bims-hylehe Biomed news
on Hypoplastic left heart syndrome
Issue of 2019‒03‒31
nine papers selected by
Richard James
University of Pennsylvania


  1. Pediatr Pulmonol. 2019 Mar 28.
    Wolfe KR, Brinton J, Di Maria MV, Meier M, Liptzin DR.
      OBJECTIVES: To evaluate whether the degree of hypoxemia following stage-I and stage-II palliative surgeries predicts neurodevelopmental outcomes at 14 months of age in children with single ventricle congenital heart disease (SVCHD).DESIGN: We analyzed longitudinal data from two Pediatric Heart Network (PHN) randomized controlled trials, with a total of 328 subjects. Oxygen saturations, measured via pulse oximetry, at time of discharge from stage-I and stage-II surgeries were the primary predictors of interest, and Bayley Scales of Infant Development-II (BSID-II) scores at 14 months old were the primary outcome measure. Relevant covariates from previously-published PHN studies were also included in regression models.
    RESULTS: Oxygen saturations at time of discharge from stage-I and stage-II surgeries were not related to BSID-II scores. Having one or more oxygen saturation measurements below 80% was also not associated with BSID-II scores, and neither was change in oxygen saturations over time. These relationships were not altered by inclusion of relevant covariates.
    CONCLUSIONS: In this large cohort of children with SVCHD, oxygen saturations post-stage-I and post-stage-II palliation surgeries as measured via pulse oximetry were not associated with neurodevelopmental outcomes at 14 months of age. The relationship between oxygen saturations and neurodevelopment in SVCHD is likely complex, and neurodevelopment is known to be affected by a number of factors. Pulse oximetry may also be an insufficient proxy for cerebral oxygen delivery. Clinically, pulse oximetry readings during the interstage and post-stage-II surgery periods are not a reliable predictor of future neurodevelopmental risk.
    Keywords:  blood oxygenation; neurodevelopment; pediatric heart network; pulse oximetry; single ventricle
    DOI:  https://doi.org/10.1002/ppul.24275
  2. J Thorac Cardiovasc Surg. 2019 Mar;pii: S0022-5223(18)33142-8. [Epub ahead of print]157(3): 1168-1177.e2
    Romer AJ, Tabbutt S, Etheridge SP, Fischbach P, Ghanayem NS, Reddy VM, Sahulee R, Tanel RE, Tweddell JS, Gaies M, Banerjee M, Retzloff L, Zhang W, Patel AR.
      OBJECTIVES: Our primary aims were to describe the contemporary epidemiology of postoperative high-grade atrioventricular block (AVB), the timing of recovery and permanent pacemaker (PPM) placement, and to determine predictors for development of and recovery from AVB.METHODS: Patients who underwent congenital heart surgery from August 2014 to June 2017 were analyzed for AVB using the Pediatric Cardiac Critical Care Consortium registry. Predictors of AVB with or without PPM were identified using multinomial logistic regression. We used these predictors to model the probability of PPM for the subgroup of patients with intraoperative complete AVB.
    RESULTS: We analyzed 15,901 surgical hospitalizations; 422 (2.7%) were complicated by AVB and 162 (1.0%) patients underwent PPM placement. In patients with transient AVB, 50% resolved by 2 days, and 94% resolved by 10 days. In patients who received a PPM, 50% were placed by 8 days and 62% were placed by 10 days. Independent risk factors associated with PPM compared with resolution of AVB were longer duration of cardiopulmonary bypass (relative risk ratio, 1.04; P = .023) and a high-risk operation (relative risk ratio, 2.59; P < .001). Among patients with complete AVB originating in the operating room, those with the highest predicted probability of PPM had a PPM placed only 77% of the time.
    CONCLUSIONS: In this cohort, postoperative AVB complicated almost 3% of congenital heart surgery cases and 1% of patients underwent PPM placement. Because almost all patients (94%) with transient AVB had resolution by 10 days, our results suggest there is limited benefit to delaying PPM placement beyond that time frame.
    Keywords:  congenital heart disease; database; pacemaker; pediatric
    DOI:  https://doi.org/10.1016/j.jtcvs.2018.09.142
  3. J Genet Couns. 2019 Mar 25.
    Shikany AR, Parrott A, James J, Madueme P, Nicole Weaver K, Cassidy C, Khoury PR, Miller EM.
