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Abstract Volume: 1 Issue: 3 ISSN:

Spontaneous Coronary Artery Dissection: A Case Series
Mahmood Hasan Khan*, Shahab Uddin Talukder1, Sohail Ahmed2, Tamzeed Ahmed1, Md. Shamsul Alam1, A Q M Reza1, Aparajita Karim1, Poppy Bala1, Nighat Islam1, Dr. Azfar Hossain Bhuiyan1

 

1 Department of Clinical & Interventional Cardiology, Evercare Hospital Dhaka.

2 Department of Cardiothoracic &Vascular Surgery, Evercare Hospital Dhaka.

*Corresponding Author: Dr. Mahmood Hasan Khan, Department of Clinical & Interventional Cardiology, Evercare Hospital Dhaka.

Received Date:  October 20, 2020

Publication Date: November 01, 2020


Abstract

Spontaneous coronary artery dissection (SCAD) is a rare, infrequent & often missed clinical entity in patients presenting with the acute coronary syndrome (ACS). Patients of SCAD can present with different clinical presentation ranging from myocardial ischemia to myocardial infarction, different types of fatal arrhythmias and even death. Frequently missed due to lack of angiographic recognition, documentations are the major causes of under-diagnosis. With the advancement of diagnostic modalities including intracoronary imaging, there has been an improved diagnosis of SCAD. These series aim to showcase this rare clinical entity as well as make awareness among the Cardiologists to diagnose & further management of the patients to reduce morbidities & mortality among the patients with acute coronary syndrome.

Keywords: Spontaneous coronary artery dissection (SCAD), acute coronary syndrome, myocardial infarction, coronary angiogram

Spontaneous Coronary Artery Dissection: A Case Series

Case Summary
Case 1:

Mr. X, 38 years hypertensive, diabetic & current smoker patient was evaluated for a coronary angiogram. He sustained acute anterior wall STEMI 01 month back, with it he got himself admitted to a local hospital. There he was diagnosed as a case of acute STEMI (Anterior) with late presentation and was treated with anti-platelets, statin, other anti-ischaemic drugs & sub-cutaneous low molecular weight heparin. After stabilization, he was discharged with the advice of a coronary angiogram.

In our center, he was at first clinically evaluated in the outpatient department. There he gave complaints of exertional SOB (NYHA class II/III) since his myocardial infarction (MI). His vitals showed, Pulse: 88/ min, regular; BP: 130/80 mmHg without any postural drop, Heart: S1 & S2 were audible without any added sound, Lungs: Clear without any added sound, Respiratory Rate: 20/ min, Temperature: 98?, SPO2: 94% in room air. He was evaluated for CoVid19. His RT-PCR for CoVid was found Negative. His ECG showed Old anterior wall MI. He was advised for a chest X-ray P/A view & Echo screening. His chest X-ray showed mild cardiomegaly without any other remarkable abnormality. His echo screening showed dilated LA & LV, with regional wall motion abnormality, severe LV systolic dysfunction (LVEF: 25-30%) & Mild MR. Then he got himself admitted for coronary evaluation by coronary angiogram.

His initial blood picture showed,

Hb: 15.3 gm/dl,

TLC: 12.19x109/L,

Platelet: 373x109/L.

Serum creatinine: 0.91 mg/dl,

urea: 30 mg/dl,

sodium: 138 mmol/L,

potassium: 4.3 mmol/L,

chloride: 103 mmol/L,

bicarbonate: 32 mmol/L.

His viral markers were found negative.
Figure 1: Electrocardiograph of the patient showing old anterior MI.


His coronary angiogram was done through the right radial approach under local anesthesia with a 5F Tiger diagnostic catheter & it showed: spontaneous spiral dissection from proximal to mid segment of type IV LAD involving second diagonal branch which was occluded from its proximal segment. In LCx there was a 95% stenosis in the mid-segment with a good size first obtuse marginal branch having 50% of ostial stenosis. His RCA was also totally occluded from its mid-segment which was a dominant vessel. So, coronary angiographically, a triple vessel coronary artery disease was made & considering lesion morphology, character & co-morbidities revascularization with CABG was advised. 

Figure 2: Coronary angiogram of the patient showing spontaneous coronary artery dissection.

 

Case 2:

Mr. Y, 54 years normotensive, non-diabetic & known COPD patient was evaluated for a coronary angiogram. He has a history of positive TMT and was treated with anti-platelets, statin & other anti-ischaemic drugs. 

