bims-hylehe Biomed news
on Hypoplastic left heart syndrome
Issue of 2019‒01‒27
five papers selected by
Richard James
University of Pennsylvania


  1. J Cardiovasc Med (Hagerstown). 2019 Jan 22.
    Flocco SF, Dellafiore F, Caruso R, Giamberti A, Micheletti A, Negura DG, Piazza L, Carminati M, Chessa M.
      AIMS: The aim of this study was to assess the impact of a transition clinic model on adolescent congenital heart disease (CHD) patients' health perception outcomes. The transition clinic model consists of multidisciplinary standardized interventions to educate and support CHD patients and represents a key element in the adequate delivery of care to these individuals during their transition from childhood to adulthood. Currently, empirical data regarding the impact of transition clinic models on the improvement of health perceptions in CHD adolescent patients are lacking.METHODS: A quasi-experimental design was employed. Quality of life, satisfaction, health perceptions and knowledge were assessed at the time of enrolment (T0) and a year after enrolment (T1), respectively. During the follow-up period, the patients enrolled (aged 11-18 years) were involved in the CHD-specific transition clinic model (CHD-TC).
    RESULTS: A sample of 224 CHD adolescents was enrolled (60.7% boys; mean age: 14.84 ± 1.78 years). According to Warnes' classification, 22% of patients had simple heart defect, 56% showed moderate complexity and 22% demonstrated severe complexity. The overall results suggested a good impact of the CHD-TC on adolescents' outcomes, detailing in T1 the occurrence of a reduction of pain (P < 0.001) and anxiety (P < 0.001) and an improvement of knowledge (P < 0.001), life satisfaction (P < 0.001), perception of health status (P < 0.001) and quality of life (P < 0.001).
    CONCLUSION: The CHD-TC seems to provide high-quality care to the patient by way of a multidisciplinary team. The results of the present study are encouraging and confirm the need to create multidisciplinary standardized interventions in order to educate and support the delivery of care for CHD adolescents and their families.
    DOI:  https://doi.org/10.2459/JCM.0000000000000770
  2. Pediatr Cardiol. 2019 Jan 22.
    Loomba RS, Frommelt PC, Anderson RH, Flores S, Tweddell JS.
      Isomerism, or heterotaxy, impacts morbidity and mortality after various stages of univentricular palliation. Timing of Fontan completing in these patients based on preoperative factors has not been investigated previously. The aim of this study was to determine the impact of preoperative factors on various outcomes including length of hospital stay and duration of chest tubes. A cross-sectional study was conducted. Patients with isomerism having undergone Fontan at the Children's Hospital of Wisconsin between 1998 and 2014 were identified. Preoperative, operative, and postoperative data were collected on these patients. Linear regression analysis was conducted to determine preoperative characteristics associated with various postoperative outcomes. Receiver operator curve analysis was also performed to determine the sensitivity and specificity of age and pre-Fontan arterial oxygen saturation in predicting increased length of hospitalization and increased duration of chest tubes. Younger age and lower pre-Fontan arterial oxygen saturation were associated with increased length of hospitalization while younger age, lower pre-Fontan arterial oxygen saturation, interrupted inferior caval vein, and worse pre-Fontan atrioventricular valve regurgitation were associated with increased length of chest tubes. Age, arterial oxygen saturation, pulmonary arteriovenous malformations, interruption of the inferior caval vein, and atrioventricular valve regurgitation should all be taken into consideration when timing Fontan completion in patients with isomerism. Arterial oxygen saturations between 82 and 84% with an approximate age of 3 years appear to be a time at which it is reasonable to consider Fontan in patients with isomerism.
    Keywords:  Cardiac surgical procedures; Congenital heart defects; Fontan procedure; Heterotaxy syndrome; Isomerism; Pediatrics
    DOI:  https://doi.org/10.1007/s00246-019-02060-z
  3. J Am Heart Assoc. 2019 Jan 22. 8(2): e010893
    Öhman A, El-Segaier M, Bergman G, Hanséus K, Malm T, Nilsson B, Pivodic A, Rydberg A, Sonesson SE, Mellander M.
