Case Author(s): Yonglin Pu, M.D., Ph.D., Tom R. Miller, M.D., Ph.D. , 03/22/03 . Rating: #D2, #Q5

Diagnosis: Spontaneous Coronary Artery Dissection

Brief history:

27-year-old woman with chest pain.

Images:

Myocardial Imaging(Rest/Pharmacologic-stress/SPECT), Bull's eye and three-dimensional stress and rest images

View main image(mi) in a separate image viewer

View second image(mi). Myocardial imaging, SPECT slices

View third image(an). Coronary Arterogram

View fourth image(an). Left Ventriculogram

Full history/Diagnosis is available below

Diagnosis: Spontaneous Coronary Artery Dissection

Full history:

A 27-year-old white woman with no significant past medical history presented to an outside hospital with episodic substernal chest pain. The pain, described as a non-radiating “dull pressure,” initially began about 6 months ago. It was initially exertional in nature, with relief after sitting or resting. Over the past 6 weeks, the pressure increased in frequency and severity. Three days prior to admission, she described pain at rest, with newly associated shortness of breath, nausea, and diaphoresis. She denied any other heart failure symptoms, palpitations, or syncope or presyncope. She smoked one pack per day for 8 yrs., and admitted to only occasional alcohol use. She denied illicit drug use. Her only additional cardiac risk factor was a positive family history for coronary artery disease (Father, MI/CABG, 40’s; Mother, MI, 50’s). She denied any medication use.

Radiopharmaceutical:

2.7 mCi TI-201 chloride intravenously and 20.2 mCi Tc-99m sestamibi intravenously

Findings:

Standard myocardial perfusion images were obtained after resting injection of Tl-201. Subsequently, an intravenous infusion of adenosine was performed with injection of Tc-99m sestamibi. There is a large predominantly reversible perfusion defect of moderate severity involving the lateral wall, consistent with myocardial ischemia with a small component of myocardial infarction. Gated Tc-99m sestamibi images (not shown) demonstrate mild global hypokinesis with moderate to severe hypokinesis of the lateral wall. There is mild transient ischemic dilatation of the left ventricle as well as moderate left ventricular enlargement. The left ventricular ejection fraction was mildly reduced at 40%.

Selective coronary arterograms demonstrated significant spontaneous dissection of the left anterior descending and left circumflex coronary artery, involving the distal left main coronary artery. The left ventricogram demonstratee a normal size left ventricle. The left ventricular ejection fraction was 60%. There was also mild hypokinesis in the anterolateral wall.

Discussion:

Spontaneous coronary artery dissection is extremely uncommon, with approximately 150 cases reported since 1931. In those cases, the average age of patients was 40 years old with females outnumbering males 4:1.

Young females in puerperium and people with atherosclerotic disease are more likely to get spontaneous coronary artery dissection, although some cases are idiopathic. Overall, prognosis is poor, with 70% of the patients diagnosed at necroscopy and 30% at angiography.

80% of single-vessel disease cases are in the left anterior descending coronary artery, and multi-vessel involvement with left main coronary artery disease has also been described. Women mostly have spontaneous coronary artery dissection in the left anterior descending artery and left main coronary artery while men usually have isolated right coronary artery involvement. The pattern and/or severity of presentation and prognosis are related to the vessels involved. For example, left main, left anterior descending and multi-vessel disease have the poorest prognosis.

Treatments to this disease are on a case-by-case basis. One series of 5 patients treated medically with ASA, Beta-blockade, Nitrates had no recurrence of ischemia in 4 of the 5 cases. Data derived from use after acute presentations of undiagnosed spontaneous coronary artery dissection demonstrated that thrombolysis has mixed results. Some patients were treated successfully. Also, some reports demonstrated thrombolytic therapy induced extension of dissection. Therefore, it is generally NOT recommended.

Coronary artery bypass graft has conferred marked survival benefit in many series. Some authors recommend it for all patients. It is best suited for left main, multi-vessel disease, refractory or recurrent myocardial ischemia. Percutaneous intervention has also been proven to be mortality benefit. It is an ideal therapy in single-vessel spontaneous coronary artery dissection if left main coronary artery is not involved there is not report about the dissection extension with antiplatelet therapy for the stent care.

References: 1. Bizzarri F, Mondillo S, Guerrini F, Barbati R, Frati G, Davoli G. Spontaneous acute coronary dissection after cocaine abuse in a young woman. Can J Cardiol 2003 Mar 15;19(3):297-9 2. Kay IP, Williams MJ. Spontaneous coronary artery dissection: long stenting in a patient with polycythemia vera. Int J Cardiovasc Intervent 1999;2(3):191-193 3. Maresta A, Varani E, Balducelli M, Vecchi G. Spontaneous coronary dissection of all three coronary arteries: a case description with medium-term angiographic follow-up Ital Heart J 2002 Dec;3(12):747-51

Special thanks to Dr. Tuan Nguyen, internal medicine resident at Barnes-Jewish hospital for his great contribution on this digital teaching file.

Major teaching point(s):

See discussion section.

Differential Diagnosis List

1. Aortic dissection with coronary artery involvement. 2.Iatrogenic dissection due to coronary angiography or angioplasty or cardiac surgical procedures or manipulation. 3. Marfan’s Syndrome. 4. Anomalous coronary arteries and coronary artery fistulas.

ACR Codes and Keywords:

References and General Discussion of Myocardial Imaging (Anatomic field:Heart and Great Vessels, Category:Organ specific)

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Case number: mi023

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