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Anomalous origin of the left coronary artery from the pulmonary trunk
International Journal of Cardiology 201 (2015) 165–167
Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Letter to the Editor
Anomalous origin of the left coronary artery from the pulmonary trunk Wei Hua Wu a,1, Jing Ping Sun b,1, Lan Ma a, Xiao Yi Xie a, Xing Sheng Yang b, Cheuk-Man Yu b,⁎ a b
Echocardiographic Department, Shanghai Chest Hospital, Shanghai Jiaotong University, Shanghai, China Division of Cardiology, Department of Medicine and Therapeutics, Prince of Wales Hospital, The Chinese University of Hong Kong, Hong Kong
a r t i c l e
i n f o
Article history: Received 29 July 2015 Accepted 1 August 2015 Available online 4 August 2015 Keywords: Congenital heart disease Anomalous origin coronary artery Echocardiography
A 59 years old female admitted to our hospital with complaints of chest pressure for 5 years and syncope twice. Physical examination was unremarkable except the 3/6 grade systolic murmur at apical area. Heart rate was 70/minuet. Blood pressure was measured as 120/80 mm Hg. Electrocardiographic Holter monitor showed multiple atrial and ventricular premaster beats with short array ventricular tachycardia. Chest X-ray revealed cardiomegaly and pulmonary congestion. When echocardiographic examination was performed, apical 4chamber view showed that left atrium and ventricle were significantly enlarged with a border line systolic function (EF = 50%); there is a color Doppler flow in ventricular septum that communicated with left ventricular cavity continuously; the blood flow was back to septum during systole and into LV cavity during diastole. Parasternal short axis view showed plenty of intra septum blood flow (Fig. 1, videos 1, 2). There is an abnormal blood flow retrospectively into the main pulmonary artery and the orifice of left coronary artery could not be found in the aortic root, which leads to suspicion of anomalous origin left coronary artery. Because the origin and course of anomalous coronary arteries must be defined, we performed cardiac 64-slice multidetector computed tomography (MDCT), which showed that the right coronary artery
⁎ Corresponding author at: Institute of Vascular Medicine (IVM), Institute of Innovative Medicine (IIM), Heart Education And Research Training (HEART) Centre, Li Ka Shing Institute of Health Sciences (LiHS), S.H. Ho Cardiovascular Disease and Stroke Centre, and Division of Cardiology, Department of Medicine and Therapeutics, Prince of Wales Hospital, The Chinese University of Hong Kong, Shatin, N.T., Hong Kong. E-mail address: [email protected] (C.-M. Yu). 1 Co-first author.
http://dx.doi.org/10.1016/j.ijcard.2015.08.041 0167-5273/© 2015 Elsevier Ireland Ltd. All rights reserved.
