World Journal for Pediatric and Congenital Heart Surgery http://pch.sagepub.com/
Anomalous Origin of Circumflex Coronary Artery From Right Pulmonary Artery Associated With Coarctation of the Aorta: A Case Report of Surgical Treatment Roman Sekelyk, Yaroslav Mykychak, Oleh Fedevych and Illya Yemets World Journal for Pediatric and Congenital Heart Surgery 2014 5: 97 DOI: 10.1177/2150135113505294 The online version of this article can be found at: http://pch.sagepub.com/content/5/1/97
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On behalf of:
World Society for Pediatric and Congential Heart Surgery
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Case Report
Anomalous Origin of Circumflex Coronary Artery From Right Pulmonary Artery Associated With Coarctation of the Aorta: A Case Report of Surgical Treatment
World Journal for Pediatric and Congenital Heart Surgery 2014, Vol 5(1) 97-99 ª The Author(s) 2014 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/2150135113505294 pch.sagepub.com
Roman Sekelyk, MD1, Yaroslav Mykychak, MD1, Oleh Fedevych, MD1, and Illya Yemets, MD, PhD1
Abstract Origin of the circumflex coronary artery (Cx) from the pulmonary arteries is an extremely rare anomaly. We describe a two-month-old female patient with anomalous origin of the Cx from the right pulmonary artery associated with coarctation of the aorta. Reimplantation of the anomalous Cx to the aorta and coarctation repair were performed. There were no postoperative complications, and the patient was discharged in satisfactory condition. Keywords coronary artery anomaly, coronary artery imaging, coarctation, pulmonary arteries Submitted May 26, 2013; Accepted August 06, 2013.
Introduction Anomalous origin of the circumflex coronary artery (Cx) from the pulmonary artery (ACxPA) is an extremely rare coronary anomaly.1-3 The clinical course of ACxPA may not always be favorable, and patients need surgical treatment in early infancy.1 The anomaly is often associated with another congenital cardiac malformations.1 The literature search revealed that only a few cases of ACxPA associated with aortic coarctation were reported up to date.3-5
Patients and Methods A two-month-old female patient (weight, 3.6 kg) with coarctation of aorta was admitted to the Ukrainian Children’s Cardiac Center, Kyiv, Ukraine. Diagnosis of aortic coarctation was confirmed by echocardiography. Left ventricle ejection fraction (LVEF) was 58% and peak systolic coarctation gradient was 92 mm Hg, also a coronary anomaly was suspected. The patient was referred to catheterization laboratory and underwent balloon dilatation of coarctation of aorta. The procedure was successful, but the control angiography immediately after balloon dilation revealed anomalous origin of the Cx (diameter, 1 mm) from the posterior aspect of the right pulmonary artery 5 mm distal to pulmonary artery bifurcation (Figure 1A). Cardiac magnetic resonance imaging demonstrated moderate subendocardial hypoperfusion of ventricular septum and papillary muscles of the left ventricle. No large subendocardial regions of delayed hyperenhancement
were detected. Considering the signs of hypoperfusion and potentially poor prognosis for this type of coronary anomaly and remaining tubular hypoplasia in B segment of aortic arch, a surgical repair was performed. The infant underwent an operation on the ninth day after admission. Operation was performed through a median sternotomy using hypothermic (28 C) cardiopulmonary bypass and histidine-tryptophan-ketoglutarate (Custodiol HTK; Ko¨hler Chemie GmbH, Germany) cardioplegic solution. Cardioplegic solution was introduced antegradely into the aortic root and retrogradely into the coronary sinus. The Cx originating from the posterior surface of right pulmonary artery was visualized (Figure 2). Button-shaped excision of the Cx ostium was performed. After obtaining enough mobilization, Cx ostium was reimplanted to noncoronary sinus of aorta. Reconstruction of the right pulmonary artery was performed with glutaraldehydetreated pericardial patch. Concomitant aortic arch reconstruction with extended anastomosis was performed.
1
Cardiac Surgery Department, Ukrainian Children’s Cardiac Center, Kyiv, Ukraine Corresponding Author: Yaroslav Mykychak, Cardiac Surgery Department, Ukrainian Children’s Cardiac Center, Chornovola 28/1, Kyiv, 01135 Ukraine. Email: [email protected]
Downloaded from pch.sagepub.com at Scientific library of Moscow State University on January 10, 2014
98
World Journal for Pediatric and Congenital Heart Surgery 5(1)
Figure 1. A, Preoperative angiography revealed CoAo and normal R and LAD coronary arteries with anomalous origin of the Cx from the posterior aspect of R pulmonary artery and retrograde flow from the circumflex artery to the pulmonary artery A. B, Postoperative angiography demonstrated the Cx origin from ascending aorta without aortic arch obstruction. CoAo indicates coarctation of aorta; Cx, circumflex coronary artery; LAD, left anterior descending; R, right.
