bims-hylehe Biomed news
on Hypoplastic left heart syndrome
Issue of 2018‒10‒14
six papers selected by
Richard James
University of Pennsylvania


  1. Am J Cardiol. 2018 Oct 01. pii: S0002-9149(18)31325-0. [Epub ahead of print]122(7): 1222-1230
    Mechak JT, Edwards EM, Morrow KA, Swanson JR, Vergales J.
      Prematurity increases pre- and postoperative mortality in children with congenital heart disease. There are no large, multicentered, studies that have evaluated this relation specifically in neonates with hypoplastic left heart syndrome (HLHS). We sought to determine the impact of gestational age (GA) on survival to Stage 1 palliation surgery and hospital discharge in infants with HLHS. We reviewed data from 1,913 neonates with HLHS born at or transferred to a Vermont Oxford Network expanded member hospital in the United States from 2009 to 2014. Demographic, diagnostic, and surgical codes, and outcome data within the Vermont Oxford Network database were used to determine the effect of GA and birth weight on survival to Stage 1 palliation surgery and hospital discharge. Risk models were developed controlling for common confounders to determine the relative risk of GA on the observed outcomes. These data demonstrate that, when compared with 39-week infants, those born at earlier GA were less likely to survive until surgery; <34 weeks adjusted risk ratio (ARR) for survival: 0.47 (95% confidence interval 0.37 to 0.60), 34 to 35 weeks ARR 0.73 (0.62 to 0.87), and 36 to 37 weeks ARR 0.88 (0.83 to 0.94). Higher GA also positively correlated with survival to hospital discharge, although there was no difference in 34 to 35-week infants and 36 to 37-week infants. In conclusion, these data show that GA was an independent risk factor for survival to Stage 1 palliation surgery and survival to hospital discharge. However, there is no significant difference in survival to hospital discharge between infants born in 34 to 37 weeks gestation.
    DOI:  https://doi.org/10.1016/j.amjcard.2018.06.033
  2. J Heart Lung Transplant. 2018 Aug 11. pii: S1053-2498(18)31597-3. [Epub ahead of print]
    Miera O, Germann M, Cho MY, Photiadis J, Walter EMD, Hetzer R, Berger F, Schmitt KRL.
      BACKGROUND: The majority of children supported with ventricular assist devices (VADs) are bridged to heart transplantation. Although bridge to recovery has been reported, low recovery patient numbers has precluded systematic analysis. The aim of this study was to delineate recovery rates and predictors of recovery and to report on long-term follow-up after VAD explantation in children.METHODS: Children bridged to recovery at our institution from January 1990 to May 2016 were compared with a non-recovery cohort. Clinical and echocardiographic data before and at pump stoppages and after VAD explantation were analyzed. Kaplan‒Meier estimates of event-free survival, defined as freedom from death or transplantation after VAD removal, were determined.
    RESULTS: One hundred forty-nine children (median age 5.8 years) were identified. Of these, 65.2% had cardiomyopathy, 9.4% had myocarditis, and 24.8% had congenital heart disease. The overall recovery rate was 14.2%, and was 7.1% in patients with dilated cardiomyopathy. Predictors of recovery were age <2 years (recovery rate 27.8%, odds ratio [OR] 5.64, 95% confidence interval [CI] 2.0 to 16.6) and diagnosis of myocarditis (rate 57.1%; OR 17.56, 95% CI 4.6 to 67.4). After a median follow-up of 10.8 years, 15 patients (83.3%) were in Functional Class I and 3 (16.7%) in were in Class II. Mean left ventricular ejection fraction was 53% (range 28% to 64%). Ten- and 15-year event-free survival rates were both 84.1 ± 8.4%.
    CONCLUSIONS: Children <2 years of age and those diagnosed with myocarditis have the highest probability of recovery. Long-term survival after weaning from the VAD was better than after heart transplantation, as demonstrated in the excellent long-term stability of ejection fraction and functional class.
    DOI:  https://doi.org/10.1016/j.healun.2018.08.005
  3. J Pediatr. 2018 Oct 02. pii: S0022-3476(18)31230-7. [Epub ahead of print]
    Cloete E, Bloomfield FH, Sadler L, de Laat MWM, Finucane AK, Gentles TL.
      OBJECTIVE: To establish the impact that timing of diagnosis and place of birth have on neonatal outcomes in those with readily treatable critical congenital heart disease.STUDY DESIGN: This was a population-based study with a complete national cohort of live-born infants with transposition of the great arteries and aortic arch obstruction in New Zealand between 2006 and 2014. Timing of diagnosis, place of birth, survival to surgery, in-hospital events, and neonatal mortality were reviewed. Live births with a gestation of ≥35 weeks and without associated major extracardiac anomalies were included for analysis.