(RCA) originated from the aorta, but the left coronary artery (LCA) originated from the pulmonary artery; there are plenty of anastomosis branches between the right and left coronary arteries (Fig. 2). Catheterization and angiographic study were planned urgently. Aortic root and pulmonary artery angiographies showed dilatation of the right coronary artery which originated from the aorta with plenty of collateral arteries between right and left coronaries, through which the blood flows retrospectively into anomalous original left coronary (Video 3). Bypass surgery was successfully performed without any complications. The surgeon found that the LCA originated from the main pulmonary artery, and the orifice of the LCA was located above the pulmonary valve about 10 mm; ligated the LCA close to its connection with the main pulmonary artery, and internal mammary artery bypass to the LCA was performed. The patient improved quickly after operation and is still being followed up with normal left ventricular systolic function. Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary abnormality associated with early infant mortality and adult sudden death. The incidence of ALCAPA is estimated at 1/300,000 live births comprising between 0.24% and 0.46% of all congenital heart defects. Left untreated, about 90% of infants die in the first year of life as a result of intractable left ventricular failure [1]. In a review of 25 infant and adult cases, Kaunitz [2] noted in the adult cases, RCA dilation and significant development of collateralization from the RCA to the LCA. This became the first distinction proposed between infants who die in the first year and those who survive to adulthood. Several pathophysiologic mechanisms have been evoked to explain why ALCAPA is well tolerated in the early neonatal period and why some patients survive until adulthood. During fetal and early neonatal life, the pulmonary pressure equals systemic pressures. This leads to antegrade flow in the anomalous left coronary artery and the normal RCA. Gradual decline in the pulmonary pressure after birth results in low perfusion pressure, hypoxic blood, and reversal of flow in the LCA [3]. In the infant type of ALCAPA, there is little or no collateral flow between the RCA and the LCA. When reversal of flow in the LCA is established, reduced blood supply to the myocardium leads to myocardial infarction [3]. In the adult type, reversal of flow in the LCA triggers formation of interarterial collaterals. With time, marked dilatation of the RCA and LCA causes preferential blood flow into the low-pressure pulmonary system rather than into the high-resistance myocardium. This results in a coronary steal phenomenon [3]. Over the past 2 decades, the number of reported patients with ALCAPA over age 50 years has increased. This diagnosis in an older
166
W.H. Wu et al. / International Journal of Cardiology 201 (2015) 165–167
Fig. 1. A. Atypical parasternal short axis view showed anomalous left coronary artery with retrospective flow arrow (arrow). B. Continuous wave Doppler recorded from short axis view showed coronary flow into intra-septum during cardiac cycle, predominately in diastole. C. Apical 4-chamber view showed left atria and ventricle were significantly enlarged with a color Doppler flow in ventricular septum (long arrow) communicated with left ventricular cavity continuously, the blood flow was back to septum during systole (short arrow,) and D. into LV cavity during diastole (short arrow).
cohort correlates with advances in echocardiography and the introduction of cardiac CT and MRI as new noninvasive techniques for evaluation of coronary anatomy. Cardiac CT and MRI are useful not only in diagnosis, but may also offer prognostic information, allowing risk stratification, and be utilized for long-term follow-up imaging. Echocardiography and MRI also can evaluate myocardial function, ischemia and viability. Imaging has reached such an advanced stage of sophistication and precision that these techniques may eventually allow for the establishment of definite clinical guidelines for managing this congenital condition. Transthoracic echocardiography is an essential tool for the diagnosis of ALCAPA. In pediatric patients, the echocardiographic diagnosis of this anomaly has improved. However, in the adult group the location of the right coronary artery ostium is always difficult to detect by transthoracic two-dimensional imaging because of a low spatial resolution with poor penetration through the chest wall [4]. Cardiac computed tomography coronary angiogram and cardiovascular magnetic resonance provide excellent visualization of coronary artery anomalies. They complete coronary angiography by providing detailed anatomic information of origin, course, and relationship of the anomalous coronary artery. Our case was a 59 year old who presented with atypical chest pain. Chest X ray showed enlarged heart and pulmonary congestion. Anomalous origin of the left coronary artery from the pulmonary artery was diagnosed by the echocardiography initially, and confirmed by the CT and angiogram.
Most of the patients with ALCAPA present with findings of decreased left ventricular function and mitral regurgitation secondary to myocardial ischemia and papillary muscle damage, respectively [4]. Immediate coronary reimplantation and early improvement of the left ventricular function are essential for recovery. Surgical repair outcomes are quite excellent in most patients. After successful repair, ventricular function, dilatation, and mitral regurgitation improve gradually [5]. The patient reported here underwent successful surgical treatment. At the time of this writing, she remains asymptomatic more than 2.5 years post operation. Conclusion ALCAPA is a rare and life-threatening condition. The availability of newer diagnostic modalities correlates with an increasing incidence in an older cohort. A comprehensive review of adult ALCAPA suggests that older patients experience less frequent life-threatening presentations and sudden death. Nevertheless, surgical correction should be considered for all patients. Finally, this review emphasizes the lack of adequate comparative surgical and follow-up data in reported adults, highlighting the need for this information in future studies [6]. Conflict of interest statement The authors report no relationships that could be construed as a conflict of interest. Supplementary data to this article can be found online at http://dx. doi.org/10.1016/j.ijcard.2015.08.041.
