Spontaneous coronary artery dissection (SCAD) is a rare cause of acute myocardial infarction and sudden death. It typically occurs in healthy women during the peripartum period or in the presence of connective tissue disorders.^1–4 Although the lesion is often fatal and the diagnosis established postmortem, in recent years there have been reports of successful medical, percutaneous and surgical interventions.⁵ We describe a case of an extensive spontaneous left coronary artery dissection that led to cardiogenic shock and that was successfully treated with multiple stent implantation. It occurred in a young healthy woman on oral contraceptives subsequent to a recent interruption of pregnancy.

Case Report. A 23-year-old female was taken to the emergency room due to the sudden onset of mid-sternal excruciating chest pain that occurred while working at her desk and that lasted 1 hour. She gave no prior history of cardiovascular disease, connective tissue disorders or trauma. She smoked less than 10 cigarettes per day for the last 5 years. Two months before admission, she underwent an elective abortion at 14 weeks and was subsequently placed on oral contraceptive therapy, sequential and monophasic.
An electrocardiogram showed ST-elevation of the precordial leads and of lead I and aVL. On examination, the patient was still complaining of chest pain, shortness of breath and diaphoresis. Her arterial pressure was 80/50 mmHg, and her heart rate 130 beats/minute; heart auscultation revealed an S3 gallop and Killip II congestive heart failure. Shortly thereafter, ventricular fibrillation occurred and was successfully defibrillated with 360 joules. Initial management included intravenous morphine, heparin, dobutamine and lidocaine.
Echocardiography showed severe impairment of the left ventricular contractility (ejection fraction < 30%), with akinesia of the anterolateral, septal and apical walls. She was immediately transferred to the catheterization laboratory for coronary angiography. The left coronary tree showed distal subocclusion of the left main, and only the left circumflex (LCx) was opacified, revealing TIMI 2 flow (Figure 1). The right coronary artery (RCA) was free of any disease. An intra-aortic balloon pump was inserted. Because of her poor clinical status, percutaneous coronary intervention (PCI) was performed while notifying the cardiac surgeon on duty. Wiring the occluded left anterior descending (LAD) was technically challenging and, when accomplished, led to TIMI 2 flow in the vessel. Reinjection revealed an extensive dissection involving the left main and the LAD down to its mid-portion, including the main diagonal branch and the proximal part of the LCx (Figure 2).
Through a 6 Fr left Judkins guiding catheter, a total of 6 stents were directly implanted to cover the large dissection. The left main was stabilized with a 4.0 x 13 mm Bx Velocity stent (Cordis Corp., Miami, Florida). Another 3.5 x 18 mm Bx Velocity stent was deployed at the ostium of the LCx. Then, the LAD was treated with 4 more stents, including a 3.5 x 15 mm BeStent 2 (Medtronic, Inc., Minneapolis, Minnesota) in the proximal part, a 3.5 x 13 mm Bx Velocity in the middle portion and two 3.0 x 8 mm Tetra stents (Guidant Corp., Indianapolis, Indiana) sequentially implanted in the distal portion of the LAD and at the ostium of the main diagonal branch.
At the end of the procedure, a complete sealing of the dissection was achieved with restoration of TIMI 3 flow in the left coronary tree (Figure 3). A bolus of 0.25 mcg/kg of abciximab was administered, followed by a 0.125 mcg/kg/m 12-hour infusion. The patient’s hemodynamic status improved and she became asymptomatic. Her blood pressure rose to 110/70 mmHg, and she was transferred to the intensive care unit where her condition continued to improve over the following days.
The patient’s peak CK level was 6530 IU/L with an MB fraction of 528 IU/L 6 hours after symptom onset. Electrocardiography showed a resolving anterolateral infarction. No laboratory evidence was found for thrombophilia, coagulative or connective tissue disorders. An echocardiogram performed 1 week afterstenting demonstrated residual anterior and septal akinesia, with an ejection fraction of 40%. A repeat cardiac catheterization done before discharge showed widely patent left main, LAD and diagonal branch arteries with mild “stent-jailing” of the ostium of the LCx. Her clinical course was uneventful and she was discharged on day 10 on ticlopidine 250 mg b.i.d., aspirin 165 mg per day and lisinopril 5 mg b.i.d.
Two months later, the patient suffered an episode of chest pain during physical exertion. She underwent coronary angiography that revealed a total occlusion of the ostial LCx, collateralized by the RCA, without significant restenosis at the other treated coronary sites. Left ventriculography showed apical akinesia and anterolateral hypokinesia, with an ejection fraction of 46%. A thallium study showed a fixed defect in the anterolateral wall and mild ischemia in the posterolateral wall. Atenolol 25 mg per day and transdermal nitrates were added to her therapy; a subsequent maximal treadmill test was negative for symptoms and signs of ischemia. The risks and benefits of a repeat revascularization procedure on the LCx in an asymptomatic patient were presented to the patient. A conservative approach was subsequently chosen. The 8-month follow up angiography showed recanalization of the previously occluded ostial LCx, with a residual 75% in-stent stenosis, and disappearance of the collateral circulation from the RCA and persistent patency of the other stented segments. Eighteen months after the patient’s first admission, she was hospitalized for unstable angina with signs of inferior ischemia during the electrocardiogram. Coronary angiography showed a dissection of the posterior descending branch of the RCA, while the left coronary tree was unchanged. A 2.5/18 Cypher™ stent (Cordis Corp.) was implanted. After 9 months, a control coronary angiogram showed a widely patent posterior descending branch. More than 4 years after the index procedure, the patient is leading an unencumbered lifestyle, is symptom-free, and had a maximal negative treadmill test. She is on aspirin and beta-blocker therapy, and some antidepressive treatment is also cyclically necessary.

