bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2019‒10‒13
ten papers selected by
Richard James
University of Pennsylvania


  1. Curr Probl Pediatr Adolesc Health Care. 2019 Oct 08. pii: S1538-5442(19)30088-4. [Epub ahead of print] 100649
    Fierman AH.
      
    DOI:  https://doi.org/10.1016/j.cppeds.2019.100649
  2. J Neonatal Perinatal Med. 2019 Oct 01.
    Hoffman JIE, Hons BS.
      BACKGROUND: False negative pulse oximeter results occur in new born infants with critical congenital heart disease who have an oximeter saturation ≥95%. Some of these infants have abnormal physical findings but others do not.OBJECTIVES: To determine the causes of false negative results.
    METHODS: Mathematical analysis of determinants of arterial oxygen saturation and discussion of oximeter bias.
    RESULTS: False negative oximeter results are not rare; the sensitivity of pulse oximetry screening for critical congenital heart disease is about 80%. The high saturation may be due to a very small right-to-left shunt at the time of study, a relatively high cardiac output and mixed venous saturation, or to positive bias in oximeter readings. It may also be due to some critical congenital heart lesions that do not show desaturation at the time of testing.
    CONCLUSIONS: A diagnosis of a normal heart based on a negative oximeter test is presumptive, and requires careful follow-up for 1-2 weeks after birth.
    Keywords:  Mixed venous saturation; cardiac output; oximeter bias; right-to-left shunt
    DOI:  https://doi.org/10.3233/NPM-190297
  3. J Palliat Care. 2019 Oct 09. 825859719874765
    Morell E, Thompson J, Rajagopal S, Blume ED, May R.
      BACKGROUND: The majority of children with advanced heart disease in the inpatient setting die in an intensive care unit under 1 year of age following multiple interventions. While pediatric cardiology and palliative care provider attitudes have been described, little is known about pediatric cardiothoracic surgeon attitudes toward palliative care in children with advanced heart disease.OBJECTIVE: To describe perspectives of pediatric cardiothoracic surgeons regarding palliative care in pediatric heart disease.
    DESIGN: Cross-sectional web-based national survey.
    RESULTS: Of the 220 surgeons who were e-mailed the survey, 36 opened the survey and 5 did not meet inclusion criteria (n = 31). Median years of practice was 23.5 (range: 12-41 years), and 87.1% were male. Almost all (90%) reported that they had experience consulting palliative care. While 68% felt palliative care consultation was initiated at the appropriate time, 29% felt it occurred too late. When asked the appropriate timing for palliative care consultation in hypoplastic left heart syndrome, 45% selected "at time of prenatal diagnosis" and 30% selected "when surgical and transcatheter options have been exhausted." Common barriers to palliative care involvement included the perception of "giving up" (40%) and concern for undermining parental hope (36%).
    CONCLUSIONS: While a majority of pediatric cardiothoracic surgeons are familiar with palliative care, there is variation in perception of appropriate timing of consultation. Significant barriers to consultation still exist, including concern that parents will think they are "giving" up, undermining parental hope, and influence of palliative care on the medical care team's approach.
    Keywords:  congenital heart disease; pediatric palliative care
    DOI:  https://doi.org/10.1177/0825859719874765
  4. Obstet Gynecol. 2019 Oct 08.
    Cuneo BF, Olson CA, Haxel C, Howley L, Gagnon A, Benson DW, Kaizer AM, Thomas JF.
      OBJECTIVE: To evaluate a fetal telecardiology program in a medically underserved area.METHODS: We conducted a prospective case series of pregnant women at 18-38 weeks of gestation with risk factors for fetal congenital heart disease. Obstetric ultrasonographers performed fetal echocardiograms (local site) that were read in real time. The results were given to the mother by a fetal cardiologist at a children's hospital 243 miles and two mountain passes away (distant site). We evaluated the fetal telecardiology program in five domains: 1) education of obstetric ultrasonographers before initiation of telecardiology services, 2) process and efficiency, 3) patient satisfaction, 4) economic effects, and 5) accuracy of cardiac diagnosis and success of risk stratification.
