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Clinician Update
Pregnancy-Related Spontaneous Coronary Artery Dissection
Ram Vijayaraghavan, MD; Subodh Verma, MD, PhD; Nandini Gupta, MD; Jacqueline Saw, MD
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C
ase 1: A 34-year-old gravida 2,
para 2 woman was admitted with
anterior ST-segment–elevation myocardial infarction 10 days postpartum.
Coronary angiography showed left
main (LM) dissection with occlusion
of the proximal left anterior descending (LAD) artery (Figure 1A), and her
ejection fraction was 35%. Bare metal
stents were successfully implanted
from the LM into the proximal LAD
and diagonal arteries. Antegrade extension of dissection into the circumflex
was left untreated because her ischemia
resolved. However, repeat angiography
at 5 months with optical coherence
tomography showed in-stent restenosis
in the LM, stent-strut malapposition
in the proximal LAD (Figure 1B), and
residual circumflex dissection, prompting subsequent coronary artery bypass
grafting (CABG).
Case 2: A 40-year-old woman
(gravida 2, para 2) presented with
non–ST-segment–elevation myocardial
infarction 10 days after elective cesarean section. Coronary angiography
showed proximal LAD dissection with
an occluded midsegment and collateralization from the right coronary artery
(Figure 1C), and her ejection fraction
was 40%. After the initial angioplasty,
extensive dissections from the proximal to distal LAD and diagonal arteries
were noted. She underwent emergency
bailout CABG with anastomosis of
the left internal mammary artery to
the distal LAD and saphenous vein
graft to the diagonal artery. Follow-up
computed tomography angiography at
3 months demonstrated mild residual
stenosis of the proximal LAD with a
patent left internal mammary artery,
but the saphenous vein graft to the
diagonal artery was occluded.
Case 3: A 37-year-old woman
(gravida 2, para 2) presented with
non–ST-segment–elevation myocardial infarction 9 days after an uneventful vaginal delivery at 38 weeks.
Her troponin I peaked at 26 μmol/L.
Coronary angiography showed proximal LAD dissection with 80% stenosis (Figure 1D), and her ejection
fraction was 40%. She was treated
conservatively because her chest pain
resolved. Repeat angiography at 5 days
showed improvement of stenosis to
50% (Figure 1E). Computed tomography angiography at 14 months showed
almost complete normalization of the
proximal LAD. She was found incidentally to have fibromuscular dysplasia of
her external iliac artery (Figure 1F).
Introduction
Pregnancy-related (P-) spontaneous
coronary artery dissection (SCAD) is
a rare and potentially lethal complication of pregnancy. It is estimated that
1 in 16 000 pregnancies is complicated
by acute myocardial infarctions in
the United States; up to one fourth of
these are due to P-SCAD.1,2 Although
the true prevalence of overall SCAD is
unknown and older reports suggested
that pregnancy caused a substantial proportion of SCAD, contemporary series
estimated that P-SCAD accounted for
only <5% of SCAD cases.3,4
SCAD is defined as a separation
within the arterial wall by intramural hematoma, which can occur by
an intimal rupture initiating medial
dissection or more commonly by a
spontaneous intramedial hemorrhage
such as that resulting from disruption of the vasa vasorum (Figure 2).5,6
P-SCAD can occur during pregnancy (as early as 2 weeks after
From the Division of Cardiology, Rouge Valley Health System, Scarborough, ON, Canada (R.V.); Division of Cardiac Surgery, Keenan Research Centre
for Biomedical Science and Li Ka Shing Knowledge Institute of St. Michael’s Hospital, Toronto, ON, Canada (S.V.); University of Ottawa, Ottawa, ON,
Canada (N.G.); and Division of Cardiology, Vancouver General Hospital, Vancouver, BC, Canada.
Correspondence to Jacqueline Saw, MD, FRCPC, FACC, Clinical Associate Professor, University of British Columbia, Division of Cardiology,
Vancouver General Hospital, 2775 Laurel St, 9th Floor, Vancouver, BC, V5Z 1M9, Canada. Email [email protected] or Subodh Verma, MD, PhD,
FRCSC, FAHA, Professor, University of Toronto, Division of Cardiac Surgery, 8th Floor Bond Wing, St. Michael’s Hospital, Toronto, ON, Canada.
E-mail [email protected]
(Circulation. 2014;130:1915-1920.)