      Left ventricular outflow tract obstruction (LVOTO) malformations exhibit higher heritability than other cardiac lesions and cardiac screening is encouraged for first-degree relatives. This study sought to determine the uptake of familial cardiac screening in families with an infant with an LVOTO and assess parental knowledge regarding genetics and heritability of LVOTO. A chart review of the period 2010-2015 identified 69 families who received genetic counseling regarding a diagnosis of LVOTO in an infant. Surveys assessing familial cardiac screening and parental knowledge were completed by a parent in 24 families (completion rate of 35%). Forty percent (36/89) of all at-risk first-degree family members completed cardiac screening. The presence of additional congenital malformations in the affected infant was the only significant factor reducing the uptake of familial cardiac screening (p = 0.003). The reported uptake of screening for subsequent at-risk pregnancies was 11/12 (92%) compared to 25/77 (32%) of living at-risk relatives. Survey respondents answered seven knowledge questions with an average score of 5.2 and all correctly identified that LVOTO can run in families. Uptake of familial cardiac screening is occurring in less than half of at-risk individuals, despite parents demonstrating basic knowledge and receiving genetic counseling. Follow-up counseling in the outpatient setting to review familial screening recommendations should be considered to increase uptake and optimize outcomes.
    Keywords:  congenital heart disease; familial cardiac screening; genetic counseling; genetics; left ventricular outflow tract obstruction; parental knowledge; recurrence risk
    DOI:  https://doi.org/10.1002/jgc4.1117
  4. Eur Heart J. 2019 Mar 25. pii: ehz136. [Epub ahead of print]
    Roos-Hesselink J, Baris L, Johnson M, De Backer J, Otto C, Marelli A, Jondeau G, Budts W, Grewal J, Sliwa K, Parsonage W, Maggioni AP, van Hagen I, Vahanian A, Tavazzi L, Elkayam U, Boersma E, Hall R.
      AIMS: Reducing maternal mortality is a World Health Organization (WHO) global health goal. Although maternal deaths due to haemorrhage and infection are declining, those related to heart disease are increasing and are now the most important cause in western countries. The aim is to define contemporary diagnosis-specific outcomes in pregnant women with heart disease.METHODS AND RESULTS: From 2007 to 2018, pregnant women with heart disease were prospectively enrolled in the Registry Of Pregnancy And Cardiac disease (ROPAC). Primary outcome was maternal mortality or heart failure, secondary outcomes were other cardiac, obstetric, and foetal complications. We enrolled 5739 pregnancies; the mean age was 29.5. Prevalent diagnoses were congenital (57%) and valvular heart disease (29%). Mortality (overall 0.6%) was highest in the pulmonary arterial hypertension (PAH) group (9%). Heart failure occurred in 11%, arrhythmias in 2%. Delivery was by Caesarean section in 44%. Obstetric and foetal complications occurred in 17% and 21%, respectively. The number of high-risk pregnancies (mWHO Class IV) increased from 0.7% in 2007-2010 to 10.9% in 2015-2018. Determinants for maternal complications were pre-pregnancy heart failure or New York Heart Association >II, systemic ejection fraction <40%, mWHO Class 4, and anticoagulants use. After an increase from 2007 to 2009, complication rates fell from 13.2% in 2010 to 9.3% in 2017.
    CONCLUSION: Rates of maternal mortality or heart failure were high in women with heart disease. However, from 2010, these rates declined despite the inclusion of more high-risk pregnancies. Highest complication rates occurred in women with PAH.
    Keywords:  Cardiomyopathies; Congenital Heart Disease; Maternal mortality; Pregnancy; Pregnancy complications
    DOI:  https://doi.org/10.1093/eurheartj/ehz136
  5. Clin Nutr ESPEN. 2019 Apr;pii: S2405-4577(18)30613-2. [Epub ahead of print]30 190-198
    Ruys CA, Bröring T, van Schie PEM, van de Lagemaat M, Rotteveel J, Finken MJJ, Oostrom KJ, Lafeber HN.
      BACKGROUND: Children born very preterm are at risk for cognitive deficits and motor impairment. Enhanced protein intake immediately after very preterm birth has been associated with favorable growth and improved neurodevelopment. It is unknown whether increased protein intake after discharge from the hospital affects long-term neurodevelopment.OBJECTIVE: The primary objective was to assess neurodevelopment from infancy to 8 years in preterm-born children who received either protein-enriched formula (PDF), standard term formula (TF), or human milk (HM) after discharge. The secondary objective was to assess the correlation between outcomes obtained at 24 months corrected age (CA) and at 8 years.
