J Thromb Thrombolysis DOI 10.1007/s11239-013-1038-1
Anomalous left main coronary artery originating from the pulmonary artery Said Alsidawi • Faisal Khan
Ó Springer Science+Business Media New York 2013
Introduction Coronary artery anomalies are rare congenital diseases. Most are usually diagnosed in the first few years of life due to symptoms of ischemia, heart failure or arrhythmia. We present a unique case of anomalous left main coronary artery (LM) originating from the main pulmonary artery (PA) in an elderly patient (Fig. 1). Case presentation An 81 year-old Caucasian female presented to the hospital with dyspnea on exertion and was found to be in new onset atrial fibrillation. She has a past medical history of hypertension and has led an active life till her symptoms of dyspnea started. An echocardiogram was performed as a part of the work up showing an ejection fraction of 35 % with moderate mitral valve regurgitation (Fig. 2). Given her new onset cardiomyopathy and reluctance to undergo invasive work up, a pharmacologic nuclear myocardial perfusion stress test was performed showing a moderate sized area of anterolateral wall mixed ischemia and scar with an ejection fraction of 36 % (Fig. 3). A coronary angiography was recommended but patient was still reluctant and preferred a non-invasive work-up. Thus, a cardiac CT angiography was recommended. This showed an anomalous left main coronary artery originating from the proximal main pulmonary artery just beyond the
Said Alsidawi and Faisal Khan contributed equally to this work. S. Alsidawi (&) F. Khan Division of Cardiovascular Health and Disease, University of Cincinnati College of Medicine, Cincinnati, OH, USA e-mail: [email protected]
pulmonic valve. The contrast density in the left main coronary artery is slightly higher than in the pulmonary artery and matches the contrast density in the arterial circulation indicating collateral filling of the left coronary system from either the aorta or right coronary artery (Fig. 4). She was started on beta-blockers and lisinopril. She underwent a successful electrical cardioversion preceded by a transesophageal echocardiogram to rule out left atrial appendage clot. She was started and warfarin and discharged. On follow-up 3 months later, she remained in sinus rhythm with significant improvement in her symptoms. A repeated echocardiogram showed significant improvement in the left ventricular ejection fraction to 55 % with only mild mitral regurgitation. On six-month follow-up she remained asymptomatic with improved functional capacity.
Discussion Anomalous origin of the LM from the PA is a very rare condition accounting for approximately 0.25–0.5 % of all congenital heart disease, with more than 90 % of the cases present in the first year of life. The incidence does not vary geographically. It is not considered an inheritable congenital cardiac defect. It is usually an isolated cardiac anomaly but, in rare incidences, has been described with patent ductus arteriosus (PDA) and ventricular septal defect [1]. Shortly after birth, when such anomaly is present, left coronary artery flow reverses and enters the pulmonic trunk due to the low pulmonary vascular resistance (coronary steal phenomena). As a result, left ventricular myocardium remains under-perfused. Consequently, the combination of left ventricular dysfunction and significant mitral valve insufficiency leads to congestive heart failure symptoms [2].
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S. Alsidawi, F. Khan
Fig. 1 CT angiography showing the anomalous left main artery originating from the pulmonary artery from a cross sectional view
Fig. 3 3D reconstruction imaging showing the anomalous origin of the left main artery
Fig. 4 3D reconstruction imaging showing the anomalous origin of the left main artery with contrast density in the left main that is higher than that in the pulmonary artery and matches that in the right coronary artery Fig. 2 CT angiography showing the anomalous left main artery originating from the pulmonary artery from a coronal view
Most patients are usually diagnosed in the first few years of life due to symptoms of ischemia, heart failure or arrhythmia. Rarely, some kids outgrow their symptoms and remain asymptomatic till adulthood due to extensive collateral circulations. However, it is estimated that the mortality during the first year of life is as high as 80 %. On physical exam, a holo-systolic murmur consistent with mitral valve regurgitation is common. Left ventricular dilation is evidenced by a displaced apex laterally and downward. Rarely, a continuous murmur can be detected on the left sternal border but this cannot be distinguished from a PDA murmur.
