Atenolol versus losartan in children and young adults with Marfan's syndrome

BACKGROUND : Aortic-root dissection is the leading cause of death in Marfan's syndrome. Studies suggest that with regard to slowing aortic-root enlargement, losartan may be more effective than beta-blockers, the current standard therapy in most centers. METHODS : We conducted a randomized trial comparing losartan with atenolol in children and young adults with Marfan's syndrome. The primary outcome was the rate of aortic-root enlargement, expressed as the change in the maximum aortic-root-diameter z score indexed to body-surface area (hereafter, aortic-root z score) over a 3-year period. Secondary outcomes included the rate of change in the absolute diameter of the aortic root; the rate of change in aortic regurgitation; the time to aortic dissection, aortic-root surgery, or death; somatic growth; and the incidence of adverse events. RESULTS : From January 2007 through February 2011, a total of 21 clinical centers enrolled 608 participants, 6 months to 25 years of age (mean [+/- SD] age, 11.5 +/- 6.5 years in the atenolol group and 11.0 +/- 6.2 years in the losartan group), who had an aorticroot z score greater than 3.0. The baseline-adjusted rate of change (+/- SE) in the aortic-root z score did not differ significantly between the atenolol group and the losartan group (-0.139 +/- 0.013 and -0.107 +/- 0.013 standard-deviation units per year, respectively; P = 0.08). Both slopes were significantly less than zero, indicating a decrease in the degree of aortic-root dilatation relative to body-surface area with either treatment. The 3-year rates of aortic-root surgery, aortic dissection, death, and a composite of these events did not differ significantly between the two treatment groups. CONCLUSIONS : Among children and young adults with Marfan's syndrome who were randomly assigned to losartan or atenolol, we found no significant difference in the rate of aorticroot dilatation between the two treatment groups over a 3-year period

Nature and frequency of genetic disease

No abstract available

Lesson learned from the International Registry of Acute Aortic Dissection (IRAD)

Acute aortic dissection (AAD) is a life-threatening disorder that is challenging to diagnose and with defined optimal therapies and outcomes. It is associated with high morbidity and mortality rates, in spite of new management approaches developed over the past few decades. In this article we review lessons learned from the 20-year IRADRegistry experience and its nearly 100 publications. The International Registry of Acute Aortic Dissection (IRAD) is an ongoing registry, which was established in 1996 with the aim to collect data and provide information on this rare disease in order to provide better decision making on clinical practice and surgical management. Acute aortic dissection, including intramural hematoma, is defined as onset of symptoms within 14 days to hospital admission. After 20 years of enrollment, more than 8000 patients with AAD have been collected worldwide, roughly two-thirds with type Aand one-third with type B. Data shows trend for a reduced overall in hospital mortality over the years, in particular for those operated on for type AAAD, due to the improvement in operative technologies, organ perfusion and surgeons taking on even high-risk patients. In contrast, for type B AADendovascular repair has been more frequently adopted in recent years but without a significant demonstrable reduction of in hospital mortality, although an increased survival rate at 5 years was observed. Data suggest an increase in surgical intervention treatment for both type Aand B AAD, particularly in B due to expansion of endovascular treatment. While expanded surgical repair for type Ahas shown a trend for a better outcome, this was not observed for type B patients. In addition, debate continues about endovascular treatment of uncomplicated type B and IMH