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MANAGEMENT OF SCAD

Management of SCAD
Dr Tharusha Gunawardena

Introduction:

Spontaneous coronary artery dissection (SCAD) refers to blood entering and separating the layers of the coronary arterial wall to form a false lumen. This leads to external compression of the true coronary lumen, restricting coronary blood flow and leading to coronary insufficiency. Whilst SCAD is considered a “generally” uncommon cause of acute coronary syndrome (0.1-0.4% of all ACS cases), it contributes to a third of ACS presentations in women <50 (it is the commonest cause of ACS during pregnancy). It is therefore important to consider and recognise SCAD in patients presenting with acute chest pain. The Mayo Clinic registry of 87 consecutive patients with SCAD reported a mean age of 43 years; 82% were female. No cause was identified in 45% of all cases, highlighting that many cases of SCAD remain unexplained.

SCAD should be distinguished from atherosclerotic dissections arising from plaque rupture events or erosions allowing blood to enter the intimal space and from iatrogenic dissections arising during coronary procedures. Certain SCAD subtypes can be challenging to distinguish angiographically, so further delineation with intracoronary imaging is advisable.

Predisposing factors:

  • female sex
  • peripartum period
  • fibromuscular dysplasia (FMD)
  • connective tissue disease
  • hormonal therapy
  • emotional or stressful triggers (such as exercise, vomiting, sympathomimetic drugs such as cocaine and amphetamines)

The patients usually do not have traditional atherosclerotic risk factors, though there is a comparable prevalence of hypertension and hypercholesterolemia in the SCAD cohort compared to the atherosclerotic ACS population. A Canadian cohort study suggested that migraines (33%), anxiety (20%), and depression (20%) were relevant comorbid associations.

 

Clinical presentation:

Whilst patients may present with typical ACS symptoms of chest discomfort, diaphoresis and nausea, symptoms may seem to have a more abrupt onset. SCAD has also been known to present with acute heart failure, arrhythmia or cardiac arrest. Up to 50% may present with STEMI. Lobo et al (2019) identified 53 SCAD patients among a cohort of 5208 STEMI patients. Those SCAD patients were found to have been more likely (than those with atherosclerotic disease) to develop cardiogenic shock or have left main stem (LMS) or left anterior descending (LAD) disease.

Pregnancy and SCAD:

Pregnant patients with cardiac conditions are managed with a greater degree of anxiety and wider concern. Patients with pregnancy-associated SCAD unfortunately tend to have a more severe clinical course. Most cases occur in the first four weeks post-partum, but can occur at any time during the pregnancy or post-partum.

Angiography should NOT be avoided solely based on concerns regarding fetal radiation, given the clear benefit to the mother of early angiography to confirm the diagnosis and the relative low level of fetal radiation with the appropriate precautions (Hayes et al, JACC 2020).

Management is largely as for non-pregnant patients, but patients should be managed in a multidisciplinary capacity including obstetricians. There will be special considerations for longer term medications, including choice of betablockers and avoidance of ACE-inhibitors.

Despite limited data on outcomes, if a patient has had SCAD then they are generally advised against pregnancy even if the initial SCAD was not pregnancy related (ESC 2018). The sequelae of a previous SCAD, including LV function, will affect individual risk. The theoretical risk of recurrence would necessitate management as a high-risk pregnancy, with close collaboration between obstetricians and cardiologists.

A Mayo Clinic registry study (Gulati 2015) looked at 31 pregnancies in 22 SCAD women resulting in 19 live births, with two patients experiencing recurrence of SCAD but the majority having uncomplicated pregnancies.

Diagnosis:

SCAD is underdiagnosed and this is in part likely due to the heterogeneity of appearances in presentation. 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. There are various different classifications encompassing the different appearances, a commonly used one as below:

Figure 1: the four commonly used subtypes in the angiographic classification of SCAD.

  • Type 1 describes the pathognomonic presence of contrast within arterial wall delineating multiple radiolucent lumens.
  • Type 2 describes diffuse and smooth stenosis of usually >20 mm length with varying severity.
  • Type 3 describes focal or tubular stenosis, which mimics atherosclerosis and requires intracoronary imaging to confirm the presence of intramural hematoma.
  • Type 4 includes total occlusion of the vessel

Source: Djokovic A, Krljanac G, Matic P, Zivic R, Djulejic V, Marjanovic Haljilji M, Popovic D, Filipovic B, Apostolovic S. Pathophysiology of spontaneous coronary artery dissection: hematoma, not thrombus. Front Cardiovasc Med. 2023 Oct 20;10:1260478. doi: 10.3389/fcvm.2023.1260478. PMID: 37928766; PMCID: PMC10623160.

