bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2019‒06‒02
nine papers selected by
Richard James
University of Pennsylvania


  1. J Am Heart Assoc. 2019 Jun 04. 8(11): e011284
    Zielonka B, Snarr BS, Liu MY, Zhang X, Mascio CE, Fuller S, Gaynor JW, Spray TL, Rychik J.
      Background Healthcare resource utilization is substantial for single-ventricle cardiac defects ( SVCD ), with effort commencing at time of fetal diagnosis through staged surgical palliation. We sought to characterize and identify variables that influence resource utilization for SVCD from fetal diagnosis through death, completed staged palliation, or cardiac transplant. Methods and Results Patients with a prenatal diagnosis of SVCD at our institution from 2004 to 2011 were screened. Patients delivered with intent to treat who received cardiac care exclusively at our institution were included. Primary end points included the total days hospitalized and the numbers of echocardiograms and cardiac catheterizations. Subanalysis was performed on survivors of completed staged palliation on the basis of Norwood operation, dominant ventricular morphology, and additional risk factors. Of 202 patients born with intent to treat, 136 patients survived to 6 months after completed staged palliation. The median number of days hospitalized per patient-year was 25.1 days, and the median numbers of echocardiograms and catheterizations per patient-year were 7.2 and 0.7, respectively. Mortality is associated with increased resource utilization. Survivors had a cumulative length of stay of 57 days and underwent a median of 21 echocardiograms and 2 catheterizations through staged palliation. Right-ventricle-dominant lesions requiring Norwood operation are associated with increased resource utilization among survivors of staged palliation. Conclusions For fetuses with SVCD , those with dominant right-ventricular morphology requiring Norwood operation demand increased resource utilization regardless of mortality. Our findings provide insight into care for SVCD , facilitate precise prenatal counseling, and provide information about the resources utilized to successfully manage SVCD .
    Keywords:  congenital heart disease; fetal echocardiography; hypoplastic left heart syndrome; resource utilization; single ventricle
    DOI:  https://doi.org/10.1161/JAHA.118.011284
  2. Cardiol Young. 2019 May 28. 1-8
    Furlong-Dillard JM, Miller BJ, Sward KA, Neary AI, Hardin-Reynolds TL, Jeffers G, Clay BA, Truong DT, Miller TA, Jones CE, Lambert LM, Bailly DK.
      BACKGROUND: Children with congenital heart disease are at high risk for malnutrition. Standardisation of feeding protocols has shown promise in decreasing some of this risk. With little standardisation between institutions' feeding protocols and no understanding of protocol adherence, it is important to analyse the efficacy of individual aspects of the protocols.METHODS: Adherence to and deviation from a feeding protocol in high-risk congenital heart disease patients between December 2015 and March 2017 were analysed. Associations between adherence to and deviation from the protocol and clinical outcomes were also assessed. The primary outcome was change in weight-for-age z score between time intervals.
    RESULTS: Increased adherence to and decreased deviation from individual instructions of a feeding protocol improves patients change in weight-for-age z score between birth and hospital discharge (p = 0.031). Secondary outcomes such as markers of clinical severity and nutritional delivery were not statistically different between groups with high or low adherence or deviation rates.
    CONCLUSIONS: High-risk feeding protocol adherence and fewer deviations are associated with weight gain independent of their influence on nutritional delivery and caloric intake. Future studies assessing the efficacy of feeding protocols should include the measures of adherence and deviations that are not merely limited to caloric delivery and illness severity.
    Keywords:  Feeding; congenital heart disease; protocol; weight-for-age z score
    DOI:  https://doi.org/10.1017/S1047951119000222
  3. Cardiol Young. 2019 May 30. 1-7
    Bates KE, Glatz AC, Giglia TM, Natarajan SS, Ravishankar C, Stagg A, Rome JJ.
