left atria1 guidewire can prevent the catheter from entering the left atria1 appendage(the most frequent location of thrombus). Second,its flow-directed passagefrom the The efficacy of PTMC is well documented.However, left atrium to left ventricle can minimize manipulation of it has somerisks of complications.2-4Complications fre- the catheter in the left atrium. quently reported include mortality, emergency surgery, From our experience,thrombus in the left atria1 ap cardiac tamponade, systemic thromboembolism, mitral pendageis not an absolute contraindication for PTMC. regurgitation and atria1 septal defect. The complication In selectedpatients with MS who have small and fKed rates of systemic thromboembolism range from 0 to left atria1appendagethrombus, PTMC could be careful4%.2-4Hence, left atria1 thrombus was consideredas an ly performedwith the Inoue balloon catheter with acceptabsolutecontraindication for PTMC. However, all previ- ably low complication risks. ous studiesdependon transthoracic echocardiographyto detect left atria1 thrombus.2-4The diagnostic accuracy 1. Inoue K, ChvakiT, Nakamura T, Kitamura F, Miyamoto N. Clinical applicaof transvenousmitral commissurotomyby a new balloon catheter. J Thorac of transthoracic echocardiography to detect left atria1 tion CardiouascSurg 1984;87:394-402. thrombus is not satisfactory.5T6 Becausetransesophageal 2. Vahanian A, Michel PL, Cormier B, Vitoux B, Michel X, Slama M, Sarano echocardiography improves the diagnostic sensitivity of LE, Trabelsi S, Ismail MB, Acar .I. Resultsof percutaneousmitral commissurotoin 200 patients. Am J Cardiol 1989;63:847-852. left atria1 thrombus, more patients with MS would be my 3. Nobuyoshi M, Hamasaki N, Kimura T, Nosaka H, Yokoi H, Yasumoto H, found to have left atria1 thrombus. The issueof whether Horiuchi H, Nakashima H, Shindo T, Mori T, Miyamoto AT, Inoue K. Indicapatients with small and fmed thrombus confined to the tions,complications,and short-termclinical outcomeof percutaneoustransvenous mitral commissurotomy.Circulation 1989;80:782-792. left atria1appendageshould be deniedthe potential bene- 4. Hung JS, Chern MS, Wu JJ, Fu M, Yeh KH, Wu YC, Chern WJ, Chua S, Lee fits of PTMC is worth examining. Our preliminary expe- CB. Short- and long-term results of catheter balloon percutaneoustransvenous commissurotomy.Am J Cardiol 1991;67:854-862. rience may shed some light on this crucial point. All 6 mitral 5. AschenbergW, Schluter M, Kremer P, Schroder E, Siglow V, Bleifeld W. casesin this serieshad successfulPTMC without clinical- Transesophagealtwc+dimentionalechocardiographyfor the detectionof left atria1 appendagethrombus.J Am Coil Cardiol 1986;7:163-166. ly evident thromboembolic complication. 6. Kronzon I, Tunick PA, GlassmanE, Slater J, Schwinger M, Freedberg RS. PTMC with the Inoue balloon catheter has lower Tramesophageal echocardiographyto detectatria1clots in candidatesfor percutathromboembolic rates (0% to 1.4%)than do other balloon neous transseptal mitral balloon valvuloplasty. .I Am Co// Cardiol 1990;16: catheters (3 to 4%) in the literature.2-4The specialchar- 1320-1322. 7. Hung JS, Lin FC, Chiang CW. Successfulpercutaneoustransvenouscather acter of the Inoue balloon catheter may explain the lower balloon mitral commissurotomyafter warfarin therapy and resolutionof left atria1 rates of thromboembolic complication. First, its coiled thrombus.Am J Cardiol 1989;64:126-128. atria1 level in all 6 cases; however, only 1 had significant oxygen step-up, with Qp/Qs = 1.4 during the cardiac catheterization study.