Comment
Figure 2. Operative view of anomalous origin of the Cx from the posterior aspect of RPA. Ao indicates aorta, RVOT, right ventricular outflow tract; RPA, right pulmonary artery.
Results There were no complications in early postoperative period, and patient was discharged from the hospital 18 days after admission and 9 days postoperatively. Five months after the operation, echocardiography showed good left ventricular contractility with an LVEF of 65% and a peak systolic gradient at aortic anastomosis of 15 mm Hg. Control angiography demonstrated the Cx origin from the ascending aorta with neither obstruction nor kinking (Figure 1B).
Anomalous origin of the Cx from the right pulmonary artery associated with coarctation of the aorta is a very rare pathology.1-3 The symptoms of the ACxPA may present in different periods of life or patients may remain asymptomatic with anomaly being discovered accidentally during diagnostic procedures. There are no standard indications or operative techniques for such patients. Bolognesi et al described exceptional case of ischemic heart disease due to the origin of the Cx from the pulmonary artery in a 50-year-old woman.5 She had undergone surgery for aortic coarctation at 16 years of age. Patient was referred to the hospital with chest pain and dyspnea after light exercise. Selective coronary angiography revealed the coronary anomaly. Honey et al described a 13-year-old patient with coarctation of aorta.4 A cardiac catheterization study showed ACxPA. Aortic coarctation repair was performed previously. There was no clinical or electrocardiographic evidence of impaired myocardial perfusion, but it was thought rational to correct the anomaly by closing the orifice of Cx with direct suture one year later. Chopra et al reported a 15-year-oldfemale patient who underwent surgical correction of an aortopulmonary window at 13 months.2 Fourteen years later, she presented with dyspnea on exertion associated with angina. A coronary artery bypass grafting of the anomalous Cx and ligation of its anomalous origin in the pulmonary artery were performed. Sarioglu et al described a ten-year-old female patient who underwent surgical repair of the aortic coarctation at four years of age.3 Six years later, she presented with chest pain during exercise. Cardiac catheterization demonstrated retrograde filling of the Cx from the left anterior descending and right coronary artery, with drainage into the right pulmonary artery. Alexi-Meskishvili et al reported two infants with ACxPA who had to be operated at ages 40 days and 30 days because of severe myocardial dysfunction.1 The aortic reimplantation of the anomalous Cx was performed in the first patient, and simple ligation via left thoracotomy was chosen in the second case.
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Sekelyk et al
99
Although our patient had no signs of severe ischemia, we believe that because of mostly unfavorable clinical course of ACxPA, an elective surgical treatment should be considered on establishing a diagnosis. Declaration of Conflicting Interests The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding The author(s) received no financial support for the research, authorship, and/or publication of this article.
References 1. Alexi-Meskishvili V, Dahnert I, Hetzer R, Lange PE, Karl TR. Origin of the circumflex coronary artery from the pulmonary artery in infants. Ann Thorac Surg. 1998;66(4): 1406-1491.
2. Chopra PS, Reed WH, Wilson AD, Rao PS. Delayed presentation of anomalous circumflex coronary artery arising from pulmonary artery following repair of aortopulmonary window in infancy. Chest. 1994;106(6): 1920-19222. 3. Sarioglu T, Kinoglu B, Saltik L, Eroglu A. Anomalous origin of circumflex coronary artery from the right pulmonary artery associated with subaortic stenosis and coarctation of the aorta. Eur J Cardiothorac Surg. 1997;12(4): 663-665. 4. Honey M, Lincoln JCR, Osborne MP, de Bono DP. Coarctation of aorta with right aortic arch report of surgical correction in 2 cases: one with associated anomalous origin of left circumflex coronary artery from the right pulmonary artery. Br Heart J. 1975;37(9): 937-945. 5. Bolognesi R, Alfieri O, Tsialtas D, Manca C. Surgical treatment of the left circumflex coronary artery from the pulmonary artery in an adult patient. Ann Thorac Surg. 2003;75(5): 1642-1643.
