bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2019–05–05
twelve papers selected by
Richard James, University of Pennsylvania
  1. Perceived Health Mediates Effects of Physical Activity on Quality of Life in Patients With a Fontan Circulation.
  2. Prediction of heart failure and death in an adult population of Fontan patients.
  3. Long-term health-related burden of adult congenital heart diseases in Hong Kong.
  4. Adverse Delivery Events in Pregnant Women With Congenital Heart Defects: Wish You an Easy Delivery.
  5. Too little too late? Communication with patients with congenital heart disease about challenges of adult life.
  6. Maternal Comorbidities and Complications of Delivery in Pregnant Women With Congenital Heart Disease.
  7. Pediatric palliative care in the medical neighborhood for children with medical complexity.
  8. Real-world healthcare utilization in adult congenital heart disease: a systematic review of trends and ratios.
  9. RACHS - ANZ : A Modified Risk Adjustment in Congenital Heart Surgery Model for Outcome Surveillance in Australia and New Zealand.
  10. Surgery of single ventricles in humanitarian practice: surgery for which patients?
  11. 3D bioprinting of vascular conduits for pediatric congenital heart repairs.
  12. Surveillance of Congenital Heart Defects among Adolescents at Three U.S. Sites.
  1. Am J Cardiol. 2019 Apr 10. pii: S0002-9149(19)30398-4. [Epub ahead of print]
    Perceived Health Mediates Effects of Physical Activity on Quality of Life in Patients With a Fontan Circulation.
    APPROACH-IS consortium and the International Society for Adult Congenital Heart Disease (ISACHD)
      Patients with a Fontan circulation are at risk of a sedentary lifestyle. Given the direct relationship between physical activity and health, promotion of physical activity has the potential to improve outcomes, including quality of life (QOL). This study aimed to describe self-reported physical activity levels in adult Fontan patients and examine associations between physical activity, perceived health status and QOL. The sample consisted of 177 Fontan patients (Mage = 27.5 ± 7.6 years, 52% male) who reported their physical activity, perceived health status, and QOL as part of the cross-sectional Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease - International Study. Descriptive statistics and univariate analyses of variance with planned contrasts were computed to describe physical activity characteristics. Mediation analyses tested whether perceived health status variables mediated the association between physical activity and QOL. Forty-six percent of patients were sedentary while only 40% met international physical activity guidelines. Higher physical activity was associated with younger age, lower NYHA class, higher perceived general health, and greater QOL. Patients who commuted by walking and engaged in sports reported better perceived health and QOL. Mediation analyses revealed that perceived general health but not NYHA functional class mediated the association between physical activity and QOL (αβ = 0.22, 95% confidence interval = 0.04 to 0.49). In conclusion, Fontan patients likely benefit from regular physical activity, having both higher perceived general health and functional capacity; greater perceived health status may contribute to enhanced QOL. In conclusion, these data support the pivotal role of regular physical activity for Fontan patients.
    DOI:  https://doi.org/10.1016/j.amjcard.2019.03.039
  2. Cardiol Young. 2019 Apr 30. 1-8
    Prediction of heart failure and death in an adult population of Fontan patients.
    Jan Thorben Sieweke, Arash Haghikia, Christian Riehle, Carolin Klages, Muharrem Akin, Tobias König, Carolin Zwadlo, Jens Treptau, Andreas Schäfer, Johann Bauersachs, Mechthild Westhoff-Bleck.
       BACKGROUND: Late Fontan survivors are at high risk to experience heart failure and death. Therefore, the current study sought to investigate the role of non-invasive diagnostics as prognostic markers for failure of the systemic ventricle following Fontan procedure.
    METHODS: This monocentric, longitudinal observational study included 60 patients with a median age of 24.5 (19-29) years, who were subjected to cardiac magnetic resonance imaging, echocardiography, cardiopulmonary exercise testing, and blood analysis. The primary endpoint of this study was decompensated heart failure with symptoms at rest, peripheral and/or pulmonary edema, and/or death.
    RESULTS: During a follow-up of 24 months, 5 patients died and 5 patients suffered from decompensated heart failure. Clinical (NYHA class, initial surgery), functional (VO2 peak, ejection fraction, cardiac index), circulating biomarkers (N-terminal pro brain natriuretic peptide), and imaging parameters (end diastolic volume index, end systolic volume index, mass-index, contractility, afterload) were significantly related to the primary endpoint. Multi-variate regression analysis identified afterload as assessed by cardiac magnetic resonance imaging as an independent predictor of the primary endpoint (hazard ratio 1.98, 95% confidence interval 1.19-3.29, p = 0.009).
