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Table of Contents
Year : 2021  |  Volume : 10  |  Issue : 1  |  Page : 23-25

Multi-vessel coronary artery dissection in a patient with congenital aortopathy

1 Rajaie Cardiovascular Medical and Research Center, Iran University of Medical Sciences, Tehran, Iran
2 Intervention Research Center, Rajaie Cardiovascular Medical and Research Center, Tehran, Iran

Date of Submission04-Dec-2020
Date of Decision09-Jan-2021
Date of Acceptance09-Feb-2021
Date of Web Publication29-Jun-2021

Correspondence Address:
Dr. Sedigheh Saedi
Rajaie Cardiovascular Medical and Research Center, Vali-AsrAve, Adjacent to Mellat Park, Tehran
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/rcm.rcm_46_20

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Spontaneous coronary artery dissection (SCAD) is a well-documented cause of acute coronary syndrome and could have life-threatening consequences. We report the case of young female with a prior history of cardiac surgery due to congenital aortic stenosis presenting with multi-vessel SCAD. She was successfully managed with medical treatment.

Keywords: Fibromuscular dysplasia, myocardial infarction, spontaneous coronary artery dissection, women

How to cite this article:
Fakhrabadi AA, Zanganehfar ME, Saedi S, Maleki M, Ansari MJ, Pouraliakbar HR. Multi-vessel coronary artery dissection in a patient with congenital aortopathy. Res Cardiovasc Med 2021;10:23-5

How to cite this URL:
Fakhrabadi AA, Zanganehfar ME, Saedi S, Maleki M, Ansari MJ, Pouraliakbar HR. Multi-vessel coronary artery dissection in a patient with congenital aortopathy. Res Cardiovasc Med [serial online] 2021 [cited 2022 Dec 9];10:23-5. Available from: https://www.rcvmonline.com/text.asp?2021/10/1/23/319790

  Introduction Top

Spontaneous coronary artery dissection (SCAD) is a rare but well-described cause of acute coronary syndrome (ACS). SCAD is described as a spontaneous tear in the coronary arterial endothelial lining by nonatherosclerotic causes. The prevalence is estimated to be about 1%–4% of all ACS patients and is implicated in 0.5% of sudden cardiac deaths.[1],[2] SCAD is believed to be the etiologic factor for myocardial infarction in about one-third of women younger than 50 years of age.[2],[3] In SCAD, myocardial ischemia results from intimal disruption and development of intramural hematoma (IMH) rather than atherosclerotic type intraluminal thrombus formation. Diagnosis is generally made during coronary catheterization. Thorough knowledge and careful interpretations of the angiographic findings are of paramount importance. SCAD is classified into three main types based on angiographic appearance:

  • Type 1: A typical dissection flap or a linear filling defect is seen in the vessel
  • Type 2: Long segment (usually >20 mm), smooth, diffused narrowing, and stenosis of variable severity is observed. This type is subclassified into Type 2A consisting of diffuse arterial narrowing and IMH between two normal proximal and distal segments and 2B when the narrowing extends to the distal end of the artery
  • Type 3: Mimics atherosclerotic type focal or tubular stenosis (usually <20 mm in length) and requires optical coherence tomography or intravascular ultrasound to diagnose the IMH and false lumen formation.[4]

We report the rare case of multi-vessel SCAD with various subtypes of the coronary dissection occurring concomitantly in a young female. She was treated with full medical therapy and showed remarkable recovery in the follow-up.

  Case Report Top

A 40-year-old female with a previous history of aortic valve replacement at the age of 15 years due to congenital aortic stenosis and later aortic valve cleaning and replacement of ascending aorta with tube graft due to severe pannus ingrowth and ascending aorta aneurysm at age 37 presented to the emergency department with new-onset exertional chest discomfort. She had no coronary artery disease risk factors or recent history of pregnancy. She negated the use of any drugs except for warfarin and aspirin.

Physical examination was within the normal limits, and there were no finding in favor of connective tissue diseases. Electrocardiography (ECG) showed nonspecific ST-T changes. Laboratory tests including inflammatory markers were all in the normal range except for a slightly elevated serum Troponin-I level. Echocardiography revealed preserved left ventricular function without regional wall motion abnormality. Mechanical aortic valve had normal motion and hemodynamic study, and no evidence of thrombosis and the aorta had no dissection flap.

An aortic and coronary computed tomographic angiography (CTA) performed before her last surgery was entirely normal; however, due to episodes of chest pain, CTA was done and showed evidence multi-vessel coronary artery dissection with IMH formation in most coronary territories. There were circumferential hypodense lesions from origin of the left main artery extending to the left anterior descending (LAD), left circumflex (LCx), and obtuse marginalis (OM) branches. Linear density was visualized in the distal parts of the right coronary artery (RCA) in favor of intimal dissection flap. Aorta and its main branches were normal with no evidence of dissection. Selective coronary angiography revealed coronary dissection in three vascular beds and each with a different subtype. LAD had type 2B dissection, large OM and LCx had type 2A, and the RCA showed Type 1 coronary dissection [Figure 1], [Figure 2], [Figure 3].
Figure 1: Coronary computed tomography findings and angiographic images of a Type 2B intramural hematoma of the left anterior descending coronary artery at time of presentation

