The pregnant woman with heart disease: management of pregnancy and delivery

Women with heart disease are at risk of cardiac complications during pregnancy and delivery. Risk assessment should be performed in these women, and the management of pregnancy and delivery should be planned accordingly. Depending on the risk, women should be cared for in specialised centres, regional centres or a combination of both. Multidisciplinary teams must be involved in the care of these women. Adequate organisation of care and communication between the team members is important to prevent complications

Feasibility of Ultrasound Measurements of Peripheral Sensory Nerves in Head and Neck Area in Healthy Subjects

Background: Current diagnostic methods for nerve compression headaches consist of diagnostic nerve blocks. A less-invasive method that can possibly aid in the diagnosis is ultrasound, by measuring the cross-sectional area (CSA) of the affected nerve. However, this technique has not been validated, and articles evaluating CSA measurements in the asymptomatic population are missing in the current literature. Therefore, the aim of this study was to determine the feasibility of ultrasound measurements of peripheral extracranial nerves in the head and neck area in asymptomatic individuals.Methods: The sensory nerves of the head and neck in healthy individuals were imaged by ultrasound. The CSA was measured at anatomical determined measurement sites for each nerve. To determine the feasibility of ultrasound measurements, the interrater reliability and the intrarater reliability were determined.Results: In total, 60 healthy volunteers were included. We were able to image the nerves at nine of 11 measurement sites. The mean CSA of the frontal nerves ranged between 0.80 ± 0.42 mm2 and 1.20 ± 0.43 mm2, the mean CSA of the occipital nerves ranged between 2.90 ± 2.73 mm2 and 3.40 ± 1.91 mm2, and the mean CSA of the temporal nerves ranged between 0.92 ± 0.26 mm2 and 1.40 ± 1.11 mm2. The intrarater and interrater reliability of the CSA measurements was good (ICC: 0.75–0.78).Conclusions: Ultrasound is a feasible method to evaluate CSA measurements of peripheral extracranial nerves in the head and neck area. Further research should be done to evaluate the use of ultrasound as a diagnostic tool for nerve compression headache

Diagnosis of Guillain–Barré syndrome in children and validation of the Brighton criteria

To describe the key diagnostic features of pediatric Guillain–Barré syndrome (GBS) and validate the Brighton criteria. Retrospective cohort study of all children (<18 years) diagnosed with GBS between 1987 and 2013 at Sophia Children’s Hospital, Erasmus MC, Rotterdam. Clinical information was collected and the sensitivity of the Brighton criteria was calculated. 67 children (35 boys) were included, with a median age of 5.0 years [interquartile range (IQR) 3.0–10.0 years]. Bilateral limb weakness was present at hospital admission in 93% of children, and at nadir in all patients. Children presented with tetraparesis in 70% or with paraparesis in 23%. Reduced reflexes in paretic limbs were observed at hospital admission in 82% and during follow-up in all children. The progressive phase lasted median 6 days (IQR 3–8 days) and less than 4 weeks in all children. A monophasic disease course was seen in 97%, including 5 children with a treatment-related fluctuation. Two children had a later relapse at 9 weeks and 19 weeks after onset. 77% of the children showed an elevated protein level in CSF. Nerve conduction studies showed evidence for a poly(radiculo)neuropathy in 91% of the children. 46 children had a complete data set, the sensitivity of the Brighton criteria level 1 was 72% (95% CI 57–84) and 96% (95% CI 85–99) for level 2 and 98% (95% CI 88–100) for level 3. The majority of the pediatric GBS patients presented in this cohort fulfilled the current diagnostic criteria

Fertility, pregnancy and delivery in women after biventricular repair for double outlet right ventricle

Objectives: To investigate outcome of pregnancy and fertility in women with double outlet right ventricle (DORV). Methods: Using 2 congenital heart disease registries, 21 female patients with DORV (aged 18-39 years) were retrospectively identified. Detailed recordings of each patient and their completed (>20 weeks gestation) pregnancies were recorded. Results: Overall, 10 patients had 19 pregnancies, including 3 spontaneous miscarriages (16%). During the 16 live birth pregnancies, primarily (serious) noncardiac complications were observed, e.g. premature labor/delivery (n = 7 and n = 3, respectively), small for gestational age (n = 4), preeclampsia (n = 2) and recurrence of congenital heart disease (n = 2). Except for postpartum endocarditis and deterioration of subpulmonary obstruction, only mild cardiac complication pregnancies were recorded. Two women with children reported secondary female infertility. Several menstrual cycle disorders were reported: secondary amenorrhea (n = 4), primary amenorrhea (n = 3) and oligomenorrhea (n = 2). Conclusion: Successful pregnancy in women with DORV is possible. Primarily noncardiac complications were observed and only few (minor) cardiac complications. Infertility and menstrual cycle disorders appear to be more prevalent. Copyrigh