The Four Subtype! of AnoyalTus Origin of the l+t Main Coronary k&from the Right Aort~c Smus (or from the Right Coronary William C. Roberts, MD, and Jamshid Shirani, MD ost published reports of coronary arterial anomalies concern a single patient or only a small group of M patients, Although much good and useful information, of course,can be derived from the study of a single patient, multiple casesof a major coronary anomaly are required to observethe various subgroupsof a single major anomaly. We have studied at necropsy 17 patients in whom the left main coronary artery (LMCA) arosefrom either the right aortic sinusor the most proximal portion of the right coronary artery.1-5After its origin, the LMCA coursedto the left side of the heart by 1 of 4 routes, and the clinical consequencesof suchcoursesare describedin this report. Pertinent clinical and necropsy findings in the 17 patients are summarized in Table Z, and the 4 subtypes are illustrated in Figure 1. In 2 patients (12%) (cases 1 and 2 [Table Zj), the anomalously arising LMCA coursed anterior (group A) to the right ventricular outflow tract to reach the anterior sulcus (the anteriorportion of the heart immediately anterior to the ventricular septum), where it then divided into the left anterior deFrom the Pathology Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland 20892. Manuscript received February 21, 1992; revised manuscript received March 10.1992, and accepted March 11.

scending and left circumflex coronary arteries.5 In neither patient did the coronary anomaly appear to have caused cardiac dysfunction or myocardial ischemia. In 9patients (53%) (cases 3 to II), the anomalously arising LMCA coursed in between (group B) the ascending aorta and pulmonary trunk before reaching the anterior SU~CUS.~-~ The ostium of the LMCA was slit-like in 8 patients. In 7 of the 9patients death was attributed to the coronary anomaly: sudden outside the hospital in 6, and secondary to severe intractable congestive heart failure (the result of a previous large acute myocardial infarct that had healed) in 1 (case 9). In 2patients (12%) (cases 12 and 13), the anomalous LMCA coursed within the crista supraventricularis muscle (group C) behind the right ventricular outflow cavity before reaching the anterior sulcus, and then dividing into the left anterior descending and left circumflex coronary arteries.l Neitherpatient ever had evidence of myocardial ischemia or cardiac. dysfunction. In 4 patients (23%) (cases 14 to 17), the anomalous LMCA coursed dorsal (group D) to the ascending aorta before reaching the usual area of bifurcation into the left anterior descending and Ieft circumflex coronary arteries.s*6Although 2 of the 4 patients died from cardiovasBRIEF REPORTS

119

TABLE I Clinical and Morphologic Findings in 17 Patients with Anomalous Origin of the Left Main Coronary Artery from the Right Coronary Artery or the Right Aortic Sinus No. of

Case 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17

Age (yr) &Sex 22 44 13 14 14 19 29 39 64 81 50 34 48 32 45 57 69

Origin of Anomaly LMCA Group*

M

Length of LMCA

km)

AP

SD

Death Outside Hospital

0 + 0 0

+ + + +

+ + + +

0

+

+

o+ + 0 0 0 0 0 + 0 0

0 -I+ 0 + + + 0 0

+ 0 + + + + + + + 0

F F M M M

RAS RASt RAS RAS RAS RAS

A A 8 8 El 8

4.5 3.0 1.1 1.2 -o++ 1.1

M

RAS

6

-+++

F F

RAS RAS

8 I3

M

RAS

8

F

RASt RAS RAS RAS

6 C C D

M M M M F M

RAS

D

RAS RCA

D D

2.4 2.0 4.7 4.5 3.3 4.5 3.6 3.0

Length of RCA > LCCA

Slit-Like Ostium

Major CAs > 75% J. in CSA by Plaque

HC Trauma Coronary anomaly Coronary anomaly

+ + + +

0 0 + +

0 0 0 0

Coronary anomaly

-

Cause of Death

Coronary anomaly Coronary anomaly Coronary anomaly Coronary anomaly Trauma Atherosclerotic CAD Trauma Trauma Trauma Atherosclerotic CAD Opiate addictionS Forme fruste Marfan

+ 0 0 + + + + + 0 + + +

LV Scar (1 to3+j

HW (g)

0 0 0 0

870 255 210 370 380 325 350 220 510 420 650 330 550 325 580 300 685

+

0

0

+ + + + 0 0 0 0 0 0 0 0

0 0 0 0 0 3 0 0 0 1 0 0

0 + 0 +++ 0 +++ 0 0 0 +++ 0 0

‘See Figure 1 fordescription. tCommon ostium of both RCA and LMCA. SComplications arising from the addiction. AP = angina pectoris; CA = coronaty artery; CAD = coronary artery disease; CSA = cross-sectional area; HC = hypertrophic cardiomyopathy: HW = heart weight: LCCA = left circumflex coronary artery: LMCA = left main coronary artery; LV = left ventricular; RAS = right aortic sinus; RCA = right coronary artery; SD = sudden death; + = present or positive; 0 = absent or negative; - = no information available.