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Figure 2: Coronary computed tomography, three-dimensional reconstruction and fluoroscopic images of a Type 2A intramural hematoma in the left circumflex and the marginalis branch

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Figure 3: Type 1 coronary dissection with dissection flap in the right coronary artery

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As the patient was hemodynamically stable, we decided against an invasive strategy and managed her conservatively with institution of full medical treatment, including aspirin, warfarin, statin, beta blockers, and nitrates. The patient remained stable during the course of hospitalization. The chest pain and ECG changes resolved and there were no arrhythmias or other complications. She was discharged home in good conditions with improved functional capacity. Coronary CTA was repeated in 1 month and showed almost total resolution of the previous LAD IMH with only a small residue in the proximal part. There was no change in the LCx and RCA involvement [Figure 4]. As she remained symptom-free, we decided to continue the medical therapy with short-interval clinical follow-up. Patient consent for the case report publication was obtained.
Figure 4: Follow-up coronary computed tomography showing healing in the left anterior descending coronary artery (a) and persistent intramural hematoma and the flap in the left circumflex (b) and right coronary arteries (c)

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  Discussion Top

The predisposing risk factors and pathological basis of the SCAD are not well established. Several associations have been implicated, including female sex, pregnancy, and early postpartum state and related hormonal changes, mechanical or emotional stress, systemic arteriopathies, fibromuscular dysplasia, connective tissue, and systemic inflammatory disorders and drugs abuse. However, about 20% of the cases have no predisposing factor and are listed as idiopathic.[2],[5],[6] Our patient had a history of congenital aortic disease and remote manipulation of aortic valve and proximal of aorta that could serve as an underlying etiology.

The most frequently involved coronary is the anterior descending artery (LAD). Multi-vessel involvement is uncommon and has been reported in 10%–20% of the SCAD patients. Patients with SCAD usually present with signs and symptoms similar to ACS. The most common presentation is acute severe chest pain but sudden cardiac death could occur. SCAD remains underdiagnosed or neglected on many occasions that might lead to harmful consequences for the patient.[2],[4]

While SCAD is a well-known cause of ACS, the treatment of choice still remains controversial. In general, conservative and medical management is preferred to invasive revascularization unless there are high-risk features such as left main dissection or those who are hemodynamically unstable with evidence of ongoing ischemia. In fact, inappropriate use of invasive strategies and percutaneous coronary intervention could result in worsening clinical condition or adverse outcomes.[2],[7],[8] Currently, medical management is started and maintained similar to patients with ACS. CTA is a valuable imaging modality in follow-up and shows healing process occurring in many patients over time.[8]

  Conclusion Top

SCAD is a rare yet serious cause of nonatherosclerotic myocardial infarction that should particularly be considered in young women presenting with ACS symptoms. Except for those with unstable or high-risk clinical conditions, the majority of patients are treated conservatively. The overall survival is good and most lesions heal during the follow-up; however, a potential for long-term adverse events exists.

Declaration of patient consent

The authors certify that they had obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Ethical clearance

RHC ethics committee approved of the study (reg number : 99151).

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Saw J, Humphries K, Aymong E, Sedlak T, Prakash R, Starovoytov A, et al. Spontaneous coronary artery dissection: clinical outcomes and risk of recurrence. J Am Coll Cardiol 2017;70:1148-58.  Back to cited text no. 1
Hayes SN, Kim ES, Saw J, Adlam D, Arslanian-Engoren C, Economy KE, et al. Spontaneous coronary artery dissection: Current state of the science: A scientific statement from the American Heart Association. Circulation 2018;137:e523-57.  Back to cited text no. 2
Saw J, Aymong E, Mancini GB, Sedlak T, Starovoytov A, Ricci D. Nonatherosclerotic coronary artery disease in young women. Can J Cardiol 2014;30:814-9.  Back to cited text no. 3
Tanaka A. Shedding light on pathophysiology of spontaneous coronary artery dissection. JACC Cardiovasc Imaging 2019;12:2489-91.  Back to cited text no. 4
Gilhofer TS, Saw J. Spontaneous coronary artery dissection: A review of complications and management strategies. Expert Rev Cardiovasc Ther 2019;17:275-91.  Back to cited text no. 5
Almaddah NK, Morsy MS, Dishmon D, Khouzam RN. Spontaneous coronary artery dissection: An often unrecognized cause of acute coronary syndrome. Cleve Clin J Med 2019;86:252-6.  Back to cited text no. 6
Alfonso F, Paulo M, Lennie V, Dutary J, Bernardo E, Jiménez-Quevedo P, et al. Spontaneous coronary artery dissection: Long-term follow-up of a large series of patients prospectively managed with a “conservative” therapeutic strategy. JACC Cardiovasc Interv 2012;5:1062-70.  Back to cited text no. 7
Adlam D, Alfonso F, Maas A, Vrints C, Writing Committee. European Society of Cardiology, acute cardiovascular care association, SCAD study group: A position paper on spontaneous coronary artery dissection. Eur Heart J 2018;39:3353-68.  Back to cited text no. 8


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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