Question 3: 87 yr old female with history of hypertension had negative stress echo, presents with acute chest pain. What’s the diagnosis?

Dx-Aneurysm of the Circumflex Coronary Artery

In this patient the cause of the coronary artery aneurysm was atherosclerotic disease and could best be managed by surgical intervention. The patient ultimately decided against any intervention.

What is a coronary artery aneurysm?

• A coronary aneurysm is defined as a 50% or greater increase in coronary arterial diameter compared to adjacent arterial segments
• Most commonly involves the right coronary artery (RCA) and second most common site is the left anterior descending coronary artery (LAD).

Coronary Artery Aneurysms: Common Causes

• There are a range of causes of coronary artery aneurysms that are listed in the following chart. The categories can best be defined as ranging from unusual vascular conditions (Kawasaki disease, Takayasu aortitis), to atherosclerotic disease to iatrogenic or traumatic causes (cardiac catheterization) to infection.
• Atherosclerotic disease is most common cause in the USA, while Kawasaki disease is most common cause worldwide

Coronary Artery Aneurysms: Common Etiologies

• Kawasaki’s disease
• Atherosclerosis
• Takayasu’s vasculitis
• Infection
• Congenital
• Trauma
• Polyarteritis nodosa
• Loeys Dietz Syndrome
• May also be associated with cocaine use, SLE, syphilis and rheumatic fever

Kawasaki Disease: Facts

• Occurs in children younger than 5 yrs with peak under age 2 yrs
• Male > female by 1.5x
• More common in Asians especially Japanese
• Acute febrile systemic illness in children
• AKA mucocutaneous lymph node syndrome

Kawasaki Disease: Facts

• Fever for 5 days or more without a cause and four of the following
• Bilateral conjunctival injection
• Mucous membrane changes (strawberry tongue)
• Extremity abnormalities including erythema of palms or soles, edema of hands or feet
• Rash
• Cervical lymphadenopathy

Kawasaki Disease: Facts

• Cardiac abnormalities
• Pericardial effusion (30%)
• Myocarditis
• Mitral regurgitation (30%)
• Aortitis and aortic regurgitation
• Congestive heart failure
• Coronary artery aneurysms

8 mm Aneurysm of the Left Main Coronary Artery

11 year old male with a history of Kawasaki’s disease at 8 months of age. The patient complained of episodic mid sternal chest pain occasionally.

Kawasaki Disease with Giant Coronary Artery Aneurysms

12 year old male with a hx of Kawasaki disease being treated with coumadin and aspirin. CTA was requested to define the status of the coronary arteries. CTA demonstrated multiple RCA aneurysms measuring up to 9mm in size. Portions of the RCA wall have begun to calcify

What is the accuracy of MDCT/CTA in detection coronary artery aneurysms?

"Multislice spiral computed tomography was performed in 16 adolescents and young adults who had Kawasaki's disease to assess coronary artery abnormalities. Adequate images were obtained for 96% of major coronary segments. The sensitivity of multislice spiral computed tomography to detect coronary artery aneurysms was 100%, and that for significant stenosis and occlusions was 87.5%, whereas false-positive results due to severe calcification was present in 5 arteries and those due to cardiac motion artifact were present in 2, resulting in a specificity of 92.5%."

Assessment of coronary artery abnormalities by multislice spiral computed tomography in adolescents and young adults with Kawasaki disease.
Kanamaru H et al.
Am J Cardiol - 15-FEB-2005; 95(4): 522-5

Takayasu Disease or Takayasu Arteritis

• Granulomatous inflammatory vasculitis that typically affects medium and large vessels like the aorta and its branches (left subclavian artery most common)
• Typical CT pattern is concentric wall thickening of vessels with resultant stenosis of the vessel. Occasionally aneurysmal dilatation may occur
• The coronary arteries may be involved in up to 15% of cases
• CTA with 3D mapping is ideal for vascular mapping
• Patient population usually under age 30 and more common in females by 8:1
• Patients are usually treated with corticosteroids. If unsuccessful then cyclophosphamide and methotrexate are used. Angioplasty or bypass reserved for select cases.

Takayasu’s Aortitis with Left Main Coronary Artery Aneurysm

16 year old male with a history of Takayasu aortitis and severe chest pain. The CTA was ordered to rule out an aortic dissection. CTA detected no dissection but defined aneurysmal dilatation of the left main coronary artery.

Coronary Artery Aneurysms: Illustrated Cases

• Kawasaki’s disease
• Atherosclerosis
• Takayasu’s vasculitis
• Infection
• Congenital
• Trauma (including post-surgical)Polyarteritis nodosa
• Loeys Dietz Syndrome
• May also be associated with cocaine use, SLE, syphilis and rheumatic fever

Aneurysm of Circumflex Coronary Artery

27 year old female with symptoms of increasing fatigue and pulsation in the left chest and neck. CTA demonstrates a persistent left SVC and aneurysm of the circumflex coronary artery.

Thrombosed Right Coronary Artery Aneurysm

68 yr old male with long standing hx of coronary artery disease. Patient had increasing angina. CTA detected near occlusion of LAD and a 3 cm RCA aneurysm. Subsequent catheter angiography did not detect the aneurysm. The patient had bypass surgery and at surgery a 3 cm thrombosed RCA aneurysm was detected.

Coronary Artery Aneurysm: Acute Clinical Presentation

Although most coronary artery aneurysms present in the non-acute setting others may present as an acute life threatening process. We have seen this in cases related to infection or due to Kawasaki’s disease. In the acute setting hemopericardium is a ominous sign of rupture or pending rupture

Contained Rupture of an LAD Aneurysm and Hemopericardium

19 yr old male with onset of left shoulder pain beginning 3 weeks earlier and now becoming severe chest pain. Patient arrived in the ER hypotensive. Patient has a hx of Dengue fever 10 years earlier.

Aneurysmal Dilatation of the LAD and CX Coronary Arteries

40 yr old female with hx of surgical correction of anomalous LCA from pulmonary artery at age 11. Patient presents with exertional chest discomfort that has been increasing. CTA demonstrates aneurysmal dilatation of LAD and circumflex coronary arteries.

Coronary Artery Button Aneurysm s/p Aortic Root Replacement

20 yr old female with Marfan Syndrome and prior aortic root replacement with St. Jude composite graft for aortic root aneurysm. Patient developed sharp chest pain following exercise and a CTA was requested. CTA demonstrates aneurysm dilatation of RCA at surgical site. “Button Aneurysm” of RCA found at surgery and repaired.

Conclusion

With the increased use of coronary CTA one should be aware of the CTA appearance of coronary artery aneurysms.