjacent adult hospital and then moved to our hospital for intensive care and management. From 2014, these patients were delivered at a co-located theatre in our hospital with immediate atrial septectomy. The hybrid approach was occasionally used with preference for the Norwood procedure for suitable candidates.
    RESULTS: One-year survival after Norwood procedure was 62.5% and 80% for Era-I and Era-II (P = not significant (ns)), respectively, and 41% of patients were categorized as high risk using conventional criteria. Survival at 1 year differed significantly between high-risk and standard-risk patients (P = 0.01). For high-risk patients, survival increased from 42% to 65% between eras (P = ns). In the R/I-IAS subgroup (n = 15), 11 underwent Norwood procedure after emergency atrial septectomy. Of these, seven born at the adjacent adult hospital had 40% survival to stage II versus 60% for the four born at the colocated theatre. Delivery in a colocated theatre reduced the birth-to-cardiopulmonary bypass median time from 445 (150-660) to 62 (52-71) minutes.
    CONCLUSION: Reported surgical outcomes are comparable to multicenter reports and international databases. Proactive management for risk factors such as R/I-IAS may contribute to improved overall outcomes.
    Keywords:  Norwood procedure; high-risk characteristics; hypoplastic left heart syndrome; restrictive atrial septum
    DOI:  https://doi.org/10.1177/2150135119852319
  4. World J Pediatr Congenit Heart Surg. 2019 Jul;10(4): 492-498
    St Louis JD, Tchervenkov CI, Jonas RA, Sandoval N, Zhang H, Jacobs JP, Talwar S, Halees ZA, Finucane K, Kirklin JK.
      The World Database for Pediatric and Congenital Heart Surgery was created to provide a resource for centers to be able to perform complex outcomes analyses of children undergoing repair of a congenital heart defect. In just under two years, the World Society for Pediatric and Congenital Heart Surgery (WSPCHS) has amassed over 13,000 procedures from 55 centers into the database. This Proceedings of the 3rd World Database Symposium held at the 6th Scientific Meeting of the WSPCHS summarizes the presentations of international experts in the fields of outcomes analysis and care of children with congenital heart surgery.
    Keywords:  congenital heart disease (CHD); congenital heart surgery; database (all types); mortality); outcomes (includes morbidity
    DOI:  https://doi.org/10.1177/2150135119852320
  5. World J Pediatr Congenit Heart Surg. 2019 Jul;10(4): 416-423
    West C, Maul T, Feingold B, Morell VO.
      BACKGROUND: Investigations of ventricular dominance and outcomes after the Fontan procedure have shown conflicting results. This may be due to the inclusion of multiple modifications of the Fontan or the omission of recently identified complications of the procedure. We examined the association between right ventricular dominance (RVD) and morbidity/mortality in a contemporary cohort following the extracardiac (EC) Fontan.METHODS: We studied all pediatric patients at our center who underwent a predominantly fenestrated EC Fontan from 2004 to 2016. Outcomes assessed were freedom from (1) Fontan failure (death, takedown, listing for transplantation) and (2) complication (arrhythmia requiring medication, postoperative pacemaker, or implantable cardioverter defibrillator requirement, stroke, thrombosis in the Fontan circuit, protein losing enteropathy, plastic bronchitis, New York Heart Association class >2). We defined the perioperative period as occurring before hospital discharge or within 30 days of the Fontan.
    RESULTS: A total of 137 patients (median age: 34 months, 62% male, 60% RVD) underwent the EC Fontan. Median duration of follow-up was 5.8 years (interquartile range: 2.4-9.0). Freedom from any event was 82.5% (RVD = 77%, LVD = 91%, χ2(1) = 5.03, P = .025) and RVD was associated with reduced event-free survival (hazard ratio: 2.94, P = .02). No confounders were identified. In the perioperative period, RVD was associated with reduced complication-free survival (P = .004). After this period, RVD was associated with reduced failure-free survival (P = .003).
    CONCLUSIONS: In this contemporary, single-center cohort of EC Fontan patients, RVD was associated with inferior outcomes.