123
Cardiac computed tomography remains the test of choice for making the diagnosis due to its ability in showing the coronary arteries origins and course with excellent resolution. Echocardiogram may identify abnormal origin of the left coronary artery from the main pulmonary artery. There is retrograde flow into the pulmonary trunk that is directed in an unusual orientation within the main pulmonary artery distinguishing it from PDA [3]. Cardiac MRI is another way of making the diagnoses and showing the subendocardial fibrosis in the anteroseptal areas along with the anomalous origin of the LM coronary artery [4]. Cardiac catheterization and angiography can also be used to make the diagnosis [5]. Right coronary arteriography usually demonstrates a dilated right coronary artery
Anomalous left main coronary artery
system with collateral circulation to the left coronary artery which shunts into the PA. However, if collaterals have not occurred, identification of the anomalous left coronary artery may not be possible. Medical management should focus on heart failure management. All patients should be treated with angiotensin-converting-enzyme inhibitors as well as betablockers if no contraindications. Aggressive afterload reduction should be avoided, however, so that collateral flow to the left coronary system is not compromised. Surgical intervention remains the treatment of choice despite the potential for percutaneous catheter interventions. Many techniques were suggested. In the past, ligation of the LM was used without bypass with full dependence on collaterals circulation. This resulted in variable outcomes depending on the degree of collateral flow between the RCA and the LM. More recent surgical techniques include ligating the LM coronary artery with bypass from the subclavian artery [6] or a saphenous venous graft attached to the aorta [7]. Re-implantation of the LM to the aorta after cutting the origin from the PA is also possible but more complicated [8]. Takeuchi procedure (creation of an aortopulmonary window and an intrapulmonary tunnel extending from the anomalous ostium to the window) was suggested by Takeuchi et al. in 1997 as another surgical option but represents a more complex surgery [9]. Most institutions currently prefer ligating the LM in its origin with bypassing the vessel with either an arterial or venous graft as this holds good surgical outcome with fewer complications. Intraoperative transesophageal echocardiogram is important to evaluate the severity of mitral valve insufficiency and the need for repair. Overall, survival is very good if surgical intervention and medical management are considered as soon as the diagnosis is established.
Conflict of interest
No conflict of interest to report.
References 1. Arciniegas E, Farooki ZQ, Hakimi M, Green EW (1980) Management of anomalous left coronary artery from the pulmonary artery. Circulation 62(2 Pt 2):I180–I189 2. Su LS, Burkhart HM, O’Leary PW, Dearani JA (2011) Mitral valve arcade with concomitant anomalous left coronary artery from the pulmonary artery. Ann Thorac Surg 92(6):e121–e123 3. Pisacane C, Pinto SC, De Gregorio P, Pisacane F, Monda V, Caso P, Calabro` R (2006) ‘‘Steal’’ collaterals: an echocardiographic diagnostic marker for anomalous origin of the left main coronary artery from the pulmonary artery in the adult. J Am Soc Echocardiogr 19(1):107.e3–107.e6 4. Secinaro A, Ntsinjana H, Tann O, Schuler PK, Muthurangu V, Hughes M et al (2011) Cardiovascular magnetic resonance findings in repaired anomalous left coronary artery to pulmonary artery connection (ALCAPA). J Cardiovasc Magn Reson 13:27 5. Juan CC, Hwang B, Lee PC, Meng CC (2011) Diagnostic application of multidetector-row computed tomographic coronary angiography to assess coronary abnormalities in pediatric patients: comparison with invasive coronary angiography. Pediatr Neonatol 52(4):208–213 6. Champsaur G, Bozio A, Joffre B et al (1980) Anomalous origin of the left coronary artery from the pulmonary artery. Treatment by left subclavian-left main coronary artery anastomosis. Nouv Presse Med 9(16):1167–1169 7. el-Said GM, Ruzyllo W, Williams RL et al (1973) Early and late result of saphenous vein graft for anomalous origin of left coronary artery from pulmonary artery. Circulation 48(1 Suppl):III2–III6 8. Erdinc M, Hosgor K, Karahan O (2011) Repair of anomalous origin of the left coronary artery arising from right pulmonary artery with rolled-conduit-extended reimplantation in an adult. J Card Surg 26(6):604–607 9. Takeuchi S, Imamura H, Katsumoto K et al (1979) New surgical method for repair of anomalous left coronary artery from pulmonary artery. J Thorac Cardiovasc Surg 78(1):7–11
