Cardiology Intensive Board Review, 3 ed.

Aorta

Craig R. Asher • Gian M. Novaro

QUESTIONS

Case 1 (Questions 1 to 3)

A 78-year-old woman is referred to cardiology clinic for management of aortic regurgitation. The patient has no cardiac risk factors except mild hypertension (HTN) on monotherapy and has not previously undergone cardiac testing. A review of systems is notable for recent onset of headaches and myalgias.

Physical Examination

Blood pressure (BP)—138/78 mmHg in both arms; pulse—62 bpm.

Funduscopic examination reveals no changes consistent with hypertensive retinopathy. The heart examination is notable for a normal S1 and increased intensity S2 (A2). An S4 gallop, II/VI diastolic decrescendo murmur heard best at the right sternal border, and III/VI early-peaking systolic ejection murmur heard at the left sternal border are present. There is no systolic ejection click. The carotid pulse is of normal intensity and contour and the pulses in the upper and lower extremities are strong and equal.

Electrocardiogram (ECG) reveals sinus rhythm with nonspecific ST changes.

1.What is the most likely explanation for the patient’s heart murmur?

a.Bicuspid aortic valve with severe aortic regurgitation

b.Bicuspid aortic valve with severe aortic stenosis and moderate aortic regurgitation

c.Degenerative severe aortic valve stenosis and moderate aortic regurgitation

d.Aortic dilatation with moderate aortic regurgitation and mild aortic stenosis

A transthoracic echocardiogram (TTE) is performed showing normal left ventricular (LV) size and function with a trileaflet aortic valve. Aortic measurements are as follows: sinus of Valsalva—4.0 cm; sinotubular junction—4.4 cm; mid-ascending aorta—4.5 cm with moderate effacement of the sinotubular junction. Peak and mean aortic gradients are 22/13 mmHg with moderate (2+) aortic regurgitation. A small circumferential pericardial effusion is present. Laboratory tests reveal an erythrocyte sedimentation rate of 74.

2.What additional test would be most helpful in determining the etiology of the patient’s aortic dilatation and aortic regurgitation?

a.Coronary angiogram and aortography

b.Magnetic resonance angiography (MRA) of the great vessels

c.Computed tomographic angiography (CTA) of the ascending aorta

d.Transesophageal echocardiography (TEE)

3.What is the most likely diagnosis to explain the patient’s aortic dilatation and aortic regurgitation? See Figure 6.1.

a.Takayasu arteritis

b.Degenerative aortic disease

c.Connective tissue disorder

d.Giant cell arteritis

Figure 6.1

Case 2 (Questions 4 to 6)

An 18-year-old woman presents for her annual physical examination. She had a brother with Marfan syndrome who was 24 years old when he died suddenly. She is active and asymptomatic.

Physical Examination

5 feet 7 inches (170 cm) and 150 pounds (68 kg).

Arm span-to-height ratio = 1.07.

Head and neck examination is notable for a high-arched palate and a slit-lamp examination shows ectopia lentis. Musculoskeletal examination is notable for a pectus carinatum and positive wrist and thumb sign. Cardiac examination is notable for a mitral valve click and a soft murmur of mitral regurgitation.

4.What additional testing is needed to determine whether this young woman has Marfan syndrome?

a.TTE

b.Genetic testing

c.CT angiogram of the aorta

d.No additional testing

A TTE is performed that shows mitral valve prolapse with mild (1+) mitral regurgitation. The aortic root is dilated at 5.0 cm with effacement of the sinotubular junction and a mid-ascending aortic measurement of 3.6 cm. The aortic valve is trileaflet with no aortic regurgitation.

5.What is the most important recommendation to be made to this patient?

a.Repeat the TTE in 6 months

b.Initiate a β-blocker

c.Avoid strenuous exertion, contact sports, and pregnancy

d.Elective aortic replacement

The patient wishes to schedule surgery but prefers to wait 2 months until the end of the school year. Her father who is an internal medicine physician has read about the potential benefit of angiotensin receptor blockers (ARBs) for patients with aneurysms and asks your advice regarding treatment.