    Keywords:  CHD; Fontan; complications; congenital heart disease; outcomes; regression analysis; right; statistics; survival analysis; univentricular heart; ventricle
    DOI:  https://doi.org/10.1177/2150135119843887
  6. Am J Hum Biol. 2019 Jul 18. e23297
    Kim HJ, Jae SY, Choo J, Yoon JK, Kim SH, Königstein K, Schmidt-Trucksäss A, Franklin BA.
      OBJECTIVES: There is little evidence on interrelationships between physical activity, sedentary behaviors, and health-related quality of life (HRQOL) among adolescents with congenital heart disease (CHD). We hypothesized that exercise capacity would have a mediating effect on the associations of either physical activity or sedentary behavior with HRQOL.METHODS: Adolescents with complex CHD (n = 111) were consecutively recruited from an outpatient clinic in a general hospital in South Korea. Physical activity and sedentary behavior were assessed using the global physical activity questionnaire. Exercise capacity was directly measured by peak oxygen uptake using a symptom-limited maximal treadmill exercise test. HRQOL was evaluated by both adolescents and their parents using the Pediatric Quality of Life Inventory questionnaire.
    RESULTS: The self-reported and parent proxy-reported HRQOL were positively associated with physical activity (ß = 0.16, P = .003; ß = 0.12, P = .049) and exercise capacity (ß = 0.63, P < .001; ß = 0.66, P < .001), but not with sedentary behavior in adjusted regression models. When both variables were entered in the same regression models, only exercise capacity remained significantly associated with the self-reported (ß = 0.50, P = .008) and parent proxy-reported HRQOL (ß = 0.62, P = .003). Exercise capacity acted as a full mediator variable on the relationship between physical activity and HRQOL (P < .05 for both).
    CONCLUSIONS: The present findings suggest that exercise capacity mediates the association between physical activity and HRQOL, highlighting the importance of improving exercise capacity to potentially enhance HRQOL in adolescents with complex CHD.
    DOI:  https://doi.org/10.1002/ajhb.23297
  7. Am J Obstet Gynecol. 2019 Jul 13. pii: S0002-9378(19)30903-2. [Epub ahead of print]
    Easter SR, Rouse CE, Duarte V, Hynes JS, Singh MN, Landzberg MJ, Valente AM, Economy KE.
      BACKGROUND: Though consensus guidelines on the management of cardiovascular disease (CVD) in pregnancy reserve cesarean delivery for obstetric indications, there is a paucity of data to support this approach.OBJECTIVES: To compare cardiovascular and obstetric morbidity in women with cardiovascular disease (CVD) according to plan for vaginal birth or cesarean delivery.
    STUDY DESIGN: We assembled a prospective cohort of women delivering at an academic tertiary care center with a protocolized multidisciplinary approach to management of CVD between September 2011 and December 2016. Our practice is to encourage vaginal birth in women with CVD unless there is an obstetric indication for cesarean delivery. We allow women attempting vaginal birth a trial of Valsalva in the second stage with the ability to provide operative vaginal delivery if pushing leads to changes in hemodynamics or symptoms. Women were classified according to planned mode of delivery-either vaginal birth or cesarean delivery. We then used univariate analysis to compare adverse outcomes according to planned mode of delivery. The primary composite cardiac outcome of interest included sustained arrhythmia, heart failure, cardiac arrest, cerebral vascular accident, need for cardiac surgery or intervention, or death. Secondary obstetric and neonatal outcomes were also considered.
    RESULTS: We included 276 consenting women with congenital heart disease (68.5%), arrhythmias (11.2%), connective tissue disease (9.1%), cardiomyopathy (8.0%), valvular disease (1.4%) or vascular heart disease (1.8%) at or beyond 24 weeks gestation. Seventy-six percent (n=210) planned vaginal birth and 24% (n=66) planned cesarean delivery. Women planning vaginal birth had lower rates of left ventricular outflow tract obstruction, multiparity, and preterm delivery. All women attempting vaginal birth were allowed to Valsalva. Among planned vaginal deliveries 86.2% (n=181) were successful with a 9.5% operative vaginal delivery rate. Five women underwent operative vaginal delivery for the indication of cardiovascular disease without another obstetric indication at the discretion of the delivering provider. Four of these patients tolerated trials of Valsalva ranging from 15 to 75 minutes prior to delivery. Adverse cardiac outcomes were similar between planned vaginal birth and cesarean delivery groups (4.3% v. 3.0%, p=1). Rates of postpartum hemorrhage (1.9% v. 10.6%, p<0.01) and transfusion (1.9% v. 9.1%, p=0.01) were lower in the planned vaginal birth group. There were no differences in adverse cardiac, obstetric or neonatal outcomes in the cohort overall or the subset of women with high-risk CVD or a high burden of obstetric comorbidity.
