Spontaneous coronary artery dissection

escardio.org/Journals/E-Journal-of-Cardiology-Practice/Volume-14/spontaneous-coronary-artery-dissection

Vol. 14, N° 38 - 22 Feb 2017

Dr. Shahid Aziz

Spontaneous coronary artery dissection is an important cause of acute

myocardial infarction, especially in young women. The pathophysiology
and treatment differ significantly from patients with plaque instability
due to atherosclerosis. This review highlights the importance of
recognising the angiographic subtypes of spontaneous coronary artery dissection, the
role of intravascular imaging and indications for revascularisation. The association with
fibromuscular dysplasia and pregnancy is discussed, along with evidence from large
clinical registries on the risk of recurrent cardiac events.

Acute Coronary Syndromes

Introduction
Spontaneous coronary artery dissection (SCAD) is a rare non-atherosclerotic cause of
acute coronary syndromes (ACS). More cases are now being identified due to increased
awareness and earlier use of invasive angiography in patients presenting with acute
chest pain.

It is important to recognise SCAD, as patient characteristics and management differ

substantially from typical ACS cases. SCAD patients are often younger, and more likely
to be female without the classic cardiovascular risk factors. Results of revascularisation
with percutaneous coronary intervention (PCI) and coronary artery bypass grafting
(CABG) are suboptimal. Conservative management is the preferred option with
spontaneous healing of the dissection in the majority of uncomplicated cases.

Pathophysiology
SCAD is caused by sudden disruption of the coronary artery wall, resulting in
separation of the inner intimal lining from the outer vessel wall. The trigger is thought
to be either an intimal tear or bleeding from the vasa vasorum, resulting in intramural
haematoma. Pressure-driven expansion of the haematoma causes propagation of the
dissection plane with formation of a true lumen, and a thrombus containing a false
lumen.

SCAD should be distinguished from dissection caused by plaque rupture in patients

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with atherosclerosis or catheter-induced iatrogenic dissections. Patients with SCAD
have fragile arterial walls with no atheroma or calcification to limit propagation of
dissection. Therefore, cases with SCAD will often have more extensive dissections, and
non-affected coronary artery segments appear smooth and disease-free on angiography.

Epidemiology
SCAD is estimated to be responsible for 0.1% to 0.4% of all ACS cases. [1] It is an
important cause of ACS in young women, responsible for up to 25% of all ACS cases in
women <50 years of age. [2]

The Mayo Clinic registry of 87 consecutive patients with SCAD reported a mean age of
43 years; 82% were female. [3] No cause was identified in 45% of all cases, highlighting
that many cases of SCAD remain unexplained. The commonest identified predisposing
factors were postpartum, fibromuscular dysplasia (FMD), connective tissue disease and
hormonal therapy. Potential stressors include extreme physical exertion, particularly in
young male patients, intense emotional stress, sympathomimetic drugs (such as
cocaine, amphetamines), childbirth and Valsalva-like activities (such as coughing,
retching, vomiting). Triggers for SCAD are thought to increase shear stress on the
coronary artery wall, often mediated by elevated catecholamine levels and intra-
abdominal pressure. [4]

Clinical presentation and diagnosis

ST-elevation myocardial infarction (MI) is present in up to 50% of patients presenting
with SCAD, with non-ST-elevation MI in the rest. Most patients with SCAD will have an
elevated troponin level. Life-threatening ventricular arrhythmias and sudden cardiac
death are recognised early complications.

The diagnosis of SCAD is made at the time of coronary angiography. Findings can be
graded into three types.

Type 1

The classic description is of a longitudinal filling defect, representing the radiolucent

intimal flap. There is often contrast staining of the arterial wall with appearance of a
double lumen (Figure 1).

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Figure 1. Coronary angiogram image from a 47-year-old female admitted with chest
pain, dynamic inferior ST-elevation and raised troponins. The AP projection shows
Type 1 SCAD involving a large proximal obtuse marginal branch. There is a long
proximal dissection flap with reduced TIMI 1 flow in the distal vessel. The patient
settled with conservative treatment and was discharged home seven days after
admission.

Type 2

Diffuse long smooth tubular lesions (due to intramural haematoma) with no visible
dissection plane that can result in complete vessel occlusion. Lesions are typically >30
mm in length with an abrupt change in vessel diameter between normal and diseased
segments. There is no response to intracoronary nitrates and there are no
atherosclerotic lesions in other coronary segments.