© 2014 American Heart Association, Inc.
Circulation is available at http://circ.ahajournals.org
DOI: 10.1161/CIRCULATIONAHA.114.011422
1915
1916 Circulation November 18, 2014
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Figure 1. A, Case 1: left main dissection (+) with smooth stenosis extending into the left anterior descending coronary artery (LAD),
which is occluded proximally (*). B, Optical coherence tomography at follow-up showing severe stent strut malapposition in the proximal
LAD. C, Case 2: extensive dissection with multiple radiolucent lumen (*) originating from the ostial LAD and occluded in the midsegment
(arrow). D, Case 3: proximal LAD dissection with smooth 80% stenosis, which improved 5 days later to ≈50% stenosis (E) with contrast
hang-up in the arterial wall (arrow). F, Fibromuscular dysplasia changes of the right external iliac artery in case 3.
conception)2 or postpartum, defined
as within 6 weeks of delivery by the
World Health Organization.7 The term
peripartum SCAD has variable and
limited definitions, ranging from the
third trimester of pregnancy to 3 to 5
months postpartum.8–10 We have also
observed late P-SCAD several months
postpartum,9,11 even as late as 11 and
16 months postpartum, especially in
women still breastfeeding.3,11 Thus, the
term P-SCAD is preferred for broader
inclusion and may be categorized as
antepartum, early postpartum (within
6 weeks of delivery), late postpartum
(6 weeks to 12 months), and very late
postpartum (12–24 months).
Multiple
predisposing
causes
of P-SCAD have been described.
Estrogen and progesterone surges
during pregnancy (especially recurrent
exposures with multiparity) can cause
structural changes with recurrent and
chronic accumulation of medial degeneration such as that from decreased
collagen synthesis and increased media
mucopolysaccharide content, causing weakening of the tunica media.
Histopathological features include
fragmented and disorganized elastic
and collagen fibers, microcystic mucinous pools, cystic medial necrosis,
and inflammatory infiltration at arterial dissection planes.8 In particular,
periadventitial eosinophilic infiltration was observed, but it is unclear
if this was reactive to or causative of
SCAD.12 Aside from pregnancy-related
structural changes, fibromuscular dysplasia was recently discovered to be
strongly associated with SCAD and
was reported with P-SCAD.11 Other
systemic predisposing causes such as
connective tissue disorders (eg, EhlerDanlos type IV, Marfan syndrome) and
inflammatory conditions (eg, systemic
lupus erythematosus, ulcerative colitis)
may also be associated with P-SCAD
but are infrequently linked. The physiological changes during pregnancy,
with >50% increase in cardiac output,
and the hemodynamic effects of laborintensifying vascular shear stresses
can also precipitate P-SCAD when
superimposed on arterial structural
changes related to pregnancy or other
predisposing cases. The association of
breastfeeding with late and very late
postpartum P-SCAD is intriguing and
may suggest that hormonal changes
Vijayaraghavan et al Pregnancy-Related SCAD 1917
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Figure 2. A, Normal coronary arterial wall. B, Spontaneous coronary artery dissection (SCAD) with intramural hematoma in the arterial
wall without intimal rupture, which may be related to vasa vasorum rupture. Predisposing histopathological arterial wall abnormalities of
periadventitial inflammation, cystic medial necrosis, fibromuscular dysplasia, and medial degeneration are depicted. C, SCAD with intimal
rupture depicted as the cause of dissection and intramural hematoma.
with lactation13 compound the effects
of pregnancy.
Clinical Presentation
and Diagnosis
The mean presenting age with P-SCAD
was 33 years in the 3 largest medical
literature review series, with timing at
a mean of 23 to 24 days postpartum or
32 weeks’ gestation. The vast majority (>85%) were multiparous, with a
mean parity of 2.4 to 2.8 and gravidity of 2.7 to 3.1.8,10,14 Clinical presentations included chest pain, dyspnea,
acute myocardial infarction, congestive heart failure, ventricular arrhythmia (including sudden cardiac death),
and cardiogenic shock. Such presentations in pregnant or postpartum women
should prompt early invasive coronary
angiography to rule out P-SCAD, with
precautions to minimize radiation
exposure in antepartum women.
