CASE REPORT – CONGENITAL
Interactive CardioVascular and Thoracic Surgery 20 (2015) 283–285 doi:10.1093/icvts/ivu347 Advance Access publication 6 November 2014
Asymptomatic anomalous origin of left anterior descending artery from the pulmonary artery and multiple atherosclerotic stenoses revealed by silent ischaemia Gurbuz Murata, François Cellierb, Bertand Leobonc and Nicolas Boudoua,* a b c
Department of Cardiology, Rangueil University Hospital, Toulouse, France Department of Cardiology, Henri Mondor Hospital, Aurillac, France Department of Cardiothoracic Surgery, Rangueil University Hospital, Toulouse, France
* Corresponding author. Department of Cardiology, Rangueil University Hospital, 1 avenue Jean Poulhès, 31059 Toulouse, France. Tel: +33-5-61323310; fax: +33-5-61323325; e-mail: [email protected] (N. Boudou). Received 17 July 2014; received in revised form 18 September 2014; accepted 22 September 2014
Abstract Anomalous origin of the left coronary artery from the pulmonary artery is a rare congenital disease responsible for a high mortality rate in childhood. Here, we report for the first time the case of an asymptomatic 61-year old patient showing a combination of anomalous origin of the left anterior descending artery from pulmonary artery and atherosclerotic stenosis in both the right coronary artery and the left circumflex artery without anomalous origin. Keywords: Anomalous origin of the left coronary artery from the pulmonary artery • Anomalous origin of the left anterior descending artery from pulmonary artery • Atherosclerosis • Congenital heart disease • Coronary bypass
INTRODUCTION Bland–Garland–White syndrome, or anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) syndrome, is a rare congenital heart disease with an incidence of 1 in 300 000 live births and found in 0.5% of children with congenital heart disease [1]. There is usually no manifestation before birth because systemic and pulmonary artery pressures are equal. Clinical presentation depends on coronary artery topography. In cases with a dominant left coronary artery, ischaemic congestive heart failure or mitral insufficiency by annular dilatation reveals this disease early. Without treatment, the mortality rate is very high and death occurs during childhood [2]. When the right coronary artery (RCA) is dominant, sudden death of up to 90% of patients is observed at a mean age of 35 years and results from myocardial ischaemia, heart failure or malignant arrhythmia [3]. Rarely, this disease can be revealed at a later stage. We report here an original case of an anomalous origin of left anterior descending artery from the pulmonary artery (ALADAPA) associated with atherosclerotic stenosis in the RCA and the left circumflex artery (LCX) revealed by diffuse silent ischaemia before carotid endarterectomy.
Carotid ultrasound revealed a severe stenosis of the left internal carotid, requiring surgical treatment. The patient underwent a preoperative cardiological evaluation. He did not report any symptom before the stroke and was not taking any medication. Transthoracic echocardiography was normal. Myocardial SPECT revealed five anterior ischaemic segments and the coronary angiography showed severe stenosis of the RCA and the second marginal of the LCX (Fig. 1A and B, white arrows). The left anterior descending artery (LAD) appeared as a chronic total occlusion with collateral filling [Fig. 1B (red arrow) and C], and seemed to arise from the pulmonary artery. Coronary computed tomography (CT) showed an isolated anomalous origin of the LAD from the pulmonary artery without any atherosclerotic stenosis (Fig. 2A). The heart team’s strategy was to treat the left carotid stenosis first and then to perform myocardial revascularization by coronary artery bypass graft (CABG). In accordance with the latest guidelines, CABG was performed a few weeks following the left carotid endarterectomy, with the left mammary artery to the LAD, right mammary artery to the second posterolateral branch and the saphenous vein to the posterior descending artery. Intra-operative findings confirmed origin of LAD from pulmonary artery (Fig. 2B). The left ventricular ejection fraction remained normal and graft patency was assessed by cardiac CT within days following the surgical procedure (Fig. 2C).