Discussion. Coronary artery dissection has been described as a complication of aortic dissection, blunt chest trauma, cardiac catheterization, coronary angioplasty or bypass graft surgery.¹ SCAD usually occurs in young women, particularly during the peripartum period or in association with oral contraceptive therapy,^1,2,6,7 though young men are not spared.^8,9 Multiparity seems to be a predisposing factor, whereas no clear association has been found with traditional coronary risk factors, although it has also been observed in young smokers and hypertensive patients.^1,5,10 Recently, SCAD has also been decribed in association with prolonged and forceful retching.¹¹ The most frequently involved vessel is the LAD, though left main and multivessel dissections have been described.⁵ Spontaneous dissection of bilateral internal mammary arterial grafts has also been observed in a single patient.¹²
The pathogenesis is still unclear and subject to debate. In the peripartum period, there is an altered hemodynamic status with an increase in cardiac output and blood volume, both augmenting the mechanical stress on the arterial wall. Coupled with an adaptive endocrine metabolism, this may lead to smooth muscle cell hypertrophy, loosening of ground substance and fragmentation of reticular fibers. An accumulation of eosinophils, with liberation of lytic proteases in the media, has been shown to contribute to vessel wall damage.^2,10,13 Furthermore, excess progesterone has been postulated to induce a weakness in the tunica media by structural modification in the coronary vessel wall.¹⁴
Disruption of the vasa vasorum with intramedial hemorrhage and dissection has been found in autopsy studies.^15,16 The use of oral contraceptives is also considered a contributor^6,7 since high estrogen levels may be responsible for low collagen synthesis and subsequently predisposes women to spontaneous dissection.^17,18 In the present case, this condition was present, but it is unclear whether the premature interruption of pregnancy could duplicate normal full-term delivery in terms of endocrine pattern predisposing to SCAD.
To the best of our knowledge, no such case has been previously reported in the literature. In a comprehensive, observational review, Koul et al.⁵ reported an overall mortality for spontaneous coronary dissections of 38%, mostly occurring within the first few hours from the onset of symptoms. In the surviving patients, various treatment modalities — surgery, angioplasty with or without stent implantation, cardiac transplant and medical therapy — have been shown to be of benefit, with initial high success rates. Medical treatment appears to be appropriate if the patient has no hemodynamic instability and chest pain quickly disappears. In such cases, the dissection occasionally can completely heal, as confirmed by follow-up angiography. Surgical or percutaneous revascularization is mandatory for patients with persistent or recurring symptoms, since the 3-year mortality rate in this setting for the untreated patients is 20%.^5,19
Of a total of 58 cases with SCAD reported by Koul, there were 14 patients with left main involvement; of these, 5 (35.7%) died, 4 suddenly or soon after the onset of symptoms, and 1 after a massive medically treated acute myocardial infarction. Interestingly, the 8 survivors were either sent on to CABG (4 patients), surgical extrusion of the hematoma (1 patient), cardiac transplant (3 patients) or stenting (1 patient), and were treated from 1989 on.⁵
Our case represents the second reported patient with left main and bifurcation involvement treated with stenting. Thrombolysis is relatively contraindicated in patients with SCAD because, although it has been shown to lyse the thrombus in the false lumen, it may also increase the parietal hemorrhage and further extend the dissection.⁵ At presentation, our patient was in cardiogenic shock, thus an aggressive therapeutic approach was mandatory. Thrombolytic treatment is not considered a first-line choice because of the risk of increasing intraparietal hemorrhaging and so forth, extending the dissection.²⁰ Our report underlines the crucial importance of maintaining a high degree of suspicion for SCAD.
A coronary angiography and complete revascularization had to be performed in order to offer our patient a favorable outcome. CABG could have been an option but, in the setting of acute myocardial infarction with shock, it has high operative mortality and complications.^21–23 Moreover, it has been shown to be associated with poorer results in cases of dissected coronary arteries.^24–25 The time required to have the surgical suite ready, particularly in off-hours, is certainly much longer than that required to perform ad hoc angioplasty.
The entire dissection was stented in order to stabilize the extensively damaged coronary segments. Although such extensive stenting increases the risk of subacute thrombosis and restenosis, the main goal of the procedure was to quickly stabilize and effectively reverse the patient’s life-threatening hemodynamic status. The observation of a totally occluded ostial LCx after 2 months supported the presence of a coronary thrombus or exaggerated remodeling. We decided to be conservative and wait at least until the 6-month follow up when the other stented segments may have restenosed.
In that case, percutaneous intervention could have been a bridge to delayed surgical revascularization. The 8-month coronary angiography, showing recanalization of the previously occluded LCx, confirmed our first hypothesis of a subacute stent thrombosis associated with neointimal hyperplasia. Considering the absence of symptoms and signs of ischemia, it was felt that further intervention was unnecessary and potentially hazardous because of the risk of involving the left main and proximal LAD. The later occurrence of a further dissection in another section of the patient’s coronary tree also points to the causative mechanism of a genetic disorder of the extracellular matrix organization, such as lysyl oxidase (LOX) deficiency,26 which we were not able to investigate in our patient at that time.
An aggressive diagnostic approach is warranted also in young women on oral contraceptives who present with acute coronary syndromes and who have had an elective or spontaneous abortion. An interventional procedure, particularly primary stenting, should nowadays be considered the first therapeutic option in patients with suitable anatomy. Premature interruption of pregnancy followed by oral contraception should be added to the list of clinical profiles associated with SCAD. The risk of coronary dissection may extend beyond the trigger event due to some not yet well-known alterations in the connective tissue of some SCAD patients.

Acknowledgment. We wish to acknowledge Dr. John Lasala of the George Washington University in Saint Louis, Missouri, for his thoughtful revision of the manuscript.