The gold standard imaging is coronary angiography for SCAD; computed
tomography angiography has inadequate spatial resolution to visualize
SCAD in smaller coronary arteries,
especially those <2 mm in diameter.6
Given the possibility of iatrogenic
catheter-induced dissection in patients
with arterial fragility prone to SCAD,
cautious and meticulous angiographic
techniques are warranted with careful
attention to pressure tracing, avoidance
of deep intubation (especially with the
radial approach in which iatrogenic
LM dissections were reported with
SCAD),3 gentle contrast injections (to
minimize propagation of dissection),
and consideration of initial nonselective
injections to visualize the LM. Despite
these measures, propagation of dissection may still occur, and prompt recognition and management are imperative.
The angiographic appearance, categorization, and algorithm for SCAD
diagnosis have been described previously.15 The stereotypical appearance
of contrast arterial wall stains (type
1 SCAD) is present in fewer than one
third of cases3; instead, the most common angiographic appearance is a long
diffuse stenosis (type 2 SCAD) without intimal disruption, as depicted in
our cases. Intravascular ultrasound or
optical coherence tomography is useful
to confirm SCAD in nonstereotypical
cases. Vascular calcification and other
changes typical of atherosclerosis are
1918 Circulation November 18, 2014
usually absent. Patients with P-SCAD
also have a tendency for proximal coronary involvement (including the LM
and LAD), multivessel dissections,
and worse left ventricular ejection
fraction compared with patients with
non–P-SCAD.9
Management
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An algorithm to guide management,
including percutaneous coronary
intervention (PCI) and surgical interventions (eg, CABG, extracorporeal
membrane oxygenation or left ventricular assist device), is described
in Figure 3. Conservative therapy is
preferred in the management of stable
SCAD patients,6,16 especially because
the vast majority of dissected segments
heal spontaneously (We reported 79 of
79 cases of healing on repeat angiograms after 4 weeks).3 The primary
factors necessitating revascularization
are the presence of ongoing ischemia/
infarction, hemodynamic instability,
and involvement of the LM, which,
unfortunately, are frequently encountered with P-SCAD.
PCI is typically the first-line revascularization strategy for SCAD for
feasible anatomy except for LM
and ostial LAD dissection, in which
case CABG is generally preferred.
Unfortunately, PCI for SCAD is
fraught with challenges, with technical success rates of ≈65%.3,17 Even if
PCI is technically successful, extension of dissections occurs in 25% to
60% of cases, and long-term durable
results were achieved in only ≈30%.3,17
Wiring into the true lumen can be challenging, especially in the presence of
intimal disruption. Confirmation of
true lumen position may be achieved
with intravascular ultrasound or optical coherence tomography, before
angioplasty or stenting. Strategies
to minimize hematoma extension
include implanting a very long stent
that extends 5 to 10 mm on both sides
of the dissection and stenting the distal edge first (to limit apical vessel
extension), followed by the proximal
edge and then the middle section.6,18
Optical coherence tomography or
intravascular ultrasound is also useful
to achieve better short- and long-term
results by ensuring adequate dissection coverage and appropriate sizing.
Drug-eluting stents are typically used,
given the extensive lengths required
and the risks of subsequent restenosis observed.3 In addition, because
intramural hematoma spontaneously
resorbs over weeks, subacute and late
malapposition (as shown in case 1)
with the risk of stent thrombosis is a
potential concern. Thus, drug-eluting
bioabsorbable stents may have possible advantages.
CABG is typically reserved for
patients with LM dissection or
patients in whom PCI is not feasible
or is unsuccessful. Similar to PCI,
there are technical challenges with
CABG, including isolating suitable
nondissected graft insertion sites or
surgically approximating vessel layers at the site of graft insertion. Longterm durability with surgical grafts for
SCAD was reported to be poor. In the
Mayo Clinic series, 11 of 15 grafts
were occluded at the long-term follow-up,17 probably reflecting the natural history of spontaneous healing of
dissected segments, resulting in eventual graft failure from competitive
flow. However, the suboptimal longterm graft patency should not detract
from choosing CABG in patients in
Figure 3. Recommended management algorithm for pregnancy-related (P-) spontaneous coronary artery dissection (SCAD). CABG
indicates coronary artery bypass graft; Circ, circumflex; CP, chest pain; ECMO, extracorporeal membrane oxygenation; IABP, intra-aortic
balloon pump; ICD, implantable cardioverter-defibrillator; LAD, left anterior descending artery; LM, left main; LVAD, left ventricular assist
device; Med tx, medical treatment; PCI, percutaneous coronary intervention; VF, ventricular fibrillation; and VT, ventricular tachycardia.