W.H. Wu et al. / International Journal of Cardiology 201 (2015) 165–167
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Fig. 2. Computer tomography reconstructive imaging: A and B showed the right coronary artery originating from aorta, but left coronary artery originating from pulmonary artery. C and D showed plenty anastomosis branches between right and left coronary arteries.
References [1] J.D. Keith, The anomalous origin of the left coronary artery from the pulmonary artery, Br. Heart J. 21 (1959) 149–161. [2] P.E. Kaunitz, Origin of left coronary artery from the pulmonary artery: review of the literature and report of two cases, Am. Heart J. 33 (1947) 182–206. [3] D.J. Driscoll, M.R. Nihill, C.E. Mullins, D.A. Cooley, D.G. McNamara, Management of symptomatic infants with anomalous origin of the left coronary artery from the pulmonary artery, Am. J. Cardiol. l47 (1981) 642–648.
[4] A. Dodge-Khatami, C. Mavroudis, C.L. Backer, Anomalous origin of the left coronary artery from the pulmonary artery: collective review of surgical therapy, Ann. Thorac. Surg. 74 (2002) 946–955. [5] R.B. Case, A.G. Morrow, W. Stainsby, J.O. Nestor, Anomalous origin of the left coronary artery: physiologic defect and suggested surgical treatment, Circulation 17 (6) (1958) 1062–1068. [6] J.M. Yau, R. Singh, E.J. Halpern, D. Fischman, Anomalous origin of the left coronary artery from the pulmonary artery in adults: a comprehensive review of 151 adult cases and a new diagnosis in a 53-year-old woman, Clin. Cardiol. 34 (4) (2011 Apr) 204–210.
Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Letter to the Editor
Anomalous origin of the left coronary artery from the pulmonary trunk Wei Hua Wu a,1, Jing Ping Sun b,1, Lan Ma a, Xiao Yi Xie a, Xing Sheng Yang b, Cheuk-Man Yu b,⁎ a b
Echocardiographic Department, Shanghai Chest Hospital, Shanghai Jiaotong University, Shanghai, China Division of Cardiology, Department of Medicine and Therapeutics, Prince of Wales Hospital, The Chinese University of Hong Kong, Hong Kong
a r t i c l e
i n f o
Article history: Received 29 July 2015 Accepted 1 August 2015 Available online 4 August 2015 Keywords: Congenital heart disease Anomalous origin coronary artery Echocardiography
A 59 years old female admitted to our hospital with complaints of chest pressure for 5 years and syncope twice. Physical examination was unremarkable except the 3/6 grade systolic murmur at apical area. Heart rate was 70/minuet. Blood pressure was measured as 120/80 mm Hg. Electrocardiographic Holter monitor showed multiple atrial and ventricular premaster beats with short array ventricular tachycardia. Chest X-ray revealed cardiomegaly and pulmonary congestion. When echocardiographic examination was performed, apical 4chamber view showed that left atrium and ventricle were significantly enlarged with a border line systolic function (EF = 50%); there is a color Doppler flow in ventricular septum that communicated with left ventricular cavity continuously; the blood flow was back to septum during systole and into LV cavity during diastole. Parasternal short axis view showed plenty of intra septum blood flow (Fig. 1, videos 1, 2). There is an abnormal blood flow retrospectively into the main pulmonary artery and the orifice of left coronary artery could not be found in the aortic root, which leads to suspicion of anomalous origin left coronary artery. Because the origin and course of anomalous coronary arteries must be defined, we performed cardiac 64-slice multidetector computed tomography (MDCT), which showed that the right coronary artery
⁎ Corresponding author at: Institute of Vascular Medicine (IVM), Institute of Innovative Medicine (IIM), Heart Education And Research Training (HEART) Centre, Li Ka Shing Institute of Health Sciences (LiHS), S.H. Ho Cardiovascular Disease and Stroke Centre, and Division of Cardiology, Department of Medicine and Therapeutics, Prince of Wales Hospital, The Chinese University of Hong Kong, Shatin, N.T., Hong Kong. E-mail address: [email protected] (C.-M. Yu). 1 Co-first author.