6.What is the postulated mechanism of action whereby ARBs reduce progression of aortic disease in Marfan syndrome?

a.Reduction of BP through angiotensin II type 1 receptor blockade

b.Complete blockade of the renin–angiotensin–aldosterone system

c.Reduction in activity of transforming growth factor (TGF)-β

d.Increase production of matrix metalloproteinases (MMPs)

Case 3 (Questions 7 and 8)

A 67-year-old man with long-standing HTN presents to the emergency room (ER) with sudden-onset chest pain described as ripping in quality, subsiding since its onset. He underwent a cardiac catheterization 6 months previously that showed a 40% lesion in the mid-left anterior descending coronary artery. His medications include aspirin, gemfibrozil, and nifedipine.

Physical Examination

He appears diaphoretic. HR—110 bpm; BP—106/54 mmHg (right arm); 72/35 mmHg (left arm). Jugular venous pressure—12 cm H2O. Heart sounds are soft and there is no audible systolic or diastolic murmur. Left radial and brachial pulses are weak.

ECG on presentation shows ST elevations in the inferior leads and low voltage.

Chest X-ray (CXR) shows cardiomegaly with a globular-shaped heart and interstitial edema.

7.Which of the following is the first diagnostic test that should be performed?

a.Cardiac enzymes

b.MRA of the aorta

c.TTE

d.Cardiac catheterization

A TTE was performed showing a pericardial effusion and signs of cardiac tamponade. A CT angiogram is also performed and shown in Figure 6.2.

8.What intervention should be performed next for the management of this patient?

a.Cardiac catheterization

b.Emergent aortic surgery

c.Swan-Ganz catheterization

d.Pericardiocentesis

Case 4 (Questions 9 and 10)

A 36-year-old man with a bicuspid aortic valve develops sudden onset of headache, mental status changes, and unequal pupils. He is rushed to an ER and a head CT scan is done that shows an intracranial bleed. BP on presentation is 158/78 mmHg.

Figure 6.2

Except for a history of HTN, he has no known medical problems and no history of drug abuse. A visit to his physician’s office 1 week earlier revealed a BP of 120/75 mmHg on metoprolol and ramipril.

9.What is the most likely reason for the patient’s intracranial bleed?

a.Hypertensive crisis

b.Aortic dissection

c.Cerebral aneurysm rupture

d.Mycotic aneurysm associated with endocarditis

10.What other structural abnormality is most commonly associated with coarctation of the aorta?

a.Atrial septal defect

b.Ventricular septal defect

c.Pulmonary stenosis

d.Mitral valve prolapse

Case 5 (Questions 11 and 12)

A 74-year-old man presents to the ER with upper back pain ongoing for 3 hours. The pain is described as sharp and severe occurring at rest. He has no associated symptoms of shortness of breath, chest pain, or presyncope. His past medical history is notable for a coronary artery bypass graft (CABG) 2 years previously, HTN, and ongoing tobacco use. At the time of his CABG, he was noted to have a 4.4-cm ascending aortic aneurysm that was not repaired. His medications include aspirin, an angiotensin-converting enzyme inhibitor, and a β-blocker.

Physical Examination

BP—180/110 mmHg.

Pulse rate—90 bpm.

Lung and cardiac examinations are unremarkable and no cardiac murmur is heard. The abdomen is mildly tender with no bruit. Pulses are equal but diminished in the lower extremities.

ECG shows sinus rhythm with nonspecific ST changes and an old inferior myocardial infarction (MI).

Laboratory tests including cardiac enzymes, liver function tests, amylase, and lipase are normal.

11.What is the most appropriate diagnostic procedure to perform next?

a.TTE

b.TEE

c.CTA of the chest and abdomen

d.Aortography

A CT scan demonstrates an ascending aortic aneurysm of 4.8 cm and a descending thoracic aortic aneurysm of 6.0 cm but no evidence of dissection. There is no abdominal aortic aneurysm (AAA). The patient continues to have ongoing pain despite high doses of β-blockers, sodium nitroprusside, and opioid analgesics.

ECG and cardiac enzymes remain normal.

D-dimer level is >500 ng/mL.

12.What is the most appropriate next decision for management?

a.Cardiac catheterization

b.MRA of the chest/aorta

c.TEE

d.Intensify medical therapy

Case 6 (Question 13)

A 62-year-old man presents for a routine annual examination. He has a history of HTN that is managed with monotherapy. He is active and has no symptoms.

Physical Examination

BP—162/88 mmHg in both arms. Pulse rate—70 bpm. Heart and lung examination is unremarkable. Abdominal examination reveals a pulsatile mass.

