Differences at surgery between patients with bicuspid and tricuspid aortic valves.

AIM: To determine differences in surgical procedures and clinical characteristics at the time of surgery between native bicuspid aortic valves (BAV) and tricuspid aortic valves (TAV) in patients being followed up after aortic valve surgery (AVS). METHODS: In this retrospective cohort study in a non-academic hospital, we identified patients who had a surgeon's report of the number of native valve cusps and were still being followed up. We selected patients with BAV and TAV, and used multivariable regression analyses to identify associations between BAV-TAV and pre-specified clinical characteristics. RESULTS: Of 439 patients, 140 had BAV (32%) and 299 TAV (68%). BAV patients were younger at the time of surgery (mean age 58.6 ± 13 years) than TAV patients (69.1 ± 12 years, p < 0.001) and were more often male (64% vs 53%; p = 0.029). Cardiovascular risk factors were less prevalent in BAV than in TAV patients at the time of surgery (hypertension (31% vs 55%), hypercholesterolaemia (29% vs 58%) and diabetes (7% vs 16%); all p < 0.005). Concomitant coronary artery bypass grafting (CABG) was performed less often in BAV than in TAV patients (14% vs 39%, p < 0.001), even when adjusted for confounders (adjusted odds ratio (adj.OR) 0.45; 95% CI: 0.25-0.83). In contrast, surgery of the proximal aorta was performed more often (31% vs 11%, respectively, p < 0.001; adj.OR 2.3; 95% CI: 1.3-4.0). CONCLUSIONS: Whereas mechanical stress is the supposed major driver of valvulopathy towards AVS in BAV, prevalent cardiovascular risk factors are a suspected driver towards the requirement for AVS and concomitant CABG in TAV, an observation based on surgical determination of the number of valve cusps.

A ventricular septal defect with a giant appendiform aneurysm of the membranous septum.

We report a case of a 39-year-old female with a ventricular septal defect (VSD) and a giant appendiform aneurysm of the membranous septum, illustrated by echocardiography and magnetic resonance imaging. From the literature a short review of the prevalence of spontaneous closure of VSDs together with the possible complications of persisting VSDs is presented. Since patients stay at risk in later years, follow-up at regular intervals is advised.

Anti-Tumour Necrosis Factor Therapy for Paediatric Crohn's Disease: Improved Benefits Through Treatment Optimisation, Deeper Understanding of Its Risks, and Reduced Costs due to Biosimilar Availability.

Antibodies directed to tumour necrosis factor-α (TNF-α) are very effective in treating paediatric Crohn's disease (CD). Over the last few years, research has provided important new insights into how to optimise this treatment's effectiveness. Research on predictors for anti-TNF treatment responsiveness has revealed potential markers, but data on their accuracy in paediatric CD patients are lagging behind. Also, new evidence has become available on the safety profile of anti-TNF antibodies that suggests the assumed increased malignancy risk seen in patients on anti-TNF and thiopurine combination treatment may be linked more to thiopurine use and not to anti-TNF treatment. In addition, the early results of CT-P13, an infliximab biosimilar, in CD patients confirm the expected similarity with its originator. Thus, the effectiveness of anti-TNF antibody treatment is slowly improving, its malignancy risk is lower than assumed, and its costs are reduced by the introduction of equally effective biosimilars. Together, these trends allow for a more prominent role for anti-TNF antibodies in future treatment of paediatric CD.

Top-down Infliximab Study in Kids with Crohn's disease (TISKids): an international multicentre randomised controlled trial.

INTRODUCTION: Crohn's disease (CD) is a chronic inflammatory disease predominantly affecting the gastrointestinal tract. CD usually requires lifelong medication and is accompanied by severe complications, such as fistulae and strictures, resulting in surgery. Infliximab (IFX) is very effective for treating paediatric patients with CD, but is currently only registered for therapy refractory patients-the so-called step-up strategy. We hypothesise that using IFX first-line, that is, top-down, will give more mucosal healing, fewer relapses, less complications, need for surgery and hospitalisation. METHODS AND ANALYSIS: This international multicentre open-label randomised controlled trial includes children, aged 3-17 years, with new-onset, untreated CD with moderate-to-severe disease activity (weighted Paediatric Crohn's Disease Activity Index (wPCDAI)>40). Eligible patients will be randomised to top-down or step-up treatment. Top-down treatment consists of 5 IFX infusions combined with azathioprine (AZA). After these 5 infusions, patients will continue AZA. Patients randomised to step-up will receive standard induction treatment, either oral prednisolone or exclusive enteral nutrition, combined with AZA as maintenance treatment. The primary outcome is clinical remission (wPCDAI<12.5) at 52 weeks without need for additional CD-related therapy or surgery. Total follow-up is 5 years. Secondary outcomes include clinical disease activity, mucosal healing by endoscopy (at week 10 and optionally week 52), faecal calprotectin, growth, quality of life, medication use and adverse events. ETHICS AND DISSEMINATION: Conducted according to the Declaration of Helsinki and Good Clinical Practice. Medical-ethical approval will be obtained for each site. TRIAL REGISTRATION NUMBER: NCT02517684; Pre-results.