    RESULTS: A total of 166 live-born infants with transposition of the great arteries and 87 with aortic arch obstruction were included. Antenatal detection increased from 32% in the first 3 years to 47% in the last 3 years (P = .05). During the same period, neonatal mortality decreased from 9% to 1% (P = .02). No deaths occurred after surgical intervention. An antenatal diagnosis was associated with decreased mortality (1/97 [1%] vs 11/156 [7%]; P = .03) and birth outside the surgical center was associated with increased risk of mortality (11/147 [7%] vs 1/106 [1%]; P = .02). Those with an antenatal diagnosis required fewer hours of mechanical ventilation (P = .02) and had shorter durations of hospital stay (P = .05) compared with those diagnosed >48 hours after birth.
    CONCLUSIONS: The mortality risk for transposition of the great arteries and critical aortic arch obstruction is greatest before cardiac surgery. Improved antenatal detection allowing delivery at a surgical center is associated with reduced mortality.
    Keywords:  birth defects; cardiovascular disorders; newborn infant
    DOI:  https://doi.org/10.1016/j.jpeds.2018.08.056
  4. Resuscitation. 2018 Oct 03. pii: S0300-9572(18)30887-6. [Epub ahead of print]
    Scholefield BR, Silverstein FS, Telford R, Holubkov R, Slomine BS, Meert KL, Christensen JR, Nadkarni VM, Dean JM, Moler FW.
      BACKGROUND: Separate trials to evaluate therapeutic hypothermia after paediatric cardiac arrest for out-of-hospital and in-hospital settings reported no statistically significant differences in survival with favourable neurobehavioral outcome or safety compared to therapeutic normothermia. However, larger sample sizes might detect smaller clinical effects. Our aim was to pool data from identically conducted trials to approximately double the sample size of the individual trials yielding greater statistical power to compare outcomes.METHODS: Combine individual patient data from two clinical trials set in forty-one paediatric intensive care units in USA, Canada and UK. Children aged at least 48 hours up to 18 years old, who remained comatose after resuscitation, were randomized within 6 hours of return of circulation to hypothermia or normothermia (target 33.0 °C or 36.8 °C). The primary outcome, survival 12 months post-arrest with Vineland Adaptive Behaviour Scales, Second Edition (VABS-II) score at least 70 (scored from 20-160, higher scores reflecting better function, population mean = 100, SD = 15), was evaluated among patients with pre-arrest scores ≥70.
    RESULTS: 624 patients were randomized. Among 517 with pre-arrest VABS-II scores ≥70, the primary outcome did not significantly differ between hypothermia and normothermia groups (28% [75/271] and 26% [63/246], respectively; relative risk, 1.08; 95% confidence interval [CI], 0.81 to 1.42; p = 0.61). Among 602 evaluable patients, the change in VABS-II score from baseline to 12 months did not differ significantly between groups (p = 0.20), nor did, proportion of cases with declines no more than 15 points or improvement from baseline [22% (hypothermia) and 21% (normothermia)]. One-year survival did not differ significantly between hypothermia and normothermia groups (44% [138/317] and 38% [113/ 297], respectively; relative risk, 1.15; 95% CI, 0.95 to 1.38; p = 0.15). Incidences of blood-product use, infection, and serious cardiac arrhythmia adverse events, and 28-day mortality, did not differ between groups.
    CONCLUSIONS: Analysis of combined data from two paediatric cardiac arrest targeted temperature management trials including both in-hospital and out-of-hospital cases revealed that hypothermia, as compared with normothermia, did not confer a significant benefit in survival with favourable functional outcome at one year.
    CLINICAL TRIAL REGISTRATION: THAPCA-OH ClinicalTrials.gov number, NCT00878644. THAPCA- IH ClinicalTrials.gov number, NCT00880087.
    Keywords:  Paediatric Cardiac Arrest; Randomised Controlled Trials; Targeted Temperature Management; Therapeutic Normothermia; Therapeutic hypothermia
    DOI:  https://doi.org/10.1016/j.resuscitation.2018.09.011
  5. J Biomed Inform. 2018 Oct 04. pii: S1532-0464(18)30197-7. [Epub ahead of print]
    Klein AZ, Sarker A, Cai H, Weissenbacher D, Gonzalez-Hernandez G.
      BACKGROUND: Although birth defects are the leading cause of infant mortality in the United States, methods for observing human pregnancies with birth defect outcomes are limited.OBJECTIVE: The primary objectives of this study were (i) to assess whether rare health-related events-in this case, birth defects-are reported on social media, (ii) to design and deploy a natural language processing (NLP) approach for collecting such sparse data from social media, and (iii) to utilize the collected data to discover a cohort of women whose pregnancies with birth defect outcomes could be observed on social media for epidemiological analysis.
