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


  1. World J Pediatr Congenit Heart Surg. 2019 01;10(1): 66-71
    Anderson JB, Brown DW, Lihn S, Mangeot C, Bates KE, Van Bergen AH, Rudd NA, Hanke S, Tweddell J, Lannon C.
      BACKGROUND: The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) formed to improve outcomes in infants with hypoplastic left heart syndrome. The collaborative sought to (1) decrease mortality, (2) reduce growth failure, and (3) reduce hospital readmissions due to major medical problems during the interstage period between discharge following stage 1 palliation (S1P) and admission for stage 2 palliation (S2P).METHODS: The NPC-QIC is a learning network, coproduced by parents and clinicians, of 65 pediatric cardiology centers that contribute clinical data on care processes and outcomes to a shared registry. The adapted Breakthrough Series Model structure brings teams together regularly to review data, share lessons, and plan improvements. Outcomes are monitored using statistical process control methods.
    RESULTS: Between 2008 and 2016, interstage mortality decreased by >40%, from 9.5% to 5.3%. Identification and use of a nutrition bundle led to improved infant growth, with a 28% reduction in interstage growth failure. The rate of serious hospital readmissions was low and did not significantly change. Importantly, a formed partnership with the parent group Sisters by Heart fostered the coproduction of tools and strategies and an emphasis on data transparency and outcomes.
    CONCLUSIONS: The NPC-QIC's initial efforts led to improvements in interstage growth and mortality. The NPC-QIC has modeled the use of data for improvement and research, the value of coproduction with parents, and the concept "all teach, all learn," demonstrating the power of the learning network model.
    Keywords:  Norwood; hypoplastic left heart syndrome; nutrition; quality improvement
    DOI:  https://doi.org/10.1177/2150135118815023
  2. World J Pediatr Congenit Heart Surg. 2019 01;10(1): 72-80
    Martin GR, Anderson JB, Vincent RN.
      The IMproving Pediatric and Adult Congenital Treatments (IMPACT) Registry and the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) are two efforts initiated to improve outcomes in the congenital heart disease field. The IMPACT Registry is focused on evaluating the use, risks, adverse events (AEs), and outcomes associated with diagnostic and common interventional catheterization procedures in all children and adults with congenital heart disease. Utilizing a modular approach, the common procedures include diagnostic cardiac catheterization, atrial septal defect device closure, patent ductus arteriosus device closure, pulmonary valvuloplasty, aortic valvuloplasty, balloon and stent angioplasty of coarctation of the aorta, pulmonary artery balloon stent angioplasty, transcatheter pulmonary valve replacement, and electrophysiology procedures including radiofrequency ablation. To date, important observations on the common procedures have been made and a risk stratification methodology has been created to allow comparisons between centers in AEs and quality improvement activity. The registry is open to international participation. The NPC-QIC was developed to reduce mortality and improve the quality of life of infants with Hypoplastic Left Heart Syndrome (HLHS) during the interstage period between discharge from the Norwood operation and admission for the bidirectional Glenn procedure. Mortality in the interstage has been reduced by 44%. The IMPACT Registry and the NPC-QIC have demonstrated value to the congenital heart disease community. The IMPACT Registry, however, has not yet demonstrated an impact on patient outcomes. The NPC-QIC, which combines both a registry with a learning collaborative with specific aims, key drivers, and change strategies, has made more significant gains with reductions in variation, growth failures, and mortality.
    Keywords:  cardiac catheterization/intervention; congenital heart disease; databases; outcomes
    DOI:  https://doi.org/10.1177/2150135118815059
  3. Eur J Prev Cardiol. 2019 Mar 01. 2047487319830531
    Villani GQ, Villani A, Zanni A, Sticozzi C, Maceda DP, Rossi L, Pisati MS, Piepoli MF.