In our center, he was at first clinically evaluated in the outpatient department. There he gave complaints of exertional angina (CCS class II/III). His vitals showed, Pulse: 80/ min, regular; BP: 130/70 mmHg without any postural drop, Heart: S1 & S2 were audible without any added sound, Lungs: Clear without any added sound, Respiratory Rate: 17/ min, Temperature: 98?, SPO2: 97% in room air. He was evaluated for CoVid19. His RT-PCR for CoVid was found Negative. His ECG was within the normal limit. He was advised for a chest X-ray P/A view, Echo screening & TMT. His chest X-ray was unremarkable. His echo screening showed no regional wall motion abnormality, good LV & RV systolic function (LVEF: 65%) & DRA (Grade-I). Then he got himself admitted for coronary evaluation by coronary angiogram.

His initial blood picture showed, Hb: 16.1 gm/dl, TLC: 10.88x109/L, Platelet: 287x109/L. Serum creatinine: 1.17 mg/dl, sodium: 138 mmol/L, potassium: 3.9 mmol/L, chloride: 99 mmol/L, bicarbonate: 30 mmol/L. His viral markers were found negative.

Figure 3: Electrocardiograph of the patient showing normal findings.

His coronary angiogram was done through the right radial approach under local anesthesia with a 5F Tiger diagnostic catheter & it showed: spontaneous spiral dissection from proximal to mid segment of dominant RCA. In LCx there was a mild narrowing in the proximal segment of the first obtuse marginal branch & 50% narrowing in the proximal segment of a good-sized second obtuse marginal branch. His LAD was a type IV vessel without any disease. So, coronary angiographically, a single vessel coronary artery disease was made & considering lesion morphology, character & co-morbidities revascularization with PCI was advised.

Figure 4: Coronary angiogram of the patient showing spontaneous coronary artery dissection.
 

Discussion

SCAD is defined as a non-traumatic and non-iatrogenic separation of the coronary arterial walls, creating a false lumen (1). This separation can occur between the intima and media or between the media and adventitia, with intramural hematoma (IMH) formation within the arterial wall that compresses the arterial lumen, decreasing ante-grade blood flow and subsequent myocardial ischemia or infarction (1,2). Spontaneous coronary artery dissection, also termed as dissecting aneurysm, intramural hemorrhage, or hematoma, is an uncommon clinical  entity.(3-7) 

The sign & symptoms that raise the possibility of SCAD are as follows:

  

Clinical features that raises suspicion of SCAD

-Myocardial infarction in young women (especially age ≤50)

-Absence of traditional cardiovascular risk factors

-Little or no evidence of typical atherosclerotic lesions in coronary arteries

-Peripartum state

-History of fibromuscular dysplasia

-History of relevant connective tissue disorder: Marfan’s syndrome, Ehler Danlos syndrome, Cystic Medial Necrosis, Fibromuscular Dysplasia

-History of relevant systemic inflammation: Systemic Lupus Erythematosus, Crohn’s disease, Ulcerative colitis, Polyarteritis Nodosa, Sarcoidosis

-Precipitating stress events, either emotional or physical (intensive exercise)

 

Table 1: Depicting the clinical features that raise suspicion of SCAD
 

It usually tends to involve the outer media and causes luminal occlusion by pushing the inner media against the opposing wall. Clotted blood filling the false lumen may simulate coronary thrombosis at the naked eye, masking the dissection. Thus, the accurate scenario of this clinical condition may be underestimated. A review of published reports showed that 69% of the cases were diagnosed at necropsy. (7) Among them 80% of the cases were women, and more than 25% of these were in the peripartum period. (8-22) Involvement of the right coronary artery seems to be more common in men, whereas left anterior descending coronary artery involvement is frequent in women. Coronary artery dissection affects young adults; in a series of cases that were diagnosed before death. According to De Maio et al (6), the mean age was 46 years in males and 38 years in females. The clinical presentation of this includes the entire spectrum of the acute coronary syndrome and is primarily related to the extent of the dissection and the vessel involved. Nonetheless, sudden death without pre- evidence of myocardial infarction is much more frequent. (23) Survival is possible if obstruction of the lumen is incomplete, or an uncomplicated myocardial infarction. (24-27) Spontaneous healing of a coronary dissection has also been shown to occur, on both clinical and histological evidence.(13,26) When secondary causes are excluded, (11, 15, 24, 28-36) the etiology of it remains uncertain. Among the risk factors, hypertension has rarely been reported. (11) Pregnancy or oral contraceptives induced arterial wall changes have been well documented (37) hormonal and hemodynamic factors also may contribute to the weakening of the tunica media, thereby correlate well with a higher incidence of spontaneous coronary dissection in the puerperium(8-16,38,39) .A true pattern of cystic medial necrosis has been described in the involved coronary artery as the cause of spontaneous coronary artery dissection. (40)

In many published cases, a fairly diffuse adventitial and periadventitial inflammatory reaction consisting mainly of eosinophils was observed.(4,29,30) Because of the presence of these "periarteritis-like" adventitial change, it was proposed that the dissection is a result of the lytic action of protease released from eosinophils. (30)  The in vivo diagnosis of spontaneous coronary artery dissection by selective angiography depends on the visualization of two lumina separated by a radiolucent intimal flap. (7) This procedure entails the risk of cardiac arrest by injection of the hypertonic medium into the false lumen, thus aggravating the dissection. Some cases may not be recognized if an intimal tear does not occur or if the true lumen is severely narrowed or if the false lumen is occluded by a clot.

