Survival with acute primary coronary artery dissection: A case report and review of the literature

The Journal

of Emergency

Medicine, Vol12, No 2, pp 193-198, 1994 Copyright 0 1994 Hevia science Ltd Printed in the USA. All rig&r rwcrved cv364679/w $6.00 + .oo

07364679(93)mo114

Selected Tbpks Cardiology

Commentary

SURVIVAL WITH ACUTE PRHUARY CORONARY ARTERY DISSECTION: A CASE REPORT AND REVIEW OF THE LITERATURE Lou Anne Plumhoff Wellford,

MD,

and Theodore

Martin Kelly, MD

Joint Miliiary Medical Centers, Emergency Medicine Residency, San Antonio, Texas Lou Anne Wellford, MD, Department of Emergency Medidne, Brooke Army Medical Center, Fort Sam Houston,

Reprint Address:

TX 78234

of cardiac diseaseor anginal symptoms who presentedwith an acutenon-Q-waveMI (NQWMI), and who was ultimately found to have a primary coronary artery dissection.

0 Abstract-Primary coronary artcry clissrdon is a rare etidooy of acute myoeamUal infarction (AIM) that often has devwhhg conseqm. We present the case of a 46-year-old male swvlvor of primary coronary artery di!+ section who was diagnosed angiogr8phkaUy. He s&feral a non-Q-wave Ml bat had no -wtwbk 0 cllmge64huhghblcome.Areviewoftklitemtlueof primary coronary artery dimecti011,particularly those diagnosed 8ntamortem, is provided.

0 Keywordx - -on;

CASE REPORT

A 46-year-oldCaucasianmale presentedto the emergencydepartment(ED) with the complaint of chest pain. The patient had just completed his vigorous daily training exerciseof a 4-mile run, sit-ups, and push-ups,and had proceededto isometrichandexerciseswhenhenotedthe onsetof mild, dull, retrosternal chestpressure.He had a total of six episodesof the pain, eachlasting approximately2 minutes, with waxing and waning over a 2-hour period, and there wereseveralperiodsof completepain resolution.He had been pain free for the past hour. The patient deniedshortnessof breath,nausea,diaphoresis,palpitations, or radiation of the pain. Risk factors for ischemic heart diseasewere absent. He had smokedtwo packs of cigarettesa day for 6 years,but hadquit 25yearsago. His last cholesterol levelwas 200mg/dL, and he had no history of diabetesor hypertension,and a family history unremarkablefor the presenceof cardiovasculardisease. He had no prior history of cardiac diseaseand no

coronaryartery;primary; spoa-

taneons INTRODUCTION

Coronary artery dissectionhas beenreportedin the literatureasa very rareentity that presentsassudden deathor asmassivemyocardialinfarction (MI) leading to death;usually this diagnosishasbeenmadeat autopsy.We fmd only 40 casesof primary or spontaneouscoronaryartery dissection(SCAD) in the English literature that were diagnosedantemortemby angiography. We present a case of a previously healthy 46-year-oldmale without any prior history The views expressed in this article are those of the authors and do not reflect the official policy or position of the Department of the Army, Department of Defense, or the United States Government.

RECXIVBD: 10 Jamary 1992; FINAL ACCEPTED: 20 August 1993

SUBMISSION

RECEIVED:

193

9 August 1993;

Lou Anne P. Wellford and Theodore M. Kelly

194

history of cocaineor other substanceuse. He was taking no medicationsand had no allergies. On physicalexamination, vital signswere within normal limits (BP 15002 torr, P 49 beats/min, R 18 breathsimin, 36.4OC[T 97.6OF]).The patient was comfortableat the time of examination,without respiratory distressor diaphoresis.He had normal carotid pulseswithout bruits and had no evidenceof jugular venous distention. Peripheral pulses were symmetric and strong. On cardiac examination, he had a regularrhythm with a normal Sl and S2 and the absenceof third and fourth heartsounds.Lungs wereclearto auscultation,and the abdomenwasbenign. Therewas no cyanosis,clubbing, or edemaof the extremities. Initial evaluationincluded an electrocardiogram (ECG) showinga sinusbradycardiaat 47 bpm without ischemic changes(Figure 1). Portable chest roentgenogramshowedmild cardiomegalywithout acutepulmonary disease.Room air pulse oximetry revealedan oxygensaturationof 99%. Initial complete blood counts, electrolytes, and coagulation studieswerenormal.

