Detection of Coronary Artery Anomalies in Tetralogyof Fallot Using a Specific Angiographic Protocol

Dhiraj Gupta, MD, DM, Anita Saxena, MD, DM, Shyam Sunder Kothari, MD, DM,Rajnish Juneja, MD, Mira Rajani, MD, Sanjeev Sharma, MD, and

Panangipalli Venugopal, MS, MCh

Tetralogy of Fallot (TOF) is known to be associatedwith anomalies in the origin and/or course of cor-

onary arteries.1–4 These anomalies can add signifi-cantly to morbidity and mortality during surgicalrepair and need to be clearly delineated before sur-gery.5–7 Not only are earlier angiographic, surgical,and autopsy studies limited by small numbers, butnone have also studied whether these anomalies occurindependent of other pathologic abnormalities seenwith TOF. The ideal modality to detect these anoma-lies is not clear. We conducted this study using aprespecified angiographic protocol to estimate the in-cidence and pattern of coronary anomalies in patientswith TOF and to look for any possible associationswith other abnormalities.

• • •From 1989 to 1998, 1,486 consecutive patients

with TOF who underwent cardiac catheterization andangiography as a part of their presurgical work-up atour institution were included in this study. There were1,109 males (74.6%) and median age was 3.5 years(range 1 month to 51 years). Only those subjects whomet the criteria of Van Praagh et al8 for the diagnosisof TOF were included in the study. Patients withangiographic and/or echocardiographic demonstrationof biventricular conus (implying the diagnosis of dou-ble outlet right ventricle) were excluded as were pa-tients with pulmonary atresia with ventricular septaldefects.

All patients underwent aortic root angiography inthe left anterior oblique projection by power-injectinga large bolus of contrast (1.00 ml/kg) through anangiographic flow catheter (Pigtail, Cordis, Johnson &Johnson Co., Roden, The Netherlands). Care wastaken to avoid positioning the catheter tip in the non-coronary sinus so as to better opacify the coronaryarteries. Cine angiographic recordings were takenwith a 35-mm camera using a shutter speed of 50frames/s. The degree of left anterior oblique tilt wastailored in individual cases from 45° to 60° to clearlyseparate the origin of the left and right coronary ar-teries.9 In patients in whom coronary anatomy couldnot be clearly delineated by the flush aortogram (n5152, 10.2%), selective coronary angiography in mul-tiple views (left anterior oblique, 45° or 30° right

anterior oblique, and lateral) was performed. Sevenadditional patients underwent selective coronary an-giography to rule out concomitant atherosclerotic cor-onary artery disease because they were.40 years old.

For analysis, only developmental coronary anom-alies involving the origin and/or the course of either ofthe coronary arteries were included.9 Presumably, ac-quired coronary anomalies like hypertrophied conalbranch and coronary artery-to-pulmonary artery col-laterals were excluded.4,9

Detailed angiographic study was performed con-currently that included right and left ventricular anddescending aortic angiograms. Angiographic featuresof patients with coronary anomalies were comparedwith the patients without these anomalies. The vari-ables compared included details of pulmonary artery

From the Departments of Cardiology, Cardiac Radiology, and Car-diothoracic Surgery, All India Institute of Medical Sciences, NewDelhi, India. Dr. Saxena’s address is: Department of Cardiology, AllIndia Institute of Medical Sciences, Ansari Nagar, New Delhi-110029, India. E-mail: anitasaxena@hotmail.com. Manuscript re-ceived June 1, 2000; revised manuscript received and accepted July24, 2000.

TABLE 1 Distribution of Different Coronary Anomalies (n 5104)

Type of Anomaly Number

Percent ofCoronaryAnomalies

Percentof TOF

(n 5 1,486)

Anterior descendingartery from rightcoronary artery/sinus

39 37.5 2.6

Accessory anteriordescending arteryfrom right coronaryartery/sinus

27 25.9 1.80

Single coronary arteryfrom left coronarysinus

15 14.4 1.01

Single coronary arteryfrom right coronarysinus

5 4.8 0.35

Both coronary arteriesfrom left coronarysinus

8 7.7 0.54

Both coronary arteriesfrom right coronarysinus

5 4.8 0.35

Others 5 4.8 0.35Left circumflex arteryfrom right coronarysinus

3 2.9 0.21

Right coronaryartery from leftcoronary sinus, leftcoronary arteryfrom right coronarysinus

1 0.9 0.07

Anomalous origin ofthe left coronaryartery from thepulmonary artery

1 0.9 0.07

241©2001 by Excerpta Medica, Inc. All rights reserved. 0002-9149/01/$–see front matterThe American Journal of Cardiology Vol. 87 JANUARY 15, 2001 PII S0002-9149(00)01330-8

anatomy, presence of right-sided aortic arch, presenceand size of aortopulmonary collaterals, presence anddegree of aortic regurgitation, presence of additional

ventricular septal defects, right ventricular function,and tricuspid regurgitation. Chi-square test and Stu-dent’st test were used for comparisons as applicable.

