Maternal and fetal outcomes in pregnancies complicated by Marfan syndrome.

OBJECTIVES: Information to guide counselling and management for pregnancy in women with Marfan syndrome (MFS) is limited. We therefore conducted a UK multicentre study. METHODS: Retrospective observational study of women with MFS delivering between January 1998 and March 2018 in 12 UK centres reporting data on maternal and neonatal outcomes. RESULTS: In total, there were 258 pregnancies in 151 women with MFS (19 women had prior aortic root replacements), including 226 pregnancies ≥24 weeks (two sets of twins), 20 miscarriages and 12 pregnancy terminations. Excluding miscarriages and terminations, there were 221 live births in 139 women. Only 50% of women received preconception counselling. There were no deaths, but five women experienced aortic dissection (1.9%; one type A and four type B-one had a type B dissection at 12 weeks and subsequent termination of pregnancy). Five women required cardiac surgery postpartum. No predictors for aortic dissection could be identified. The babies of the 131 (65.8%) women taking beta-blockers were on average 316 g lighter (p<0.001). Caesarean section rates were high (50%), particularly in women with dilated aortic roots. In 55 women, echocardiographic aortic imaging was available prepregnancy and postpregnancy; there was a small but significant average increase in AoR size of 0.84 mm (Median follow-up 2.3 months) CONCLUSION: There were no maternal deaths, and the aortic dissection rate was 1.9% (mainly type B). There with no identifiable factors associated with aortic dissection in our cohort. Preconception counselling rates were low and need improvement. Aortic size measurements increased marginally following pregnancy.

Achieving high-quality care: a view from NICE.

The National Institute for Health and Care Excellence (NICE) was established in 1999 to provide evidence-based guidance. The task of producing guidance by reviewing primary research data and using an advisory committee to develop evidence-based recommendations, is not straightforward. Guidance production is, however, less challenging than the task of putting evidence-based recommendations into practice.NICE is very sensitive to this challenge as, since 1999, over 1500 pieces of NICE guidance have been published. A number of pieces of guidance relate to heart disease, including pharmaceutical agents, new medical technologies and clinical guidelines. Examples include guidelines on acute heart failure and atrial fibrillation, and advice on technologies including edoxaban and implantable cardioverter defibrillators.The research evidence is clear that a change in practice rarely comes about as a result of simply disseminating guidance on best practice. Simple dissemination is particularly ineffective if the guidance has not been produced by a well-respected, credible organisation. It is also clear from the literature that implementation is more successful when more than one approach is taken, and when there is alignment between efforts at organisational, local and national levels.At an organisational level, there should be support from the Board for quality improvement, with ongoing measurement of progress. Resources should be provided for targeted change programmes, particularly where new guidance suggests improvements are required. A systematic process for putting change in place should include identifying barriers to change, agreeing interventions to overcome the barriers and drive forward improvement and planning for implementation and evaluation.

Surgical clip migration following laparoscopic cholecystectomy as a cause of cholangitis.

Gallstone disease is a common surgical presentation, and laparoscopic cholecystectomy is the favoured method of surgical management. Ligation of the cystic duct is usually performed with surgical clips, which have the potential to migrate into the common bile duct with time. This paper describes a case of cholangitis secondary to clip migration in a 42-year-old male patient 9 years after the initial laparoscopic cholecystectomy. Magnetic resonance cholangiopancreaography imaging revealed a surgical clip lodged in dilated common bile duct. The patient was managed successfully by endoscopic retrograde cholangiopancreatography.