Figure 1. Electrocardiogram

at presentation

revealing

While in the ED, the patientremainedstablewithout any further episodesof chestpain. It wasdecided amongthe emergencyphysiciansto dischargethe patient with closecardiologyfollow-up arranged.However,in consultationwith the cardiologist,it wasdecided to admit the patient given his youth, active military career,and the needto assessworldwidedeployability. He was admitted to the coronary care unit wherehe continuedto be pain free with stable vital signs.Initial serumcardiacenzymeanalysisrevealeda creatinephosphokinase(CPK) value of 656 U/L with CK-MB of 66 U/L (MB% = 10); initial lactate dehydrogenase(LDH) was 202 U/L and reached a peak of 258 U/L. Peak CPK values reached714U/L with CK-MB = 77 U/L (MB% = 11).The patienthad no ECG abnormalitiesthroughout his stay. On the secondhospitalday, the patientunderwent cardiac catheterization.He was found to havenormal left main, left anterior descending(LAD), and right coronary arteries.There was a 2-cm area of intimal dissection of an obtuse marginal branch (OMB) of the circumflex artery (Figure2). The area

sinus bmdycardla

wlthout evidence

of Ischemia or infarction.

Coronary Artery Dissection

Figure 2. Cineanglogmm

demonstrating

rpiml dissection

of dissectionwas well beyond the cathetertip and was not thought to be secondaryto the catheterization. Therewas minimal luminal irregularity, consistent with atheroma proximal to the dissection,but the circumflex artery was otherwisefree of atheroscleroticdisease.There wasno evidenceof aneurysm formation, but there was focal inferior akinesisof cardiacwall motion. The patient did well throughout the remainderof his hospital stay without further chestpain. He had no complicatingcongestiveheartfailure or dysrhythmias and completed cardiac rehabilitation without difficulty. The patient was dischargedhomein good condition. DISCUSSION

Approximately 1.5 million peoplesuffer acutemyocardial infarction (AMI) yearly, and it is estimated that one-fourth of all deathsannually in the United Statesaredueto AM1 (1). Myocardial infarction has beenattributed to numerouscauses,but by far the most commonof theseis atheroscleroticluminal narrowing, often with superimposedthrombosis. The developmentof thrombus adjacentto or on an atheroscleroticplaquemay lead to critical narrowing or total occlusionof a vessel,therebyleadingto infarction of the myocardiumsuppliedby that vessel.Identification and treatmentof suchpatientscanoften be

of an obtuse marginal

branch of th# ahwmf&x

ertwy.

aidedby knowledgeof the presenceof atherosclerotic diseasein that patient or of risk factors for ischemic cardiacdisease,suchas hypertension,diabetesmellitus, hypercholesterolemia,tobacco use, and family history of atheroscleroticdisease.Lessthan 6% of all AM1 occur in patientswithout theserisk factors and with normal coronary vessels(1). In thesepatients, etiologiesof AM1 includevasospasm,emboli, arteritis, trauma, congenitalanomalies,and, rarely, coronaryarterydissections. Coronary arterial dissectionsare most commonly due to extensionfrom a proximal aortic dissection (2,3) but havealso beendescribedsecondaryto cardiac catheterization(3,4), percutaneoustransluminal coronary angioplasty(3), cardiac surgery(coronary artery bypassgrafting, valve replacement[3,5,6]), and cardiopulmonaryresuscitation(7). Primary coronary artery dissectionsare much lesscommon than secondarydissections,andtheir etiologyremainsunclear.Associationof primary coronaryartery dissections with Marfan’s syndrome(3,8), trauma (9,10), and the puerperium(2,3,6,11-21)have beennoted. DeMaio and colleagues(22) identified three groups associatedwith primary coronaryartery dissections: thosewith atheroscleroticdisease,thoseperi- or postpartum, andthosepresumedto beidiopathic. In general, thesedissectionspredominatein young females (female:male 3 : 1) and, as in our case,thereis usually minimalor no atheroscleroticdiseasepresentby cardiaccatheterizationor autopsy.