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Anomalous Origin of Circumflex Coronary Artery From Right Pulmonary Artery Associated With Coarctation of the Aorta: A Case Report of Surgical Treatment Roman Sekelyk, Yaroslav Mykychak, Oleh Fedevych and Illya Yemets World Journal for Pediatric and Congenital Heart Surgery 2014 5: 97 DOI: 10.1177/2150135113505294 The online version of this article can be found at: http://pch.sagepub.com/content/5/1/97
Published by: http://www.sagepublications.com
On behalf of:
World Society for Pediatric and Congential Heart Surgery
Additional services and information for World Journal for Pediatric and Congenital Heart Surgery can be found at: Email Alerts: http://pch.sagepub.com/cgi/alerts Subscriptions: http://pch.sagepub.com/subscriptions Reprints: http://www.sagepub.com/journalsReprints.nav Permissions: http://www.sagepub.com/journalsPermissions.nav
>> Version of Record - Jan 8, 2014 What is This?
Downloaded from pch.sagepub.com at Scientific library of Moscow State University on January 10, 2014
Case Report
Anomalous Origin of Circumflex Coronary Artery From Right Pulmonary Artery Associated With Coarctation of the Aorta: A Case Report of Surgical Treatment
World Journal for Pediatric and Congenital Heart Surgery 2014, Vol 5(1) 97-99 ª The Author(s) 2014 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/2150135113505294 pch.sagepub.com
Roman Sekelyk, MD1, Yaroslav Mykychak, MD1, Oleh Fedevych, MD1, and Illya Yemets, MD, PhD1
Abstract Origin of the circumflex coronary artery (Cx) from the pulmonary arteries is an extremely rare anomaly. We describe a two-month-old female patient with anomalous origin of the Cx from the right pulmonary artery associated with coarctation of the aorta. Reimplantation of the anomalous Cx to the aorta and coarctation repair were performed. There were no postoperative complications, and the patient was discharged in satisfactory condition. Keywords coronary artery anomaly, coronary artery imaging, coarctation, pulmonary arteries Submitted May 26, 2013; Accepted August 06, 2013.
Introduction Anomalous origin of the circumflex coronary artery (Cx) from the pulmonary artery (ACxPA) is an extremely rare coronary anomaly.1-3 The clinical course of ACxPA may not always be favorable, and patients need surgical treatment in early infancy.1 The anomaly is often associated with another congenital cardiac malformations.1 The literature search revealed that only a few cases of ACxPA associated with aortic coarctation were reported up to date.3-5
Patients and Methods A two-month-old female patient (weight, 3.6 kg) with coarctation of aorta was admitted to the Ukrainian Children’s Cardiac Center, Kyiv, Ukraine. Diagnosis of aortic coarctation was confirmed by echocardiography. Left ventricle ejection fraction (LVEF) was 58% and peak systolic coarctation gradient was 92 mm Hg, also a coronary anomaly was suspected. The patient was referred to catheterization laboratory and underwent balloon dilatation of coarctation of aorta. The procedure was successful, but the control angiography immediately after balloon dilation revealed anomalous origin of the Cx (diameter, 1 mm) from the posterior aspect of the right pulmonary artery 5 mm distal to pulmonary artery bifurcation (Figure 1A). Cardiac magnetic resonance imaging demonstrated moderate subendocardial hypoperfusion of ventricular septum and papillary muscles of the left ventricle. No large subendocardial regions of delayed hyperenhancement
were detected. Considering the signs of hypoperfusion and potentially poor prognosis for this type of coronary anomaly and remaining tubular hypoplasia in B segment of aortic arch, a surgical repair was performed. The infant underwent an operation on the ninth day after admission. Operation was performed through a median sternotomy using hypothermic (28 C) cardiopulmonary bypass and histidine-tryptophan-ketoglutarate (Custodiol HTK; Ko¨hler Chemie GmbH, Germany) cardioplegic solution. Cardioplegic solution was introduced antegradely into the aortic root and retrogradely into the coronary sinus. The Cx originating from the posterior surface of right pulmonary artery was visualized (Figure 2). Button-shaped excision of the Cx ostium was performed. After obtaining enough mobilization, Cx ostium was reimplanted to noncoronary sinus of aorta. Reconstruction of the right pulmonary artery was performed with glutaraldehydetreated pericardial patch. Concomitant aortic arch reconstruction with extended anastomosis was performed.
1
Cardiac Surgery Department, Ukrainian Children’s Cardiac Center, Kyiv, Ukraine Corresponding Author: Yaroslav Mykychak, Cardiac Surgery Department, Ukrainian Children’s Cardiac Center, Chornovola 28/1, Kyiv, 01135 Ukraine. Email: [email protected]
Downloaded from pch.sagepub.com at Scientific library of Moscow State University on January 10, 2014
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World Journal for Pediatric and Congenital Heart Surgery 5(1)
Figure 1. A, Preoperative angiography revealed CoAo and normal R and LAD coronary arteries with anomalous origin of the Cx from the posterior aspect of R pulmonary artery and retrograde flow from the circumflex artery to the pulmonary artery A. B, Postoperative angiography demonstrated the Cx origin from ascending aorta without aortic arch obstruction. CoAo indicates coarctation of aorta; Cx, circumflex coronary artery; LAD, left anterior descending; R, right.