CASE DESCRIPTION The clinical presentation of the ALADAPA syndrome in the adult population varies. Symptoms are related to chronic myocardial
© The Author 2014. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.
CASE REPORT
DISCUSSION A 61-year old patient was admitted to the neurology department for acute ischaemic stroke. His cardiovascular risk factors were type II diabetes mellitus, smoking, arterial hypertension and obesity.
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G. Murat et al. / Interactive CardioVascular and Thoracic Surgery
Figure 1: Coronary angiography showing stenosis of the right coronary artery (A) and the second marginal branch of the left circumflex artery (B, white arrow). The left anterior descending artery appears to have a chronic total occlusion with collateral filling (B and C, red arrows).
Figure 2: (A) Computed coronary tomography (CT) showing an anomalous isolated origin of the left anterior descending artery from the pulmonary artery. (B) Intraoperative findings confirmed the origin of left anterior descending artery from pulmonary artery (white arrow). The aorta is cannulated. (C) After surgery, cardiac CT showed graft patency (white arrow). Ao: aorta; LAD: left anterior descending; PA: pulmonary artery.
ischaemia leading to ventricular arrhythmia, syncope, dyspnoea, angina or heart failure [4]. CT angiography can detect the ALADAPA. The usual treatment comprises surgical correction by CABG or rerouting through an aorto-pulmonary window (Takeuchi procedure). Here, CABG was required because of the combination of atherosclerotic stenosis and ALADAPA. The longterm outcome after revascularization is excellent [5]. We report an original case of coronary artery disease revealed by silent myocardial ischaemia resulting from an association of ALADAPA and atherosclerosis, with a late manifestation (patient aged over 60 years) and treated by CABG. To date, at the 1-year follow-up, the patient remains asymptomatic without residual myocardial ischaemia. Conflict of interest: none declared.
REFERENCES [1] Krexi L, Sheppard MN. Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), a forgotten congenital cause of sudden death in the adult. Cardiovasc Pathol 2013;22:294–7. [2] Wesselhoeft H, Fawcett JS, Johnson AL. Anomalous origin of the left coronary artery from the pulmonary trunk. Its clinical spectrum, pathology, and pathophysiology, based on a review of 140 cases with seven further cases. Circulation 1968;38:403–25. [3] Frapier J-M, Leclercq F, Bodino M, Chaptal P-A. Malignant ventricular arrhythmias revealing anomalous origin of the left coronary
artery from the pulmonary artery in two adults. Eur J Cardiothorac Surg 1999;15:539–41. [4] Safaa AM, Du LL, Batra R, Essack N. A rare case of adult type ALCAPA syndrome: presentation, diagnosis and management. Heart Lung Circ 2013;22: 444–6. [5] Cochrane AD, Coleman DM, Davis AM, Brizard CP, Wolfe R, Karl TR. Excellent long-term functional outcome after an operation for anomalous left coronary artery from the pulmonary artery. J Thorac Cardiovasc Surg 1999;117:332–42.
eComment. Combined surgical strategies for anomalous connection of coronary artery to pulmonary artery in adults Authors: Arda Ozyuksel, Cihangir Ersoy, Tijen Alkan Bozkaya and Atif Akcevin Department of Cardiovascular Surgery, Medipol University, Istanbul, Turkey doi: 10.1093/icvts/ivu412 © The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved. We read with great interest the article by Gurbuz et al. [1]. We congratulate them on their successful effort in the surgical treatment of such a rare case, presenting with both carotid artery stenosis and coronary artery disease accompanied by an anomalous origin of the left coronary artery from pulmonary artery (ALCAPA). In fact, the traditionally-named ALCAPA leads to a left-to-right shunting into pulmonary arteries, resulting in ventricular ischaemia [2]. Although the terminology defines an origin of left anterior descending artery (LAD) from pulmonary artery, the actual flow direction of the blood is from the coronary circulation to main pulmonary artery. This reversed coronary flow leads to a coronary steal phenomenon. In general, in such cases, re-establishment of a dual coronary circulation is the preferred treatment modality [2].