Lou Anne P. Wellford and Theodore M. Kelly

196

mortem diagnosis(Table 1) (2,4,6,11,16,18,20-39). Forty of thesepatientswere diagnosedangiographitally; the remainingpatientwas diagnosedat pathological examination of the excisedheart posttransplant (11). In patients surviving coronary artery dissection,AM1 is by far the most common presentation. Thirty-four (83%) of these 41 patients presentedwith AM1 as the initial diagnosisleading to their catheterization;five other patients underwent

Myocardial infarction secondaryto primary coronary arterydissectionis exceedinglyrare.While coronary artery dissectionmay presentasAM1 or assudden death, suddendeathaccountsfor the majority of presentations,thus leading to diagnosisusually at autopsy.Of 112casesof coronaryartery dissection identified by National Library of Medicine MEDLINE CD-ROM search of English language journals (2-6, 11, 15-50),thereare 41 casesof anteTable 1. Antemortem

Diagnoses

Author/Date Forker et al, 1973 Westbrook, 1975 Razavi, 1975 Palanck et al, 1977 Ciraulo and Chesne, 1978 Shaver et al, 1978 Molloy and Ablett, 1980 Mathieu et al, 1984 lskandrian et al, 1985

of Coronary Artery Dlsaection Age/Sex

Mark et al, 1985 DeJunco et al, 1985 Ramamurti et al, 1985 Bonnet et al, 1988

58lM 28/F 31/F 31/M 55/M 28/F 42/F 33/F 43/M 59/M 80/M 50/F 52/M 42lF 32/F

Vicari et al, 1988 Thayer et al, 1987

33/F 39/F

Nishikawa et al, 1988

28/M 54/M 48/F 28/F 45/M 58/M 40/F 58lM 45lM 58lM 37/M 18/M 50/M 48/F 49/F 31/F 39/F 58/F 41 IF 41/F 48/F 33/F

Vacek et al, 1987 Shioi et al, 1989 Gonzalez et al, 1989 Forker et al, 1973 DeMaio et al, 1989

Keon et al, 1989 Front et al, 1990 Curie1 et al, 1990 Himbert et al, 1991 Yeoh et al, 1991 Benham et al, 1991

41/F ZOIM

42lF

Vessel RCA LAD LAD,LM,Cx RCA RCA LAD&x LM,LAD,Cx LM RCA RCA RCA PDA RCA Dx(LAD) cx LAD LAD LM RCA RCA RCA LAD LAD LAD RCA RCA Lit LAD,Dx LAD RCA LAD RCA&ID kz LAD LAD,Dx LM,LAD RCA LM,lAD LM,Cx K

AMI Antlnf InfLat AntLat Inf Inf AntLat AntSeptal AntSeptal Old inf AntLat InfLat Ant AntLat AntSep + Lat Inf Inf Inf AntSep Old ant Inf Antlnf + + + + + + + + + + + Ant Inf AntSep AntLat Ant AntLat

ECG changes

+ + + + + + +’ + -2 -2 -2 +’ + -3 + 7 + +

+ + +6 + + +’

LAD = leftanteriordescending; LM = left main: RCA = right coronary artery; Dx = diagonal; Cx = circumflex; PDA = posterior descending: Ant = anterior: Inf = inferior; Sep = septal; Lat = lateral ‘Antemortem diagnosis but did not survive dissection. ‘Presented with anaina, not AMI. 3Non-Q-waveMI. ‘Received thrombolytics-streptokinase in Ramamurti’s patient, t-PA in Benham’s patient. ‘Diagnosed at pathological exam of excised heart at transplant, not angiographically.