In CanSCAD, the most common angiographic appearance was Type 2 (60.2% of presentations)

Figure 2: Intracoronary imaging of SCAD:

Spontaneous coronary artery dissection (SCAD) hallmarks in intracoronary imaging. FL, false lumen; IMH, intramural haematoma; TL, true lumen. Arrow indicates myoendothelial lamina (intimo-medial flap).

SOURCE: Macaya, Fernando & Salinas, Pablo & Gonzalo, Nieves & Fernández-Ortiz, Antonio & Macaya, Carlos & Escaned, Javier. (2018). Spontaneous coronary artery dissection: Contemporary aspects of diagnosis and patient management. Open Heart. 5. e000884. 10.1136/openhrt-2018-000884.

Management:

The Initial treatment given to standard ACS patients are applicable to SCAD, with the initial use of dual antiplatelet agents, heparin and beta-blockers to preserve patency of the true lumen and prevent thrombotic occlusion.

As most dissected segments will heal spontaneously, conservative management is advisable in stable patients with SCAD. PCI is the preferred revascularisation strategy, but is associated with significant challenges and has reported success rates of <50%. Therefore recommendations to intervene are limited to certain scenarios:

  • complete vessel occlusion with TIMI 0 flow, which is unlikely to resolve with medical treatment alone
  • left main stem involvement
  • ongoing ischaemia
  • recurrent chest pain
  • haemodynamic instability
  • sustained ventricular arrhythmias.

GPIIb/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.

PCI in SCAD

As should be common practice, conscientious consideration of careful catheter manipulation, coaxial positioning and contrast injections are especially important in SCAD, as the risk of iatrogenic dissection is greater. Coronary angiography provides poor visualisation of intramural haematoma, and intravascular imaging with OCT or IVUS is recommended in all PCI cases. Whilst OCT may be used with caution, given the necessity for a strong contrast clearing injection, it could be considered superior given its great resolution than IVUS. It gives more detail to confirm guidewire position in the true lumen, detail the site of intramural haematoma and intimal tear, facilitate appropriate vessel sizing and optimise stent expansion.

Technical difficulties include careful negotiation of the guidewire to ensure it is truly luminal, to avoid dissection or haematoma extension and side branch occlusion.

Stent placement can result in haematoma propagation and loss of vessel flow. The data suggests that SCAD cases disproportionately require multiple additions of long lengths of stents. There is also a greater long-term risk of stent malappposition following reabsorption of the intramural haematoma and therefore conferring a longer term risk of late stent complications.

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 a reported series suggesting that perhaps PCI for SCAD would be safer in surgical centres.

Cutting balloons in SCAD:

Given the additional difficulties of stent implantation including intramural haematoma propagation and side branch compromise, there are case reports of patients being treated with cutting or scoring balloons to “release” the haematoma.

Figure 3: A case report detailing pre and post cutting balloon treatment of SCAD

  1. Complete occlusion of the mid-section of the LAD artery and a TIMI flow score of 0-1 due to SCAD (arrow).
  2. Coronary angiography showing 2.5-mm cutting balloon dilated in the proximal and distal segments of LAD artery (arrow).
  3.  Coronary angiography showing coronary flow restored to TIMI flow 3 after ballooning (arrow).

Source: Zghouzi M, Moussa Pacha H, Sattar Y, Alraies MC. Successful Treatment of Spontaneous Coronary Artery Dissection With Cutting Balloon Angioplasty. Cureus. 2021

Other case reports of the use of cutting/scoring balloons in SCAD:

Author Year Age Sex Location Treatment Imaging follow-up
Terzian et al. [10] 2019 34 F LAD Scoring balloon angioplasty Coronary angiogram at 9 months
Kaya et al. [11] 2019 46 F LAD Cutting balloon angioplasty Coronary CTA at 1 and 6 months
Sharma et al. [12] 2019 53 F RCA Cutting balloon angioplasty NA
Main et al. [13] 2019 62 F Large diagonal branch Cutting balloon angioplasty ECG in 6 weeks
Bresson et al. [14] 2019 36 F Proximal LAD Stenting and cutting balloon angioplasty NA
McGrath et al. [15] 2018 51 F LM, LAD, LCX Stenting and cutting balloon angioplasty NA
Noguchi et al. [16] 2018 42 M LAD, LCX Cutting balloon angioplasty and stenting Coronary angiogram at 6 months
Ito et al. [17] 2017 46 F LAD Cutting balloon angioplasty Coronary CTA at 3 months
Alkhouli et al. [18] 2016 50 F LAD Cutting balloon angioplasty and stenting NA
Yumoto et al. [19] 2014 47 F LAD Cutting balloon angioplasty Coronary angiogram at 6 months
Uema et al. [20] 2013 42 F LAD Cutting balloon angioplasty NA

 

Post SCAD screening:

A third to half of SCAD patients will also be diagnosed with Fibro Muscular Dysplasia; therefore SCAD patients should have CT/MR angiography for extra-coronary vascular abnormalities. Some studies have reported FMD in 11-86% of SCAD patients.