ECG shows sinus rhythm and a complete right bundle branch block.

Abdominal ultrasound shows an infrarenal AAA of 4.2 cm.

13.What is the most appropriate management step?

a.Initiate a β-blocker and repeat ultrasound in 6 months

b.Initiate a β-blocker and repeat ultrasound in 3 months

c.No medical therapy and repeat ultrasound in 1 year

d.No medical therapy and repeat ultrasound in 2 years

Case 7 (Questions 14 and 15)

A 76-year-old man presents to the ER with severe sharp chest pain that began 2 hours previously. He has a history of HTN and had CABG 3 years ago after an MI. He continues to smoke. The CABG was performed off-pump because of severe atheroma in the ascending aorta seen by intraoperative TEE. The patient’s pain has not subsided with the initiation of IV heparin, nitroglycerin, and β-blockers. The pain is different in character from the pain before his MI.

Physical Examination

BP—160/94 mmHg.

Pulse—76 bpm.

Heart and lung sounds are normal. Pulses are diminished in the lower extremities.

ECG shows a left bundle branch block.

Cardiac enzymes are normal × 1.

Despite severe atheroma in the aorta, the physician taking care of the patient is not convinced that he does not have an acute coronary syndrome and performs a cardiac catheterization. It shows that the grafts are patent and there is no culprit lesion in the native vessels. He then decides to perform aortography and a focal outpouching is seen in the aortic wall in the distal ascending aorta (Fig. 6.3A). Contrast dye collects slowly in this region. The patient’s chest pain is intensifying. A TEE is also performed (Fig. 6.3B).

Figure 6.3

14.What is the correct diagnosis?

a.Aortic dissection

b.Aortic aneurysm

c.Intramural hematoma

d.Penetrating aortic ulcer

15.What is the most appropriate next management step to take?

a.Medical management with β-blockers and afterload reduction

b.Transfer to the operating room immediately for replacement of the ascending aorta

c.Medical management and obtain a CT chest/aorta

d.Medical management and obtain an MRA of the aorta

Case 8 (Questions 16 and 17)

A 45-year-old woman presents with discomfort in her left leg with walking, dizziness, headaches, and a cold right hand. She has no chest pain or shortness of breath. There is no significant past medical history and she does not smoke.

Physical Examination

BP—170/82 mmHg (left arm) and 140/68 mmHg (right arm). Lung sounds are clear. Cardiac examination is notable for a normal S1 and S2 and II/VI diastolic decrescendo murmur at the left sternal border. The right brachial pulse is diminished and lower extremity pulses are diminished. A bruit is heard over the left carotid artery and right subclavian artery.

16.What test would be most useful for diagnosing the patient’s condition?

a.TEE

b.Carotid duplex ultrasound

c.Magnetic resonance imaging (MRI) of the head

d.Angiography

17.What is the most likely diagnosis?

a.Behçet disease

b.Relapsing polychondritis

c.Giant cell arteritis

d.Takayasu arteritis

Case 9 (Questions 18 to 21)

A 21-year-old woman with Turner syndrome has a history of surgical repair for periductal coarctation of the aorta at age 4 years. Her presentation was for HTN and heart failure, both of which resolved after the procedure. She was lost to follow-up after childhood and recently reestablished with a cardiologist. She has been experiencing dyspnea and claudication in the past year.

Physical Examination

BP—188/94 mmHg (left arm); 192/100 mmHg (right arm); 100/60 mmHg (right leg).

Pulses are notable for normal upper extremity and reduced lower extremity pulses with a brachial–femoral delay. Cardiac examination is notable for normal S1 and paradoxically split S2 with an ejection click and S4 gallop. A continuous murmur III/VI in intensity is heard under the left scapula.

