Clinical Review & Education

JAMA Clinical Challenge

A Young Woman With Chest Pain

Quentin R. Youmans, MD; Erin D. Unger, MD; Keith H. Benzuly, MD

Figure. Clinical findings from 12-lead electrocardiography (left) and right anterior oblique cranial angiography of the left anterior descending artery during diastole (right).

A 36-year-old healthy gravida 3, para 3 woman with a history of asthma and elective cesarean
delivery 11 days prior presented to the emergency department with 3 days of exertional chest WHAT TO DO NEXT
pain. The pain was described as crushing and substernal, but she denied radiation or asso-
ciated dyspnea, nausea, vomiting, or diaphoresis. On physical examination, her blood pres- A. Administer thrombolytic therapy
sure was 167/105 mm Hg bilaterally, heart rate was 71/min, respiratory rate 18/min, and oxy-
gen saturation 98% on room air. Initial laboratory evaluation revealed a troponin I level of
B. Continue medical therapy alone,
0.12 ng/mL (reference range, 0.00-0.04 ng/mL). An electrocardiogram showed normal si-
with a β-blocker and antiplatelet
nus rhythm with diffuse ST-segment depressions but without q waves or ST-segment el-
therapy
evation (Figure, left). Computed tomography angiography did not show pulmonary em-
bolism or aortic dissection. Echocardiography demonstrated a left ventricular ejection fraction
of 53%, with apical akinesis and no valvular disease. C. Perform percutaneous coronary
The patient was started on aspirin and ticagrelor, heparin infusion, and metoprolol tar- intervention (PCI)
trate for treatment of non–ST-segment elevation myocardial infarction. She underwent coro-
nary angiography (Figure, right; Video). D. Refer for coronary artery bypass
graft (CABG) surgery

Diagnosis Discussion
Spontaneous coronary artery dissection (SCAD) SCAD is increasingly recognized as an etiology of acute
coronary syndrome (ACS). The prevalence of SCAD in ACS
What to Do Next is estimated to be between 1% and 4% but may be under-
B. Continue medical therapy alone, with a β-blocker and antiplate- recognized and underdiagnosed.1 Women account for more than
let therapy 90% of cases, 2 and the prevalence has been reported to be
The keys to the correct diagnosis are the epidemiology and as high as 35% in women younger than 50 years with ACS. 1
unique angiographic characteristics of SCAD. Expert consensus Conditions that adversely affect vessel wall integrity or in-
recommends medical therapy crease shear stress are thought to increase the risk of dis-
for this condition. Thromboly- section.3 Fibromuscular dysplasia is present in 25% to 86% of
Video
sis carries risk of propagation, patients in cohort studies. 1 Pregnancy, including multiparity,
as does mechanical revascularization with PCI or CABG surgery; is another important risk factor, with most cases occurring within
these interventions are thus reserved for select patients. 4 weeks of delivery.1

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 JAMA Clinical Challenge Clinical Review & Education

In atherosclerotic coronary artery disease, plaque rupture or
erosion initiates the intravascular thrombotic cascade, causing
ischemia. The pathophysiology of SCAD is less well defined. One
hypothesis is that hematoma within the coronary artery wall initi-
ates the dissection.4 Intramural hematoma may form through
a tear in the intima or by spontaneous bleeding from the vasa
vasorum.5 The true coronary lumen is compressed, and thrombus
formation may also occur. Ultimately, this leads to myocardial
ischemia or infarction.1
The clinical presentation of SCAD is often indistinguishable
from that of atherosclerotic ACS. Patients report chest pain and
may have ischemic electrocardiographic abnormalities or elevated
levels of cardiac biomarkers. Rarely, patients with SCAD present
with ventricular arrhythmias or sudden cardiac death. 6 ACS,
whether atherosclerotic or due to SCAD, often warrants coronary
angiography. SCAD can have 1 of 3 angiographic appearances. Type
1 indicates multiple visible lumens, with contrast staining of the
arterial wall; type 2, diffuse stenosis and an abrupt change in artery
caliber at either end of that stenosis; and type 3, focal or tubular
stenosis that mimics artherosclerosis.7 Type 1 is pathognomonic for
SCAD, type 2 most common, and type 3 most deceiving. The lesion
in this case is most consistent with type 2 SCAD. A high index of
suspicion is necessary to distinguish SCAD from atherosclerotic
coronary artery disease or vasospasm.
Treatment recommendations for SCAD are primarily based on
expert consensus. β-blockers are widely recommended for acute
and long-term management of SCAD, because they decrease coro-
nary artery wall stress and myocardial oxygen demand.1 A prospec-
tive cohort study demonstrated a significant decrease in recur-
rence of SCAD in patients treated with β-blockers.6 Antiplatelet
therapy, whether single- or dual-agent, is reasonable given the
known benefits of aspirin in other forms of ACS.8 Statin therapy is
appropriate for patients when indicated for primary prevention of
atherosclerotic cardiovascular disease.1
Revascularization can be technically difficult or hazardous and
should be reserved for patients for whom intensive medical therapy
has failed or for those who have high-risk clinical features (eg, left
main coronary artery involvement, persistent ischemia, ventricular
arrhythmias, or shock). PCI is technically difficult because of the po-
tential of entering the false lumen with the guidewire and carries risk
of extending the dissection proximally or distally.8 CABG surgery has
been performed successfully, but data are limited to case reports and
small series. There may be a high incidence of graft failure, and CABG
surgery is not protective for recurrent SCAD. Thus, CABG surgery
should be reserved for patients with multiple or proximal dissec-
tions, those with left main coronary artery disease not amenable to
PCI, or those for whom PCI has failed.1 Thrombolytic therapy should
be avoided because of the risk of worsening coronary artery intra-
mural hematoma.9
In a recent study of 310 patients, the rate of de novo SCAD
recurrence was 11%, with a median time to recurrence of 4 years.10
Vessel tortuosity is the only risk factor identified for recurrence.1
Follow-up is generally clinically driven; repeat imaging may be use-
ful if patients have recurrent symptoms or other evidence of recur-
rent ischemia.
Patient Outcome
The patient was prescribed metoprolol succinate, aspirin, and
clopidogrel. Her chest pain resolved, and her troponin I level
peaked at 1.25 ng/mL. At 4-month follow-up, an echocardiogram
showed resolution of the apical wall motion abnormality. She
remained active and asymptomatic at 9-month follow-up.

ARTICLE INFORMATION
Author Affiliations: Department of Medicine,
Northwestern University Feinberg School of
Medicine, Chicago, Illinois (Youmans); McGaw
Medical Center of Northwestern University,
Chicago, Illinois (Unger); Bluhm Cardiovascular
Institute, Northwestern University Feinberg School
of Medicine, Chicago, Illinois (Benzuly).
Corresponding Author: Keith H. Benzuly, MD,
Bluhm Cardiovascular Institute, Northwestern
University Feinberg School of Medicine, 251 E
Huron St, Feinberg Pavilion, Ste 8-503, Chicago, IL
60611 (k-benzuly@northwestern.edu).
Section Editor: Mary McGrae McDermott, MD,
Senior Editor.
Published Online: November 19, 2018.
doi:10.1001/jama.2018.17045
Conflict of Interest Disclosures: None reported.
Additional Contributions: We thank the patient for
providing permission to share her information.
Submissions: We encourage authors to submit
papers for consideration as a JAMA Clinical
Challenge. Please contact Dr McDermott at
mdm608@northwestern.edu.
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