Question 1

While discussing health maintenance with a patient whom you recently diagnosed with a thoracic aortic aneurysm (TAA), you advise them to avoid all the following except?

Accepted Answer

A)  Daily walking exercise 
B)  Weight lifting 
C)  Isometric yoga 
D)  Contact sports A

Question 2

In addition to an annual screening echocardiogram of the ascending aorta, CT or MRI angiography of the entire aorta is recommended at what frequency for all first-degree relatives of individuals diagnosed with a thoracic aortic aneurysm/dissection (TAAD)?

Accepted Answer

A)  2 to 3 years 
B)  4 to 5 years 
C)  6 to 7 years 
D)  8 to 10 years B

Question 3

Which of the following valvular conditions is seen in patients genetically predisposed to developing familial thoracic aortic aneurysms and dissections (TAAD)?

Accepted Answer

A)  Bicuspid aortic valve 
B)  Mitral valve prolapse 
C)  Tricuspid aortic valve 
D)  Aortic regurgitation A

Question 4

Which of the following is not part of the aortic dissection bundle questions included in the initial history for all patients complaining of chest pain?

Accepted Answer

A)  Does the patient have Loeys-Dietz syndrome or a family history of Loeys-Dietz syndrome? 
B)  Does the patient's family history have a history of aortic dissection? 
C)  Does the patient have Marfan syndrome or a family history of Marfan syndrome? 
D)  Do physical findings suggest the patient may have undiagnosed Marfan syndrome?

Question 5

All genetic mutations resulting in familial thoracic aortic aneurysms and dissections (TAAD) appear to be inherited in an autosomal dominant manner. What is the risk that parents, siblings, and offspring of a proband will be affected?

Accepted Answer

A)  25% 
B)  50% 
C)  75% 
D)  100%

Question 6

While managing the care of your 18-year-old patient with a thoracic aneurysm with dissection (TAAD), your patient reveals to you that his grandfather suffered from the same condition but was not diagnosed until the age of 63 when he first developed symptoms. What is the phenomenon in which one individual in a family may present with symptoms at a young age, whereas another individual may present at an elderly age?

Accepted Answer

A)  Pentrance 
B)  Genetic heterogeneity 
C)  Genocopy 
D)  Variable expressivity

Question 7

A 22-year-old male presents to you with a chief complaint of chest pain. During your history and physical you review the aortic dissection bundle questions to which you gather a single "yes" answer. What is the appropriate next step in determining your diagnosis?

Accepted Answer

A)  Order an EKG 
B)  Order an emergent CT scan 
C)  Order a screening MRI within the next few days 
D)  Order a trans-thoracic echocardiogram

Question 8

After performing an annual screening echocardiogram on a patient with a known history of a thoracic aortic aneurysm (TAA) with a previous ascending aorta dimension of 3.0 cm the patient is now found to have an ascending aorta dimension of 4.4 cm. What is the appropriate management of this patient?

Accepted Answer

A)  Continue annual screening 
B)  Increase screening frequency to every 6 months 
C)  Refer the patient for prophylactic surgical repair 
D)  Order a CT scan to confirm the echocardiogram findings

Question 9

While examining a patient whom you suspect may have a thoracic aortic aneurysm (TAA) based on familial history, which of the following ocular abnormalities may you expect to find during your physical exam?

Accepted Answer

A)  Livedo reticularis 
B)  Retinitis pigmentosa 
C)  Iris floccule 
D)  Pinguecula

Question 10

While examining a 20-year-old white male during a routine physical he reveals to you that his younger brother was recently diagnosed with a thoracic aortic aneurysm with dissection (TAAD). Which of the following is not an acceptable method to screen your patient for a possible TAAD?

Accepted Answer

A)  MRI 
B)  Chest x-ray 
C)  CT 
D)  Echocardiogram

Exam 16: Familial Thoracic Aortic Aneurysms and Dissections

While discussing health maintenance with a patient whom you recently diagnosed with a thoracic aortic aneurysm (TAA) , you advise them to avoid all the following except?

In addition to an annual screening echocardiogram of the ascending aorta, CT or MRI angiography of the entire aorta is recommended at what frequency for all first-degree relatives of individuals diagnosed with a thoracic aortic aneurysm/dissection (TAAD) ?

Which of the following valvular conditions is seen in patients genetically predisposed to developing familial thoracic aortic aneurysms and dissections (TAAD) ?

Which of the following is not part of the aortic dissection bundle questions included in the initial history for all patients complaining of chest pain?

All genetic mutations resulting in familial thoracic aortic aneurysms and dissections (TAAD) appear to be inherited in an autosomal dominant manner. What is the risk that parents, siblings, and offspring of a proband will be affected?

A 22-year-old male presents to you with a chief complaint of chest pain. During your history and physical you review the aortic dissection bundle questions to which you gather a single "yes" answer. What is the appropriate next step in determining your diagnosis?

After performing an annual screening echocardiogram on a patient with a known history of a thoracic aortic aneurysm (TAA) with a previous ascending aorta dimension of 3.0 cm the patient is now found to have an ascending aorta dimension of 4.4 cm. What is the appropriate management of this patient?

While examining a patient whom you suspect may have a thoracic aortic aneurysm (TAA) based on familial history, which of the following ocular abnormalities may you expect to find during your physical exam?

While examining a 20-year-old white male during a routine physical he reveals to you that his younger brother was recently diagnosed with a thoracic aortic aneurysm with dissection (TAAD) . Which of the following is not an acceptable method to screen your patient for a possible TAAD?