18.Which of the following statements is correct regarding the echocardiographic image from this patient obtained from the suprasternal notch (Fig. 6.4)?

a.Pressure gradients are usually underestimated using the simplified Bernoulli equation

b.Pressure gradients are usually accurate using the simplified Bernoulli equation

c.Pressure gradients shown are not consistent with severe coarctation

d.Presence of systolic and diastolic flow is consistent with severe coarctation

Figure 6.4

19.Which of the following statements is correct regarding the echocardiographic image from this patient obtained from the abdominal aorta (Fig. 6.5)?

a.The hallmark of coarctation is the presence of low systolic velocities

b.The presence of coarctation cannot be determined without knowing the timing of the pulse delay relative to aortic ejection

c.The hallmark of coarctation is the presence of persistent antegrade flow in diastole

d.The presence of coarctation cannot be determined without additional Doppler images proximal to the coarctation site

Figure 6.5

20.Which of the following recommendations is most appropriate regarding reintervention in this patient (Fig. 6.6)?

a.Surgery is generally recommended

b.No intervention should be performed until maximal medical therapy is attempted

c.Balloon angioplasty with or without stents is generally recommended

d.There is no consensus and either surgery or balloon aortoplasty (with or without stenting) are equal options

Figure 6.6

21.Which of the following is an indication for aortic coarctation intervention?

a.Symptomatic patient with dyspnea at rest, regardless of the coarctation gradient

b.Asymptomatic patient with normal BP at rest and with exercise and a peak-to-peak gradient across the coarctation site of 19 mmHg

c.Asymptomatic patient with a peak-to-peak gradient across the coarctation site of 15 mmHg with extensive collaterals

d.Symptomatic patient with a bicuspid aortic valve and severe aortic regurgitation undergoing aortic valve replacement and a peak-to-peak gradient across the coarctation of 15 mmHg

Case 10 (Question 22)

A 30-year-old man is referred to cardiology clinic for evaluation of a heart murmur. He had an uneventful childhood except that on four separate occasions he fractured his arms or legs requiring multiple surgical repairs. He has no family history of heart disease or congenital abnormalities.

Physical Examination

Normal stature. Vital signs are normal. Arm span-to-height ratio is normal.

No pectus deformity, scoliosis, wrist sign. Cardiac examination is notable for a decreased S1 and normal S2 intensity with a III/VI diastolic decrescendo murmur and no gallops. Pulses are normal and the extremities are hypermobile. No abnormality of the skin is present.

TTE shows a dilated sinus of Valsalva of 4.7 cm with severe aortic regurgitation. Left ventricular ejection fraction (LVEF) = 65% with LV dimensions of (diastole 6.4 cm; systole 5.1 cm).

22.What is the most likely diagnosis for this patient?

a.Ehlers-Danlos syndrome

b.Marfan syndrome

c.Osteogenesis imperfecta

d.Homocystinuria

Case 11 (Questions 23 and 24)

A 65-year-old man presents to the ER with severe, tearing lower back pain that started while he was shoveling snow. He has a history of poorly controlled HTN and coronary artery disease with a stent to the left anterior descending coronary artery 4 months previously. Other medical problems include severe O2-dependent chronic obstructive pulmonary disease.

Physical Examination

BP—190/110 mmHg.

Pulse—90 bpm.

Cardiac examination is notable for a normal S1 and S2 with an S4 gallop and II/VI early-peaking systolic ejection murmur at the left sternal border.

ECG shows sinus rhythm and no ST changes.

An initial set of cardiac enzymes is normal.

23.What is the most appropriate initial medical therapy?

a.Intravenous metoprolol

b.Intravenous diltiazem

c.Intravenous enalapril

d.Intravenous nitroprusside

A CT angiogram is performed (Fig. 6.7).

24.Which of the following statements regarding the appearance of this form of acute aortic syndrome is most accurate?

a.There is low attenuation of the aortic wall

b.It is not continuous

c.Intimal calcium is nondisplaced

d.It is circumferential or crescentic

Case 12 (Questions 25 to 28)

A 44-year-old man is admitted to the hospital because of a left hemisphere stroke with right arm and leg weakness. He has no history of HTN or smoking, although his total cholesterol level is 334. ECG shows sinus rhythm. Carotid duplex ultrasound shows less than 20% obstruction bilaterally. Head CT demonstrates a recent stroke in the left cortex in the region of the middle cerebral artery. A TTE shows normal valves, chamber sizes, and LV function.