Outlook/prognosis of SCAD:

Two single-centre registries have provided important data on the natural history and prognosis of SCAD.

Saw et al. 2014 reported the results of a single-centre Canadian registry of 164 patients with SCAD.

  • 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.
  • 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. 2012 reported the results for 189 patients with first presentation of a SCAD episode. 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. The PCI failure rate was 53%
  • 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).

These findings are in keeping with previous reports suggesting that patients should receive careful in-hospital monitoring for up to one week due to the risk of further, recurrent events. This contrasts with atherosclerotic ACS cases where guidelines emphasise an early intervention and discharge approach.

In patients with recurrent SCAD, 75%-90% of those 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.

Take home messages:

  • SCAD is an important cause of ACS and should be considered particularly in young women without traditional cardiovascular risk factors.
  • 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 LMS involvement, complete vessel occlusion, ongoing chest pain or haemodynamic instability will require coronary revascularisation. 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 failure and complications.
  • Although long-term prognosis is excellent, the risk of recurrent SCAD events is significant, with an average rate of 5% per year.

References:

  • Saw J, Starovoytov A, Humphries K, et al. Canadian Spontaneous Coronary Artery Dissection Cohort Study. ESC Congress 2018; Munich, Germany. August 30, 2018.
  • Saw J., Aymong E., Sedlak T., Buller C. E., Starovoytov A., Ricci D., Robinson S., Vuurmans T., Gao M., Humphries K., & Mancini G. B. Spontaneous coronary artery dissection: association with predisposing arteriopathies and precipitating stressors and cardiovascular outcomes. Circ Cardiovasc Interv, 2014, Oct. 7 (5): 645-55.
  • Lobo AS, Cantu SM, Sharkey SW et al. Revascularisation in Patients with Spontaneous Coronary Artery Dissection and ST-Segment Elevation Myocardial Infarction. J Am Coll Cardiol 2019;74(10):1290-1300
  • Hayes SN, Tweet MS, Adlam D et al. Spontaneous Coronary Artery Dissection: JACC State-of-the-Art Review. J Am Coll Card 2020;76(8):961-984
  • Tweet MS, Hayes SN, Gulati R et al. Pregnancy after spontaneous coronary artery dissection: a case series. Ann Intern Med 2015;162(8):598-600
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  • Saw J, Aymong E, Sedlak T et al. Spontaneous Coronary Artery Dissection: Association with Predisposing Arteriopathies and Precipitating Stressors and Cardiovascular Outcomes. Circ Cardiovasc Interv 2014;7(5):645655
  • Swamy, Pooja & Parwani, Purvi & Mamas, Mamas & Bharadwaj, Aditya. (2020). Role of Intravascular Imaging in the Diagnosis and Treatment of Spontaneous Coronary Artery Dissection. Current Cardiovascular Imaging Reports. 13. 10.1007/s12410-020-09547-x.
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  • Djokovic A, Krljanac G, Matic P, Zivic R, Djulejic V, Marjanovic Haljilji M, Popovic D, Filipovic B, Apostolovic S. Pathophysiology of spontaneous coronary artery dissection: hematoma, not thrombus. Front Cardiovasc Med. 2023 Oct 20;10:1260478. doi: 10.3389/fcvm.2023.1260478. PMID: 37928766; PMCID: PMC10623160.
  • Fenestration of a spontaneous coronary hematoma with life-threatening dissection: a case of recurrent and multivessel spontaneous coronary artery dissections in a young female [Article in French] Terzian Z, Demian H, Karrillon GJ, Aubry P. Ann Cardiol Angeiol (Paris) 2019;68:382–388.
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  • Novel use of cutting balloon to manage compressive subintimal hematoma during left main stenting in a patient with spontaneous coronary artery dissection. McGrath BM, Vo MN. Clin Case Rep. 2018;6:1291–1295.
  • Usefulness of cutting balloon angioplasty prior to stenting with intravascular ultrasound imaging guidance for spontaneous multi-vessel coronary artery dissection including the left main coronary artery. Noguchi M, Obunai K, Fukui Y, Okumura H, Watanabe H. Intern Med. 2018;57:1867–1871.
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  • Zghouzi M, Moussa Pacha H, Sattar Y, Alraies MC. Successful Treatment of Spontaneous Coronary Artery Dissection With Cutting Balloon Angioplasty. Cureus. 2021 Mar 4;13(3):e13706. doi: 10.7759/cureus.13706. PMID: 33824840; PMCID: PMC8016528.
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