FlGUREl.Giagrm~the4~lypesofalomdom~oftheMmaln coronrv~(MCA)fr#nhrigM awlkskus.A=antdqL=~; UDCA=leRmteriordeumhgwronmy~LCCA=leRclramGexcoro~A~4P=-R=&M corowy~RVGT= fight-outlbwtmt.

C

120

THE AMERICAN JOUFWL OF CARDIOLOGY VOLUME 70

JULY 1, 1992

cular disease (atherosclerosis in 1, and Marfan-type aortic disease in the other), in none could the cardiac problems be attributed to the coronary anomaly. This brief report indicates that if an anomalouslyarising LMCA coursesanterior (group A) to the right ventricular outflow tract, behind the right ventricular outflow tract (infract-&al) (group C) or dorsal (group D) to the ascendingaorta, symptomsof cardiac dysfunction or myocardial &hernia do not result. In contrast, if the anomalously arising LMCA coursesbetween (group B) the pulmonary trunk and ascendingaorta, symptomsof myocardial ischemia usually occur, and death is a frequent consequence.

1. Roberts WC, Dicicco BS, Wailer BF, Kishel JC, McManus BM, Dawson SL, HunsakerJC III, Luke JL. Origin of the left main from the right coronary or from the right aortic sinuswith intramywardial tunnelingto the left sideof the heart via the ventricular septum:the caseagainstclinical significanceof myocardial bridge or coronary tunnel. Am Heart J 1982;104:306-308. 2. Barth WC III, Roberts WC. Left main coronary artery originating from the right sinusof Valsalva andcoursingbetweenthe aorta and pulmonarytrunk. JAm Cdl Cardiol 1986;1:366-313. 3. Roberts WC. Major anomaliesof coronary arterial origin seenin adulthood. Am Heart J 1986;111:941-963. 4. Kragel AH, Roberts WC. Anomalous origin of either the right or left main coronary artery from the aorta with subsequentcoursing between aorta and pulmonary trunk: analysisof 32 necropy cases.Am J Cardiol1988;62:771-777. 5. Roberts WC, Kragel AH. Anomalous origin of either the right or left main coronary artery from the aorta without coursing of the anomalistically arising artery betweenaorta and pulmonary trunk. Am J Cardiol 1988;62:1263-1267. 6. Wailer BF, Reis RL, McIntosh CL, Epstein SE, Roberts WC. The Marfan cardiovascular diseasewithout the Marfan syndrome. Cheer 1980;77:533-540.

Congenital Hypoplasia of Both Right and Left Circumflex Coronary Arteries William C. Roberts, MD, and Brian N. Glick, MD* hen the right coronary artery is dominant, i.e., it coursesto the crux of the heart, the left circumflex W coronary artery is usually quite small and therefore may From the Pathology Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda,Maryland. Manuscript acceptedMarch 1,1992. *Cardiology Fellow, Georgetown University Medical Center, Washington,D.C.

be consideredhypoplastic (Figure 1). Conversely,when the left circumflex is the dominant coronary artery, i.e., it coursesto the crux of the heart, the right coronary artery is usually small and therefore may be consideredhypoplastic (Figure 1). Hypoplasia of both right and left circumflex coronary arteries in the sameheart, however,is a rare occurrence(Figure 1). Examination of 3,400 hearts during the last 8 years disclosedat least 8 to have hypo-

Dominant

BRIEF REPORTS 121

The four subtypes of anomalous origin of the left main coronary artery from the right aortic sinus (or from the right coronary artery).

left atria1 guidewire can prevent the catheter from entering the left atria1 appendage(the most frequent location of thrombus). Second,its flow-direct...
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