Ann Thorac Surg 2005;79:347-348
© 2005 The Society of Thoracic Surgeons
Case report
Anomalous Origin of the Right Coronary Artery From the Pulmonary Artery Combined With Aortic Valve Stenosis
Torsten Bossert, MDa,*,
Thomas Walther, MD, PhDa,
Nicolas Doll, MDa,
Jan F. Gummert, MD, PhDa,
Martin Kostelka, MD, PhDa,
Friedrich W. Mohr, MD, PhDa
a Department of Cardiac Surgery, University of Leipzig Heart Center, Leipzig, Germany
Accepted for publication August 19, 2003.
* Address reprint requests to Dr Bossert, Department of Cardiac Surgery, University of Leipzig Heart Center, Strümpellstr 39, D-04289 Leipzig, Germany
tbossert11{at}aol.com
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Abstract
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We report the case of a 41-year-old patient presenting with anomalous of origin of the right coronary artery from the pulmonary artery (ARCAPA) and severe aortic valve stenosis. Surgical correction was performed by partial (T-shaped) sternotomy. After replacing the bicuspid aortic valve, the right coronary artery ostium was directly reimplanted into the aortic root. The main pulmonary artery was reconstructed using an autologous pericardial patch. Postoperative recovery was uneventful. Four cases involving an association of the bicuspid aortic valve and ARCAPA have been reported in the literature. This potential relationship should be considered whenever diagnosing a bicuspid aortic valve.
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Introduction
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Patients with anomalous origin of the right coronary artery (ARCAPA) or the left coronary artery from the pulmonary artery (ALCAPA) incidentally present with a wide spectrum of clinical symptoms, such as sudden cardiac death, heart failure, angina, or myocardial infarction [1–3].
A 41-year-old man was referred to our hospital for aortic valve replacement. Physical examination revealed a systolic murmur. Transthoracic echocardiography revealed severe calcified bicuspid aortic valve stenosis, left ventricular hypertrophy, and an enlarged left ventricle chamber. Left ventricular function was normal (ejection fraction, 0.70), but right ventricular function was slightly reduced. Cardiac catheterization confirmed the diagnosis of severe calcified aortic stenosis with an orifice area of 0.5 cm2 and a peak systolic gradient of 71 mm Hg. Pulmonary artery pressures were within the reference range. Selective coronary arteriography revealed ARCAPA and a dilated vasculature of the right coronary artery (Fig 1).
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Fig 1. Coronary arteriogram showing the anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) and the left coronary artery (LCA). The aortic valve is calcified.
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Surgical correction was performed by an upper partial (inverted T-shaped) sternotomy. Direct cannulation using a wire-reinforced aortic cannula (Edwards Fem-Flex; Irvine, CA) and an oval-shaped, wire-reinforced, two-stage venous cannula (Medtronic Oval VC2; Grand Rapids, MI) for the right atrium was performed. The venous cannula was tunneled through a subxiphoid incision to minimize materials in the operative field.
The operation was performed under moderate hypothermia (32°C) and cold antegrade crystalloid cardioplegic arrest (Bretschneider HTK solution, 30 mL/kg). Venting was achieved by the main pulmonary artery. The ARCAPA, which had a diameter of 2.5 mm at its origin, was identified, dissected, and excised with a sufficiently sized cuff, fully mobilized, and translocated to the ascending aorta. After transverse aortotomy the stenosed bicuspid aortic valve was replaced with a 27-mm ATS Medical Open Pivot mechanical heart valve prosthesis (ATS Medical, Minneapolis, MN). Reimplantation of the ARCAPA was performed directly without tension using a continuous suture (Prolene 5–0). The main pulmonary artery was reconstructed using an autologous pericardial patch.
Aortic cross-clamp duration was 75 minutes. Deairing was achieved through the aortotomy and then continuously through a needle vent in the aortic root. Weaning from extracorporal circulation was unproblematic. Transesophageal echocardiography proved good right ventricular and aortic valve function. The sternum was closed using 6 wires. The patient's postoperative course was uneventful, and he has been asymptomatic with good echocardiographic right and left ventricular function at 18 months follow-up.
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Comment
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This is a rare report of a patient presenting with aortic valve stenosis where additional ARCAPA was incidentally diagnosed at preoperative checkup. However, three similar patients with bicuspid aortic valve stenosis and ARCAPA have been previously described [4–6]. Furthermore a rare coronary anomaly, origin of the circumflex and left coronary artery from the proximal right coronary artery or anomalous left coronary artery arising from the right sinus of Valsalva, has been described in association with bicuspid aortic valves. Patients with anomalies of the coronaries may have an associated cardiac diagnosis. Azakie and colleagues [7] recently described 67 patients studied over a 48-year period with an anomalous origin of coronaries from the pulmonary artery and associated cardiac or vessel anomalies.
Anomalous origin of the right coronary artery can lead to substantial cardiac-related problems such as ischemia, infarction, or heart failure. Thus surgical treatment is usually recommended. Standard therapy is to establish a double-ostium coronary system by translocation of the ARCAPA [8]. Direct reimplantation, as feasible and performed in our patient, is always advantageous because it can avoid any potential for thrombus formation.
In summary, we conclude that in patients undergoing replacement of a bicuspid aortic valve, the preoperative diagnosis of ARCAPA or other coronary anomalies is important, because this condition is surgically correctable and the reimplantation technique is intriguing for adult cardiovascular surgeons. Anomalous origin of coronary arteries should always be considered when diagnosing bicuspid aortic valve disease.
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References
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- Bortolotti U, Casarotto D, Betti D, De Mozzi PL, Stritoni P, Cevese PG. Anomalous origin of the right coronary artery from the main pulmonary artery. Eur J Cardiol. 1978;7:451–455[Medline]
- Bregman D, Brennan FJ, Singer A, et al. Anomalous origin of the right coronary artery from the pulmonary artery. J Thorac Cardiovasc Surg. 1976;72:626–630[Abstract]
- Marik D, Gately HL, Strauss R, Starr A. Anomalous origin of right coronary artery from pulmonary artery. J Card Surg. 1995;10:55–58[Medline]
- Schang SJ, Pepine CJ, Bemiller CR. Anomalous coronary artery origin and bicuspid aortic valve. Vasc Surg. 1975;9:67–72[Medline]
- Aoyagi S, Suzuki S, Kosuga K, Ohishi K. Anomalous origin of the right coronary artery associated with congenital bicuspid aortic valve. Kurume Med J. 1991;38:199–202[Medline]
- Azakie A, Russell JL, McCrindle BW, et al. Anatomic repair of anomalous left coronary artery from the pulmonary artery by aortic reimplantation: early survival, patterns of ventricular recovery and late outcome. Ann Thorac Surg. 2003;75:1535–1541[Abstract/Free Full Text]
- Radke PW, Messmer BJ, Haager PK, Klues HG. Anomalous origin of the right coronary artery: preoperative and postoperative hemodynamics. Ann Thorac Surg. 1998;66:1444–1449[Abstract/Free Full Text]
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Asian Cardiovasc Thorac Ann,
October 1, 2007;
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