Genetic evidence for the most common risk factors for chronic axonal polyneuropathy in the general population

BACKGROUND AND PURPOSE: Chronic axonal polyneuropathy is a common disease, but the etiology remains only partially understood. Previous etiologic studies have identified clinical risk factors, but genetic evidence supporting causality between these factors and polyneuropathy are largely lacking. In this study, we investigate whether there is a genetic association of clinically established important risk factors (diabetes, body mass index [BMI], vitamin B12 levels, and alcohol intake) with chronic axonal polyneuropathy. METHODS: This study was performed within the population‐based Rotterdam Study and included 1565 participants (median age = 73.6 years, interquartile range = 64.6–78.8, 53.5% female), of whom 215 participants (13.7%) had polyneuropathy. Polygenic scores (PGSs) for diabetes, BMI, vitamin B12 levels, and alcohol intake were calculated at multiple significance thresholds based on published genome‐wide association studies. RESULTS: Higher PGSs of diabetes, BMI, and alcohol intake were associated with higher prevalence of chronic axonal polyneuropathy, whereas higher PGS of vitamin B12 levels was associated with lower prevalence of polyneuropathy. These effects were most pronounced for PGSs with lenient significance thresholds for diabetes and BMI (odds ratio [OR](diabetes, p < 1.0) = 1.21, 95% confidence interval [CI] = 1.05–1.39 and OR(BMI, p < 1.0) = 1.21, 95% CI = 1.04–1.41) and for the strictest significance thresholds for vitamin B12 level and alcohol intake (OR (vitamin B12, p < 5e‐6) = 0.79, 95% CI = 0.68–0.92 and OR(alcohol, p < 5e‐8) = 1.17, 95% CI = 1.02–1.35). We did not find an association between different PGSs and sural sensory nerve action potential amplitude, nor between individual lead variants of PGS (p ) (< 5e‐8) and polyneuropathy. CONCLUSIONS: This study provides evidence for polygenic associations of diabetes, BMI, vitamin B12 level, and alcohol intake with chronic axonal polyneuropathy. This supports the hypothesis of causal associations between well‐known clinical risk factors and polyneuropathy

The Web-Based Advance Care Planning Program "Explore Your Preferences for Treatment and Care":Development, Pilot Study, and Before-and-After Evaluation

BACKGROUND: Web-based advance care planning (ACP) programs may support patients in thinking about and discussing their preferences for future treatment and care. However, they are not widely available, and only a limited number of programs are evidence based. OBJECTIVE: We aimed to develop and evaluate an evidence-based, interactive web-based ACP program that guides users through the process of thinking about, discussing, and recording of preferences for treatment and care. METHODS: The program "Explore your preferences for treatment and care" was developed, pilot-tested on feasibility, and subsequently evaluated; engagement in ACP was assessed before program completion and 2 months after program completion using the ACP Engagement Survey (score 1-5) among 147 persons with chronic disease. Usability (score 0-100) and user satisfaction (score 1-5) were also assessed. RESULTS: ACP engagement increased from 2.8 before program completion to 3.0 two months after program completion (P&lt;.001); contemplation about ACP increased from 2.6 to 2.8 (P=.003), and readiness for ACP increased from 2.2 to 2.5 (P&lt;.001). No changes were found for knowledge about ACP (3.0-3.2; P=.07) and self-efficacy for ACP (3.8-3.8; P=.25). The program was perceived as usable (mean 70, SD 13), attractive (mean 3.8, SD 0.7), and comprehensible (mean 4.2, SD 0.6). CONCLUSIONS: We developed an evidence-based, interactive web-based ACP program in cocreation with patients, relatives, and health care professionals. Before-and-after evaluation showed that the program can support people in taking first steps in ACP and in reflecting on preferences for treatment and care, by guiding them through the process of ACP using a stepwise approach. Participants perceived the program as usable and understandable, and they were satisfied with the program and with the amount of information. Health care professionals may use the program as a tool to start ACP discussions with their patients. The program may increase awareness of ACP.</p