bims-hylehe Biomed news
on Hypoplastic left heart syndrome
Issue of 2018‒10‒07
seven papers selected by
Richard James
University of Pennsylvania


  1. J Pediatr. 2018 Sep 26. pii: S0022-3476(18)30966-1. [Epub ahead of print]
    Pinto NM, Waitzman N, Nelson R, Minich LL, Krikov S, Botto LD.
      OBJECTIVE: To assess longitudinal estimates of inpatient costs through early childhood in patients with critical congenital heart defects (CCHDs), for whom reliable estimates are scarce, using a population-based cohort of clinically validated CCHD cases.STUDY DESIGN: Longitudinal retrospective cohort of infants with CCHDs live born from 1997 to 2012 in Utah. Cases identified from birth defect registry data were linked to inpatient discharge abstracts and vital records to track inpatient days and costs through age 10 years. Costs were adjusted for inflation and discounted by 3% per year to generate present value estimates. Multivariable models identified infant and maternal factors potentially associated with higher resource utilization and were used to calculate adjusted costs by defect type.
    RESULTS: The final statewide cohort included 1439 CCHD cases among 803 509 livebirths (1.8/1000). The average cost per affected child through age 10 years was $136 682 with a median of $74 924 because of a small number of extremely high cost children; costs were highest for pulmonary atresia with ventricular septal defect and hypoplastic left heart syndrome. Inpatient costs increased by 1.6% per year during the study period. A single birth year cohort (~50 000 births/year) had estimated expenditures of $11 902 899 through age 10 years. Extrapolating to the US population, inpatient costs for a single birth year cohort through age 10 years were ~$1 billion.
    CONCLUSIONS: Inpatient costs for CCHDs throughout childhood are high and rising. These revised estimates will contribute to comparative effectiveness research aimed at improving the value of care on a patient and population level.
    DOI:  https://doi.org/10.1016/j.jpeds.2018.07.060
  2. Heart. 2018 Sep 29. pii: heartjnl-2018-313613. [Epub ahead of print]
    Ntiloudi D, Apostolopoulou S, Vasiliadis K, Frogoudaki A, Tzifa A, Ntellos C, Brili S, Manginas A, Pitsis A, Kolios M, Karvounis H, Tsioufis C, Goudevenos J, Rammos S, Giannakoulas G, .
      OBJECTIVE: Despite the progress in the management of patients with adult congenital heart disease (ACHD), a significant proportion of patients still develop pulmonary hypertension (PH). We aimed to highlight the rate of the complications in PH-ACHD and the predicting factors of cumulative mortality risk in this population.METHODS: Data were obtained from the cohort of the national registry of ACHD in Greece from February 2012 until January 2018.
    RESULTS: Overall, 65 patients receiving PH-specific therapy were included (mean age 46.1±14.4 years, 64.6% females). Heavily symptomatic (New York Heart Association (NYHA) class III/IV) were 53.8% of patients. The majority received monotherapy, while combination therapy was administered in 41.5% of patients. Cardiac arrhythmia was reported in 30.8%, endocarditis in 1.5%, stroke in 4.6%, pulmonary arterial thrombosis in 6.2%, haemoptysis in 3.1% and hospitalisation due to heart failure (HF) in 23.1%. Over a median follow-up of 3 years (range 1-6), 12 (18.5%) patients died. On univariate Cox regression analysis history of HF hospitalisation emerged as a strong predictor of mortality (HR 8.91, 95% CI 2.64 to 30.02, p<0.001), which remained significant after adjustment for age and for NYHA functional class.
    CONCLUSIONS: Long-term complications are common among patients with PH-ACHD. Hospitalisations for HF predict mortality and should be considered in the risk stratification of this population.
    Keywords:  congenital heart disease; heart failure; secondary pulmonary hypertension
    DOI:  https://doi.org/10.1136/heartjnl-2018-313613
  3. Int J Cardiol. 2018 Sep 22. pii: S0167-5273(18)34632-1. [Epub ahead of print]
    Ohuchi H.
      