    RESULTS: The program was initiated on November 12, 2015, and here we describe its first 37 months. Over the initial training period of 3 months and about 70 examinations, obstetric ultrasonographers improved their identification of fetal congenital heart disease. Telecardiology was performed once a week and also for suspected fetal congenital heart disease or arrhythmia outside clinic hours, for a total of 455 examinations. All mothers preferred having their fetal cardiac evaluations performed locally as opposed to traveling to the distant center. The estimated cost to parents for fetal cardiac evaluation at the distant center was nine times greater than that of telecardiology ($581 vs $61). Congenital heart disease or arrhythmia was diagnosed in 28 and 15 fetuses, respectively; there was one false-negative result. All fetuses were correctly risk-stratified with respect to delivery location.
    CONCLUSIONS: Neither diagnostic quality nor patient satisfaction were sacrificed with telecardiology. The program was feasible, empowered the local health care providers and ultrasonographers, offered strong economic advantages to families, and offered the benefit of timely standard-of-care, face-to-face consultation without travel. Based on the success of this program, further studies are warranted to assess its replicability.
    DOI:  https://doi.org/10.1097/AOG.0000000000003502
  5. Ann Thorac Surg. 2019 Oct 05. pii: S0003-4975(19)31550-4. [Epub ahead of print]
    Bichell D.
      
    DOI:  https://doi.org/10.1016/j.athoracsur.2019.09.005
  6. Syst Rev. 2019 Oct 10. 8(1): 236
    Feldmann M, Ullrich C, Bataillard C, Knirsch W, Gosteli-Peter MA, Latal B, Held U.
      BACKGROUND: Over the past decades, survival rates of children born with congenital heart disease (CHD) have increased dramatically. Progress in prenatal diagnosis, less-invasive catheter techniques and perioperative intensive care as well as surgical techniques have led to an increased focus on extracardiac comorbidities, including potential neurodevelopmental sequelae associated with CHD. A growing body of literature reports impairments in early and school-age developmental outcome; however, there is a substantial variability in the spectrum of examined CHD types, assessment ages and applied test batteries. Furthermore, little information is available on executive function impairments in this population. Therefore, the aim of this systematic review is to determine the impact of CHD on intellectual outcome and executive functioning at school age and to determine risk factors for impaired outcomes by means of a systematic search.METHODS: A systematic review of literature that reports neurodevelopmental outcome in children with CHD undergoing cardiopulmonary bypass surgery. Intelligence quotient or executive function scores will be considered primary outcomes. Databases such as Cochrane, EMBASE, MEDLINE and PsycINFO will be searched.
    DISCUSSION: The results of this systematic review will summarize the current evidence on intellectual and executive function outcome after cardiopulmonary bypass surgery in school-age children with CHD. This review will thus be the basis for better patient and parental counselling and the establishment of tailored follow-up programmes and interventional trials.
    SYSTEMATIC REVIEW REGISTRATION: In accordance with the guidelines, our systematic review protocol was registered with the International Prospective Register of Systematic Reviews (PROSPERO) on January 9, 2019 (CRD42018086568). PROSPERO CRD42019118736 .
    Keywords:  Congenital heart disease; Executive function; IQ; Intellectual outcome; Neurocognitive outcome; School-age
    DOI:  https://doi.org/10.1186/s13643-019-1153-y
  7. Arq Bras Cardiol. 2019 Oct 03. pii: S0066-782X2019005019101. [Epub ahead of print]
    Avila WS, Ribeiro VM, Rossi EG, Binotto MA, Bortolotto MR, Testa C, Francisco R, Hajjar LA, Miura N.
      BACKGROUND: The improvement in surgical techniques has contributed to an increasing number of childbearing women with complex congenital heart disease (CCC). However, adequate counseling about pregnancy in this situation is uncertain, due to a wide variety of residual cardiac lesions.OBJECTIVES: To evaluate fetal and maternal outcomes in pregnant women with CCC and to analyze the predictive variables of prognosis.
    METHODS: During 10 years we followed 435 consecutive pregnancies in patients (pts) with congenital heart disease. Among of them, we selected 42 pregnancies in 40 (mean age of 25.5 ± 4.5 years) pts with CCC, who had been advised against pregnancy. The distribution of underlying cardiac lesions were: D-Transposition of the great arteries, pulmonary atresia, tricuspid atresia, single ventricle, double-outlet ventricle and truncus arteriosus. The surgical procedures performed before gestation were: Fontan, Jatene, Rastelli, Senning, Mustard and other surgical techniques, including Blalock, Taussing, and Glenn. Eight (20,0%) pts did not have previous surgery. Nineteen 19 (47.5%) pts had hypoxemia. The clinical follow-up protocol included oxygen saturation recording, hemoglobin and hematocrit values; medication adjustment to pregnancy, anticoagulation use, when necessary, and hospitalization from 28 weeks, in severe cases. The statistical significance level considered was p < 0.05.