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Anomalous left main coronary artery originating from the pulmonary artery Said Alsidawi • Faisal Khan
Ó Springer Science+Business Media New York 2013
Introduction Coronary artery anomalies are rare congenital diseases. Most are usually diagnosed in the first few years of life due to symptoms of ischemia, heart failure or arrhythmia. We present a unique case of anomalous left main coronary artery (LM) originating from the main pulmonary artery (PA) in an elderly patient (Fig. 1). Case presentation An 81 year-old Caucasian female presented to the hospital with dyspnea on exertion and was found to be in new onset atrial fibrillation. She has a past medical history of hypertension and has led an active life till her symptoms of dyspnea started. An echocardiogram was performed as a part of the work up showing an ejection fraction of 35 % with moderate mitral valve regurgitation (Fig. 2). Given her new onset cardiomyopathy and reluctance to undergo invasive work up, a pharmacologic nuclear myocardial perfusion stress test was performed showing a moderate sized area of anterolateral wall mixed ischemia and scar with an ejection fraction of 36 % (Fig. 3). A coronary angiography was recommended but patient was still reluctant and preferred a non-invasive work-up. Thus, a cardiac CT angiography was recommended. This showed an anomalous left main coronary artery originating from the proximal main pulmonary artery just beyond the
Said Alsidawi and Faisal Khan contributed equally to this work. S. Alsidawi (&) F. Khan Division of Cardiovascular Health and Disease, University of Cincinnati College of Medicine, Cincinnati, OH, USA e-mail: [email protected]
pulmonic valve. The contrast density in the left main coronary artery is slightly higher than in the pulmonary artery and matches the contrast density in the arterial circulation indicating collateral filling of the left coronary system from either the aorta or right coronary artery (Fig. 4). She was started on beta-blockers and lisinopril. She underwent a successful electrical cardioversion preceded by a transesophageal echocardiogram to rule out left atrial appendage clot. She was started and warfarin and discharged. On follow-up 3 months later, she remained in sinus rhythm with significant improvement in her symptoms. A repeated echocardiogram showed significant improvement in the left ventricular ejection fraction to 55 % with only mild mitral regurgitation. On six-month follow-up she remained asymptomatic with improved functional capacity.
Discussion Anomalous origin of the LM from the PA is a very rare condition accounting for approximately 0.25–0.5 % of all congenital heart disease, with more than 90 % of the cases present in the first year of life. The incidence does not vary geographically. It is not considered an inheritable congenital cardiac defect. It is usually an isolated cardiac anomaly but, in rare incidences, has been described with patent ductus arteriosus (PDA) and ventricular septal defect [1]. Shortly after birth, when such anomaly is present, left coronary artery flow reverses and enters the pulmonic trunk due to the low pulmonary vascular resistance (coronary steal phenomena). As a result, left ventricular myocardium remains under-perfused. Consequently, the combination of left ventricular dysfunction and significant mitral valve insufficiency leads to congestive heart failure symptoms [2].
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S. Alsidawi, F. Khan
Fig. 1 CT angiography showing the anomalous left main artery originating from the pulmonary artery from a cross sectional view
Fig. 3 3D reconstruction imaging showing the anomalous origin of the left main artery
Fig. 4 3D reconstruction imaging showing the anomalous origin of the left main artery with contrast density in the left main that is higher than that in the pulmonary artery and matches that in the right coronary artery Fig. 2 CT angiography showing the anomalous left main artery originating from the pulmonary artery from a coronal view
Most patients are usually diagnosed in the first few years of life due to symptoms of ischemia, heart failure or arrhythmia. Rarely, some kids outgrow their symptoms and remain asymptomatic till adulthood due to extensive collateral circulations. However, it is estimated that the mortality during the first year of life is as high as 80 %. On physical exam, a holo-systolic murmur consistent with mitral valve regurgitation is common. Left ventricular dilation is evidenced by a displaced apex laterally and downward. Rarely, a continuous murmur can be detected on the left sternal border but this cannot be distinguished from a PDA murmur.