Interactive CardioVascular and Thoracic Surgery 20 (2015) 283–285 doi:10.1093/icvts/ivu347 Advance Access publication 6 November 2014
Asymptomatic anomalous origin of left anterior descending artery from the pulmonary artery and multiple atherosclerotic stenoses revealed by silent ischaemia Gurbuz Murata, François Cellierb, Bertand Leobonc and Nicolas Boudoua,* a b c
Department of Cardiology, Rangueil University Hospital, Toulouse, France Department of Cardiology, Henri Mondor Hospital, Aurillac, France Department of Cardiothoracic Surgery, Rangueil University Hospital, Toulouse, France
* Corresponding author. Department of Cardiology, Rangueil University Hospital, 1 avenue Jean Poulhès, 31059 Toulouse, France. Tel: +33-5-61323310; fax: +33-5-61323325; e-mail: [email protected] (N. Boudou). Received 17 July 2014; received in revised form 18 September 2014; accepted 22 September 2014
Abstract Anomalous origin of the left coronary artery from the pulmonary artery is a rare congenital disease responsible for a high mortality rate in childhood. Here, we report for the first time the case of an asymptomatic 61-year old patient showing a combination of anomalous origin of the left anterior descending artery from pulmonary artery and atherosclerotic stenosis in both the right coronary artery and the left circumflex artery without anomalous origin. Keywords: Anomalous origin of the left coronary artery from the pulmonary artery • Anomalous origin of the left anterior descending artery from pulmonary artery • Atherosclerosis • Congenital heart disease • Coronary bypass
INTRODUCTION Bland–Garland–White syndrome, or anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) syndrome, is a rare congenital heart disease with an incidence of 1 in 300 000 live births and found in 0.5% of children with congenital heart disease [1]. There is usually no manifestation before birth because systemic and pulmonary artery pressures are equal. Clinical presentation depends on coronary artery topography. In cases with a dominant left coronary artery, ischaemic congestive heart failure or mitral insufficiency by annular dilatation reveals this disease early. Without treatment, the mortality rate is very high and death occurs during childhood [2]. When the right coronary artery (RCA) is dominant, sudden death of up to 90% of patients is observed at a mean age of 35 years and results from myocardial ischaemia, heart failure or malignant arrhythmia [3]. Rarely, this disease can be revealed at a later stage. We report here an original case of an anomalous origin of left anterior descending artery from the pulmonary artery (ALADAPA) associated with atherosclerotic stenosis in the RCA and the left circumflex artery (LCX) revealed by diffuse silent ischaemia before carotid endarterectomy.
Carotid ultrasound revealed a severe stenosis of the left internal carotid, requiring surgical treatment. The patient underwent a preoperative cardiological evaluation. He did not report any symptom before the stroke and was not taking any medication. Transthoracic echocardiography was normal. Myocardial SPECT revealed five anterior ischaemic segments and the coronary angiography showed severe stenosis of the RCA and the second marginal of the LCX (Fig. 1A and B, white arrows). The left anterior descending artery (LAD) appeared as a chronic total occlusion with collateral filling [Fig. 1B (red arrow) and C], and seemed to arise from the pulmonary artery. Coronary computed tomography (CT) showed an isolated anomalous origin of the LAD from the pulmonary artery without any atherosclerotic stenosis (Fig. 2A). The heart team’s strategy was to treat the left carotid stenosis first and then to perform myocardial revascularization by coronary artery bypass graft (CABG). In accordance with the latest guidelines, CABG was performed a few weeks following the left carotid endarterectomy, with the left mammary artery to the LAD, right mammary artery to the second posterolateral branch and the saphenous vein to the posterior descending artery. Intra-operative findings confirmed origin of LAD from pulmonary artery (Fig. 2B). The left ventricular ejection fraction remained normal and graft patency was assessed by cardiac CT within days following the surgical procedure (Fig. 2C).
CASE DESCRIPTION The clinical presentation of the ALADAPA syndrome in the adult population varies. Symptoms are related to chronic myocardial
© The Author 2014. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.
