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CASE REPORT

CLINICAL CASE

Spontaneous Coronary ArteryDissection in Levo-Transpositionof the Great Arteries

Avinash V. Sharma, MD, MPH,a,* Raja Zaghol, MD,a,* Ana Barac, MD, PHD,b Itsik Ben-Dor, MD,b

Anitha John, MD, PHDc

ABSTRACT

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Spontaneous coronary artery dissection (SCAD) is an uncommon form of acute coronary syndrome (ACS). With the

increasing recognition of this condition presenting as ACS in females, clinicians should always remain vigilant for it.

This case describes a patient with congenital heart disease presenting with SCAD. (Level of Difficulty: Advanced.)

(J Am Coll Cardiol Case Rep 2019;1:146–50) © 2019 The Authors. Published by Elsevier on behalf of the

American College of Cardiology Foundation. This is an open access article under the CC BY-NC-ND license

(http://creativecommons.org/licenses/by-nc-nd/4.0/).

CASE PRESENTATION

A 38-year-old gravida 6 para 5015 Hispanic femalepresented 10 days postpartum with chest pain andshortness of breath for 2 days. On arrival, cardiactroponin was elevated to 4.6 ng/ml, and electrocar-diography (ECG) showed T-wave inversions in lateralleads (Figure 1).

EARNING OBJECTIVES

Understanding coronary anatomy and elec-trocardiographic changes in levo-transposition of the great arteries is criticalto evaluating patients with SCAD.SCAD continues to be a more recognizableform of acute coronary syndrome in theyounger female population.Conservative management is preferred inpatients with SCAD without hemodynamiccompromise.

N 2666-0849

m the aDepartment of Internal Medicine, MedStar Washington Hospital C

titute, Medstar Washington Hospital Center, Washington, DC; and the

stem, George Washington University School of Medicine, Washington, DC

rk. The authors have reported that they have no relationships relevant to

nuscript received May 28, 2019; accepted June 5, 2019.

MEDICAL HISTORY

Her history was significant for levo-transposition ofthe great arteries (L-TGA) and subpulmonary andpulmonary valve stenosis with a secundum atrialseptal defect status post-closure device implanted 12years previously. Her most recent echocardiogramduring pregnancy demonstrated mild right ventricu-lar dysfunction with moderate to severe multilevelpulmonary stenosis.

DIFFERENTIAL DIAGNOSIS

Findings included pulmonary embolism, acute coro-nary syndrome (ACS) including an acute myocardialinfarction, coronary vasospasm, and aorticdissection.

INVESTIGATIONS

Coronary angiography revealed spontaneous coro-nary artery dissection (SCAD) involving the left maincoronary artery, extending into the left circumflex

https://doi.org/10.1016/j.jaccas.2019.06.016

enter, Washington, DC; bMedStar Heart and VascularcDivision of Cardiology, Children’s National Health

. *Drs. Shah and Zaghol contributed equally to this

the contents of this paper to disclose.

AB BR E V I A T I O N S

AND ACRONYM S

ACS = acute coronary

syndrome

AMI = acute myocardial

infarction

Ao = aorta

ASD = atrial septal defect

CC-TGA = congenitally

corrected transposition of the

great arteries

CMR = cardiac magnetic

resonance imaging

ECG = electrocardiogram

L-TGA = levo-transposition of

the great arteries

MRI = magnetic resonance

imaging

PV = pulmonic ventricle

RV = right ventricle

SCAD = spontaneous coronary

artery dissection

SV = systemic ventricle

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and the left anterior descending coronary arterieswithout significant obstruction (Figure 2). Cardiacmagnetic resonance imaging (Figures 3 and 4) showedno significant findings in the left (pulmonary)ventricle supplied by the dissected arteries but ratherrevealed an area of transmural enhancement on theanterolateral segment of the mid to distal portion ofthe right (systemic) ventricle (Figure 4), thought to berelated to chronic right (systemic) ventricular failurefrom her underlying L-TGA.