Type 3

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Multiple focal tubular lesions due to intramural haematoma that mimic atherosclerosis.
Intravascular imaging is required to make the diagnosis.

SCAD is often under-recognised and incorrectly classified as due to atherosclerosis if

diagnosis is reliant upon visualisation of the classic dissection flap. [5] Type 2 SCAD is
the most common, resulting in compression of the lumen due to intramural haematoma
with no intimal flap (67% of cases). Type 1 SCAD with classic dissection flap occurs in
29%, and Type 3 SCAD mimicking atherosclerosis is relatively uncommon, occurring in
4% of cases. [6] Therefore, relying upon the identification of contrast wall staining
(Type 1 SCAD) on coronary angiography would result in a significant number of cases
being missed. Imaging of the vessel wall with intravascular ultrasound (IVUS) or optical
coherence tomography (OCT) is required to make the diagnosis of Type 2 and Type 3
SCAD. Diffuse long coronary lesions in a young female patient with otherwise smooth
coronary arteries should always promote consideration of intramural haematoma in the
differential diagnosis.

The left anterior descending artery is the most frequently affected vessel, with
multivessel dissections in up to 20%-25% of cases. Coronary dissections are more
common in the mid- to distal segments, often involving side branches. Eleid et al.
showed that coronary artery tortuosity on coronary angiography is more common in
patients with SCAD (78% vs. 17% in controls), and severe coronary tortuosity is a
marker for recurrent SCAD events. [7]

Patients with SCAD are thought to have more fragile coronary artery walls. Meticulous
attention to angiographic technique is required to avoid catheter-induced dissection.
Deep catheter intubation should be avoided, with careful monitoring of pressure traces.
Gentle contrast injection to achieve adequate vessel opacification is important to
minimise the risk of dissection propagation.

Management
Conservative management is preferred in stable patients with SCAD as most dissected
segments will heal spontaneously (Figure 2). Medical therapy is based upon opinion,
with no randomised clinical trials in this area. Initial treatment is similar to standard
ACS patients with the use of dual antiplatelet agents, heparin and beta-blockers to
preserve patency of the true lumen and prevent thrombotic occlusion. Glycoprotein
IIb/IIIa inhibitors have also been used without complications. However, these agents
could potentially delay healing of the intramural haematoma and lead to dissection
extension. Thrombolytic agents should not be used due to an increased risk of bleeding
and extension of intramural haematoma.

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Figure 2. Repeat coronary angiogram for the same patient as in Figure 1 at three
months post-discharge, showing complete vessel healing with no residual dissection.

Dual antiplatelet therapy with aspirin and clopidogrel is generally accepted, with no
data on the role of more potent antiplatelet agents, such as ticagrelor and prasugrel.
Although statins are important for ACS treatment in patients with atheroma, the benefit
in SCAD is unknown.

Beta-blockers are recommended in all patients, with the potential to reduce arterial
shear stress, facilitate healing and reduce long-term recurrence.

Indications for revascularisation include: complete vessel occlusion with thrombolysis

In myocardial infarction (TIMI) 0 flow which is unlikely to resolve with medical
treatment alone, left main stem involvement, ongoing ischaemia, recurrent chest pain,
haemodynamic instability and sustained ventricular arrhythmias.

PCI is the preferred revascularisation strategy, but is associated with significant

challenges and has reported success rates of <50%. [4,6] Technical difficulties include
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negotiating the guidewire into the true lumen, dissection or haematoma extension and
side branch occlusion. Stent placement can result in haematoma propagation and loss
of vessel flow. Coronary angiography provides poor visualisation of intramural
haematoma, and intravascular imaging with OCT or IVUS is recommended in all PCI
cases. OCT has superior resolution to IVUS: it can confirm guidewire position in the
true lumen, detail the site of intramural haematoma and intimal tear, facilitate
appropriate vessel sizing and optimise stent expansion. [8]

A conservative approach to stent implantation is preferred, with stenting of the

proximal segment to seal the dissection entry, if present, and to limit the risk of
dissection/haematoma propagation. Distal dissections can be left untreated if coronary
flow is good with no significant obstruction. Late follow-up has demonstrated that these
spontaneous distal dissections heal with reabsorption of the intramural haematoma.
Drug-eluting stents are typically used in cases involving long lengths of stents to reduce
the risk of restenosis. However, there is an increased risk of stent malapposition
following reabsorption of the intramural haematoma, which may predispose to late
stent thrombosis.

