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DOI:10.2214/AJR.05.0521
AJR 2006; 186:1192-1193
© American Roentgen Ray Society

An Unusual Coronary Artery Anomaly in Tetralogy of Fallot Shown on MDCT

Gurpreet Singh Gulati, Charanjeet Singh, Shyam Sunder Kothari and Sanjiv Sharma

All India Institute of Medical Sciences New Delhi, India 110029

An 18-year-old woman was admitted with complaints of dyspnea on exertion and cyanosis since birth. Physical examination showed clubbing and an ejection systolic murmur. ECG revealed findings suggestive of tetralogy of Fallot. Angiocardiography showed right ventricular hypertrophy, a subaortic ventricular septal defect, aortic override, and infundibular (right ventricular outflow) obstruction. In addition, angiography of the aortic root and coronary sinus injections in multiple views showed a single coronary artery arising from the left coronary sinus. The right coronary artery was seen to arise from the proximal segment of the left anterior descending artery (Figs. 1A and 1B). However, its further course in relation to the underlying cardiac structures was not clearly defined.


Figure 1
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  		Fig. 1A —18-year-old woman with tetralogy of Fallot. Catheter angiogram shows aortic root injection in left anterior oblique and cranial view. Right coronary sinus is empty (white arrow) and single coronary artery (black arrow) arises from left coronary sinus. Artery then divides into left anterior descending (LAD) and left circumflex (LCx) arteries. Anomalous right coronary artery (asterisks) arises from proximal LAD.

 

Figure 2
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  		Fig. 1B —18-year-old woman with tetralogy of Fallot. Catheter angiogram shows selective left coronary artery injection in right anterior oblique view. Anomalous right coronary artery (asterisks) originates from LAD and is foreshortened in this projection.

 
The patient then underwent coronary angiography on a 16-MDCT scanner (Sensation 16, Siemens) with retrospective ECG gating (heart rate, 72 beats/min). Eighty milliliters of nonionic contrast material was injected IV at 3.5 mL/sec. CT showed that the right coronary sinus was empty. The right coronary artery, after arising from the proximal left anterior descending artery, was coursing anterior to the right ventricular outflow tract to enter the right atrioventricular groove (Figs. 1C and 1D). These findings were confirmed at operation.


Figure 3
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  		Fig. 1C —18-year-old woman with tetralogy of Fallot. MDCT volume-rendering technique (VRT) shows heart viewed from cranial aspect. Single coronary artery initially divides into left circumflex (LCx) and left anterior descending (LAD) arteries. Anomalous right coronary artery (asterisks) originates from LAD and is seen to course toward right side, anterior to right ventricular outflow tract (RVOT). Ao = aorta, PA = pulmonary artery, RV = right ventricle, LV = left ventricle.

 

Figure 4
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  		Fig. 1D —18-year-old woman with tetralogy of Fallot. MDCT VRT as viewed from cranial and right anterior oblique aspect. Relation of anomalous right coronary artery (asterisks) to RVOT is depicted. Artery then enters right atrioventricular groove between right atrium (RA) and RV.

 
Surgical repair for tetralogy of Fallot consists of closure of the ventricular septal defect and repair of the infundibular obstruction. An anomalous coronary artery crossing the right ventricular outflow tract is at risk for injury during transannular infundibular repair. The incidence of a surgically relevant anomalous coronary artery in tetralogy of Fallot is 5-12% [1]. These anomalies occur because of abnormal development of endothelial buds (which give rise to future coronary arteries) from the base of the truncus arteriosus. The most common coronary anomaly in tetralogy of Fallot is a left anterior descending artery arising from the right coronary sinus and crossing the right ventricular outflow tract. Although a right coronary artery arising from the left anterior descending artery has been reported in patients with coronary artery disease, its occurrence in the setting of tetralogy of Fallot has been reported in only two cases [2].

Catheter angiography is usually performed preoperatively for identification of coronary anomalies. However, angiography is limited in that it cannot reliably show the relationship of the aberrant vessel to the underlying cardiac structures. MDCT is rapidly emerging as a credible alternative for the visualization of coronary anomalies [3]. ECG-gated volumetric acquisition of the entire heart in a short breath-hold allows simultaneous depiction of the cardiac and coronary anatomy. This permits generation of 3D and multiplanar reconstructions of the anatomy, allowing recognition of the relation of the anomalous coronary vessel to the cardiac chambers. In our case, MDCT visualization of the anomalous right coronary artery crossing the right ventricular outflow tract allowed safe surgical repair of the cardiac defect.

MDCT visualization of a right coronary artery arising from the left anterior descending artery has been reported in two patients with obstructive coronary artery disease [4]. To our knowledge, this is the first report of CT visualization of this anomaly in tetralogy of Fallot.


References
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 References
 

  1. Li J, Soukias ND, Carvalho JS, Ho SY. Coronary arterial anatomy in tetralogy of Fallot: morphological and surgical correlations. Heart 1998; 80:174 -183[Abstract/Free Full Text]
  2. Tchervenkov CI, Pelletier MP, Shum-Tim D, Beland MJ, Rohlicek C. Primary repair minimizing the use of conduits in neonates and infants with tetralogy or double-outlet right ventricle and anomalous coronary arteries. J Thorac Cardiovasc Surg 2000;119 : 314-323[Abstract/Free Full Text]
  3. Gilkeson RC, Ciancibello L, Zahka K. Multidetector CT evaluation of congenital heart disease in pediatric and adult patients. AJR 2003; 180:973 -980[Free Full Text]
  4. Teragawa H, Okada K, Sueda T. Anomalous origin of the right coronary artery from the left anterior descending coronary artery. Heart 2004; 90:1492[Free Full Text]

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