    METHODS: This RCT included 152 children born very preterm (gestational age ≤32 weeks) and/or with a very low birth weight (≤1500 g) of whom 102 were randomly assigned to receive PDF (n = 54) or TF (n = 48) from term age to 6 months CA. A control group of infants fed HM (n = 50) was also included. Neurodevelopmental outcomes were assessed at 24 months CA (cognitive and motor functioning; n = 123) and at 8 years (estimated Full Scale Intelligence Quotient, visual-motor skills, verbal memory, attention, and motor functioning; n = 76).
    RESULTS: The PDF and TF groups were not significantly different in neurodevelopmental outcomes. The HM group had a better cognitive score compared with the PDF group: at 24 months CA 92.9 ± 12.5 vs. 105.2 ± 18.6, P < 0.001 and at 8 years 98.1 ± 11.3 vs. 105.8 ± 9.1, P = 0.017 (P = 0.002 and P = 0.080, respectively, after adjustment for parental educational level). Correlations between outcomes at 24 months CA and 8 years were weak: r = 0.35 and r = 0.37 for cognitive and motor outcomes, respectively.
    CONCLUSIONS: PDF did not improve long-term neurodevelopmental outcomes as compared with TF. However, these results should be interpreted with caution considering the substantial attrition at follow-up. Furthermore, the correlation between outcomes at different ages was weak, emphasizing the need for long-term follow-up of nutritional intervention studies in preterm-born children.
    Keywords:  Cognition; Early nutrition; Follow-up; Motor functioning; Preterm infants; Protein
    DOI:  https://doi.org/10.1016/j.clnesp.2018.12.083
  6. Hosp Pediatr. 2019 Mar 26. pii: hpeds.2018-0155. [Epub ahead of print]
    Colwell BRL, Olufs E, Zuckerman K, Kelly SP, Ibsen LM, Williams CN.
      BACKGROUND AND OBJECTIVES: Early mobilization of critically ill children may improve outcomes, but parent refusal of mobilization therapies is an identified barrier. We aimed to evaluate parent stress related to mobilization therapy in the PICU.METHODS: We conducted a cross-sectional survey to measure parent stress and a retrospective chart review of child characteristics. Parents or legal guardians of children admitted for ≥1 night to an academic, tertiary-care PICU who were proficient in English or Spanish were surveyed. Parents were excluded if their child's death was imminent, child abuse or neglect was suspected, or there was a contraindication to child mobilization.
    RESULTS: We studied 120 parent-child dyads. Parent mobilization stress was correlated with parent PICU-related stress (rs [119] = 0.489; P ≤ .001) and overall parent stress (rs [110] = 0.272; P = .004). Increased parent mobilization stress was associated with higher levels of parent education, a lower baseline child functional status, more strenuous mobilization activities, and mobilization therapies being conducted by individuals other than the children's nurses (all P < .05). Parents reported mobilization stress from medical equipment (79%), subjective pain and fragility concerns (75%), and perceived dyspnea (24%). Parent-reported positive aspects of mobilization were clinical improvement of the child (70%), parent participation in care (46%), and increased alertness (38%).
    CONCLUSIONS: Parent mobilization stress was correlated with other measures of parent stress and was associated with child-, parent-, and therapy-related factors. Parents identified positive and stressful aspects of mobilization therapy that can guide clinical care and educational interventions aimed at reducing parent stress and improving the implementation of mobilization therapies.
    DOI:  https://doi.org/10.1542/hpeds.2018-0155
  7. Curr Pediatr Rev. 2019 Mar 20.
    Kostopoulou E, Dimitriou G, Karatza A.
      Congenital heart disease is present in almost 1% of live births and despite current progress in prenatal screening a significant percentage has delayed diagnosis or remain undiagnosed. A cardiac murmur may be the first or unique clinical sign of congenital heart disease in childhood, however less than 1% of auscultated murmurs are of organic cause. Distinguishing between an innocent and a pathologic murmur can be challenging and the experience of the examiner is crucial for identifying the distinctive properties of an innocent murmur. Timely diagnosis of an underlying cardiovascular pathology is of great significance, so that prompt management is provided and morbidity or mortality are restricted. Of similar importance is the avoidance of unnecessary anxiety for the parents and unreasonable referrals to Paediatric Cardiologists. Indications for referral include a medical history suggestive of a cardiac abnormality, such as the presence of relevant symptoms, the identification of abnormal findings on clinical examination, auscultatory findings suggestive of an organic murmur, and very young patient age. ECG and a chest x-Ray are not usually part of the diagnostic approach of a child with a cardiac murmur, as they do not increase the success rate of diagnosing heart disease, as compared to a detailed medical history accompanied by a thorough physical examination. In conclusion, the recognition of suspicious distinctive features of cardiac murmurs is crucial and requires skills based on sufficient training and experience.