Bioresorbable vascular scaffolds (BVS) offer a promising solution by providing

temporary lumen support whilst allowing vessel healing with long-term restoration of
normal vessel architecture. Theoretically, BVS may reduce the risk of late stent
malapposition by avoiding the use of a permanent metallic stent, although this has not
been formally tested in clinical studies.

CABG is considered for patients with left main stem dissections or when PCI has been
unsuccessful or is not technically feasible. The rate of emergency CABG for PCI failure is
significant, varying between 10% and 13% in reported series. [4,6] This would suggest
that PCI for SCAD should be performed, if possible, at centres with on-site cardiac
surgery.

In-hospital results following CABG for SCAD are excellent, with a reported mortality of
<2%. Follow-up angiographic studies have shown high rates of graft occlusion, possibly
due to competitive flow in the native vessel or technical difficulties with distal graft
anastomosis. In a single study with follow-up angiography at a median of 3.5 years, only
five out of 16 grafts were shown to be patent. [6]

Prognosis
Two single-centre registries have provided important data on the natural history and
prognosis of SCAD. Shaw et al. reported the results of a single-centre Canadian registry
of 164 patients (mean age 52, 92% female) with SCAD. [4] Some 80% of patients were
treated conservatively on initial presentation, and the in-hospital recurrent MI rate in
this group was 4.5%. In patients treated conservatively who underwent delayed elective
coronary angiography (≥26 days after the index event), all had spontaneous
angiographic healing. Interestingly, early angiography (within 20 days) showed
persistence of the coronary dissection, suggesting that the healing process is relatively
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slow. In the 33 patients who underwent PCI, complete success was only achieved in
36.4%. Over half of the patients undergoing PCI had procedural extension of the
dissection including, in some cases, involvement of the left main stem. Only six out of
the 164 patients underwent CABG, including three for failed PCI, with guide catheter-
induced left main dissection in two cases. There were no in-hospital mortalities. The
two-year MACE rate was 10%-17%, mainly driven by recurrent SCAD events.

Tweet et al. reported the results for 189 patients with first presentation of a SCAD
episode. [6]. Women made up 92% of them, with a mean age of 44 years. Ninety-four
patients were treated conservatively, with 10% experiencing SCAD progression at a
mean of four days after the initial admission requiring intervention. These findings are
in keeping with previous reports suggesting careful in-hospital monitoring for up to one
week due to the risk of recurrent events. This is in contrast to atherosclerotic ACS cases
where guidelines emphasise an early intervention and discharge approach. The PCI
failure rate was 53%, underscoring the suboptimal results of PCI in this challenging
group of patients.

Long-term survival was excellent, with only one death during a median follow-up of 2.3
years, although the risk of recurrent SCAD events was significant (27% at five years).
Interestingly, 75%-90% of recurrent SCAD events involved coronary segments not
affected at the time of the initial presentation. This would suggest that the risk for SCAD
involves the entire epicardial coronary artery circulation and is not limited to isolated
segments.

Collectively, these results would suggest excellent in-hospital and long-term survival,
with a significant risk of future SCAD events. Therefore, patients should be warned
about the risks of SCAD recurrence. At present, there is no effective treatment to reduce
long-term risk.

Fibromuscular dysplasia (FMD)

FMD is a rare condition that affects medium-sized arteries, with abnormal thickening of
the vessel wall causing stenosis and aneurysm formation. [9] It can involve all arterial
beds, and is most commonly seen in the renal and carotid arteries. Most patients are
female, aged between 20 and 60 years. The cause of FMD is unknown, and diagnosis is
made by angiography (invasive or non-invasive).

The classic finding is of multifocal disease with a 'string of beads' appearance due to
fibromuscular ridges causing arterial stenosis alternating with arterial dilatation. Focal
disease is less common and results in localised tubular narrowing.

