Asian Cardiovasc Thorac Ann 2007;15:418-421
© 2007 Asia Publishing EXchange Ltd
Repair of Anomalous Origin of Right Coronary Artery from the Pulmonary Artery
Hong-Wei Guo, MD,
Jian-Ping Xu, MD,
Yun-Hu Song, MD,
Han-Song Sun, MD,
Xiang-Dong Shen, MD,
Sheng-Shou Hu, MD
Department of Cardiovascular Surgery, Fu Wai Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China
For reprint information contact: Hong-Wei Guo, MD, Tel: 86 10 8839 8063, Fax: 86 10 6831 3012, Email: ghwdr{at}hotmail.com, Department of Cardiovascular Surgery, Fu Wai Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, 167 Beilishi Road, Beijing 100037, China.
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ABSTRACT
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Anomalous origin of the right coronary artery from the pulmonary artery is a rare cardiac malformation. Between July 2002 and July 2005, we operated on 4 patients with this defect. There were 2 males and 2 females, aged from 18 months to 42 years. Three patients underwent direct re-implantation of the right coronary artery into the aorta, and one had an intrapulmonary tunnel repair (intrapulmonary artery baffle with an autologous pericardial patch for tunneling to the anomalous right coronary ostium). Cardiopulmonary bypass was used in 2 patients, and an off-pump technique in the other 2. One patient had an atrial septal defect that was closed with an Amplatzer septal occluder through the right atrium under transesophageal echocardiography, without cardiopulmonary bypass. All patients survived and recovered uneventfully. Follow-up ranged from 3 to 39 months (mean, 17 months). All patients were doing well and free from symptoms, with normal exercise tolerance. Surgical correction of anomalous right coronary artery shows good early and midterm results.
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INTRODUCTION
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Anomalous origin of the right coronary artery (RCA) from the pulmonary artery (PA) is a rare congenital cardiac malformation. Most patients remain asymptomatic. However, there are cases of sudden cardiac death described in the literature, indicating a potentially malign course of the disease. To establish a double-ostium coronary system, correction of the aberrant vessel is recommended. We reviewed our experience of treating this disease surgically.
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PATIENTS AND METHODS
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Between July 2002 and July 2005, 4 patients were diagnosed with anomalous RCA from the PA prior to operation, and underwent a successful surgical repair at Fu Wai Hospital. There were 2 males and 2 females, aged from 18 months to 42 years. The clinical details of these 4 patients are given in Table 1
. Two patients were in New York Heart Association functional class I, one was in class II, and the other was in class III. Two patients (nos. 2 and 4) were asymptomatic but murmurs from associated anomalies led to echocardiographic studies. Electrocardiograms showed sinus rhythm in 3 patients and right axis deviation in one. Chest radiographs revealed cardiothoracic ratios ranging from 0.47 to 0.59 (mean, 0.53). Two-dimensional echocardiography showed the RCA originating from the posterior wall of the main PA in one patient; a coronary artery fistula extending from the left anterior descending coronary artery to the PA in one, and from the left anterior descending coronary artery to the right ventricle in another; and the other patient had a suspected anomalous left circumflex artery. Doppler examination revealed anomalous diastolic flow directed into the main PA in 3 patients. Coronary angiograms confirmed the diagnosis of anomalous origin of the RCA from the PA in all 4 patients, demonstrating collateral circulation between the RCA and left coronary artery and a coronary steal phenomenon into the PA (Figure 1). Three patients had associated cardiac anomalies: one had a patent ductus arteriosus, and 2 had an atrial septal defect (ASD). Patient no. 1 with an ASD had undergone transcatheter closure with an Amplatzer septal occluder (Starway Medical Supplies Ltd., Beijing, China) one year before the operation.
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Figure 1. Selective coronary angiography showing the right coronary artery originating from the pulmonary artery. Ao = aorta, ARCAPA = anomalous right coronary artery originating from the pulmonary artery, LCA = left coronary artery, PA = pulmonary artery.