The prognosis for these patients is poor. In a survey of 123 cases, Benham and Tillinghast found that 67% of the patients died and 33% survived, treated either surgically or medically. (41) Since sudden death is the most frequent clinical presentation, the clinician is left without the time of any potential therapeutic option in the majority of cases. Medical treatment may play a palliative role. Although the outcome for patients with spontaneous coronary dissection is considered grim, with a high mortality rate, aggressive surgical treatment in a recent series of 10 consecutive patients resulted in 100% survival. (7) Thayer et al (5) recommended coronary artery bypass grafting for all patients with spontaneous dissection, whereas De Maio et al (6) advocated this intervention only in cases of left main disease, three-vessel disease, few or recurrent ischemia. 


Conclusion

SCAD is an infrequent condition that is under-diagnosed among patients presenting with ACS. Risk factors for SCAD are multifold, including young women, fibromuscular dysplasia, systemic inflammation, connective tissue disorders and pregnancy, and often compounded by precipitating stressors. The long-term outcome of patients who survived their initial SCAD presentation is good; however, recurrent events are frequent and these patients should be followed closely by cardiovascular specialists. Treatments typically entail conservative medical management for stable patients with ischemia resolution; however, revascularization with PCI or CABG may be necessary for a small proportion of patients.

Figure 5: Management algorithm including revascularization for acute presentation of SCAD(42).

In conclusion, the possibility of a spontaneous coronary artery dissection should be considered in any young adult woman who presents with an unexpected circulatory collapse, myocardial ischemia, or infarction, without a previous history or risk factors, and not necessarily in the puerperium. Suspicion of the diagnosis may then lead to an emergency investigation of the coronary arteries, and surgical or interventional treatment. However, the high incidence of sudden death without premonitory symptoms casts doubt on the real possibility of prompt diagnosis and life-saving treatment in the majority of cases.


Disclosure
The research team did not receive any grant from any sources & declares no conflicts of interest.


References

1 Saw J. “Spontaneous coronary artery dissection”. Can J Cardiol 2013; 29: 1027-33.

2 Vrints CJ. “Spontaneous coronary artery dissection”. Heart 2010; 96: 801-8.

3 Pretty HC. “Dissecting aneurysm of coronary artery in a woman aged 42: rupture”. BMJ 1931; i:667.

4 Mathieu D, Larde D, Vasile N. “Primary dissecting aneurysm of the coronary arteries: case report and literature review”. Cardiovasc Intervent Radiol 1984;7:71-4.

5 Thayer JO, Healy RW, Maggs PR. “Spontaneous coronary artery dissection”. Ann Thorac Surg 1987;44:97-102.

6 De Maio SJ, Kinsella SH, Silverman ME. “Clinical course and long-term prognosis of spontaneous coronary artery dissection”. Am_J Cardiol 1989;64:471-4.

7 Jorgensen MB, Aharonian V, Mansukhani P, Mahrer P. “Spontaneous coronary dissection: a cluster of cases with this rare finding”. Am HeartJ 1994;127:1382-7.

8 Wells AL. “Dissecting aneurysm of coronary artery in the puerperium”. JfPathol  1960;79:404-5

9 Ascuncion CM, Hyun J. “Dissecting intramural hematoma of the coronary artery in pregnancy and the puerperium”. Obset Gynecol 1972;40:202-10.

10 Heefner WA. “Dissecting hematoma of the coronary artery: a possible complication of oral contraceptive therapy”. JAMA 1973;223:550-1.

11 Schmidt JC. “Dissecting aneurysms of coronary arteries”. Arch Pathol 1975;99:117-21.

12 Shaver PJ, Carrig TF, Baker WP. “Postpartum coronary artery dissection”. Br Heartr 1978;40:83-6.

13 Ciraulo DA, Chesen RB. “Coronary arterial dissection”. Chest 1978;73:677-9.

14 Jewitt JF. “Two dissecting coronary artery aneurysms postpartum”. NEnglJ Med 1979;298:1255-6.

15 Ehya H, Weitzner S. “Postpartum dissecting aneurysm of coronary arteries in a patient with sarcoidosis”. South Med _J 1980;73:87-8.

