Marfans Flashcards
How is marfans inherited
Autosomal dominant
What is the likelihood for cardiac disease in Marfan syndrome
80%
What accounts for the majority of the morbidity and mortality from Marfan syndrome
Aortic aneurysms and aortic dissection
What percentage of adults with Marfan syndrome have aortic root dilation
60-80%
What is the risk of aortic dissection in pregnancy with aortic root greater than 4cm
10% risk of dissection
What is the risk of aortic rupture or dissection without aortic root dilation
1%
When should patients with Marfan syndrome be counseled against pregnancy
All Marfan syndrome patients should be counseled against pregnancy or offered termination of pregnancy due to a 10% of dissection or ruptured of the aorta with aortic root more than 4cm and less than 1% risk of rupture and dissection with normal aorta
What aortic root diameter is an indicated for preconception repair before pregnancy is planned
Aortic root dimension of more than 4.5cm.
Surgery doesn’t eliminate the risk for rupture or dissection of the aorta but it decreases the risk
Marfan syndrome is Associated with what other abnormalities
Joint hypermobility, pectus excavatum, disproportionally wide arm span, scoliosis, arachnodactyl, ectopic lentis
List three important things to avoid in patients with Marfan syndrome
Avoid hypertension
Avoid tachycardia
Avoid valsalva
Goal heart rate for Marfan patients
Less than 110bpm after exercise and less than 90bpm at rest.
Use labetalol or metoprolol to maintain this heart rate
Can Epidural be administered to a patient with Marfan syndrome
Yes it can epidural is acceptable and may be recommended to minimize tachycardia due to pain and to minimize urge for Valsalva during delivery
When should the Cesarean delivery be used in Marfan syndrome
Cesarean may benefit patients with aortic root diameter greater than 4 cm, in patients with aortic root the section or heart failure