Figure 6.7

25.Which test is most likely to reveal the etiology of the patient’s stroke?

a.TTE with bubble study

b.30-Day event recorder

c.MRA of the head and neck

d.TEE

26.Which of the following medical regimens is most appropriate for a patient with a cardioembolic stroke and the following finding seen on TEE (Fig. 6.8)?

a.Statin and aspirin

b.Statin and warfarin

c.Aspirin and dipyridamole

d.Statin, antiplatelet agent, and warfarin

Figure 6.8

27.Which of the following atheromatous plaques is least likely to be associated with a cardioembolic event?

a.3-mm plaque with severe calcification and no mobile components

b.4-mm plaque with small mobile components and no calcification

c.5-mm plaque with multiple mobile components, no calcification, and a small ulceration

d.2-mm plaque with calcification, a small mobile component, and large ulceration

28.Which statement is correct regarding performing CABG in a similar patient with aortic arch atheroma and focal areas of atheroma in the ascending aorta?

a.Palpation of the aorta by the surgeon for calcified plaque correlates with findings of atheroma by TEE.

b.Alternative sites for cross-clamping or cannulation may reduce stroke risk.

c.Aortic arch endarterectomy is recommended.

d.Replacement of the ascending aorta is recommended because of increased likelihood of stroke.

Case 13 (Question 29)

A 21-year-old man is referred to a cardiology clinic for exertional dyspnea. As a child he was evaluated for a heart murmur by a pediatric cardiologist.

Physical Examination

BP—140/84 mmHg (left arm); 120/68 (right arm).

Cardiac examination is notable for a normal S1 and increased intensity S2/A2, absence of an ejection click, and III/VI systolic ejection murmur heard best in the first right intercostal space radiating to the neck and increases with expiration. There is a thrill in the suprasternal notch. The left carotid and brachial pulses are diminished relative to the right carotid and brachial pulses.

29.What diagnosis best explains the patient’s disorder?

a.Patent ductus arteriosus

b.Coarctation of the aorta

c.Supravalvular pulmonary stenosis

d.Williams syndrome

Case 14 (Questions 30 to 32)

A 30-year-old man with a history of congenital heart disease is referred to you because of symptoms of dysphagia and an abnormal CXR. As a child he was told that his CXR was abnormal because of an “aortic anomaly” and that it was benign.

Physical Examination

Vital signs are normal.

Cardiac examination is notable for normal intensity S1 and S2 heart sounds. An ejection click is heard in the right upper sternal border (RUSB) though no murmur is audible. Upper and lower extremity pulses are equal.

30.The CXR (Fig. 6.9) shows what abnormality?

a.Bovine aortic arch

b.Cervical aortic arch

c.Right-sided aortic arch

d.Aberrant right subclavian artery

Figure 6.9

31.What is the most common congenital defect associated with the aortic anomaly seen in Question 30?

a.Tetralogy of Fallot

b.Truncus arteriosus

c.Atrial septal defect

d.Transposition of the great vessels

32.The patient’s dysphagia is likely caused by what abnormality associated with his aortic anomaly?

a.Aberrant left subclavian artery and diverticulum of Kommerell

b.Coarctation of the aorta

c.Isolated aberrant right subclavian artery

d.Interrupted aortic arch

Case 15 (Questions 33 and 34)

A 30-year-old man presents for follow-up after undergoing a surgical coarctation repair at age 9. He has no records and is uncertain of the type of repair. He has not had any testing done in the past 10 years. He has used antibiotic prophylaxis for dental procedures. He takes no other medications. He feels well, is active, and has no complaints.

Physical Examination

BP—138/72 mmHg (right arm); 126/70 mmHg (left arm); pulse—80 bpm, regular.

Heart sounds are of normal intensity. An aortic ejection click is present in the RUSB. There is a brief, short duration systolic ejection murmur in the RUSB without a diastolic murmur. Upper and lower extremity pulses are equal and there is no radial or brachial to femoral delay.

33.Which test would be least appropriate for this patient?

a.TTE

b.CTA of the chest/aorta

c.Exercise treadmill stress test

d.24-Hour Holter monitoring

34.How often should the coarctation repair site be evaluated by chest imaging in this patient (CTA or MRA)?

a.Generally every 5 years

b.Generally every 10 years

c.Generally every 2 years

d.Not recommended unless there is specific concern

Case 16 (Questions 35 and 36)

A 38-year-old man is referred for a cardiac surgical evaluation for bicuspid aortic regurgitation. He has a long-standing aortic valve disorder for 10 years. He is fully active with no limitations. He plays tennis on a regular basis.