    CONCLUSIONS: These findings suggest that cesarean delivery does not reduce adverse cardiovascular outcomes and lend support to a planned vaginal birth for the majority of women with CVD including those with high-risk disease.
    Keywords:  Cardiovascular disease; maternal morbidity; operative vaginal delivery; vaginal delivery
    DOI:  https://doi.org/10.1016/j.ajog.2019.07.019
  8. J Cardiovasc Magn Reson. 2019 Jul 15. 21(1): 39
    de Lange C, Reichert MJE, Pagano JJ, Seed M, Yoo SJ, Broberg CS, Lam CZ, Grosse-Wortmann L.
      BACKGROUND: Patients with single ventricle physiology are at increased risk for developing liver fibrosis. Its extent and prevalence in children with bidirectional cavopulmonary connection (BCPC) and Fontan circulation are unclear. Extracellular volume fraction (ECV), derived from cardiovascular magnetic resonance (CMR) and T1 relaxometry, reflect fibrotic remodeling and/or congestion in the liver. The aim of this study was to investigate whether pediatric patients with single ventricle physiology experience increased native T1 and ECV as markers of liver fibrosis/congestion.METHODS: Hepatic native T1 times and ECV, using a cardiac short axis modified Look-Locker inversion recovery sequence displaying the liver, were measured retrospectively in children with BCPC- and Fontan circulations and compared to pediatric controls.
    RESULTS: Hepatic native T1 time were increased in Fontan patients (n = 62, 11.4 ± 4.4 years, T1 762 ± 64 ms) versus BCPC patients (n = 20, 2.8 ± 0.9 years, T1 645 ± 43 ms, p = 0.04). Both cohorts had higher T1 than controls (n = 44, 13.7 ± 2.9 years, T1 604 ± 54 ms, p < 0.001 for both). ECV was 41.4 ± 4.8% in Fontan and 36.4 ± 4.8% in BCPC patients, respectively (p = 0.02). In Fontan patients, T1 values correlated with exposure to cardiopulmonary bypass time (R = 0.3, p = 0.02), systolic and end diastolic volumes (R = 0.3, p = 0.04 for both) and inversely with oxygen saturations and body surface area (R = -0.3, p = 0.04 for both). There were no demonstrable associations of T1 or ECV with central venous pressure or age after Fontan.
    CONCLUSION: Fontan and BCPC patients have elevated CMR markers suggestive of hepatic fibrosis and/or congestion, even at a young age. The tissue changes do not appear to be related to central venous pressures.
    TRIAL REGISTRATION: Retrospectively registered data.
    Keywords:  Cardiovascular magnetic resonance; Fontan circulation; Liver cirrhosis; Single ventricle; T1 mapping
    DOI:  https://doi.org/10.1186/s12968-019-0545-4
  9. NPJ Digit Med. 2019 ;2 17
    Naci H, Salcher-Konrad M, Mcguire A, Berger F, Kuehne T, Goubergrits L, Muthurangu V, Wilson B, Kelm M.
