Asian Cardiovasc Thorac Ann 2005;13:307-310
© 2005 Asia Publishing EXchange Ltd
Associated Coronary Anomalies in 135 Iranian Patients with Tetralogy of Fallot
Manouchehr Hekmat, MD,
Sima Rafieyian, MD,
Mahnoush Foroughi, MD,
Mohammad M Majidi Tehrani, MD,
Mahmoud Beheshti Monfared, MD,
Seyed A Hassantash, MD
Cardiac Surgery Ward, Modarres Hospital, Beheshti University of Medical Sciences, Tehran, Iran
For reprint information contact: Manouchehr Hekmat, MD Tel: 98 21 206 2903 Fax: 98 21 206 2903 Email: rezaei_nima{at}hbi.ir, Modarres Hospital, Beheshti University of Medical Sciences, Saadat Abad, Tehran 19987, Iran.
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ABSTRACT
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Coronary artery anomalies are common among patients with tetralogy of Fallot. One hundred and thirty-five patients (80 males and 55 females) with tetralogy of Fallot who underwent repair between 1995 and 2002 were studied to determine the incidence of coronary anomalies in Iranian patients. Eight (5.9%) patients (4 males and 4 females) had a surgically relevant coronary artery anomaly: single coronary ostium in 5, origin of the left anterior descending artery from the right coronary artery in 2, and origin of the right coronary artery from the left coronary artery in 1. The surgical technique in 3 of these patients was repair of the ventricular septal defect with a transverse incision on the right ventricle, without damage to the coronary arteries. In another patient, an allograft aortic valve cylinder was inserted. In the other 4 patients with a single coronary ostium, placement of a limited transannular patch was adequate. Consideration of these anomalies during primary repair could decrease the risk of operation in such patients. However, it seems that the presence of anomalous coronary arteries does not affect incremental risk after surgical repair.
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INTRODUCTION
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Congenital coronary anomalies are a recognized cause of cardiac morbidity and mortality due to myocardial ischemia, especially in adolescents and young adults.1 The prevalence of congenital coronary anomalies in the general population has been estimated at approximately 0.85%, based on angiographic data from adults referred with chest pain.2–4 Anomalous coronary artery is more common among patients with other forms of congenital heart disease, especially those with tetralogy of Fallot (ToF), complete transposition of the great arteries, congenitally corrected transposition, or bicuspid aortic valve.5 This is the first report of the incidence of congenital coronary abnormalities in patients with ToF in Iran.
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PATIENTS AND METHODS
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This was a retrospective study over a 7-year period between 1995 and 2002. It was designed to ascertain the incidence of congenital coronary artery anomalies in patients with ToF at Modarres Hospital, one of the cardiac surgery referral centers in Tehran. One hundred and thirty-five patients with ToF underwent primary repair. There were 80 males and 55 females, ranging in age from 9 months to 40 years, with a mean age of 8.3 ± 7.2 years, and a median weight of 9.9 kg (range, 5.2–45 kg). Cardiac catheterization was carried out in all patients before repair of ToF.
Data analyses were performed using SPSS (SPSS, Inc., Chicago, IL, USA) statistical software package (version 10.0).
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RESULTS
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Associated congenital cardiac anomalies were observed in 47 of the 135 patients with ToF (34.8%). Right-sided aortic arch (12 patients) was the most common, followed by atrial septal defect (10), patent ductus arteriosus (9), and left superior vena cava (7). Eight patients (4 males and 4 females) had a surgically relevant coronary artery anomaly (5.9%). The anomalies in 6 of these patients had been diagnosed preoperatively by cardiac catheterization, whereas the anomalies in the other 2 patients were detected at operation. The mean age of these 8 patients at repair was 7.8 ± 3.3 years (range, 4–13 years). The mean right ventricle-pulmonary artery gradient was 19 mm Hg (95% confidence interval, 14.5–24 mm Hg) in ToF patients with coronary anomalies, which was much higher than the mean of 15 mm Hg (95% confidence interval, 12.5–17.5 mm Hg) in patients with normal coronary arteries. There was no early postoperative death. Actuarial freedom from re-operation at 60 months was 96.5% (95% confidence interval, 79.8–99.5%), which was similar to that in patients without coronary artery anomalies. Associated congenital cardiac anomalies were seen in 2 of the 8 ToF patients with a coronary artery anomaly: aneurysm of the sinus of Valsalva; and left superior vena cava (Table 1
).