Physical Examination

BP—120/40 mmHg; pulse—68 bpm. Cardiac examination is notable for a reduced intensity S1 and increased intensity S2/A2. Three murmurs are present, a mid-peaking crescendo/decrescendo systolic ejection murmur beginning after an ejection click, a long-duration high-pitched diastolic decrescendo murmur along the left sternal border and a low-pitched mid-diastolic rumble heard at the apex.

Stress echocardiography is performed and shows a bicuspid aortic valve with right–left fusion. There is calcification of the raphe between the conjoined leaflets. Peak/mean gradients = 30/15 mmHg. Holodiastolic flow reversal is present in the descending aorta. The aortic root measures 4.0 cm and the mid-ascending aorta is 3.9 cm with no sinotubular junction effacement. LV cavity measurements are as follows: LVIDd—5.8 cm; LVIDs—3.9 cm; LVEF—58%. There is reduction in LV cavity size with 13 metabolic equivalents of exercise and no symptoms.

35.What is the most appropriate surgical recommendation?

a.Elective aortic valve surgery and ascending aortic grafting

b.Elective aortic valve surgery without ascending aortic grafting

c.Medical therapy/observation and add a β-blocker

d.Observation only

Six months later the patient has developed dyspnea with moderate exertion and a significant reduction in exercise tolerance. Repeat echocardiogram shows similar LV cavity dimensions and LVEF calculated at 57%. Aortic root dimension is 4.0 cm and the mid-ascending aorta is 4.6 cm.

36.What is the appropriate recommendation at this time?

a.Aortic valve replacement

b.Aortic valve repair

c.Aortic valve replacement and aortic graft

d.Stress echocardiogram to confirm change in exercise tolerance

Case 17 (Questions 37 to 40)

A 66-year-old man is seen by his internist for an annual evaluation. Past medical history is notable for HTN and tobacco use. His BP is 136/80 mmHg on monotherapy. Cardiac and abdominal examinations are unremarkable.

He has a friend who was detected to have an AAA on routine screening and asks his physician whether it is indicated for him to be screened.

37.Which of the following statements regarding AAA screening is correct?

a.Consensus guidelines recommend routine screening of all women >60 years of age or older

b.Screening men >65 years old is associated with a reduction in aneurysm-related deaths compared with unscreened males of similar ages

c.Sensitivity and specificity of ultrasound screening in appropriate patients in accredited laboratories are >70%, respectively

d.After a negative screening examination in a man aged 65 years or older, a repeat examination should be performed 5 years later

A screening abdominal ultrasound is performed in this patient and shows an infrarenal AAA of 5.5 cm. The patient is advised to undergo repair though wishes to consider the option of an endovascular stent graft (EVAR) rather than an open repair.

38.Which of the following statements is true regarding recommendations for AAA repair?

a.Repair is indicated for any AAA > 5.0 cm

b.Size cutoffs for AAA repair should be based on age, weight, and height

c.Inflammatory or infectious aneurysms should be repaired at any size

d.Women should undergo repair only for AAA > 5.5 cm

39.Which statement regarding EVAR is correct?

a.Open repair and EVAR are associated with similar 30-day mortality

b.Open repair and EVAR are associated with similar long-term mortality

c.EVAR is associated with decreased late complications compared with open repair

d.EVAR is associated with less repeat interventions compared with open repair

40.Which of the following statements regarding endoleaks is correct?

a.They occur with a similar prevalence with EVAR and open procedures

b.Endoleaks may occur as a result of retrograde flow of small arterial branches back into the aneurysm sac

c.They are rare and generally do not lead to repeat procedures

d.Type IV endoleaks (leakage through graft material) are the most common

Case 18 (Questions 41 and 42)

A 38-year-old woman is admitted to the internal medicine service for chest pain. She is experiencing sharp chest pain unrelated to exertion and dyspnea for 2 days. Her family history is unknown since she was adopted. Past medical history is not well defined but notable for an uncharacterized connective tissue disorder. She was told at a younger age to avoid pregnancy.

Admission ECG and cardiac enzymes are negative. She is unable to exercise because of her symptoms and therefore is sent for an adenosine nuclear stress test. The test shows mild anteroseptal ischemia with no ECG changes.

The cardiology service is consulted for a cardiac catheterization.

Physical Examination

She is thin and in mild distress. Vital signs are normal. General examination and skin examination are notable for a translucent appearance to the skin, joint hypermobility, hyperextensible skin, and bruises. There are no findings suggestive of Marfan syndrome. Cardiac examination reveals a normal S1 and S2 and a soft diastolic decrescendo murmur at the right sternal border.