http://dx.doi.org/10.1016/j.ijcard.2015.08.041 0167-5273/© 2015 Elsevier Ireland Ltd. All rights reserved.
(RCA) originated from the aorta, but the left coronary artery (LCA) originated from the pulmonary artery; there are plenty of anastomosis branches between the right and left coronary arteries (Fig. 2). Catheterization and angiographic study were planned urgently. Aortic root and pulmonary artery angiographies showed dilatation of the right coronary artery which originated from the aorta with plenty of collateral arteries between right and left coronaries, through which the blood flows retrospectively into anomalous original left coronary (Video 3). Bypass surgery was successfully performed without any complications. The surgeon found that the LCA originated from the main pulmonary artery, and the orifice of the LCA was located above the pulmonary valve about 10 mm; ligated the LCA close to its connection with the main pulmonary artery, and internal mammary artery bypass to the LCA was performed. The patient improved quickly after operation and is still being followed up with normal left ventricular systolic function. Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary abnormality associated with early infant mortality and adult sudden death. The incidence of ALCAPA is estimated at 1/300,000 live births comprising between 0.24% and 0.46% of all congenital heart defects. Left untreated, about 90% of infants die in the first year of life as a result of intractable left ventricular failure [1]. In a review of 25 infant and adult cases, Kaunitz [2] noted in the adult cases, RCA dilation and significant development of collateralization from the RCA to the LCA. This became the first distinction proposed between infants who die in the first year and those who survive to adulthood. Several pathophysiologic mechanisms have been evoked to explain why ALCAPA is well tolerated in the early neonatal period and why some patients survive until adulthood. During fetal and early neonatal life, the pulmonary pressure equals systemic pressures. This leads to antegrade flow in the anomalous left coronary artery and the normal RCA. Gradual decline in the pulmonary pressure after birth results in low perfusion pressure, hypoxic blood, and reversal of flow in the LCA [3]. In the infant type of ALCAPA, there is little or no collateral flow between the RCA and the LCA. When reversal of flow in the LCA is established, reduced blood supply to the myocardium leads to myocardial infarction [3]. In the adult type, reversal of flow in the LCA triggers formation of interarterial collaterals. With time, marked dilatation of the RCA and LCA causes preferential blood flow into the low-pressure pulmonary system rather than into the high-resistance myocardium. This results in a coronary steal phenomenon [3]. Over the past 2 decades, the number of reported patients with ALCAPA over age 50 years has increased. This diagnosis in an older
166
W.H. Wu et al. / International Journal of Cardiology 201 (2015) 165–167
Fig. 1. A. Atypical parasternal short axis view showed anomalous left coronary artery with retrospective flow arrow (arrow). B. Continuous wave Doppler recorded from short axis view showed coronary flow into intra-septum during cardiac cycle, predominately in diastole. C. Apical 4-chamber view showed left atria and ventricle were significantly enlarged with a color Doppler flow in ventricular septum (long arrow) communicated with left ventricular cavity continuously, the blood flow was back to septum during systole (short arrow,) and D. into LV cavity during diastole (short arrow).