123
Cardiac computed tomography remains the test of choice for making the diagnosis due to its ability in showing the coronary arteries origins and course with excellent resolution. Echocardiogram may identify abnormal origin of the left coronary artery from the main pulmonary artery. There is retrograde flow into the pulmonary trunk that is directed in an unusual orientation within the main pulmonary artery distinguishing it from PDA [3]. Cardiac MRI is another way of making the diagnoses and showing the subendocardial fibrosis in the anteroseptal areas along with the anomalous origin of the LM coronary artery [4]. Cardiac catheterization and angiography can also be used to make the diagnosis [5]. Right coronary arteriography usually demonstrates a dilated right coronary artery
Anomalous left main coronary artery
system with collateral circulation to the left coronary artery which shunts into the PA. However, if collaterals have not occurred, identification of the anomalous left coronary artery may not be possible. Medical management should focus on heart failure management. All patients should be treated with angiotensin-converting-enzyme inhibitors as well as betablockers if no contraindications. Aggressive afterload reduction should be avoided, however, so that collateral flow to the left coronary system is not compromised. Surgical intervention remains the treatment of choice despite the potential for percutaneous catheter interventions. Many techniques were suggested. In the past, ligation of the LM was used without bypass with full dependence on collaterals circulation. This resulted in variable outcomes depending on the degree of collateral flow between the RCA and the LM. More recent surgical techniques include ligating the LM coronary artery with bypass from the subclavian artery [6] or a saphenous venous graft attached to the aorta [7]. Re-implantation of the LM to the aorta after cutting the origin from the PA is also possible but more complicated [8]. Takeuchi procedure (creation of an aortopulmonary window and an intrapulmonary tunnel extending from the anomalous ostium to the window) was suggested by Takeuchi et al. in 1997 as another surgical option but represents a more complex surgery [9]. Most institutions currently prefer ligating the LM in its origin with bypassing the vessel with either an arterial or venous graft as this holds good surgical outcome with fewer complications. Intraoperative transesophageal echocardiogram is important to evaluate the severity of mitral valve insufficiency and the need for repair. Overall, survival is very good if surgical intervention and medical management are considered as soon as the diagnosis is established.
Conflict of interest
No conflict of interest to report.
References 1. Arciniegas E, Farooki ZQ, Hakimi M, Green EW (1980) Management of anomalous left coronary artery from the pulmonary artery. Circulation 62(2 Pt 2):I180–I189 2. Su LS, Burkhart HM, O’Leary PW, Dearani JA (2011) Mitral valve arcade with concomitant anomalous left coronary artery from the pulmonary artery. Ann Thorac Surg 92(6):e121–e123 3. Pisacane C, Pinto SC, De Gregorio P, Pisacane F, Monda V, Caso P, Calabro` R (2006) ‘‘Steal’’ collaterals: an echocardiographic diagnostic marker for anomalous origin of the left main coronary artery from the pulmonary artery in the adult. J Am Soc Echocardiogr 19(1):107.e3–107.e6 4. Secinaro A, Ntsinjana H, Tann O, Schuler PK, Muthurangu V, Hughes M et al (2011) Cardiovascular magnetic resonance findings in repaired anomalous left coronary artery to pulmonary artery connection (ALCAPA). J Cardiovasc Magn Reson 13:27 5. Juan CC, Hwang B, Lee PC, Meng CC (2011) Diagnostic application of multidetector-row computed tomographic coronary angiography to assess coronary abnormalities in pediatric patients: comparison with invasive coronary angiography. Pediatr Neonatol 52(4):208–213 6. Champsaur G, Bozio A, Joffre B et al (1980) Anomalous origin of the left coronary artery from the pulmonary artery. Treatment by left subclavian-left main coronary artery anastomosis. Nouv Presse Med 9(16):1167–1169 7. el-Said GM, Ruzyllo W, Williams RL et al (1973) Early and late result of saphenous vein graft for anomalous origin of left coronary artery from pulmonary artery. Circulation 48(1 Suppl):III2–III6 8. Erdinc M, Hosgor K, Karahan O (2011) Repair of anomalous origin of the left coronary artery arising from right pulmonary artery with rolled-conduit-extended reimplantation in an adult. J Card Surg 26(6):604–607 9. Takeuchi S, Imamura H, Katsumoto K et al (1979) New surgical method for repair of anomalous left coronary artery from pulmonary artery. J Thorac Cardiovasc Surg 78(1):7–11
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