197

Coronary Artery Dissection

angiographyfor anginal symptoms(29,30,31).Two of the patientsdiagnosedangiographicallyhad ventricular dysrhythmias,in the face of AMI; one was ultimately pronounced dead after prolonged attempts at resuscitation(27), and the other patient wassuccessfullycardiovertedelectrically(38). Upon further review of these34 patientspresenting with AM1 secondaryto SCAD, only 19casesreport electrocardiographicfindings. Seventeenof these19patientshad classicECG findings consistent with AMI; Vicar-i’spatient had a NQWMI but had deepsymmetricT waveinversions(21),andthe other patient had an intraventricular conduction delay prior to the developmentof ventricular fibrillation (27). Thus, 89% of those patients presentingwith AM1 had ECG changesconsistentwith myocardial ischemiaor infarction, and theremainingpatienthad a significant nonperfusingventricular dysrhythmia requiring treatment. Of those caseswith reported ECG findings, there were no patients with normal electrocardiograms. In this era of thrombolytic therapy for AMI, it may becomemore important to discernthe underlying etiologyof the infarction prior to the administration of thrombolytics. To date, there are two cases in the English literature of thrombolytic administration for AM1 secondaryto coronary artery dissection, both of whom had favorableoutcomes(32,38). There is the potential, however, that thrombolysis may extenda self-limited dissection,therebyextending the areaof myocardialnecrosis.On the contrary, thrombolysis may prove to be an acceptabletreatment for AM1 secondaryto SCAD. Until this issue is clarified, it may be helpful to recognizethosepatients more likely to haveundergonecoronaryartery dissection(young, postpartum, no risk factors for ASHD), and to considerearly angiographyin these patients,if available, prior to the administration of t hrombolytics. Finally, this caseonceagainillustratesthe possible role of exertionor trauma in the developmentof coronary artery dissections.Uehlinger(9) previouslyreported two casesof SCAD following severeexertion in soldiers,while Nalbandianand Chason’s(10) patient hadthe onsetof symptomsafter shovelingsnow. Other authors have pointed out the increasedincidencein women after labor. Our patient had completeda routineexerciseregimenandcommencedisometric exerciseswhenhis symptomsbegan.

Unfortunately, the fact remainsthat SCAD is rare and exceedinglydifficult, if not impossible,to diagnoseclinically. The possibility of a SCAD may be raisedin the particular high-risk groups mentioned above (i.e., young, postpartum,no risk factors for ASHD), but the definitive diagnosisremainsan angiographicone. The sensitivityof availabletests(history, physicalexamination, chestx-ray study, and ECG) for the detectionof this entity, with the exceptionof angiography, are so low as to prohibit the diagnosis.Thus, the potential existsfor not recognizingthosepatients presentingwith AMI, especiallyNQWMI, secondary to SCAD and for dischargingthese.patientsto home. This may account for a portion of the 4vo to 8% (51,52) of patients with AM1 who are discharged from EDs throughout this country, and may representa sourceof irreduciblerisk in the battle to minimize thepercentageof AMIs dischargedhome.There may be a fixed percentageof AM1 patientswho will be impossibleto diagnosein the ED becauseof very atypical presentationsor exceedinglyrare etiologies for AMI. Thus, it is impossibleto reducethe percentageof “missedAMIs” to nil, despiteour bestefforts. SUMMARY We presenta caseof a 46-year-oldmale who suffered a NQWMI secondaryto a coronaryarterydissection. Our caseis uniquein that this is the first caseof AM1 secondaryto SCAD reportedin the Englishliterature in which therewereno ECG changesconsistentwith infarction or ischemia, and in which the diagnosis of AM1 was basedsolely on enzymechangesand catheterizationfindings. In addition, ours is the only survivor with involvement of the circumflex artery solely, and only the third reported case overall in the English literature of isolated circumflex artery dissection.This report highlights the increasingrecognition of SCAD antemortem and the potential needfor recognitionof thosepatientswith AMI secondaryto SCAD prior to routine interventionssuch asthrombolysis. authorsthank Doctors Glenn Mitchell and A. Landon Wellford for their advice and expertise in the manuscript preparation.

Acknowledgments-The

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