41.What do you recommend?

a.Coronary angiography

b.No further testing

c.Cardiac MRI to assess for coronary anomalies

d.CTA of the chest and aorta

42.What should be done to more definitively characterize the patient’s condition?

a.Diagnosis based on clinical criteria

b.DNA sequencing

c.Blood analysis for chromosomal abnormalities

d.Clinical laboratory markers of inflammation

Case 19 (Questions 43 to 45)

A 24-year-old man is referred to cardiology clinic for consultation regarding an aortic aneurysm. He has a family history of ascending aortic aneurysms. Echocardiography showed a trileaflet aortic valve and mid-ascending aortic aneurysm of 4.4 cm that is confirmed by CTA.

Physical Examination

BP—120/64 mmHg; pulse—70 bpm. Head and neck examination is notable for widely set eyes. The oropharynx is shown (Fig. 6.10). Cardiac and skin examinations are normal.

Figure 6.10

43.What is the patient’s diagnosis?

a.Cogan syndrome

b.Loeys-Dietz syndrome

c.Ormond disease

d.Behçet disease

44.What is the gene defect associated with this condition?

a.COL3A1

b.TGFBR1 or TGFBR2

c.ACTA2

d.FBN1

45.What further recommendation is correct regarding management of this patient?

a.Start a β-blocker

b.Start an ARB

c.Recommend aortic repair

d.Observation only

Case 20 (Question 46)

A 40-year-old man is transferred via air ambulance to a level 1 trauma center after a motor vehicle accident (MVA). He had a head-to-head collision with another car traveling at a high speed and was propelled forward but was partially restrained by his seat belt and deployed air bags.

Physical Examination

BP—162/62 mmHg (right arm); HR—110 bpm. General condition notable for intubated, sedated man with a cervical collar. Cardiac examination is notable for normal heart sounds and a soft systolic murmur. Lower extremities are mottled and pulseless.

ECG shows sinus tachycardia with no ST changes.

CXR shows no broken ribs bilaterally, a widened mediastinum, and left pleural effusion.

46.Which of the following diagnoses are most likely for this patient?

a.Complete aortic transection

b.Ascending aortic dissection and cardiac tamponade

c.Descending aortic partial transection with pseudoaneurysm

d.Descending aortic intramural hematoma/dissection

Case 20 (Questions 47 and 48)

A 79-year-old man with HTN underwent a routine CXR and is found to have a thoracic aneurysm. He is asymptomatic with well-controlled BP on a β-blocker. A CT angiogram of the chest is performed showing a descending thoracic aortic aneurysm of 5.4 cm distal to the left subclavian artery with a chronic dissection. The aneurysmal segment does not extend into the abdomen. The ascending aorta is 4.5 cm in the mid-portion and 4.0 cm in the mid-arch. The patient has no other comorbid conditions.

47.What recommendation is most appropriate for this patient based on current guidelines?

a.Open repair is recommended

b.TEVAR (thoracic endovascular aortic repair) is recommended

c.Medical therapy is recommended

d.An elephant trunk procedure is recommended

48.Which statement is correct comparing TEVAR with open repair for thoracic aortic aneurysms?

a.TEVAR is associated with higher rates of hospital mortality

b.TEVAR is associated with higher risks of paraplegia

c.TEVAR is associated with a 30-day risk of endoleaks of 10%

d.TEVAR is associated with lower long-term mortality in randomized trials

Case 21 (Questions 49 and 50)

A 45-year-old woman with a history of fibromuscular dysplasia presents to the ER with an acute ST-elevation inferior MI. She is taken immediately to the cardiac catheterization laboratory for primary percutaneous intervention of the right coronary artery (RCA). The first injection of the RCA shows a dissection extending from the ostium to the posterior descending artery. A subsequent aortogram after stenting of the RCA is performed (Fig. 6.11).

Figure 6.11

49.What does the aortogram show?

a.Aortic root aneurysm

b.Aortic root pseudoaneurysm

c.Anomalous RCA from the left cusp

d.Aortic root localized dissection

50.What further evaluation or management do you recommend for this patient?

a.Observation and medical therapy

b.Open heart surgery

c.Intravascular ultrasound of the RCA

d.TEE