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The operation was performed through a median sternotomy under general anesthesia with bicaval cannulation and hypothermic cardiopulmonary bypass in 2 patients; an off-pump technique was used in the other 2. Three patients underwent direct aortic re-implantation, and one had intrapulmonary tunnel repair. Myocardial protection was achieved with antegrade cardioplegia through the aortic root after cross clamping the aorta. Due to an abundance of intercoronary collaterals, delivery of cardioplegic solution could be simplified by placing gentle digital pressure over the RCA as the cardioplegic solution was infused into the ascending aorta. Before the RCA was excised, the right atrium was explored and a well-developed coronary sinus was seen. Venting was achieved through the main PA. For direct re-implantation, the main PA was divided, the anomalous origin of the RCA was excised with a button of the PA, and subsequently mobilized. The right coronary button was anastomosed to the aorta. The defect in the PA sinus was closed with an autologous pericardial patch. For intrapulmonary tunnel repair, an aortopulmonary window was created with a 4-mm puncher in the left wall of the aorta and in the right wall of the PA. An autologous pericardial patch was used to construct an intrapulmonary tunnel between the aortopulmonary window and the anomalous origin of the RCA. The anterior wall of the PA was widened with an autologous patch. Associated procedures included closure of the PDA in one patient, and ASD closure with an Amplatzer septal occluder (Starway Medical Supplies Ltd., Beijing, China) through the right atrium under transesophageal echocardiography, without cardiopulmonary bypass, in another.
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RESULTS
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There were no deaths or postoperative complications. All patients recovered uneventfully. The mean postoperative hospital stay was 7.3 ± 0.5 days. Follow-up in all 4 patients ranged from 3 to 39 months (mean, 17 months). The exercise tolerance of all patients improved to normal, and all were in New York Heart Association functional class I and free from symptoms.
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DISCUSSION
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Anomalous origin of the RCA from the PA clearly contrasts with Bland-White-Garland syndrome (anomalous origin of the left coronary artery from the PA) in which patients often show mitral insufficiency, signs of anterolateral myocardial ischemia, and heart failure early in childhood. When symptomatic, the clinical presentation of patients with anomalous origin of the RCA is mixed, including dyspnea, fatigue, congestive heart failure, angina, myocardial infarction, and even cardiac arrest. Two of our 4 patients had symptoms, and the other two had a murmur from the associated anomalies of ASD or PDA, which led to echocardiography. Both female patients were early middle-aged adults and symptomatic, but no such sex and age divide has been described in the literature.
The development of a large network of intercoronary collateral vessels between the left coronary artery and the RCA meets the metabolic needs of the myocardium fed by the anomalous artery, and the direction of flow within the RCA depends on the pulmonary vascular resistance and the degree of development of collateral circulation. Only one patient in our series was diagnosed by two-dimensional echocardiography preoperatively; however, 3 showed anomalous diastolic flow directed into the main PA on Doppler examination. Thus anomalous origin of a coronary artery from the PA should be suspected when anomalous diastolic flow directed into the main PA is found on Doppler examination. Coronary angiography is the gold standard for diagnosis of anomalous origin of the RCA from the PA. The existence of associated defects helps in making an early diagnosis, thus permitting surgical correction in a more timely fashion, as in 2 of our patients.1,2
In general, a corrective operation is recommended for this cardiac malformation, even in asymptomatic patients, for two reasons.3–5 First, with re-implantation of the aberrant vessel into the aorta, a double-ostium coronary system is re-established, with a potentially lower risk of sudden death. Second, the operation provides relief from coronary (pulmonary) steal, which may be responsible for myocardial ischemia. We chose direct ostial transfer for patients with anomalous origin of the RCA from the anterior wall of the PA, and intrapulmonary tunnel repair for those with anomalous origin of the RCA from the posterior wall of the PA. An off-pump technique is only suitable for patients with the RCA originating from the anterior wall of the PA. The right atrium was explored in patients who had cardiopulmonary bypass, but not in off-pump patients. Our series of 4 patients showed good early results after surgical intervention, but more cases and longer follow-up are required to confirm the benefits of this operation.
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REFERENCES
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- Maluf MA, Smith M, Abellan DM, Troster EJ, Takaoka F, Rati M, et al. Anomalous origin of the right coronary artery from the pulmonary artery in association with a ventricular septal defect. Tex Heart Inst J 1997;24:226–9.[Medline]
- Casillas JA, de Leon JP, Villagra F, Checa SL, Sanchez PA, Gomez R, et al. Aortopulmonary window with anomalous origin of the right coronary artery from the pulmonary trunk. Tex Heart Inst J 1986;13:325–31.[Medline]
- Bossert T, Walther T, Doll N, Gummert JF, Kostelka M, Mohr FW. Anomalous origin of the right coronary artery from the pulmonary artery combined with aortic valve stenosis. Ann Thorac Surg 2005;79:347–8.[Abstract/Free Full Text]
- Marik D, Gately HL, Strauss R, Starr A. Anomalous origin of right coronary artery from pulmonary artery. J Card Surg 1995;10:55–8.[Medline]
- Radke PW, Messmer BJ, Haager PK, Klues HG. Anomalous origin of the right coronary artery: preoperative and postoperative hemodynamics. Ann Thorac Surg 1998;66:1444–9.[Abstract/Free Full Text]
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ABSTRACT
INTRODUCTION