16 Bonnet J, Aumailley M, Thomas D, Grosgogeat Y, Broustet J, Bricaud H. “Spontaneous coronary artery dissection: case report and evidence for a defect in collagen metabolism”. Eur HeartJ 1986;7:904-9.

17 Lovitt WV, Corzine WJ Jr. “Dissecting intramural hemorrhage of anterior descending branch of left coronary artery”. Arch Pathol 1952;54:458-62.

18 Ashley PF. “Dissecting aneurysm of the coronary artery: case report”. Del Med3' 1965;37:86-8.

19 Brody GL, Burton JF, Zawadski ES, French AJ. “Dissecting aneurysms of the coronary artery”. N Engl _J Med 1965; 273: 1-6.

20 Palomino SJ. “Dissecting intramural hematoma of left coronary artery in the puerperium”. Am J Clin Pathol 1969; 51:119-25.

21 Pisano RV, Taylor MB, Sopher IM. “Dissecting coronary artery aneurysm. A report of two cases”. _J Forensic Sci 1978;1:18-25.

22 Bac DJ, Lotgering FK, Verkaaik APK, Deckers JW. “Spontaneous coronary artery dissection during pregnancy and postpartum”. Eur Heart 7 1995;16:136-8.

23 Cocco P, Thiene G, Corrado D, Lodovichetti G, Pennelli N. “Ematoma (aneurisma) dissecante spontaneo delle coronarie e morte improvvisa”. G Ital Cardiol 1990;20: 795-800.

24 Bulkley BH, Roberts WC. “Dissecting aneurysm (hematoma)limited to coronary artery: a clinico-pathologic study of six patients”. Am_J Med 1973;55:747-56.

25 Mark D, Kong Y, Whalen R. “Variant angina and spontaneous coronary artery dissection”. Am _J Cardiol 1985;56: 485-6.

26 Van Der Bel-Khan J. “Recurrent primary coronary artery dissecting aneurysm (hematoma)”. Am J Clin Pathol 1982; 78:394-8.

27 Palank E, Dawson J, Cowen G, Tysinger J. “Primary dissecting aneurysm of the right coronary artery”. Chest 1977; 72:774-6.

28 McKeon F. “Dissecting aneurysm of the coronary artery in arachnodactyly”. Br Heart3r 1960;22:434-6.

29 Barrett DL. “Isolated dissecting aneurysm of the coronary artery: report of a case apparently due to hypersensitivity angiitis”. Ohio State MedJ7 1969;65:830-2.

30 Robinowitz M, Virmani R, McAllister H. “Spontaneous coronary artery dissection and eosinophilic inflammation: a cause and effect relationship?” Am J Med 1982;72: 923-8.

31 Grady AE, Cowley MJ, Vetrovec GW. “Traumatic dissecting coronary arterial aneurysm with subsequent complete healing”. Am _ Cardiol 1985;55:1424-5.

32 Boland J, Limet R, Trotteur G, Legrand V, Kulbertus H. “Left main coronary dissection after mild chest trauma. Favorable evolution with fibrinolytic and surgical therapies”. Chest 1988;93:213-4.

33 Morise AP, Hardin NJ, Bovill G. “Coronary artery dissection secondary to coronary arteriography: presentation of three cases and review of literature”. Cathet CardiovascDiagn 1981;7:283-96.

34 Murphy DA, Craver JM, King SB. “Distal coronary artery dissection following percutaneous transluminal coronary angioplasty”. Ann Thorac Surg 1984;37:473-8.

35 Roy JJ, Klein HZ. “Dissecting aneurysm of the coronary artery”. JAMA 197 1;218:1047-8.

36 Baker P, Keyhani-Rofagha S, Graham RL, Sharma HM. “Dissecting hematoma (aneurysm) of coronary arteries”. AmJ7Med 1986;80:317-9.

37 Manolo-Estrella P, Barker A. “Histopathologic findings in human aortic media associated with pregnancy: a study of 16 cases”. Arch Pathol Lab Med 1967;83:336-41.

38 Irey NS, Manion WC, Taylor HB. “Vascular lesions in women taking oral contraceptives”. JArch Pathol 1970;89: 1-8.

39 Azam MN, Roberts DH, Logan WFWE. “Spontaneous coronary artery dissection associated with oral contraceptive use”. Intr J Cardiol 1995;48:195-8.

40 Boschetti AE, Levine A. “Cystic medionecrosis with dissecting aneurysm of coronary arteries”. Arch Intern Med 1958; 102:562-70.

41 Benham R, Tillinghast S. “Thrombolytic therapy in spontaneous coronary artery dissection”. Clin Cardiol 1991;14: 611-4.

42 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;7:645-55.


Volume 1 Issue 3 November 2020

©All rights reserved by Dr. Mahmood Hasan Khan.

 

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