      BACKGROUND: Mobile computing and communication technologies in health services and information (so-called mHealth) have modified the traditional approach in the follow-up of patients with implantable cardiac devices, increased patient engagement and empowerment, reduced healthcare costs and improved patients' outcome. Recent developments in mobile technology, with the introduction of smartphone-compatible devices that can measure various health parameters and transfer automatically generated data, have increased the potential application of remote monitoring and the interest towards mHealth. However, little is known about the patients' interest and expectations of this new technology.OBJECTIVE: The patients' interest in the possibility of receiving data from their implantable cardiac device, clinical and health advice via remote monitoring on their smartphones were investigated.
    METHODS: A questionnaire entitled 'Expectations for future possibility of self-management of device data' (Likert scale scored) was submitted to 300 consecutive implantable cardiac device outpatients. The questionnaire was focused on collecting patients' expectations in receiving direct information regarding their implantable cardiac device status (item 1, five questions), their own clinical status (item 2, seven questions) and advice on healthy lifestyle promotion (item 3, nine questions). Patient characteristics associated with greater interest towards mHealth were also investigated.
    RESULTS: Questionnaires were completed by 268 patients (221 men, aged 69 ± 14 years). The Cronbach test reported an alpha value of 0.98 for item 1, 0.94 for item 2 and 0.97 for item 3. Patients declared to be mainly interested in the device interventions (62%) and in severe arrhythmia occurrence (61%), followed by data on heart failure severity (54%) and their performed physical activity (48%). Patients showed very little interest in ECG tracing (37%), but the lowest interest was expressed towards healthy lifestyle promotion advice (<40%). A higher education degree and the presence of the caregiver positively affected the interest towards remote monitoring information ( P < 0.001).
    CONCLUSIONS: The patients' interests were mainly directed at receiving information related to technical data of the implantable cardiac device and not to the overall management of the disease, underlying the insufficient awareness of patients towards the key role of self-control health status and the promotion of a healthy lifestyle.
    Keywords:  cardiac implantable electronic devices; heart failure; mHealth; remote monitoring
    DOI:  https://doi.org/10.1177/2047487319830531
  4. J Cardiovasc Dev Dis. 2019 Feb 23. pii: E10. [Epub ahead of print]6(1):
    Grossfeld P, Nie S, Lin L, Wang L, Anderson RH.
      Hypoplastic left heart syndrome occurs in up to 3% of all infants born with congenital heart disease and is a leading cause of death in this population. Although there is strong evidence for a genetic component, a specific genetic cause is only known in a small subset of patients, consistent with a multifactorial etiology for the syndrome. There is controversy surrounding the mechanisms underlying the syndrome, which is likely due, in part, to the phenotypic variability of the disease. The most commonly held view is that the "decreased" growth of the left ventricle is due to a decreased flow during a critical period of ventricular development. Research has also been hindered by what has been, up until now, a lack of genetically engineered animal models that faithfully reproduce the human disease. There is a growing body of evidence, nonetheless, indicating that the hypoplasia of the left ventricle is due to a primary defect in ventricular development. In this review, we discuss the evidence demonstrating that, at least for a subset of cases, the chamber hypoplasia is the consequence of hyperplasia of the contained cardiomyocytes. In this regard, hypoplastic left heart syndrome could be viewed as a neonatal form of cardiomyopathy. We also discuss the role of the endocardium in the development of the ventricular hypoplasia, which may provide a mechanistic basis for how impaired flow to the developing ventricle leads to the anatomical changes seen in the syndrome.
    Keywords:  Jacobsen syndrome; cardiac myocyte; endocardium; hyperplasia; hypoplastic left heart syndrome; neural crest cell
    DOI:  https://doi.org/10.3390/jcdd6010010
  5. Semin Pediatr Surg. 2019 Feb;pii: S1055-8586(19)30003-4. [Epub ahead of print]28(1): 11-17
    Pilkington M, Egan JC.
      More children with congenital heart disease are surviving and require noncardiac surgery. A high-yield summary of congenital heart anatomy and pathophysiology is presented to contextualize these patients for surgeons. Preoperative planning including risk stratification, anesthetic management and timing of elective surgery are discussed. Specific intraoperative considerations for particular cases, such as the use of laparoscopy and thoracic surgery, are reviewed. Postoperative outcomes and recommended management required to mitigate complications are summarized.