      Computational modelling has made significant progress towards clinical application in recent years. In addition to providing detailed diagnostic data, these methods have the potential to simulate patient-specific interventions and to predict their outcome. Our objective was to evaluate to which extent patient-specific modelling influences treatment decisions in coarctation of the aorta (CoA), a common congenital heart disease. We selected three cases with CoA, two of which had borderline indications for intervention according to current clinical guidelines. The third case was not indicated for intervention according to guidelines. For each case, we generated two separate datasets. First dataset included conventional diagnostic parameters (echocardiography and magnetic resonance imaging). In the second, we added modelled parameters (pressure fields). For the two cases with borderline indications for intervention, the second dataset also included pressure fields after virtual stenting simulations. All parameters were computed by modelling methods that were previously validated. In an online-administered, invitation-only survey, we randomized 178 paediatric cardiologists to view either conventional (control) or add-on modelling (experimental) datasets. Primary endpoint was the proportion of participants recommending different therapeutic options: (1) surgery or catheter lab (collectively, "intervention") or (2) no intervention (follow-up with or without medication). Availability of data from computational predictive modelling influenced therapeutic decision making in two of three cases. There was a statistically significant association between group assignment and the recommendation of an intervention for one borderline case and one non-borderline case: 94.3% vs. 72.2% (RR: 1.31, 95% CI: 1.14-1.50, p = 0.00) and 18.8% vs. 5.1% (RR: 3.09, 95% CI: 1.17-8.18, p = 0.01) of participants in the experimental and control groups respectively recommended an intervention. For the remaining case, there was no difference between the experimental and control group and the majority of participants recommended intervention. In sub-group analyses, findings were not affected by the experience level of participating cardiologists. Despite existing clinical guidelines, the therapy recommendations of the participating physicians were heterogeneous. Validated patient-specific computational modelling has the potential to influence treatment decisions. Future studies in broader areas are needed to evaluate whether differences in decisions result in improved outcomes (Trial Registration: NCT02700737).
    Keywords:  Congenital heart defects; Health policy
    DOI:  https://doi.org/10.1038/s41746-019-0085-1
  10. World J Pediatr Congenit Heart Surg. 2019 Jul;10(4): 454-463
    Overman DM, Jacobs ML, O'Brien JE, Kumar SR, Mayer JE, Ebel A, Clarke DR, Jacobs JP.
      BACKGROUND: The completeness and accuracy of data contained within clinical databases and registries is critical to the reliability of reports emanating from these platforms. Therefore, vigorous data verification processes are a core competency of any mature database or registry. The Society of Thoracic Surgeons Congenital Heart Surgery Database (STS CHSD) has conducted audits of participant data for just over ten years. This report documents the validity of data elements within the STS CHSD.METHODS: We review the various elements of a robust audit process, detail the STS CHSD audit methodology, and report completeness and agreement rates for all adjudicated fields in the most recently completed audit.
    RESULTS: The rate of completeness for general data elements was 97.6% and the rate of agreement was 97.4%. The rate of completeness for variables in the mortality review was 100% and the rate of agreement was 99.3%.
    CONCLUSIONS: The STS CHSD audit is a highly structured and reproducible process. The most recently completed audit documents a very high level of completeness and accuracy of data variables, particularly those most germane to outcomes measurement.
    Keywords:  congenital heart disease; data verification; database; health policy; health professional affairs; medical audit; outcomes; quality care
    DOI:  https://doi.org/10.1177/2150135119845256
  11. Adv Neonatal Care. 2019 Jul 11.
    Staver MA, Moore TA, Hanna KM.
      BACKGROUND: The neonatal intensive care unit (NICU) can cause significant psychological distress in a mother. There is no common definition of maternal distress in the NICU currently in use.PURPOSE: To develop a clear conceptual understanding of maternal distress in the NICU using conceptual definitions and empirical findings.
    METHODS/SEARCH STRATEGY: A literature search was conducted using EBSCOhost, MEDLINE, CINAHL, PsychINFO, and Google Scholar. The concept analysis was guided by Walker and Avant's (2011) guide.
    FINDINGS/RESULTS: Maternal distress in the NICU consists of a combination of depressive, anxiety, trauma, and posttraumatic stress symptoms. The symptoms occur together on a spectrum and present differently in each mother. The antecedents to maternal distress are a NICU hospitalization and a perceived interruption to the transition to motherhood. Consequences of maternal distress in the NICU are issues with developing a healthy maternal-infant bond, adverse infant development, and decreased maternal quality of life.
    IMPLICATIONS FOR PRACTICE: A complete understanding of maternal distress in the NICU will lead to increased awareness of adverse mental health states in this population.
    IMPLICATIONS FOR RESEARCH: Identification of mothers at risk for maternal distress in the NICU, as well as the identification of antecedents and consequences related to the mother and the infant from maternal distress in the NICU. Using a single, clear definition of maternal distress in the NICU population will lead to a more cohesive body of literature.
    DOI:  https://doi.org/10.1097/ANC.0000000000000642