    Keywords:  cardiac murmurs; congenital heart disease; innocent murmurs; organic murmurs; transthoracic echocardiography.
    DOI:  https://doi.org/10.2174/1573396315666190321105536
  8. Front Pediatr. 2019 ;7 65
    von Scheidt F, Meier S, Krämer J, Apitz A, Siaplaouras J, Bride P, Kaestner M, Apitz C.
      Background: Impaired exercise capacity is a common feature of congenital heart disease (CHD). In adults with CHD, it has been shown that impaired heart rate response during exercise may contribute to exercise limitation. Systematic data in children and adolescents on this topic is limited. We therefore purposed to assess heart rate response during treadmill exercise testing in children and adolescents with CHD compared to healthy controls. Methods: One hundred and sixty three children and adolescents (103 with CHD, median age 15 years and 60 age-matched controls) performed cardiopulmonary exercise testing and were included in this study. Beyond peak oxygen consumption, increase in heart rate from resting level to peak exercise (heart rate reserve) and decrease of heart rate after peak exercise (heart rate recovery) were measured. Chronotropic index was defined as percentage of age predicted maximal heart rate reserve. According to data from adults on bicycle exercise, chronotropic incompetence was assumed for chronotropic index below 0.8. Results: While resting heart rate was similar between both groups, peak heart rate, heart rate reserve as well as chronotropic index were lower in the CHD group than in controls. Chronotropic index was lowest in patients with single ventricle hemodynamics and correlated with peak oxygen consumption. Heart rate recovery was impaired in the CHD group 1 and 2 min after peak exercise compared to controls and correlated with peak oxygen consumption. Chronotropic index below 0.8 was a relatively frequent finding even in the control group suggesting that the threshold of 0.8 appears inadequate for the identification of chronotropic incompetence using treadmill exercise testing in children. After normalizing to the 2.5th chronotropic index percentile of the control group we obtained a chronotropic incompetence threshold of 0.69. Conclusion: As an adjunct to measurement of peak oxygen consumption, heart rate response to exercise appears to be a physiologically important diagnostic parameter in children and adolescents with CHD. However, interpretation of heart rate response needs to consider specific age characteristics and the mode of exercise test. Our data may help future studies on chronotropic incompetence using treadmill ergometer protocols in children and adolescents.
    Keywords:  cardiopulmonary exercise testing; chronotropic incompetence; congenital heart disease; heart rate recovery; heart rate response
    DOI:  https://doi.org/10.3389/fped.2019.00065
  9. Health Serv Res. 2019 Mar 27.
    Sakai-Bizmark R, Mena LA, Kumamaru H, Kawachi I, Marr EH, Webber EJ, Seo HH, Friedlander SIM, Chang RR.
      OBJECTIVE: Regionalization directs patients to high-volume hospitals for specialized care. We investigated regionalization trends and outcomes in pediatric cardiac surgery.DATA SOURCES/STUDY SETTING: Statewide inpatient data from eleven states between 2000 and 2012.
    STUDY DESIGN: Mortality, length of stay (LOS), and cost were assessed using multivariable hierarchical regression with state and year fixed effects. Primary predictor was hospital case-volume, categorized into low-, medium-, and high-volume tertiles.
    DATA COLLECTION/EXTRACTION METHODS: We used Risk Adjustment for Congenital Heart Surgery-1 (RACHS-1) to select pediatric cardiac surgery discharges.
    PRINCIPAL FINDINGS: In total, 2841 (8.5 percent), 8348 (25.1 percent), and 22 099 (66.4 percent) patients underwent heart surgeries in low-, medium-, and high-volume hospitals. Mortality decreased over time, but remained higher in low- and medium-volume hospitals. High-volume hospitals had lower odds of mortality and cost than low-volume hospitals (odds ratio [OR] 0.59, P < 0.01, and relative risk [RR] 0.91, P < 0.01, respectively). LOS was longer for high- and medium-volume hospitals, compared to low-volume hospitals (high-volume: RR 1.18, P < 0.01; medium-volume: RR 1.05, P < 0.01).
    CONCLUSIONS: Regionalization reduced mortality and cost, indicating fewer complications, but paradoxically increased LOS. Further research is needed to explore the full impact on health care utilization.
    Keywords:  case-volume; health care utilization; mortality; pediatric cardiology
    DOI:  https://doi.org/10.1111/1475-6773.13137