    Keywords:  Congenital heart disease; Noncardiac surgery; Pediatric
    DOI:  https://doi.org/10.1053/j.sempedsurg.2019.01.003
  6. Children (Basel). 2019 Feb 25. pii: E34. [Epub ahead of print]6(2):
    Saxena A.
      About 1.35 million babies are born with congenital heart disease each year globally. Most of these are expected to lead normal, productive lives if they are treated in time. However, 90% of babies born with congenital heart disease live in regions where medical care is inadequate or unavailable. The privilege of early diagnosis and timely intervention is restricted to only those born in developed countries. Added to the burden of congenital heart disease is rheumatic heart disease, which remains a global health problem in many low-income and middle-income countries. Providing optimal care for all these children is a daunting task, and requires funds and proper planning at various levels of the health care system. This article describes the burden of pediatric heart disease, including lacunae in the current state, as well as challenges and opportunities for providing optimal care to this large population of children.
    Keywords:  congenital heart disease; developing countries; rheumatic heart disease
    DOI:  https://doi.org/10.3390/children6020034
  7. Cardiol Young. 2019 Feb 26. 1-10
    Bates KE, Yu S, Mangeot C, Shea JA, Brown DW, Uzark K.
      IntroductionTo identify interstage best practices associated with lower mortality, we studied National Pediatric Cardiology Quality Improvement Collaborative centres registry using a positive deviance approach.METHODS: Positive deviant and control centre team members were interviewed to identify potential interstage best practices. Subsequently, all collaborative centres were surveyed on the use of these practices to test their associations with centre mortality. Questionnaires were scored using Likert scales; the overall score was linearly transformed to a 0-100-point scale with higher scores indicating increased use of practices. Mortality was based on patients enrolled after a centre's first year in the collaborative. Centre mortality rates were divided into tertiles. Survey scores for the low mortality tertile were compared with the other tertiles.
    RESULTS: For this study, seven positive deviant and four control teams were interviewed. A total of 20 potential best practices were identified, including team composition, improvement practices, and parent involvement. Questionnaires were completed by 36/43 eligible centres, providing 1504 patients for analysis. Average survey score was 50.2 (SD 13.4). Average mortality was 6.1% (SD 4.1). There was no correlation between survey scores and mortality (r=0.14, p=0.41). The one practice associated with the low mortality tertile was frequency of discussion of interstage results: 58.3% of low mortality teams discussed results at least monthly versus 8.4% of the middle and high tertile centres (p=0.02).
    CONCLUSIONS: Low-mortality centres more frequently discuss interstage results than high-mortality centres. Heightened awareness of outcomes may influence practice; however, further study is needed to understand the variation in outcomes across centres.
    Keywords:  CHD; Hypoplastic low heart syndrome; Norwood operation
    DOI:  https://doi.org/10.1017/S1047951118002548
  8. Eur J Cardiovasc Nurs. 2019 Feb 26. 1474515119834484
    Van Bulck L, Luyckx K, Goossens E, Apers S, Kovacs AH, Thomet C, Budts W, Sluman MA, Eriksen K, Dellborg M, Berghammer M, Johansson B, Caruana M, Soufi A, Callus E, Moons P.
      BACKGROUND:: Inter-country variation in patient-reported outcomes of adults with congenital heart disease has been observed. Country-specific characteristics may play a role. A previous study found an association between healthcare system performance and patient-reported outcomes. However, it remains unknown which specific components of the countries' healthcare system performance are of importance for patient-reported outcomes.AIMS:: The aim of this study was to investigate the relationship between components of healthcare system performance and patient-reported outcomes in a large sample of adults with congenital heart disease.