    CONCLUSION: We identified distinct parameters of cardiopulmonary exercise testing, cardiac magnetic resonance imaging, and blood testing as markers for future decompensated heart failure and death in patients with Fontan circulation. Importantly, our data also identify increased afterload as an independent predictor for increased morbidity and mortality. This parameter is easy to assess by non-invasive cardiac magnetic resonance imaging. Its modulation may represent a potential therapeutic approach target in these high-risk patients.
    Keywords:  Fontan; cardiac magnetic resonance imaging; congenital heart disease; heart failure; univentricular heart
    DOI:  https://doi.org/10.1017/S1047951119000258
  3. J Med Econ. 2019 Apr 30. 1
    Long-term health-related burden of adult congenital heart diseases in Hong Kong.
    Vivian Wy Lee, Tiffany Mc Fong, Anita Kp Fung, Franco Wt Cheng.
       AIMS: This study aimed to examine long term clinical and economic and of adults with congenital heart disease (ACHD) in Hong Kong.
    METHODS: We retrospectively analyzed 336 consecutive ACHD patients who attended the Adult Congenital Heart Clinic between 1st Jan 2009 and 31st December 2014. Direct medical costs and clinical outcomes over 5 preceding years were calculated and documented. The economic evaluation was from the hospital's perspective.
    RESULTS: The median age of ACHD patients was 47 (31 - 62) years old with female predominance (61.5%). Ventricular and atrial septal defect accounted for 70% and severe ACHD 10% of the study cohort. The prevalence of arrhythmia and heart failure increased with the complexity of CHD. The total mean annual cost for managing each ACHD patients was USD 2,913. The annual cost of management of simple ACHD was USD 2,638 versus complex ACHD was USD 6,425 (p = 0.013).
    CONCLUSIONS: Our study demonstrated severe ACHD patients accounted for higher cardiovascular morbidities in arrhythmias and heart failure with higher cost of management.
    Keywords:  I10; I19; congenital heart disease; healthcare burden; long-term outcomes
    DOI:  https://doi.org/10.1080/13696998.2019.1613239
  4. J Am Coll Cardiol. 2019 May 07. pii: S0735-1097(19)33872-0. [Epub ahead of print]73(17): 2192-2194
    Adverse Delivery Events in Pregnant Women With Congenital Heart Defects: Wish You an Easy Delivery.
    Ami B Bhatt.
      
    Keywords:  congenital heart disease; pregnancy
    DOI:  https://doi.org/10.1016/j.jacc.2019.02.038
  5. Congenit Heart Dis. 2019 May 04.
    Too little too late? Communication with patients with congenital heart disease about challenges of adult life.
    Lisa X Deng, Lacey P Gleason, Katherine Awh, Abigail May Khan, David Drajpuch, Stephanie Fuller, Leah A Goldberg, Christopher E Mascio, Sara L Partington, Lynda Tobin, Adrienne H Kovacs, Yuli Y Kim.
       OBJECTIVE: To investigate the experiences and communication preferences of adult patients with congenital heart disease (CHD) in the domains of employment, insurance, and family planning.
    DESIGN: Patients ≥ 18 years of age completed a questionnaire about experiences and communication preferences regarding employment, health insurance, and family planning.
    RESULTS: Of 152 patients (median age = 33 years, 50% female, 35% with CHD of great complexity), one in four reported work-related problems due to CHD and a quarter also recalled a previous gap in health insurance. Of females, 29% experienced an unplanned pregnancy. The median importance of discussion ratings (on a 0-10 scale) were 3.5 (employment), 6.0 (insurance), and 8.0 (family planning). Few patients recalled discussions about employment (19%) or health insurance (20%). Over half recalled discussions about family planning, although males were less likely to have had these discussions than females (24% vs 86%, P < .001). Across the three domains, patients identified 16-18 years as the most appropriate age to initiate discussion, although for patients who recalled discussions, they typically occurred between 20 and 25 years.
    CONCLUSIONS: Adults with CHD commonly face employment, health insurance, and family planning challenges. However, discussions about these matters occur with less frequency than recommended and at older ages than patients would prefer. Communication about such issues should be incorporated into a comprehensive educational curriculum for adolescents during the process of transition to adult care.