    METHODS: To assess whether birth defects are mentioned on social media, we mined 432 million tweets posted by 112,647 users who were automatically detected via their public announcements of pregnancies on Twitter. To retrieve tweets that mention birth defects, we developed a rule-based, bootstrapping approach, which relies on a lexicon, lexical variants generated from the lexicon entries, regular expressions, post-processing, and manual analysis guided by distributional properties. To identify users whose pregnancies with birth defect outcomes could be observed for epidemiological analysis, inclusion criteria were (i) tweets indicating that the user's child has a birth defect, and (ii) accessibility to the user's tweets during pregnancy. We conducted a semi-automatic evaluation to estimate the recall of the tweet-collection approach, and performed a preliminary assessment of the prevalence of selected birth defects among the pregnancy cohort derived from Twitter.
    RESULTS: We manually annotated 16,822 retrieved tweets, distinguishing tweets indicating that the user's child has a birth defect (true positives) from tweets that merely mention birth defects (false positives). Inter-annotator agreement was substantial: κ = 0.79 (Cohen's kappa). Analyzing the timelines of the 646 users whose tweets were true positives resulted in the discovery of 195 users that met the inclusion criteria. Congenital heart defects are the most common type of birth defect reported on Twitter, consistent with findings in the general population. Based on an evaluation of 4,169 tweets retrieved using alternative text mining methods, the recall of the tweet-collection approach was 0.95.
    CONCLUSIONS: Our contributions include (i) evidence that rare health-related events are indeed reported on Twitter, (ii) a generalizable, systematic NLP approach for collecting sparse tweets, (iii) a semi-automatic method to identify undetected tweets (false negatives), and (iv) a collection of publicly available tweets by pregnant users with birth defect outcomes, which could be used for future epidemiological analysis. In future work, the annotated tweets could be used to train machine learning algorithms to automatically identify users reporting birth defect outcomes, enabling the large-scale use of social media mining as a complementary method for such epidemiological research.
    Keywords:  birth defects; cohort discovery; epidemiology; natural language processing; patient-reported pregnancy outcomes; social media mining
    DOI:  https://doi.org/10.1016/j.jbi.2018.10.001
  6. Zhonghua Fu Chan Ke Za Zhi. 2018 Sep 25. 53(9): 608-612
    Bao ZL, Zhang J.
      Objective: To summarize and analyze the methods of termination of pregnancy in the first and second trimester of pregnancy with severe cardiovascular disease. Methods: A retrospective analysis of 27 cases of termination of pregnancy in the first and second trimester of pregnancy in Beijing Anzhen Hospital from January 1, 2016 to December 30, 2017. All of these pregnant women were pregnancy complicated with severe cardiovascular disease in grade Ⅴ pregnancy risk. Results: (1) The age of 27 pregnant women was 22-40 years, gestational age was 6-27 weeks; cardiac function grade before induced labor was: 5 cases of grade Ⅱ, 15 cases of grade Ⅲ, 7 cases of grade Ⅳ. The cardiovascular diseases included ventricular septal defect in 9 cases, simple atrial septal defect in 3 cases, patent ductus arteriosus in 2 cases, tetralogy of Fallot in 2 cases, rheumatic valvular disease in 1 case,arrhythmia-paroxysmal atrial velocity in 2 cases, cardiomyopathy in 2 cases, hypertensive cardiopathy in 2 cases, primary pulmonary hypertension in 1 case, tissue disease complicated with pulmonary hypertension 1 case, hyperthyroid heart disease in 1 case, coronary heart disease in 1 case. Among them, 21 cases were complicated with pulmonary hypertension, and 5 cases with Eisenmenger syndrome. (2) Methods of termination of pregnancy: in 27 cases, eight cases of vacuum curettage, and 2 cases of forceps curettage under general anesthesia without intubation; rivanol intraamniotic induction of labor in 1 case; and hysterotomy delivery in 16 cases (11 cases were treated with continuous epidural anesthesia, 1 case was treated with combined spinal and epidural anesthesia, 4 cases were treated with spinal anesthesia) . (3) After termination of pregnancy, one patient with severe primary pulmonary hypertension at 19 weeks of gestation died on the 1st day postoperative. No significant changes in cardiac function were observed in other patients before and after termination of pregnancy, and all of them survived. Conclusions: In patients with severe cardiovascular disease, termination of pregnancy after pregnancy may result in maternal death even at the second trimester of pregnancy.It is strongly recommended that such patients undergo pre-pregnancy assessment and fertility counseling.If visiting doctor after pregnancy, we should organize a multidisciplinary consultation as soon as possible to assess the risk of continuing pregnancy and give medical advice. If the risk of pregnancy is high, we should terminate the pregnancy as early as possible, after making a suitable treatment plan. During termination of pregnancy, temporary or permanent contraception may be taken if the condition permits.
    Keywords:  Abortion, induced; Cardiovascular diseases; Perioperative care; Pregnancy complications, cardiovascular
    DOI:  https://doi.org/10.3760/cma.j.issn.0529-567x.2018.09.005