    METHODS:: A total of 1591 adults with congenital heart disease (median age 34 years; 51% men; 32% simple, 48% moderate and 20% complex defects) from eight European countries were included in this cross-sectional study. The following patient-reported outcomes were measured: perceived physical and mental health, psychological distress, health behaviours and quality of life. The Euro Health Consumer Index 2015 and the Euro Heart Index 2016 were used as measures of healthcare system performance. General linear mixed models were conducted, adjusting for patient-specific variables and unmeasured country differences.
    RESULTS:: Health risk behaviours were associated with the Euro Health Consumer Index subdomains about patient rights and information, health outcomes and financing and access to pharmaceuticals. Perceived physical health was associated with the Euro Health Consumer Index subdomain about prevention of chronic diseases. Subscales of the Euro Heart Index were not associated with patient-reported outcomes.
    CONCLUSION:: Several features of healthcare system performance are associated with perceived physical health and health risk behaviour in adults with congenital heart disease. Before recommendations for policy-makers and clinicians can be conducted, future research ought to investigate the impact of the healthcare system performance on outcomes further.
    Keywords:  Healthcare system performance; congenital; health services accessibility; heart defect; patient reported outcome measures
    DOI:  https://doi.org/10.1177/1474515119834484
  9. Congenit Heart Dis. 2019 Mar 01.
    Marckini DN, Samuel BP, Parker JL, Cook SC.
      BACKGROUND: Physician burnout has many undesirable consequences, including negative impact on patient care delivery and physician career satisfaction. Electronic health records (EHRs) may exacerbate burnout by increasing physician workload.OBJECTIVE: To determine burnout in adult congenital heart disease (ACHD) specialists by assessing stress associated with EHRs.
    DESIGN: Electronic survey study of ACHD providers.
    SETTING: Canada and United States.
    PARTICIPANTS: Three hundred eighty-three ACHD specialists listed on the Adult Congenital Heart Association directory between February and April 2017.
    OUTCOME MEASURES: Burnout was measured using the Maslach Burnout Inventory (MBI) to understand factors contributing to work life and EHR satisfaction. Chi-square and Wilcoxon Rank Sum tests were used for statistical analysis.
    RESULTS: Of the 383 invited participants, 110 (28.7%) completed surveys with the majority (n = 88, 80.7%) reporting from an academic medical center. Burnout was defined as high scores on the emotional exhaustion and/or depersonalization MBI subscales. When comparing the 40% (n = 44) that met criteria for burnout with those that did not, there was strong disagreement that a reasonable amount of time is spent on clerical tasks related to direct (P = .0043) or indirect (P = .0004) patient care. There was strong disagreement that EHRs increased efficiency (P = .006) or the patient portal improved patient care (P = .0215). Finally, physicians who met criteria for burnout had lower personal accomplishment scores (P = .0355).
    CONCLUSIONS: Our results suggest time spent on EHRs creates clerical burden exacerbating ACHD physician burnout. The high levels of emotional exhaustion may decrease quality of ACHD care by directing focus away from physician-patient interaction. Health care systems must develop best practice for EHR design and implementation to optimize patient advocacy and care, and decrease physician burnout.
    Keywords:  adult congenital heart disease; electronic health record; patient care; physician burnout; stress; workload
    DOI:  https://doi.org/10.1111/chd.12745
  10. Circulation. 2019 Feb 28.
    Saha P, Potiny P, Rigdon J, Morello M, Tcheandjieu C, Romfh A, Fernandes SM, McElhinney DB, Bernstein D, Lui GK, Shaw GM, Ingelsson E, Priest JR.
      BACKGROUND: Although lower-complexity cardiac malformations constitute the majority of adult congenital heart disease (ACHD), the long-term risks of adverse cardiovascular events and relationship with conventional risk factors in this population are poorly understood. We aimed to quantify the risk of adverse cardiovascular events associated with lower-complexity ACHD that is unmeasured by conventional risk factors.METHODS: A multi-tiered classification algorithm was used to select individuals with lower-complexity ACHD and individuals without ACHD for comparison amongst >500,000 British adults in the UK Biobank (UKB). ACHD diagnoses were sub-classified as "isolated aortic valve (AoV)" and "non-complex" defects. Time-to-event analyses were conducted for primary endpoints of fatal or non-fatal acute coronary syndrome (ACS), ischemic stroke, heart failure (HF), and atrial fibrillation, and a secondary combined endpoint for major adverse cardiovascular event (MACE). Maximum follow-up time for the study period was 22 years using retrospectively and prospectively collected data from the UKB.