    Keywords:  adult congenital heart disease; communication; employment; family planning; health insurance
    DOI:  https://doi.org/10.1111/chd.12778
  6. J Am Coll Cardiol. 2019 May 07. pii: S0735-1097(19)33871-9. [Epub ahead of print]73(17): 2181-2191
    Maternal Comorbidities and Complications of Delivery in Pregnant Women With Congenital Heart Disease.
    Lauren E Schlichting, Tabassum Z Insaf, Ali N Zaidi, George K Lui, Alissa R Van Zutphen.
       BACKGROUND: Pregnant women with congenital heart defects (CHDs) may be at increased risk for adverse events during delivery.
    OBJECTIVES: This study sought to compare comorbidities and adverse cardiovascular, obstetric, and fetal events during delivery between pregnant women with and without CHDs in the United States.
    METHODS: Comorbidities and adverse delivery events in women with and without CHDs were compared in 22,881,691 deliveries identified in the 2008 to 2013 National Inpatient Sample using multivariable logistic regression. Among those with CHDs, associations by CHD severity and presence of pulmonary hypertension (PH) were examined.
    RESULTS: There were 17,729 deliveries to women with CHDs (77.5 of 100,000 deliveries). These women had longer lengths of stay and higher total charges than women without CHDs. They had greater odds of comorbidities, including PH (adjusted odds ratio [aOR]: 193.8; 95% confidence interval [CI]: 157.7 to 238.0), congestive heart failure (aOR: 49.1; 95% CI: 37.4 to 64.3), and coronary artery disease (aOR: 31.7; 95% CI: 21.4 to 47.0). Greater odds of adverse events were observed, including heart failure (aOR: 22.6; 95% CI: 20.5 to 37.3), arrhythmias (aOR: 12.4; 95% CI: 11.0 to 14.0), thromboembolic events (aOR: 2.4; 95% CI: 2.0 to 2.9), pre-eclampsia (aOR: 1.5; 95% CI: 1.3 to 1.7), and placenta previa (aOR: 1.5; 95% CI: 1.2 to 1.8). Cesarean section, induction, and operative vaginal delivery were more common, whereas fetal distress was less common. Among adverse events in women with CHDs, PH was associated with heart failure, hypertension in pregnancy, pre-eclampsia, and pre-term delivery; there were no differences in most adverse events by CHD severity.
    CONCLUSIONS: Pregnant women with CHDs were more likely to have comorbidities and experience adverse events during delivery. These women require additional monitoring and care.
    Keywords:  adverse events; congenital heart defects; pregnancy; pulmonary hypertension
    DOI:  https://doi.org/10.1016/j.jacc.2019.01.069
  7. Fam Syst Health. 2019 May 02.
    Pediatric palliative care in the medical neighborhood for children with medical complexity.
    Justin A Yu, Yael Schenker, Scott H Maurer, Stacey C Cook, Dio Kavlieratos, Amy Houtrow.
      Children with medical complexity (CMC) are a medically fragile pediatric population that experience severe chronic illnesses resulting in significant health care needs, functional limitations, and health care utilization, and are at the highest risk for morbidity and mortality among all children. Furthermore, families and parents of CMC experience significant caregiver hardships and diminished quality of life. The field of pediatric palliative care has grown in recent years, in part to address the physical and psychosocial issues inherent to the care of these chronically ill children. However, as the prevalence and long-term survival of CMC increases with medical advancements, the demand for pediatric palliative care will likely exceed the capacity of current and future pediatric palliative care specialists. Therefore, alternative strategies to ensure access to essential aspects of palliative care must be considered. This article focuses on why and how high-quality palliative care should be integrated into the patient- and family-centered medical home, the ideal care delivery model for CMC and their families. We first discuss how palliative care principles naturally align with and complement the goals of the CMC medical home. Next, we detail what actions pediatric palliative care specialists can take to best support the CMC medical home as "medical neighbors." Lastly, we describe the fundamental aspects of pediatric palliative care that all clinicians caring for CMC should be able to provide, referred to as "primary pediatric palliative care." (PsycINFO Database Record (c) 2019 APA, all rights reserved).
    DOI:  https://doi.org/10.1037/fsh0000414
  8. Cardiol Young. 2019 May 03. 1-11
    Real-world healthcare utilization in adult congenital heart disease: a systematic review of trends and ratios.
    Ruben Willems, Amber Werbrouck, Julie De Backer, Lieven Annemans.