      INTRODUCTION: Interstage mortality causes are often unknown in infants with shunt-dependent univentricular defects. For 2 years, screening catheterisation was encouraged before neonatal discharge to determine if routine evaluation improved interstage outcomes.METHODS: Retrospective single-centre review of home monitoring programme from December, 2010 to June, 2012. Composite scores were created for physical examination/echocardiography risk factors; catheterisation risk factors; and interstage adverse events. Composite scores were compared between usual care and screening catheterisation groups. The ability of each risk factor composite to predict interstage adverse events, individually and in combination, was assessed with sensitivity, specificity, and receiver operating characteristic curves.
    RESULTS: There were 27 usual care and 32 screening catheterisation patients. There were no significant differences between groups except rates of catheterisation before discharge (29.6 versus 100%, p < 0.001). Usual care patients who underwent catheterisation for clinical indications had higher intervention rates (37.5 versus 3.1%, p = 0.004). Physical examination/echocardiography risk factor frequency was similar, but usual care patients with catheterisation had a higher catheterisation risk factor frequency. Interstage adverse event frequency was similar (48.2 versus 53.1%, p = 0.7). For interstage adverse event prediction, sensitivity for the physical examination/echocardiography, catheterisation, and either risk factor composites was 53.3, 72, and 80%, respectively; specificity was 59, 60, and 48%. The area under the receiver operating characteristic curve was 0.56, 0.66, and 0.64.
    CONCLUSION: Screening catheterisation evaluation offered slightly increased sensitivity and specificity, but no difference in interstage adverse event frequency. Given this small advantage versus known risks, screening catheterisations are no longer encouraged.
    Keywords:  CHD; Norwood procedure; catheterization; hypoplastic left heart syndrome
    DOI:  https://doi.org/10.1017/S1047951119000696
  4. Expert Rev Gastroenterol Hepatol. 2019 May 26. 1-16
    Komatsu H, Inui A, Kishiki K, Kawai H, Yoshio S, Osawa Y, Kanto T, Fujisawa T.
      Introduction: Hepatic fibrosis and hepatocellular carcinoma (HCC) can develop in children with congenital heart disease. Although hepatic fibrosis and HCC are prone to develop after the Fontan operation, they can also develop in patients suffering from congenital heart disease who have not undergone Fontan operation. Area covered: The history of cardiac hepatopathy including Fontan-associated liver disease is described. Patient characteristics, liver histology, imaging examinations and blood tests are reviewed to elucidate the mechanism of cardiac hepatopathy. In addition, a flowchart for the follow-up management of cardiac hepatopathy in children with congenital heart disease is proposed. Expert opinion: Congestion and low cardiac output are the main causes of cardiac hepatopathy. Advanced hepatic fibrosis is presumed to be associated with HCC. HCC can develop in both adolescents and young adults. Regardless of whether the Fontan operation is performed, children with a functional single ventricle and chronic heart failure should be regularly examined for cardiac hepatopathy. There is no single reliable laboratory parameter to accurately detect cardiac hepatopathy; hepatic fibrosis indices and elastography have shown inconsistent results for detection of this disease. Further studies using liver specimen-confirmed patients and standardization of evaluation protocols are required to clarify the pathogenesis of cardiac hepatopathy.
    Keywords:  Cardiac hepatopathy; Fontan; cirrhosis; congenital heart disease; fibrosis; hepatocellular carcinoma
    DOI:  https://doi.org/10.1080/17474124.2019.1621746
  5. Int J Cardiol. 2019 May 07. pii: S0167-5273(18)36295-8. [Epub ahead of print]
    Lytzen R, Vejlstrup N, Bjerre J, Petersen OB, Leenskjold S, Dodd JK, Jørgensen FS, Søndergaard L.
      BACKGROUND: Major congenital heart diseases (CHD) often demand intervention in the neonatal period. Prenatal diagnosis may improve mortality by eliminating the diagnostic delay; however, there is controversy concerning its true effect. We aimed to evaluate the effect of general prenatal screening on prognosis by comparing a period without general prenatal screening to a period with general prenatal screening.METHODS: We conducted a nationwide retrospective study including live born children and terminated fetuses diagnosed with major CHD. Prenatal screening was recommended only in high risk pregnancies between 1996 and 2004, whereas general prenatal screening was recommended between 2005 and 2013. We assessed the influence of general prenatal screening on all-cause mortality, cardiac death, preoperative and postoperative 30-day mortality and complication rate.