Comment
Figure 2. Operative view of anomalous origin of the Cx from the posterior aspect of RPA. Ao indicates aorta, RVOT, right ventricular outflow tract; RPA, right pulmonary artery.
Results There were no complications in early postoperative period, and patient was discharged from the hospital 18 days after admission and 9 days postoperatively. Five months after the operation, echocardiography showed good left ventricular contractility with an LVEF of 65% and a peak systolic gradient at aortic anastomosis of 15 mm Hg. Control angiography demonstrated the Cx origin from the ascending aorta with neither obstruction nor kinking (Figure 1B).
Anomalous origin of the Cx from the right pulmonary artery associated with coarctation of the aorta is a very rare pathology.1-3 The symptoms of the ACxPA may present in different periods of life or patients may remain asymptomatic with anomaly being discovered accidentally during diagnostic procedures. There are no standard indications or operative techniques for such patients. Bolognesi et al described exceptional case of ischemic heart disease due to the origin of the Cx from the pulmonary artery in a 50-year-old woman.5 She had undergone surgery for aortic coarctation at 16 years of age. Patient was referred to the hospital with chest pain and dyspnea after light exercise. Selective coronary angiography revealed the coronary anomaly. Honey et al described a 13-year-old patient with coarctation of aorta.4 A cardiac catheterization study showed ACxPA. Aortic coarctation repair was performed previously. There was no clinical or electrocardiographic evidence of impaired myocardial perfusion, but it was thought rational to correct the anomaly by closing the orifice of Cx with direct suture one year later. Chopra et al reported a 15-year-oldfemale patient who underwent surgical correction of an aortopulmonary window at 13 months.2 Fourteen years later, she presented with dyspnea on exertion associated with angina. A coronary artery bypass grafting of the anomalous Cx and ligation of its anomalous origin in the pulmonary artery were performed. Sarioglu et al described a ten-year-old female patient who underwent surgical repair of the aortic coarctation at four years of age.3 Six years later, she presented with chest pain during exercise. Cardiac catheterization demonstrated retrograde filling of the Cx from the left anterior descending and right coronary artery, with drainage into the right pulmonary artery. Alexi-Meskishvili et al reported two infants with ACxPA who had to be operated at ages 40 days and 30 days because of severe myocardial dysfunction.1 The aortic reimplantation of the anomalous Cx was performed in the first patient, and simple ligation via left thoracotomy was chosen in the second case.
Downloaded from pch.sagepub.com at Scientific library of Moscow State University on January 10, 2014
Sekelyk et al
99
Although our patient had no signs of severe ischemia, we believe that because of mostly unfavorable clinical course of ACxPA, an elective surgical treatment should be considered on establishing a diagnosis. Declaration of Conflicting Interests The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding The author(s) received no financial support for the research, authorship, and/or publication of this article.
References 1. Alexi-Meskishvili V, Dahnert I, Hetzer R, Lange PE, Karl TR. Origin of the circumflex coronary artery from the pulmonary artery in infants. Ann Thorac Surg. 1998;66(4): 1406-1491.
2. Chopra PS, Reed WH, Wilson AD, Rao PS. Delayed presentation of anomalous circumflex coronary artery arising from pulmonary artery following repair of aortopulmonary window in infancy. Chest. 1994;106(6): 1920-19222. 3. Sarioglu T, Kinoglu B, Saltik L, Eroglu A. Anomalous origin of circumflex coronary artery from the right pulmonary artery associated with subaortic stenosis and coarctation of the aorta. Eur J Cardiothorac Surg. 1997;12(4): 663-665. 4. Honey M, Lincoln JCR, Osborne MP, de Bono DP. Coarctation of aorta with right aortic arch report of surgical correction in 2 cases: one with associated anomalous origin of left circumflex coronary artery from the right pulmonary artery. Br Heart J. 1975;37(9): 937-945. 5. Bolognesi R, Alfieri O, Tsialtas D, Manca C. Surgical treatment of the left circumflex coronary artery from the pulmonary artery in an adult patient. Ann Thorac Surg. 2003;75(5): 1642-1643.
Downloaded from pch.sagepub.com at Scientific library of Moscow State University on January 10, 2014