      Background Norwood surgery provides a palliative surgical option for hypoplastic left heart syndrome and has been available in Sweden since 1993. The practice of prenatal ultrasound screening was gradually implemented in the same era, resulting in an increased prenatal detection rate. Our primary aims were to study changes in the incidence of live births, prenatal detection rate, and the termination of pregnancies over time. The secondary aims were to study the proportion of live-borns undergoing surgery and to identify factors that influenced whether surgery was or was not performed. Methods and Results Neonates with hypoplastic left heart syndrome with aortic atresia born 1990-2010 were identified through national databases, surgical files, and medical records. The fetal incidence was estimated from the period when prenatal screening was rudimentary. The study period was divided into the presurgical, early surgical, and late surgical periods. The incidence was calculated as the overall yearly incidence for each time period and sex separately. Factors influencing whether surgery was performed were analyzed using Cox-logistic regression. The incidence at live birth decreased from 15.4 to 8.4 per 100 000. The prenatal detection rate increased from 27% to 63%, and terminations increased from 19% to 56%. The odds of having surgery was higher in the late period and higher in the group with prenatal diagnosis. Conclusions We observed a decrease in incidence of live-borns with hypoplastic left heart syndrome aortic atresia. There was in increase in prenatal detection rate and an increase in termination of pregnancy. The proportion of live-borns who underwent surgery increased between time periods.
    Keywords:  fetal cardiovascular abnomality; fetal echocardiography; hypoplastic left heart syndrome; outcome
    DOI:  https://doi.org/10.1161/JAHA.118.010893
  4. Congenit Heart Dis. 2019 Jan 25.
    Abarbanell G, Tepper NK, Farr SL.
      OBJECTIVE: Women with congenital heart disease (CHD) are at increased risk of pregnancy complications and need information on safe, effective contraceptive methods to avoid unintended pregnancy. This systematic review examines evidence regarding safety of contraceptive use among women with CHD.METHODS: The PubMed database was searched for any peer-reviewed articles published through April 2018 that included safety outcomes associated with reversible contraceptive methods among women with CHD.
    RESULTS: Five articles met inclusion criteria: three studies comparing contraceptive users to nonusers and two noncomparative studies. Sample sizes ranged from 65 to 505 women with CHD. Two studies found a higher percent of thromboembolic complications among women with Fontan palliation or transposition of the great arteries using oral contraceptives. One study, among women with Fontan palliation, found no increased risk of thromboembolic complications between contraceptive users (not separated by type) and nonusers. Two studies found no endocarditis among intrauterine device users.
    CONCLUSIONS: There is a paucity of data regarding the safety of contraceptive methods among women with CHD. Limited evidence suggests an increased incidence of thromboembolic complications with use of oral contraceptives. Further studies are needed to evaluate contraceptive safety and quantify risk in this growing population. There is also limited data regarding the safety of contraceptive methods among women with CHD. Further information is needed to assist practitioners counseling women with CHD on safety of contraceptive methods.
    Keywords:  congenital heart disease; contraception; systematic review
    DOI:  https://doi.org/10.1111/chd.12752
  5. Pediatr Cardiol. 2019 Jan 24.
    van der Stelt F, Siegerink SN, Krings GJ, Molenschot MMC, Breur JMPJ.
      Cardiac catheterization is a commonly used form of imaging and treatment in pediatric patients with congenital heart disease. Traditionally, two-dimensional conventional angiography was the method used, but since 2000 three-dimensional rotational angiography (3DRA) is increasingly used in the field of cardiology in both adult and pediatric patients. To investigate the use and applications of 3DRA in pediatric congenital cardiology, literature was systematically reviewed and 29 eligible articles were found. Those showed that 3DRA is already a greatly valued diagnostic and therapeutic technique in pediatric cardiology. However, the literature misses well-designed clinical, homogeneous, multicenter, prospective studies recording data in a standardized manner. These studies are necessary to ensure proper data analysis and to investigate the true advantages of 3DRA and how it exactly benefits the patients.
    Keywords:  Cardiac catheterization; Congenital heart disease; Pediatric; Review; Rotational angiography
    DOI:  https://doi.org/10.1007/s00246-019-02052-z