    RESULTS: 1-year mortality decreased over both periods, but the decrease was greater in the screening period (Odds ratio 0.92 (CI 0.83-1.00), p = 0.047). Prenatal detection of major CHD was associated with cardiac death in the period without general screening (Hazard Ratio 2.40 (CI 1.72-3.33), p < 0.001), whereas there was no significant association once general screening was implemented. Similarly, the association between prenatal diagnosis and pre- and postoperative mortality found in the period without general screening was insignificant after the implementation of general screening.
    CONCLUSION: Mortality in major CHD decreased throughout the study, especially in the period with general prenatal screening. However, comparing a prenatally diagnosed group with a postnatally diagnosed group is vulnerable to selection bias and proper interpretation is difficult.
    Keywords:  Congenital heart disease; Fetal echocardiography; Prenatal diagnosis; Prenatal malformation screening; Prognosis
    DOI:  https://doi.org/10.1016/j.ijcard.2019.05.017
  6. Acta Paediatr. 2019 May 28.
    Moons P, Luyckx K.
      Over the past four decades, the number of papers on quality of life (QoL) in congenital heart disease (CHD) has grown exponentially. However, firm conclusions have been hard to draw, because conceptual issues occurred and methodological differences prevented direct comparisons of studies. In general, QoL in CHD patients is good and it can be even better than in healthy peers when measured as life satisfaction. When QoL is measured as physical functioning, patients with complex CHD do worse than patients with moderate or mild defects or healthy individuals. APPROACH-IS was a large-scale international project that confirmed international variations in QoL, with Australian patients reporting the best QoL. Poor QoL was predicted by older age, being a job seeker, unemployed or disabled, never having married and those with worse functional status. Perceived illness and religion and spirituality were also significant predictors for QoL. On the other hand, country-specific characteristics and healthcare system factors did not play a significant role in explaining variances in QoL of patients with CHD around the globe. Future studies should rely on a sound conceptual basis, use longitudinal research designs, be conducted in international, multi-centre settings and evaluate interventions that promote patients' QoL. This article is protected by copyright. All rights reserved.
    Keywords:  congenital heart disease; heart defects; outcomes; predictors; quality of life
    DOI:  https://doi.org/10.1111/apa.14876
  7. Circulation. 2019 May 28. CIR0000000000000682
    Lipshultz SE, Law YM, Asante-Korang A, Austin ED, Dipchand AI, Everitt MD, Hsu DT, Lin KY, Price JF, Wilkinson JD, Colan SD, .
      In this scientific statement from the American Heart Association, experts in the field of cardiomyopathy (heart muscle disease) in children address 2 issues: the most current understanding of the causes of cardiomyopathy in children and the optimal approaches to diagnosis cardiomyopathy in children. Cardiomyopathies result in some of the worst pediatric cardiology outcomes; nearly 40% of children who present with symptomatic cardiomyopathy undergo a heart transplantation or die within the first 2 years after diagnosis. The percentage of children with cardiomyopathy who underwent a heart transplantation has not declined over the past 10 years, and cardiomyopathy remains the leading cause of transplantation for children >1 year of age. Studies from the National Heart, Lung, and Blood Institute-funded Pediatric Cardiomyopathy Registry have shown that causes are established in very few children with cardiomyopathy, yet genetic causes are likely to be present in most. The incidence of pediatric cardiomyopathy is ≈1 per 100 000 children. This is comparable to the incidence of such childhood cancers as lymphoma, Wilms tumor, and neuroblastoma. However, the published research and scientific conferences focused on pediatric cardiomyopathy are sparcer than for those cancers. The aim of the statement is to focus on the diagnosis and classification of cardiomyopathy. We anticipate that this report will help shape the future research priorities in this set of diseases to achieve earlier diagnosis, improved clinical outcomes, and better quality of life for these children and their families.
    Keywords:  AHA Scientific Statements; cardiomyopathies; child; genetics; heart failure; heart transplantation; sudden death
    DOI:  https://doi.org/10.1161/CIR.0000000000000682