CASE REPORT
DISCUSSION A 61-year old patient was admitted to the neurology department for acute ischaemic stroke. His cardiovascular risk factors were type II diabetes mellitus, smoking, arterial hypertension and obesity.
284
G. Murat et al. / Interactive CardioVascular and Thoracic Surgery
Figure 1: Coronary angiography showing stenosis of the right coronary artery (A) and the second marginal branch of the left circumflex artery (B, white arrow). The left anterior descending artery appears to have a chronic total occlusion with collateral filling (B and C, red arrows).
Figure 2: (A) Computed coronary tomography (CT) showing an anomalous isolated origin of the left anterior descending artery from the pulmonary artery. (B) Intraoperative findings confirmed the origin of left anterior descending artery from pulmonary artery (white arrow). The aorta is cannulated. (C) After surgery, cardiac CT showed graft patency (white arrow). Ao: aorta; LAD: left anterior descending; PA: pulmonary artery.
ischaemia leading to ventricular arrhythmia, syncope, dyspnoea, angina or heart failure [4]. CT angiography can detect the ALADAPA. The usual treatment comprises surgical correction by CABG or rerouting through an aorto-pulmonary window (Takeuchi procedure). Here, CABG was required because of the combination of atherosclerotic stenosis and ALADAPA. The longterm outcome after revascularization is excellent [5]. We report an original case of coronary artery disease revealed by silent myocardial ischaemia resulting from an association of ALADAPA and atherosclerosis, with a late manifestation (patient aged over 60 years) and treated by CABG. To date, at the 1-year follow-up, the patient remains asymptomatic without residual myocardial ischaemia. Conflict of interest: none declared.
REFERENCES [1] Krexi L, Sheppard MN. Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), a forgotten congenital cause of sudden death in the adult. Cardiovasc Pathol 2013;22:294–7. [2] Wesselhoeft H, Fawcett JS, Johnson AL. Anomalous origin of the left coronary artery from the pulmonary trunk. Its clinical spectrum, pathology, and pathophysiology, based on a review of 140 cases with seven further cases. Circulation 1968;38:403–25. [3] Frapier J-M, Leclercq F, Bodino M, Chaptal P-A. Malignant ventricular arrhythmias revealing anomalous origin of the left coronary
artery from the pulmonary artery in two adults. Eur J Cardiothorac Surg 1999;15:539–41. [4] Safaa AM, Du LL, Batra R, Essack N. A rare case of adult type ALCAPA syndrome: presentation, diagnosis and management. Heart Lung Circ 2013;22: 444–6. [5] Cochrane AD, Coleman DM, Davis AM, Brizard CP, Wolfe R, Karl TR. Excellent long-term functional outcome after an operation for anomalous left coronary artery from the pulmonary artery. J Thorac Cardiovasc Surg 1999;117:332–42.
eComment. Combined surgical strategies for anomalous connection of coronary artery to pulmonary artery in adults Authors: Arda Ozyuksel, Cihangir Ersoy, Tijen Alkan Bozkaya and Atif Akcevin Department of Cardiovascular Surgery, Medipol University, Istanbul, Turkey doi: 10.1093/icvts/ivu412 © The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved. We read with great interest the article by Gurbuz et al. [1]. We congratulate them on their successful effort in the surgical treatment of such a rare case, presenting with both carotid artery stenosis and coronary artery disease accompanied by an anomalous origin of the left coronary artery from pulmonary artery (ALCAPA). In fact, the traditionally-named ALCAPA leads to a left-to-right shunting into pulmonary arteries, resulting in ventricular ischaemia [2]. Although the terminology defines an origin of left anterior descending artery (LAD) from pulmonary artery, the actual flow direction of the blood is from the coronary circulation to main pulmonary artery. This reversed coronary flow leads to a coronary steal phenomenon. In general, in such cases, re-establishment of a dual coronary circulation is the preferred treatment modality [2].
