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Marfan Syndrome Treatment

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Questions and Answers

What does the retrospective evaluation of children with Marfan syndrome suggest about beta-blocker treatment?

It accelerated aortic growth rate

It had no impact on aortic growth rate

It prevented aneurysm formation

It was associated with a reduced aortic growth rate (correct)

What did randomized trials comparing an ARB to a beta blocker in patients with Marfan syndrome find?

Significant reduction in aortic root growth rate with ARB compared to beta blocker

Significant reduction in clinical events with ARB compared to beta blocker

No significant difference in aortic root growth rate or clinical events between the 2 treatment groups (correct)

Higher clinical events with ARB compared to beta blocker

What did multiple trials comparing the addition of an ARB to beta-blocker therapy in patients with Marfan syndrome show?

Prevention of aneurysm formation

Acceleration of aortic root growth rates over a 3- to 5-year follow-up

Reduction of aortic root growth rates over a 3- to 5-year follow-up (correct)

No impact on aortic root growth rates over a 3- to 5-year follow-up

What was the outcome of losartan treatment in a small, nonrandomized open-label study of children with Marfan syndrome who had previously had rapid aortic root growth?

It dramatically slowed aortic root growth Signup and view all the answers

What did a meta-analysis confirm about combination therapy of ARB and beta-blocker in patients with Marfan syndrome?

Slower aortic growth rates Signup and view all the answers

What is the approximate reoperation rate over 20 years for adult patients with certain cardiac conditions?

10% to 15% Signup and view all the answers

What should symptoms like dyspnea, fatigue, edema, or ascites in adult patients indicate?

Potential right ventricular failure Signup and view all the answers

What is the likely consequence of recurrent stenosis in adult patients?

Catheter intervention to stent the proximal pulmonary arteries Signup and view all the answers

What is the potential implication of ventricular dysfunction precluding further surgery in adult patients?

Consideration of transplantation Signup and view all the answers

What may preclude heart transplantation in adult patients?

Bleeding from MAPCAs and prior pleural scars Signup and view all the answers