    RESULTS: We identified 2,006 individuals with lower-complexity ACHD and 497,983 unexposed individuals in the UKB (median [IQR] age at enrollment 58 [51,63]). Of the ACHD-exposed group, 59% were male; 51% were current or former smokers; 30% were obese; 69%, 41%, and 7% were diagnosed or treated for hypertension, hyperlipidemia, and diabetes respectively. After adjustment for 12 measured cardiovascular risk factors, ACHD remained strongly associated with the primary endpoints, with hazard ratios (HR) ranging from 2.0 (95% confidence interval [CI] 1.5-2.8, p<0.001) for ACS to 13.0 (95% CI 9.4-18.1, p<0.001) for HF. ACHD-exposed individuals with ≤2 cardiovascular risk factors had a 29% age-adjusted incidence rate of MACE in contrast to 13% in non-ACHD individuals with ≥5 risk factors.
    CONCLUSIONS: Individuals with lower-complexity ACHD had higher burden of adverse cardiovascular events relative to the general population that was unaccounted for by conventional cardiovascular risk factors. These findings highlight the need for closer surveillance of patients with mild to moderate ACHD and further investigation into management and mechanisms of cardiovascular risk unique to this growing population of high-risk adults.
    DOI:  https://doi.org/10.1161/CIRCULATIONAHA.118.037064
  11. J Am Heart Assoc. 2019 Mar 05. 8(5): e009893
    Kelly CJ, Christiaens D, Batalle D, Makropoulos A, Cordero-Grande L, Steinweg JK, O'Muircheartaigh J, Khan H, Lee G, Victor S, Alexander DC, Zhang H, Simpson J, Hajnal JV, Edwards AD, Rutherford MA, Counsell SJ.
      Background Abnormal macrostructural development of the cerebral cortex has been associated with hypoxia in infants with congenital heart disease ( CHD ). Animal studies have suggested that hypoxia results in cortical dysmaturation at the cellular level. New magnetic resonance imaging techniques offer the potential to investigate the relationship between cerebral oxygen delivery and cortical microstructural development in newborn infants with CHD . Methods and Results We measured cortical macrostructural and microstructural properties in 48 newborn infants with serious or critical CHD and 48 age-matched healthy controls. Cortical volume and gyrification index were calculated from high-resolution structural magnetic resonance imaging. Neurite density and orientation dispersion indices were modeled using high-angular-resolution diffusion magnetic resonance imaging. Cerebral oxygen delivery was estimated in infants with CHD using phase contrast magnetic resonance imaging and preductal pulse oximetry. We used gray matter-based spatial statistics to examine voxel-wise group differences in cortical microstructure. Microstructural development of the cortex was abnormal in 48 infants with CHD , with regions of increased fractional anisotropy and reduced orientation dispersion index compared with 48 healthy controls, correcting for gestational age at birth and scan (family-wise error corrected for multiple comparisons at P<0.05). Regions of reduced cortical orientation dispersion index in infants with CHD were related to impaired cerebral oxygen delivery ( R2=0.637; n=39). Cortical orientation dispersion index was associated with the gyrification index ( R2=0.589; P<0.001; n=48). Conclusions This study suggests that the primary component of cerebral cortex dysmaturation in CHD is impaired dendritic arborization, which may underlie abnormal macrostructural findings reported in this population, and that the degree of impairment is related to reduced cerebral oxygen delivery.
    Keywords:  brain imaging; cerebral blood flow; congenital heart disease; development; magnetic resonance imaging
    DOI:  https://doi.org/10.1161/JAHA.118.009893