cohort correlates with advances in echocardiography and the introduction of cardiac CT and MRI as new noninvasive techniques for evaluation of coronary anatomy. Cardiac CT and MRI are useful not only in diagnosis, but may also offer prognostic information, allowing risk stratification, and be utilized for long-term follow-up imaging. Echocardiography and MRI also can evaluate myocardial function, ischemia and viability. Imaging has reached such an advanced stage of sophistication and precision that these techniques may eventually allow for the establishment of definite clinical guidelines for managing this congenital condition. Transthoracic echocardiography is an essential tool for the diagnosis of ALCAPA. In pediatric patients, the echocardiographic diagnosis of this anomaly has improved. However, in the adult group the location of the right coronary artery ostium is always difficult to detect by transthoracic two-dimensional imaging because of a low spatial resolution with poor penetration through the chest wall [4]. Cardiac computed tomography coronary angiogram and cardiovascular magnetic resonance provide excellent visualization of coronary artery anomalies. They complete coronary angiography by providing detailed anatomic information of origin, course, and relationship of the anomalous coronary artery. Our case was a 59 year old who presented with atypical chest pain. Chest X ray showed enlarged heart and pulmonary congestion. Anomalous origin of the left coronary artery from the pulmonary artery was diagnosed by the echocardiography initially, and confirmed by the CT and angiogram.
Most of the patients with ALCAPA present with findings of decreased left ventricular function and mitral regurgitation secondary to myocardial ischemia and papillary muscle damage, respectively [4]. Immediate coronary reimplantation and early improvement of the left ventricular function are essential for recovery. Surgical repair outcomes are quite excellent in most patients. After successful repair, ventricular function, dilatation, and mitral regurgitation improve gradually [5]. The patient reported here underwent successful surgical treatment. At the time of this writing, she remains asymptomatic more than 2.5 years post operation. Conclusion ALCAPA is a rare and life-threatening condition. The availability of newer diagnostic modalities correlates with an increasing incidence in an older cohort. A comprehensive review of adult ALCAPA suggests that older patients experience less frequent life-threatening presentations and sudden death. Nevertheless, surgical correction should be considered for all patients. Finally, this review emphasizes the lack of adequate comparative surgical and follow-up data in reported adults, highlighting the need for this information in future studies [6]. Conflict of interest statement The authors report no relationships that could be construed as a conflict of interest. Supplementary data to this article can be found online at http://dx. doi.org/10.1016/j.ijcard.2015.08.041.
W.H. Wu et al. / International Journal of Cardiology 201 (2015) 165–167
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Fig. 2. Computer tomography reconstructive imaging: A and B showed the right coronary artery originating from aorta, but left coronary artery originating from pulmonary artery. C and D showed plenty anastomosis branches between right and left coronary arteries.
References [1] J.D. Keith, The anomalous origin of the left coronary artery from the pulmonary artery, Br. Heart J. 21 (1959) 149–161. [2] P.E. Kaunitz, Origin of left coronary artery from the pulmonary artery: review of the literature and report of two cases, Am. Heart J. 33 (1947) 182–206. [3] D.J. Driscoll, M.R. Nihill, C.E. Mullins, D.A. Cooley, D.G. McNamara, Management of symptomatic infants with anomalous origin of the left coronary artery from the pulmonary artery, Am. J. Cardiol. l47 (1981) 642–648.
[4] A. Dodge-Khatami, C. Mavroudis, C.L. Backer, Anomalous origin of the left coronary artery from the pulmonary artery: collective review of surgical therapy, Ann. Thorac. Surg. 74 (2002) 946–955. [5] R.B. Case, A.G. Morrow, W. Stainsby, J.O. Nestor, Anomalous origin of the left coronary artery: physiologic defect and suggested surgical treatment, Circulation 17 (6) (1958) 1062–1068. [6] J.M. Yau, R. Singh, E.J. Halpern, D. Fischman, Anomalous origin of the left coronary artery from the pulmonary artery in adults: a comprehensive review of 151 adult cases and a new diagnosis in a 53-year-old woman, Clin. Cardiol. 34 (4) (2011 Apr) 204–210.