MANAGEMENT

The patient was admitted to the cardiovascularintensive care unit and started on intravenous hepa-rin drip, aspirin, and clopidogrel therapy. Her symp-toms improved, and she was discharged with dual-antiplatelet therapy, beta-blocker (metoprolol), andstrict precautions to avoid heavy lifting, straining,and so forth.

FOLLOW-UP

She continues to follow-up with no progression ofdissection. She underwent a tubal ligation to avoidfurther pregnancies.

DISCUSSION

SCAD is a rare cause of ACS, described by non-traumatic separation of the coronary arterial wall.More than 80% to 90% of SCAD cases occur in fe-males, with a peak incidence in the fourth and fifthdecade. SCAD is estimated to account for 0.7% to4.0% of cases of ACS (1,2) and up to one-quarter ofcases in women younger than 50 years of age (3).SCAD is typically divided into 3 subtypes based onangiographic findings (4). Type 1 SCAD showscontrast dye staining of the arterial wall with multipleradiolucent lumens (Figures 2A and 2B). Type 2 showsdiffuse stenosis of varying severity and length (usu-ally >20 mm), usually with a subtle, abrupt changefrom normal arterial diameter to the stenotic lesion(Figure 2B), and type 2A variant SCAD borders normalsegments adjacent to the dissection (Figures 2A and2B). Type 3 mimics atherosclerosis with focal ortubular stenosis (<20 mm). Risk factors includerecent physical or emotional stressors, pregnancy anda postpartum period, fibromuscular dysplasia, connec-tive tissue diseases, and inflammatory conditions (5,6).

As in the present patient, chest pain is the mostprevalent symptom and is seen in almost 96% of cases(7). ECG changes are also similar to those of an acute

myocardial infarction with variability be-tween ST-segment elevations, ST-segmentdepressions, T-wave inversions, or nonspe-cific ST-segment changes (6). This patient’sECG findings included lateral T-wave in-versions more pronounced than on priorECGs (Figure 1). Importantly, most patientswith ECGs in L-TGA have Q waves in rightprecordial leads (II and III) with no Q waves inV5/V6 due to septal activation, which occursfrom right to left due to inversion of the rightand left bundles (8).

A conservative medical managementstrategy using antiplatelet agents, beta-blockers, and pain control is the currentpreferred approach (9). Revascularization isoften technically challenging with potentialcomplications (10) and is usually reserved forcases with symptomatic obstruction or he-modynamic instability (9).

Transposition of the great arteries {S, L, L}or congenitally corrected transposition of thegreat arteries is a rare congenital anomaly

comprising <1% of all congenital heart diseases,resulting in both atrioventricular and ven-triculoarterial discordance (8). In this configuration,the left atrium communicates with the morphologicright ventricle, which serves as the systemicventricle, pumping oxygenated blood through theaorta (Figure 3). Coronary artery anatomy is alsovariable, but most commonly, the coronaries arisefrom the posterior-facing sinuses of an anteriorlylocated aorta. The vessel arising from the right-sidedsinus is morphologically the left coronary artery,giving rise to anterior descending and circumflexbranches supplying the pulmonic ventricle (Figure 2Aand 2B), while the vessel arising from the left-sidedsinus is morphologically the right coronary arterysupplying the systemic ventricle (Figure 2D) (8).

Most L-TGA patients have other concomitantcongenital heart defects; tricuspid valve anomalies,septal defects, and pulmonary stenosis are the mostcommon (11). In addition, there is a high risk of heartblock in this patient population due to the anteriorpositioning of the conduction system. For patientswho have no concomitant defects, presentation isusually delayed and often related to systemic rightventricular failure (11). Despite having some systemicventricular dysfunction, this patient had remainedasymptomatic as she had been essentially “auto-banded” with pulmonary stenosis, resulting in vol-ume unloading of her systemic ventricle (Figure 4).

FIGURE 1 Investigations: Electrocardiogram

12-lead ECGs of this patient 2 years prior to admission (top) and on admission (bottom). T-wave inversions in the lateral leads (I/aVL/V5/V6)

are now more prominent. ECG ¼ electrocardiogram.