The first reported association between SCAD and FMD was made in 2005 by a group in
Vancouver, Canada. [10] They described a case series of seven women, all presenting
with acute MI, renal FMD and a distinctive pattern of coronary artery disease on

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angiography. This consisted of single-vessel (LAD in six and RCA in one) mid- to distal
diffuse obliterative disease, with abrupt demarcation from proximal normal segments.
Intravascular imaging was not performed.

The same group published results for 50 patients with similar presentation and
angiographic findings. [11] All patients underwent systematic screening for FMD with
the performance of invasive or non-invasive angiography of the renal, iliac and cerebral
vessels. FMD was detected in 86% of patients with 42% having FMD in two or more
vascular territories. The high pickup rate of FMD in this study may have been due to the
frequent use of invasive angiography, with 76% of patients undergoing invasive renal
angiography. CT or MR angiography is recognised to be less sensitive than invasive
angiography in detecting mild FMD abnormalities.

The high prevalence of FMD in patients with SCAD suggests a direct causative
association. However, additional studies are necessary for confirmation, and it is
difficult to see how this will alter current management unless there is specific treatment
for FMD or difference in prognosis.

Pregnancy-related SCAD
SCAD during pregnancy is uncommon, but can lead to acute myocardial infarction.
Cases tend to occur within six weeks of delivery, but have been reported during early
pregnancy and as late as 18 months postpartum, especially in patients still
breastfeeding. [12] Pregnancy-related SCAD accounts for a small proportion (less than
5%) of all SCAD cases and is a less important cause of SCAD than originally reported.
Hormonal changes during pregnancy with high progesterone levels can result in
weakening of the vessel wall. Increased cardiac output and circulatory volume, along
with the acute haemodynamic stress of childbirth can lead to pregnancy-related SCAD.
Patients should be screened for underlying connective tissue disease or chronic
inflammatory conditions. Most patients have a history of multiple pregnancies, and
clinical outcomes are thought to be worse with larger infarcts, lower ejection fraction,
more left main stem involvement and cardiogenic shock at presentation, compared with
non-pregnancy-related SCAD. [13] The risk of recurrence is thought to be significant,
with one small study showing that pregnancy-related SCAD occurred in one out of
seven patients who became pregnant from a 266-patient SCAD registry. This patient
developed ST-elevation MI nine weeks postpartum due to left main stem dissection
requiring emergency CABG. [12] Therefore, women of a reproductive age with a history
of SCAD due to any cause should be carefully counselled regarding the risk of recurrent
events, especially if they are planning a future pregnancy.

Conclusions
SCAD is an important cause of ACS, particularly in young women without traditional
cardiovascular risk factors.

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The pathophysiology and treatment are different to ACS caused by plaque rupture or
erosion.

Contrast staining of the vessel wall (Type 1 SCAD) is the most recognised finding on
coronary angiography. However, Type 2 SCAD due to intramural haematoma is the
commonest presentation and can be easily missed. Typical angiographic findings in
Type 2 SCAD include long mid- to distal subtotal coronary occlusions without contrast
staining of the vessel wall. There is often an abrupt change in vessel calibre between
normal and diseased segments. Intravascular imaging is required to confirm the
diagnosis.

Most coronary dissections will heal spontaneously, and conservative treatment is

recommended for uncomplicated cases.

Patients with left main stem involvement, complete vessel occlusion, ongoing chest pain
or haemodynamic instability will require coronary revascularisation. PCI results are
suboptimal in this challenging group of patients.

PCI should be performed by experienced operators, with the use of intravascular

imaging and preferably with on-site surgical backup due to the increased risk of
complications.

Many patients with SCAD will also have FMD, and patients should have imaging of
additional arterial beds to screen for FMD. It is not known whether this is a causative
association.

Although long-term prognosis is excellent, the risk of recurrent SCAD events is

significant, with an average rate of 5% per year.

References

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Notes to editor

Author:

Dr Shahid Aziz, MD, MBChB, BSc, MRCP.

Consultant Cardiologist, North Bristol NHS Trust, Southmead Hospital, Bristol, UK.

Address for correspondence:

Consultant Cardiologist, North Bristol NHS Trust, Southmead Hospital,

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Bristol, BS10 6TH, UK

Email: shahid.aziz@nbt.nhs.uk

Tel: +44 117 4146370

Author disclosure:

The author has no conflicts of interest to declare.

The content of this article reflects the personal opinion of the author/s and is not
necessarily the official position of the European Society of Cardiology.

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