FIGURE 1. A, selective right coronary sinus injection in left ante-rior oblique view showing anterior descending artery (arrow)arising from right coronary artery. B, anomalous anterior de-scending artery (arrow) from right coronary artery in right ante-rior artery view.

FIGURE 2. Flush aortogram in left anterior oblique view showingsingle coronary artery (arrow) arising from the left coronary si-nus.

FIGURE 3. Flush aortogram in left anterior oblique view showingboth coronary arteries originating from the left coronary sinus.

242 THE AMERICAN JOURNAL OF CARDIOLOGYT VOL. 87 JANUARY 15, 2001

Anomalous coronary arteries were seen in a total of104 patients (7.0%) (Table 1). The most commonanomaly noted (in 39 cases, 2.6%) was an aberrantanterior descending artery from the right coronaryartery or from the right coronary sinus (Figure 1). Inthese cases, the left coronary artery did not give originto the anterior descending artery and only continued asthe circumflex artery. In another 27 cases (1.8%), theright coronary artery or the right coronary sinus gaveorigin to an accessory anterior descending artery inaddition to the major anterior descending artery fromthe left coronary artery. The other anomalies are listedin descending order of frequency in Table 1 and areshown in Figures 2 to 6. In addition, 36 patients(2.4%) had coronary artery-to-pulmonary artery col-laterals and 3 patients (0.2%) had coronary artery-to-right ventricular fistulae.

The comparison between patients with coronaryartery anomalies (group A, n5 104) with those with-out (group B, n5 1,382) is shown in Table 2. Nodifferences were observed between the 2 groups inregards to age, sex, and arterial oxygen saturationlevels. There were no differences among the 2 groupsin regards to any of the other angiographic variables aswell (Table 2).

• • •According to the classification by Edwards,10 cor-

onary anomalies described in association with TOFconstitute anomalies of “minor” significance in that

FIGURE 4. Flush aortogram in left anterior oblique view showingboth coronary arteries originating from the right coronary sinus.

FIGURE 5. A, selective right coronary angiogram demonstratingorigin of circumflex artery (arrow) from right coronary artery. B,left anterior descending artery arising normally from the left cor-onary sinus as seen on flush aortogram.

FIGURE 6. Flush aortogram in left anterior oblique view showinglate filling of left coronary artery (arrow) draining into the pul-monary artery.

BRIEF REPORTS 243

they do not cause any symptoms. However, it is wellrecognized now that their significance lies in theirvulnerability at the time of total repair of this condi-tion. Several surgical series have described the devas-tating consequences of inadvertently ligating a previ-ously unsuspected anomalous coronary artery duringsurgery.2,5–7,11,12Thus, it is imperative to delineate thecoronary artery pattern preoperatively; this may be theonly indication of subjecting these patients to preopera-tive angiography in today’s era of echocardiography.

The reported incidence of coronary anomalies inTOF has varied widely depending on the method ofdetection. The low incidence of 2% to 5% reported bysurgical teams2,5,6,11,12can be explained by the factthat many of the anomalies are difficult to detectintraoperatively. Postmortem studies1,3 indicate ahigher incidence of 5% to 9%, which is probably dueto greater possibility of carefully examining the cor-onary arteries. However, there may be a bias towardoverestimation because of the increased surgical mor-tality associated with coronary artery anomalies. Thus,the most accurate method of assessing the true inci-dence is via angiography.3,4,9,13 In this regard, theangiographic study reported here comprises a group ofpatients unselected by autopsy and surgical consider-ations that might be considered to yield a more accu-rate estimate.

The incidence of 7.0% seen in our study agrees withthe other angiographic studies by Fellows et al3 (84patients, incidence 5%) and Dabizzi et al4 (265 patients,incidence 9%). In correlation with these reports, the mostcommon anomaly observed in our study was the originof the dominant and/or sole anterior descending arteryfrom the right coronary artery or sinus. Another commonanomaly seen in our series was the presence of an ac-cessory anterior descending artery from the right coro-nary artery in addition to the usual one arising from theleft coronary artery. Although in this latter setting aninadvertent ligation of this vessel may not have the samedire consequences as when a normally arising anterior

descending artery is absent, it is stillvaluable information for the surgeon,who can modify his approach of ven-triculotomy to avoid this vessel.

The reason for the high associa-tion of coronary artery anomalieswith TOF is unknown. It has beensuggested that anterior position (dex-troposition) of the aorta in tetralogypredisposes to an anomalous originof the anterior descending from theright coronary artery.14 However, ifthis were the only explanation, theoccurrence of the coronary anoma-lies would be expected to predomi-nate in patients with the severestforms of tetralogy because of greateraortic anteroposition or right ventric-ular overriding in these subjects.However, this is not substantiated inthe literature15 nor was this found in

our study (Table 2). Thus, in all probability, coronaryanomalies arise in isolation and are not linked to anyof the other embryo-pathologic features of TOF.