Congenital coronary anomalies included single coronary ostium in 5 patients, left anterior descending coronary artery (LAD) from the right coronary artery (RCA) in 2, and RCA from the left coronary artery (LCA) in 1. The LAD originated from the RCA and involved the right ventricular outflow tract (RVOT) in one patient (no. 3). A transverse incision was performed on the right ventricle, inferior to the LAD pathway. It was possible to resect the moderator band without damaging the LAD. Subsequently, repair of the ventricular septal defect (VSD) with a Gore-Tex patch and pulmonary valvotomy were carried out through a vertical pulmonary arteriotomy. The ventriculotomy was closed with a pericardial patch. In a 9-year-old girl with a single ostium, both coronaries originated from the anterior sinus of the aorta. The left main artery was in front of the pulmonary artery. In a 7-year-old girl, the RCA originated from the left side and covered the infundibulum. Repair of the VSD with a transverse incision on the right ventricle was performed in both of them, without damage to the coronary arteries. In a 5-year-old girl, the LAD originated from the RCA and went to the left side obliquely. The right ventricle was opened at a sinus portion. The membranous VSD was repaired, and an allograft aortic valve cylinder was inserted orthotopically because of the proximity of the coronary branch to the pulmonary arterial annulus and inability to adequately relieve the RVOT obstruction. At the same time, end-to-end anastomosis was performed between the distal end of the conduit and the divided pulmonary trunk. The remaining 4 patients had single origin of the coronaries but no RVOT involvement. Consequently, successful repair was achieved with placement of a limited transannular patch.
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DISCUSSION
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Anomalous coronaries typically originate on the contralateral side to their destination, and cross to the other side of the aorta.6 By far the most common congenital coronary anomaly is anomalous origin of the left circumflex artery from the right sinus of Valsalva or RCA.2–3,6–7 In ToF, a conotruncal defect gives rise to: VSD; aortic valve override; and narrowing of the RVOT, resulting in secondary right ventricular hypertrophy.8–9 Coronary artery anomalies are encountered in 5% to 14% of patients with ToF.5,10–15 Awareness of these anomalies is necessary to minimize complications and allow planning of the surgical approach. The coronary anomalies of greatest concern are: origin of the LAD from the RCA and certain variations of single coronary artery branching.14 In this study, anomalous coronary arteries were: single coronary ostium (50%), LAD from RCA (37.5%), and RCA from LCA (12.5%). Brizard and colleagues11 reported LAD from RCA or single RCA (61.1%), RCA from LCA or LAD (22.2%), and large RCA conal branch (16.6%). In other studies, the most common variants have been LAD arising from the RCA or separately from the right sinus of Valsalva, RCA from LCA or LAD, a large conal branch from the RCA, and single coronary artery.10
The ability to repair ToF early in life, regardless of coronary artery anatomy, is the reason why academic centers no longer perform routine angiography prior to surgery.11,16 In spite of the fact that cardiac catheterization was performed in all of our patients before repair of ToF, the anomalies in 2 patients were not recognized preoperatively. Cardiac catheterization is indicated before repair of ToF with previous palliative surgery and when the presence of aortopulmonary collateral arteries and pulmonary artery branching abnormalities are suspected.9 Although coronary anomalies may be recognized only at the time of ventriculotomy, inadvertent transsection of important arterial branches may cause postoperative morbidity and mortality.14 For several decades, the only means of diagnosing coronary artery anomalies was invasive angiography, but more recently, echocardiography with Doppler color flow has been sufficient to detail the distribution of the proximal coronary circulation in most cases.8,13–14
Abnormal coronary arteries have been found in some studies to be a risk factor for surgically related mortality.17 Consideration of these anomalies during primary repair could decrease the risk of operation in patients with ToF.18 The surgically important anomalies have been described previously.19 Many of these variants result in a major coronary artery coursing anterior to the pulmonary outflow tract, which complicates the ultimate ToF repair. Previously, the presence of a prominent artery crossing the infundibulum often led to initial palliation (using a Blalock-Taussig or central shunt) rather than repair. When an anomalous coronary artery is recognized, several surgical approaches are possible: a transverse or tailored incision paralleling the anomalous vessels; an incision caudal to the abnormal artery, with an additional pulmonary artery incision above it; a valved conduit graft from the right ventricle to the pulmonary artery, bypassing the anomalous coronary and underlying infundibular stenosis; and an incision tunneled underneath the aberrant artery.14 Repair of ToF has an excellent long-term prognosis and a 25-year survival rate of more than 94%.20
It was concluded from this study that the incidence of coronary artery anomalies in ToF patients in Iran is similar to that in other populations. Considering these anomalies during primary repair could decrease the risk of operation in such patients. Primary repair of ToF with anomalous coronary arteries can be achieved with excellent results.16 Surgically important coronary anomalies can be dealt with through the transatrial-transpulmonary approach, a transverse incision on the right ventricle, or the use of a composite valved conduit, in most cases, without major alterations in technique. Outcomes are similar to those in other patients with ToF, and it seems that the presence of anomalous coronary arteries does not affect incremental risk after this surgical strategy.
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ACKNOWLEDGMENTS
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We gratefully acknowledge the efforts of Dr. Mohammad-Kazem Arabnia, Dr. Ali-Mohammad Hallajzadeh, Dr. Mohammad Rezvan Nobahar, Dr. Hamid-Reza Taghipour, Dr. Naser Kachoueian, and Dr. Nima Rezaei, who assisted us at every step of this project, for their roles in collecting the data and writing this article.
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ABSTRACT
INTRODUCTION