Vijayaraghavan et al Pregnancy-Related SCAD 1919
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whom PCI is not feasible because, in
many life-threatening cases, it is the
only means of establishing coronary
flow and salvaging ischemic myocardium acutely. In these instances,
the surgical intervention (often with
hemodynamic support) enabled shortterm survival of these patients and
myocardial preservation.
Mechanical support with intra-aortic balloon pump is often required for
hemodynamically unstable patients.
The use of extracorporeal membrane
oxygenation and a left ventricular
assist device should be considered
for patients with persistent cardiogenic shock, typically in conjunction
with revascularization. In rare cases,
emergent heart transplantation may be
required,10 particularly if CABG is not
feasible or is unsuccessful and patients
remain unstable despite maximal
hemodynamic support.
Pharmacological
therapy
for
P-SCAD is similar to standard SCAD
therapy, as previously described.6
Intravenous heparin is routinely
started for standard short-term myocardial infarction management but
discontinued once SCAD is identified. Glycoprotein IIb/IIIa inhibitors
and thrombolytics are contraindicated because of potential extension
of intramural hematoma with these
potent agents. Long-term medical
therapy with aspirin and β-blocker
(which reduces arterial wall stress)
is appropriate. Optional agents
include
angiotensin-converting
enzyme inhibitors (for left ventricular dysfunction), statins (for preexisting dyslipidemia), and nitrates
(for heart failure, concomitant vasospasm, or residual coronary stenosis). Clopidogrel is mandatory for
patients receiving coronary stents
(for 1–12 months) and is commonly
administered for a limited duration
(<12 months) without PCI. There are
no data for novel P2Y12 antagonists
with SCAD. For patients treated conservatively, follow-up exercise testing should be considered, and repeat
coronary angiography (or computed
tomography if proximal/large arteries
dissected) should be performed if
ischemia is present to assess for subsequent revascularization necessity.
risk stratify, treat, and follow up
these patients.
Natural History
and Outcomes
Dr Saw has research funding support from
the Canadian Institutes of Health Research,
Abbott Vascular, AstraZeneca, Boston
Scientific, Servier, and St Jude Medical.
Dr Saw also has received speaking honoraria from AstraZeneca, Boston Scientific,
St. Jude Medical, and Sunovion. The other
authors report no conflicts.
Historical case reports and series suggested high mortality rates (38%–
50%), which were likely influenced
by limited options for intervention
and early reporting bias of autopsy
cases.8,14 Contemporary reports of
patients surviving to cardiac catheterization with prompt diagnosis and
management (including our 3 early
postpartum cases) showed more favorable in-hospital outcomes.9,10 However,
patients with P-SCAD appear to have
more sinister presentations (both clinically and angiographically) compared
with patients with non–P-SCAD.
P-SCAD is associated with larger
infarctions with higher peak troponin,
worse left ventricular function, congestive heart failure, and cardiogenic
shock.9 Recurrent coronary dissection in SCAD survivors is quite common, reported in 13% to 18% of larger
series.3,17 It is not known if recurrent
SCAD is higher in P-SCAD survivors.
In a recently reported small series of
SCAD survivors who subsequently
became pregnant, 1 of 7 suffered recurrent SCAD at 9 weeks postpartum
with LM dissection requiring CABG.19
Although this series was small, it highlights that recurrent SCAD can occur
with subsequent pregnancies, and
future pregnancies should be avoided
in SCAD survivors given the potential
severe complications.
Conclusions
P-SCAD is a rare but potentially
catastrophic condition. We described
3 representative cases managed differently in the hospital (conservative treatment, PCI, and CABG) to
demonstrate nuances of management decisions based on clinical
presentation and coronary anatomy.
Clinicians need to be familiar with
angiographic appearances of SCAD
for prompt diagnosis and with management strategies to appropriately
Disclosures
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Pregnancy-Related Spontaneous Coronary Artery Dissection
Ram Vijayaraghavan, Subodh Verma, Nandini Gupta and Jacqueline Saw
Circulation. 2014;130:1915-1920
doi: 10.1161/CIRCULATIONAHA.114.011422
Downloaded from http://circ.ahajournals.org/ by guest on June 17, 2017
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