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FIGURE 2 Angiography Reveals Type 1 SCAD of the Left Main Coronary Artery Extending into the Left Circumflex

(A, B) Angiography reveals type 1 SCAD of the left main coronary artery extending into the left circumflex (top arrow). Type 2 SCAD of the

mid-left anterior descending coronary artery with stenosis (B, bottom arrow) and type 2A dissection of the diagonal branch (A, bottom

arrow; B, middle arrow). (C) The aortogram shows take-off of right and left systems using an atrial septal closure device (Amplatzer, Abbott

Laboratories, Rockville, Maryland). (D) The right coronary artery supplies the systemic ventricle (anatomic right ventricle) without any

detected lesions. SCAD ¼ spontaneous coronary artery dissection.

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CONCLUSIONS

To the best of the present authors’ knowledge, this isthe first case of SCAD occurring in a patient with L-TGA. The authors are not aware of a mechanistic linkbetween these 2 rare conditions. One importantcaveat is the need to identify variant coronary anat-omy in L-TGA in addition to understanding thebaseline ECG changes that can be found typically inpatients with L-TGA. This patient did not experience

significant coronary obstruction, hemodynamicinstability, or progression of dissected lesions. Sheunderwent medical management alone and has pro-gressed well.

ADDRESS FOR CORRESPONDENCE: Dr. Anitha S.John, Division of Pediatric Cardiology, Children’sNational Health System, 111 Michigan Avenue, NW,Washington, DC 20010. E-mail: anjohn@cnmc.org.

FIGURE 3 CMR in a Vertical Long-Axis View

CMR in a vertical long-axis view highlights the systemic

ventricle (SV) and Aorta (Ao). The SV is the anatomic right

ventricle (RV) with corresponding features such as the crista

supraventricularis (arrow). CMR ¼ cardiac magnetic

resonance.

FIGURE 4 CMR in Cine

CMR in cine (A) shows systemic/anatomic right ventricle (SV)

with mitral valve in view (arrow) and pulmonic ventricle (PV).

(B) Short-axis view with late gadolinium enhancement was

significant for an area of transmural enhancement (arrow) in

SV. CMR ¼ cardiac magnetic resonance.

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2. Nishiguchi T, Tanaka A, Ozaki Y, et al. Preva-lence of spontaneous coronary artery dissection inpatients with acute coronary syndrome. Eur HeartJ Acute Cardiovasc Care 2016;5:263–70.

3. Saw J, Aymong E, Mancini GB, Sedlak T,Starovoytov A, Ricci D. Nonatherosclerotic coro-nary artery disease in young women. Can J Cardiol2014;30:814–9.

4. Saw J. Coronary angiogram classification ofspontaneous coronary artery dissection. CatheterCardiovasc Interv 2014;84:1115–22.

5. Tweet MS, Hayes SN, Pitta SR, et al. Clinicalfeatures, management, and prognosis of sponta-neous coronary artery dissection. Circulation 2012;126:579–88.

6. Saw J, Humphries K, Aymong E, Sedlak T,Prakash R, Starovoytov A, Mancini GBJ. Sponta-neous coronary artery dissection: clinical out-comes and risk of recurrence. J Am Coll Cardiol2017;70:1148–58.

7. Luong C, Starovoytov A, Heydari M, Sedlak T,Aymong E, Saw J. Clinical presentation of patientswith spontaneous coronary artery dissection.Catheter Cardiovasc Interv 2017;89:1149–54.

8. Warnes CA. Transposition of the great arteries.Circulation 2006;114:2699–709.

9. Hayes SN, Kim ESH, Saw J, et al. Spontaneouscoronary artery dissection: current state of thescience. Circulation 2018;137:e523–57.

10. Tweet MS, Eleid MF, Best PJ, et al. Sponta-neous coronary artery dissection: revascularizationversus conservative therapy. Circ Cardiovasc Interv2014;7:777–86.

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KEY WORDS coronary angiography,spontaneous coronary artery dissection,transposition of the great arteries