       BACKGROUND: As a result of medical advances, the adult congenital heart disease population is rapidly expanding. Nonetheless, most patients remain prone to increased morbidity and mortality. Therefore, long-term medical resource use is required. This systematic review aims to present the trends over the past decades of medical resource utilization in adult congenital heart disease as well as its current status, with a focus on hospitalizations, emergency department visits, outpatient cardiology visits, and visits to other healthcare professionals.
    METHODS: MEDLINE (Pubmed), Embase, and Web of Science were searched for retrospective database research publications. The ISPOR checklist for retrospective database research was used for quality appraisal. Trends over time are explored.
    RESULTS: Twenty-one articles met the inclusion criteria. All but one of the studies was conducted in Western Europe and North America. The absolute number of hospitalizations has been increasing over the last several decades. This increase is highest in patients with mild lesions, although these numbers are largely driven by hospitalizations of patients with an atrial septal defect or a patent foramen ovale. Meanwhile, outpatient cardiology visits are increasing at an even higher pace, and occur most often in geriatric patients and patients with severe lesions. Conversely, the number of hospitalizations per 100 patients is decreasing over time. Literature is scarce on other types of healthcare use.
    CONCLUSION: A strong rise in healthcare utilization is noticed, despite the mitigating effect of improved efficiency levels. As the population continues to grow, innovative medical management strategies will be required to accommodate its increasing healthcare utilization.
    Keywords:  Adult congenital heart disease; emergency department; general practitioner; healthcare utilization; hospitalization
    DOI:  https://doi.org/10.1017/S1047951119000441
  9. J Am Heart Assoc. 2019 May 07. 8(9): e011390
    RACHS - ANZ : A Modified Risk Adjustment in Congenital Heart Surgery Model for Outcome Surveillance in Australia and New Zealand.
    Australian and New Zealand Intensive Care Society Paediatric Study Group and Centre for Outcomes and Resource Evaluation
      Background Outcomes for pediatric cardiac surgery are commonly reported from international databases compiled from voluntary data submissions. Surgical outcomes for all children in a country or region are less commonly reported. We aimed to describe the bi-national population-based outcome for children undergoing cardiac surgery in Australia and New Zealand and determine whether the Risk Adjustment for Congenital Heart Surgery ( RACHS ) classification could be used to create a model that accurately predicts in-hospital mortality in this population. Methods and Results The study was conducted in all children's hospitals performing cardiac surgery in Australia and New Zealand between January 2007 and December 2015. The performance of the original RACHS -1 model was assessed and compared with an alternative RACHS - ANZ (Australia and New Zealand) model, developed balancing discrimination with parsimonious variable selection. A total of 14 324 hospital admissions were analyzed. The overall hospital mortality was 2.3%, ranging from 0.5% for RACHS category 1 procedures, to 17.0% for RACHS category 5 or 6 procedures. The original RACHS -1 model was poorly calibrated with death overpredicted (1161 deaths predicted, 289 deaths observed). The RACHS - ANZ model had better performance in this population with excellent discrimination (Az- ROC of 0.830) and acceptable Hosmer and Lemeshow goodness-of-fit ( P=0.216). Conclusions The original RACHS -1 model overpredicts mortality in children undergoing heart surgery in the current era. The RACHS - ANZ model requires only 3 risk variables in addition to the RACHS procedure category, can be applied to a wider range of patients than RACHS -1, and is suitable to use to monitor regional pediatric cardiac surgery outcomes.
    Keywords:  cardiac surgery; congenital heart disease; outcome and process assessment; pediatric; risk model
    DOI:  https://doi.org/10.1161/JAHA.118.011390
  10. Cardiol Young. 2019 May 03. 1-6
    Surgery of single ventricles in humanitarian practice: surgery for which patients?
    Marielle Gouton, Olivier Michel Bical, Joy Zoghbi, Régine Roussin, Vincent Lucet, Francine Leca.
       OBJECTIVES: To analyse the feasibility and effectiveness in humanitarian practice of surgical management of children with single-ventricle heart condition.
    METHODS: Retrospective study of children with a single ventricle, managed by the association Mécénat-Chirurgie Cardiaque since 1996, with long-term follow-up after their return home.
    RESULTS: Of the 138 children in our cohort, 119 had one or more surgeries (180 procedures): palliative surgery alone (systemic-pulmonary anastomosis or banding), 41; partial cavo-pulmonary connection, 47; total cavo-pulmonary connection (mean age 8.5 years), 31. Operative mortality is 5.5%. After a mean follow-up of 5.6 years, 18 children (13%) were lost to follow-up. Survival at 10 years is 79% in children receiving surgery (palliative only, 72%; partial cavo-pulmonary connection, 77%; total cavo-pulmonary connection, 97%) versus 29% in children with no surgical intervention. The prognosis is better for tricuspid atresia and double-inlet left ventricle (86 and 83% survival at 10 years) than for double-outlet right ventricle or complete atrio-ventricular canal defect (64 and 68% at 5 years).