    RESULTS: Only seventeen (40.5%) pregnancies had maternal and fetal uneventful courses. There were 13 (30.9%) maternal complications, two (4.7%) maternal deaths due to hemorrhage pos-partum and severe pre-eclampsia, both of them in women with hypoxemia. There were 7 (16.6%) stillbirths and 17 (40.5%) premature babies. Congenital heart disease was identified in two (4.1%) infants. Maternal and fetal complications were higher (p < 0.05) in women with hypoxemia.
    CONCLUSIONS: Pregnancy in women with CCC was associated to high maternal and offspring risks. Hypoxemia was a predictive variable of poor maternal and fetal outcomes. Women with CCC should be advised against pregnancy, even when treated in specialized care centers.
    DOI:  https://doi.org/10.5935/abc.20190197
  8. Pediatrics. 2019 Oct 10. pii: e20184114. [Epub ahead of print]
    Sigmon ER, Kelleman M, Susi A, Nylund CM, Oster ME.
      OBJECTIVES: There has long been an association between congenital heart disease (CHD) and general neurodevelopmental delays. However, the association between CHD and autism spectrum disorders (AuSDs) is less well understood. Using administrative data, we sought to determine the association between CHD and AuSD and identify specific CHD lesions with higher odds of developing AuSD.METHODS: We performed a 1:3 case-control study of children enrolled in the US Military Health System from 2001 to 2013. Children with International Classification of Disease, Ninth Revision, Clinical Modification codes for AuSD were identified as cases and matched with controls on the basis of date of birth, sex, and enrollment time frame. Each child's records were reviewed for CHD lesions and associated procedures. Conditional logistic regression determined odds ratios (ORs) and 95% confidence intervals (CIs) for comparative associations.
    RESULTS: There were 8760 cases with AuSD and 26 280 controls included in the study. After adjustment for genetic syndrome, maternal age, gestational diabetes, short gestation, newborn epilepsy, birth asphyxia, and low birth weight, there were increased odds of AuSD in patients with CHD (OR 1.32; 95% CI 1.10-1.59). Specific lesions with significant OR included atrial septal defects (n = 82; OR 1.72; 95% CI 1.07-2.74) and ventricular septal defects (n = 193; OR 1.65; 95% CI 1.21-2.25).
    CONCLUSIONS: Children with CHD have increased odds of developing AuSD. Specific lesions associated with increased risk include atrial septal defects and ventricular septal defects. These findings will be useful for counseling parents of children with CHD.
    DOI:  https://doi.org/10.1542/peds.2018-4114
  9. OMICS. 2019 Oct 08.
    Thomford NE, Bope CD, Agamah FE, Dzobo K, Owusu Ateko R, Chimusa E, Mazandu GK, Ntumba SB, Dandara C, Wonkam A.
      Artificial intelligence (AI) is one of the key drivers of digital health. Digital health and AI applications in medicine and biology are emerging worldwide, not only in resource-rich but also resource-limited regions. AI predates to the mid-20th century, but the current wave of AI builds in part on machine learning (ML), big data, and algorithms that can learn from massive amounts of online user data from patients or healthy persons. There are lessons to be learned from AI applications in different medical specialties and across developed and resource-limited contexts. A case in point is congenital heart defects (CHDs) that continue to plague sub-Saharan Africa, which calls for innovative approaches to improve risk prediction and performance of the available diagnostics. Beyond CHDs, AI in cardiology is a promising context as well. The current suite of digital health applications in CHD and cardiology include complementary technologies such as neural networks, ML, natural language processing and deep learning, not to mention embedded digital sensors. Algorithms that build on these advances are beginning to complement traditional medical expertise while inviting us to redefine the concepts and definitions of expertise in molecular diagnostics and precision medicine. We examine and share here the lessons learned in current attempts to implement AI and digital health in CHD for precision risk prediction and diagnosis in resource-limited settings. These top 10 lessons on AI and digital health summarized in this expert review are relevant broadly beyond CHD in cardiology and medical innovations. As with AI itself that calls for systems approaches to data capture, analysis, and interpretation, both developed and developing countries can usefully learn from their respective experiences as digital health continues to evolve worldwide.
    Keywords:  artificial intelligence; congenital heart defects; deep learning; digital health; eHealth; machine learning
    DOI:  https://doi.org/10.1089/omi.2019.0142