In conclusion, we have shown in this study thatcoronary artery anomalies are commonly associ-ated with TOF. These can be well delineated pre-operatively with flush aortography supplementedwith selective coronary arteriography, if required.

1. Meng CC, Eckner FA, Lev M. Coronary artery distribution in tetralogy ofFallot. Arch Surg1965;90:363–366.2. Kirklin JW, Ellis FH Jr, McGoon DC, Dushane JW, Swan HJC. Surgicaltreatment of tetralogy of Fallot by open intracardiac repair.J Thorac CardiovascSurg1959;37:22–29.3. Fellows KE, Freed MD, Keane JF, Van Praagh R, Bernhard WF, CastanedaAC. Results of routine preoperative coronary angiography in tetralogy of Fallot.Circulation 1975;51:561–566.4. Dabizzi RP, Caprioli G, Aiazzi L, Castelli C, Baldrighi G, Parenzan L,Baldrighi V. Distribution and anomalies of coronary arteries in tetralogy ofFallot. Circulation 1980;61:95–102.5. Berry BE, McGoon DC. Total correction for tetralogy of Fallot with anoma-lous coronary artery.Surgery1973;74:894–898.6. Hurwitz RA, Smith W, King H, Girod DA, Caldwell RL. Tetralogy of Fallotwith abnormal coronary artery: 1967 to 1977.J Thorac Cardiovasc Surg1980;80:129–134.7. McManus BM, Waller BF, Jones M, Epstein SE, Roberts WC. The case forpreoperative coronary angiography in patients with tetralogy of Fallot and othercomplex congenital heart diseases.Am Heart J1982;103:451–456.8. Van Praagh R, Van Praagh S, Nebesar RA, Muster AJ, Sinha SN, Paul MH.Tetralogy of Fallot: underdevelopment of the pulmonary infundibulum and itssequelae.Am J Cardiol1970;26:25–31.9. Shrivastava S, Mohan JC, Mukhopadhayay S, Rajani M, Tondon R. Coronaryartery anomalies in tetralogy of Fallot.Cardiovasc Intervent Radiol1987;10:215–218.10. Edwards JE. Anomalous coronary arteries with special reference to arterio-venous-like communications.Circulation 1958;14:1001–1006.11. Howe A, Rastelli GC, Ritter DG, DuShane JW, McGoon DC. Managementof right ventricular outflow tract in severe tetralogy of Fallot.J Thorac Cardio-vasc Surg1970;60:131–143.12. Senning A. Surgical treatment of right ventricular outflow tract stenosiscombined with ventricular septal defect and right–left shunt (“Fallot’s tetralogy”).Acta Chir Scandinav1959;117:73–78.13. O’Sullivan J, Bain H, Hunter S, Wren C. End-on aortogram: improvedidentification of important coronary artery anomalies in tetrology of Fallot.BrHeart J 1994;71:102–106.14. Longnecker CG, Reemtsma K, Creech O Jr. Anomolous coronary arterydistribution associated with tetralogy of Fallot: hazard in open cardiac repair.J Thorac Cardiovasc Surg1961;42:258–265.15. Dabizzi RP, Teodori G, Barletta GA, Caprioli G, Baldright G, Baldright V.Associated coronary and cardiac anomalies in the tetralogy of Fallot; an angio-graphic study.Eur Heart J1990;11:692–704.

TABLE 2 Comparison of Angiographic Profile of Patients With CoronaryAnomalies (group A, n 5 104) Versus Those Without (group B, n 5 1,382)

VariableGroup A

n (%)Group B

n (%)p

Value

Mean age (yrs) 4.9 6 3.6 5.3 6 5.7 0.57Male/female 79/25 999/353 0.64Hemoglobin (g/dl) 16.2 6 4.4 15.6 6 4.5 0.24Mean arterial oxygen saturation (%) 77.4 6 11.5 78.6 6 12.2 0.16Right-sided aortic arch 12 (11.5) 148 (10.7) 0.92Pulmonary artery abnormalities 0.74

Hypoplastic pulmonary trunk 4 (3.8) 30 (2.2)Pulmonary trunk bifurcation stenosis 1 (1.0) 19 (1.4)Small right and left pulmonary arteries 0 16 (1.1)

Additional ventricular septal defects 3 (2.9) 35 (2.5) 0.93Aortopulmonary collaterals (single/multiple) 30 (28.8) 370 (26.8) 0.88Large aortopulmonary collaterals 23 (22.1) 240 (17.4) 0.82Patent ductus arteriosus 3 (2.9) 46 (3.3) 0.76Aortic regurgitation 0 31 (2.2) 0.22Right ventricular dysfunction 1 (1.0) 9 (0.7) 0.81Tricuspid regurgitation 1 (1.0) 10 (0.7) 0.81

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