    CONCLUSION: The surgery of the single ventricle in humanitarian medicine allows a very satisfactory survival after one or more surgeries tending towards a total cavo-pulmonary connection as soon as possible.
    Keywords:  CHD; Fontan; single ventricle; tricuspid atresia
    DOI:  https://doi.org/10.1017/S1047951119000192
  11. Transl Res. 2019 Apr 11. pii: S1931-5244(19)30059-3. [Epub ahead of print]
    3D bioprinting of vascular conduits for pediatric congenital heart repairs.
    Wenhan Lee, Yi Hong, Guohao Dai.
      In children with congenital heart defects, surgical correction often involves the use of valves, patches or vascular conduits to establish anatomic continuity. Due to the differences between the pediatric and adult populations, tissue reconstruction in pediatric patients requires a substantially different approach from those in adults. Cardiovascular anatomy of children with congenital heart defect vary, which requires tailored surgical operations for each patient. Since grafts used in these palliative surgeries are sensitive to the local hemodynamic environments, their geometries need to be precisely designed to ensure long-term performance. Tissue engineered vascular grafts (TEVGs) have made tremendous progress over the past decade, but it remains difficult to fabricate patient- and operation-specific vascular grafts. This review summarizes historical milestones of TEVG development for repairing pediatric congenital defects and current clinical outcomes. We also highlight ongoing works on 3D bioprinting of TEVGs with complex geometries and address the current limitations of each technique. Although 3D bioprinted vascular grafts with appropriate functions are yet to be developed, some of the current researches are promising to create better patient specific tissue engineered vascular grafts in the future.
    DOI:  https://doi.org/10.1016/j.trsl.2019.03.007
  12. Am J Cardiol. 2019 Apr 10. pii: S0002-9149(19)30409-6. [Epub ahead of print]
    Surveillance of Congenital Heart Defects among Adolescents at Three U.S. Sites.
    George K Lui, Claire McGarry, Ami Bhatt, Wendy Book, Tiffany J Riehle-Colarusso, Julie E Dunn, Jill Glidewell, Michelle Gurvitz, Trenton Hoffman, Carol J Hogue, Daphne Hsu, Stan Obenhaus, Cheryl Raskind-Hood, Fred H Rodriguez, Ali Zaidi, Alissa R Van Zutphen.
      The prevalence, co-morbidities, and healthcare utilization in adolescents with congenital heart defects (CHDs) is not well understood. Adolescents (11 to 19 years old) with a healthcare encounter between January 1, 2008 (January 1, 2009 for MA) and December 31, 2010 with a CHD diagnosis code were identified from multiple administrative data sources compiled at 3 US sites: Emory University, Atlanta, Georgia (EU); Massachusetts Department of Public Health (MA); and New York State Department of Health (NY). The estimated prevalence for any CHD was 4.77 (EU), 17.29 (MA), and 4.22 (NY) and for severe CHDs was 1.34 (EU), 3.04 (MA), and 0.88 (NY) per 1,000 adolescents. Private or commercial insurance was the most common insurance type for EU and NY, and Medicaid for MA. Inpatient encounters were more frequent in severe CHDs. Cardiac co-morbidities included rhythm and conduction disorders at 20% (EU), 46% (MA), and 9% (NY) as well as heart failure at 3% (EU), 15% (MA), and 2% (NY). Leading noncardiac co-morbidities were respiratory/pulmonary (22% EU, 34% MA, 16% NY), infectious disease (17% EU, 22% MA, 20% NY), non-CHD birth defects (12% EU, 23% MA, 14% NY), gastrointestinal (10% EU, 28% MA, 13% NY), musculoskeletal (10% EU, 32% MA, 11% NY), and mental health (9% EU, 30% MA, 11% NY). In conclusion, this study used a novel approach of uniform CHD definition and variable selection across administrative data sources in 3 sites for the first population-based CHD surveillance of adolescents in the United States. High resource utilization and co-morbidities illustrate ongoing significant burden of disease in this vulnerable population.
    DOI:  https://doi.org/10.1016/j.amjcard.2019.03.044
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