    DOI:  https://doi.org/10.1016/j.ijcard.2018.09.071
  4. Pediatr Radiol. 2018 Sep 29.
    Diaz ES, Dillman JR, Veldtman GR, Trout AT.
      BACKGROUND: Focal liver lesions are common in children and adults after Fontan procedures.OBJECTIVE: To explore the relationship between liver shear stiffness, using magnetic resonance (MR) elastography, and the presence of focal liver lesions in patients after Fontan procedures (total cavopulmonary anastomosis).
    MATERIALS AND METHODS: The retrospective study was approved by the institutional review board and the requirement for informed consent was waived. By searching institutional electronic medical records, we identified all patients with a history of Fontan palliation of congenital heart disease who had undergone same-day liver MR elastography and liver MRI without and with intravenous contrast material between January 2012 and December 2017. Using imaging reports, patients were placed into two groups: 1) no focal liver lesions and 2) one or more focal liver lesions. Patient age, sex, mean liver shear stiffness (kPa) and maximum single anatomical level liver shear stiffness (kPa) were recorded. The Mann-Whitney U test was used to compare age and liver stiffness between groups, while the Fisher exact test was used to assess the impact of gender on liver lesions.
    RESULTS: Forty-eight patients met study inclusion criteria; 33 (69%) had one or more focal liver lesions. The median age was 20.0 years (IQR [interquartile range]: 10.8-29.1 years) for patients without liver lesions and 19.9 years (IQR: 17.2-27.0 years) for patients with liver lesions (P=0.49). Eleven of 21 male patients (52.4%) had liver lesions compared to 22 of 27 female patients (81.5%) (P=0.058). Mean (4.62 kPa [IQR: 4.10-5.59 kPa] vs. 4.10 kPa [IQR: 3.44-4.80 kPa]; P=0.02) and maximum (5.53 kPa [IQR: 4.64-6.56 kPa] vs. 4.50 kPa [IQR: 3.82-5.35 kPa]; P=0.009) liver stiffness were significantly higher in patients without liver lesions as compared to patients with liver lesions.
    CONCLUSION: Our study demonstrated a significant negative association between focal liver lesions and increased liver stiffness in patients following Fontan procedures.
    Keywords:  Children; Fontan procedure; Fontan-associated liver disease; Liver lesions; Liver stiffness; Magnetic resonance elastography; Young adults
    DOI:  https://doi.org/10.1007/s00247-018-4264-z
  5. J Pediatr. 2018 Sep 26. pii: S0022-3476(18)31114-4. [Epub ahead of print]
    Collins RT, Lyle RE, Rettiganti M, Gossett JM, Robbins JM, Casey PH.
      OBJECTIVE: To evaluate whether the presence of patent ductus arteriosus (PDA) in preterm infants worsens long-term neurodevelopmental outcomes.STUDY DESIGN: This was a secondary observational analysis of data from 1090 preterm low-birthweight infants in the Infant Health and Development Program (IHDP), a multicenter longitudinal cohort study of outcomes assessed from 3 to 18 years of age. Multivariable analysis was adjusted for IHDP treatment group (intervention or follow-up), birth weight, maternal race, maternal education, infant sex, maternal preconception weight, Home Observation Measurement of the Environment (HOME) total score at 12 months, neonatal health index, and gestational age.
    RESULTS: Of the 1090 patients (49% male) included in the analysis, 135 had a PDA. Mean birth weight (1322 g vs 1871 g; P < .0001) and gestational age (30.2 weeks vs 33.4 weeks, P < .0001) were lower and mean ventilator days (11.8 vs 1.3; P < .0001), vasopressor use (12.6% vs 1.2%; P < .0001), and congestive heart failure (8.9% vs 0.1%; P < .0001) were higher in the PDA group. There were no differences between the PDA and no-PDA groups in maternal education level and HOME total score at age 12 months. Multivariable analysis demonstrated no between-group differences in cognitive development or behavioral competence at age 3, 8, and 18 years.
    CONCLUSIONS: The presence of a PDA in moderately preterm, low-birthweight infants does not impact long-term neurodevelopmental outcomes.
    Keywords:  low birth weight; neurodevelopmental outcome; patent ductus arteriosus; preterm
    DOI:  https://doi.org/10.1016/j.jpeds.2018.08.004
  6. J Thorac Cardiovasc Surg. 2018 Aug 31. pii: S0022-5223(18)32272-4. [Epub ahead of print]
    Si MS.
      
    DOI:  https://doi.org/10.1016/j.jtcvs.2018.08.029
  7. BMJ Open. 2018 Sep 28. 8(9): e022755
    Dawson AJ, Krastev Y, Parsonage WA, Peek M, Lust K, Sullivan EA.
      OBJECTIVE: Cardiac disease in pregnancy is a leading cause of maternal death in high-income countries. Evidence-based guidelines to assist in planning and managing the healthcare of affected women is lacking. The objective of this research was to produce the first qualitative metasynthesis of the experiences of pregnant women with existing or acquired cardiac disease to inform improved healthcare services.METHOD: We conducted a systematic search of peer-reviewed publications in five databases to investigate the decision-making processes, supportive strategies and healthcare experiences of pregnant women with existing or acquired cardiac disease, or of affected women contemplating pregnancy. Identified publications were screened for duplication and eligibility against selection criteria, following Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. We then undertook a thematic analysis of the data relating to women's experiences extracted from each publication to inform new healthcare practices and communication.
    RESULTS: Eleven studies from six countries were included in our meta-synthesis. Four themes were revealed. Women with congenital and acquired heart disease identified situations where they had either taken charge of decision-making, lacked control or experienced emotional uncertainty when making decisions. Some women were risk aware and determined to take care of themselves in pregnancy while others downplayed the risks. Women with heart disease acknowledged the importance of specific social support measures during pregnancy and after child birth, and reported a spectrum of healthcare experiences.
    CONCLUSIONS: There is a lack of integrated and tailored healthcare services and information for women with cardiac disease in pregnancy. The experiences of women synthesised in this research has the potential to inform new evidence-based guidelines to support the decision-making needs of women with cardiac disease in pregnancy. Shared decision-making must consider communication across the clinical team. However, coordinated care is challenging due to the different specialists involved and the limited clinical evidence concerning effective approaches to managing such complex care.
    Keywords:  cardiac disease; pregnancy; qualitative meta-synthesis; shared decision-making; women centered care
    DOI:  https://doi.org/10.1136/bmjopen-2018-022755