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Primary coronary angioplasty in a patient with prior repair of an anomalous left coronary artery arising from the pulmonary artery
Cardiovascular Radiation Medicine 5 (2004) 104 – 107
Primary coronary angioplasty in a patient with prior repair of an anomalous left coronary artery arising from the pulmonary artery BPY Yan a, MC Nguyen a, HM Haqqani a, JL Lefkovits a, N Better a, P Skillington b, LE Grigg a, AE Ajani a,* a
Department of Cardiology, Royal Melbourne Hospital, Parkville, Australia, and Department of Epidemiology and Preventive Medicine, National Health and Medical Research Council, Centre of Clinical Research Excellence in Therapeutics, Monash University, Melbourne, Victoria, Australia b Department of Cardiothoracic Surgery, Royal Melbourne Hospital, Parkville, Australia Received 8 April 2004; accepted 8 April 2004
Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA or Bland-White-Garland syndrome) is a congenital defect that is usually diagnosed in neonates with a 1-year mortality approaching 90% if left untreated [1]. We report on a 56-year-old lady who first came to medical attention in 1993 after presenting with exertional angina. Her cardiac risk factors include hypertension, dyslipidemia (total cholesterol 5.9 mmol/l in 1996), and smoking. Stress perfusion scintigraphy showed inducible anterolateral ischemia. Coronary angiography revealed a giant tortuous dominant right coronary artery (RCA) with extensive collateral filling of the left coronary system up to the left main coronary artery, which drained retrogradely into the pulmonary trunk (Figs. 1 and 2). Left ventriculogram showed moderate anteroapical hypokinesis and no mitral regurgitation. The left coronary artery was reimplanted into the aorta. In 1994 and 1996, coronary angiography showed successful repair, ectatic left and RCA systems, and normal left ventricular function (Fig. 3). The patient remained well until December 2003 when she presented with chest pain and widespread ST depression. Cardiogenic shock rapidly ensued and required high dose inotropic support. She was transferred to the cardiac catheterization laboratory urgently and coronary angiogram revealed the following:
4. RCA was an extremely large vessel with multiple critical stenoses in mid and distal vessel and extensive disease in posterior descending artery (PDA). Coronary angioplasty was undertaken on the LCX artery using a 6 French JL4 guiding catheter. The lesion was crossed with a 0.014 guidewire (Balance Middleweight, Guidant) and predilated with a 4.020-mm balloon at 7 atmosphere (Stormer, Medtronic AVE). The lesion was subsequently stented with a 5.016-mm stent at 12 atmospheres (Express 2, Boston Scientific). Abxicimab (Reopro, Eli Lilly) bolus and infusion were given for extensive thrombus. TIMI III flow was restored (Fig. 5). Creatinine kinase peaked at 1508. Echocardiography showed moderate segmental systolic dysfunction with akinetic posterolateral wall and hypokinetic inferior wall. Ejection fraction was
1. large and ectatic left main coronary artery; 2. tortuous and dilated left anterior descending (LAD) artery with distal 80% stenosis; 3. left circumflex artery (LCX) was totally occluded proximally (Fig. 4); and
* Corresponding author. Tel.: +61-3-934-27000; fax: +61-3-934-72808. E-mail address: [email protected] (A.E. Ajani). 1522-1865/04/$ – see front matter D 2004 Elsevier Inc. All rights reserved. doi:10.1016/j.carrad.2004.04.003
Fig. 1. Coronary angiogram showing anomalous left coronary artery arising from the pulmonary artery (ALCAPA). A large RCA fills the LAD artery and LCX up to left main coronary artery (LMCA) into the pulmonary artery (PA).
B.P.Y. Yan et al. / Cardiovascular Radiation Medicine 5 (2004) 104–107
105
Fig. 2. Coronary angiogram (1993) of large tortuous RCA.
estimated to be 40% with moderate mitral regurgitation. A repeat stress perfusion scintigraphy performed 4 weeks postangioplasty showed extensive reversible ischemia in the LAD artery territory. The patient was well for 2 months. She represented to the emergency department three times with chest pain associated with minimal exertion. Coronary angiography performed in March 2004 revealed the LAD artery distal 80% stenosis was unchanged (Fig. 6). The LCX artery stent was widely patent. The RCA was tortuous and ectatic with diffuse severe distal disease. Angioplasty of the distal LAD artery was performed using a 6F XB 3.5-mm guiding catheter (Cordis). A 0.014 guidewire (Balance Middleweight, Guidant) was used to cross the lesion. The lesion was stented with a
Fig. 3. Coronary angiogram showing repaired left coronary artery reimplanted onto the aorta with dilated proximal LAD artery.
Fig. 4. Coronary angiogram showing occluded proximal LCX.
2.2520-mm Taxus Express stent (Boston Scientific). The stent was postdilated with a 2.515-mm balloon (Stormer, Medtronic AVE) (Fig. 6). Medical therapy has been optimized and the patient has had no recurrent symptoms.
Discussion ALCAPA is a condition rarely diagnosed in adults; however, patients may be asymptomatic due to adequate
Fig. 5. Coronary angiogram showing LCX postangioplasty and stent.
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B.P.Y. Yan et al. / Cardiovascular Radiation Medicine 5 (2004) 104–107
excellent early and late survival as well as long-term functional outcome [4]. The first reported reimplantation of an anomalous LCA from the pulmonary artery into the aorta occurred in 1974 and has since become the preferred operation in patients with ALCAPA [5,6]. Our patient remained stable following surgery until her presentation 10 years later with an acute myocardial infarction and cardiogenic shock. Acute percutaneous intervention for myocardial infarction in repaired ALCAPA has not been reported in the literature. The fact that our patient has such ectatic vessels raises the question of whether anomalous coronary arteries carry an additional risk of atherosclerotic coronary artery disease, over and above conventional risk factors (such as smoking and hypercholesterolemia in our patient). Current evidence suggests a strong relationship between accelerated atherosclerosis and coronary vessel ectasia [7]; however, a relationship between anomalous coronary arteries and coronary artery disease is yet to be established [8,9]. Myocardial perfusion scanning following aortic implantation for ALCAPA often reveals anterolateral reversible ischemia despite normal left ventricular function [10]. This may impact on clinical management since the interpretation of myocardial perfusion scanning may be difficult. Our patient developed ongoing ischemic symptoms following angioplasty and stenting of her LCX with anteroapical wall reversible ischemia on stress sestamibi perfusion scan. She underwent successful stenting to her distal LAD. Percutaneous intervention in anomalous coronary arteries such as repaired ALCAPA poses a new challenge for the interventional community. New technologies such as drugeluting stents are untested in this rare patient subgroup.
References
Fig. 6. Coronary angiogram showing distal LAD artery stenosis preand postintervention.
collateral circulation [2]. The usual presentations in adulthood are myocardial ischemia associated with cardiomyopathy, severe mitral regurgitation, or sudden death [3]. This case demonstrates the challenge of interventional cardiology in patients with anomalous coronary arteries. Anomalous ectatic vessels can often pose technical problems such as adequately sizing stents. Our patient initially presented with symptoms of exertional angina and was diagnosed at coronary angiography. She underwent reimplantation surgery, which carries
[1] 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 – 45. [2] Fierens C, Budts W, Denef B, Van De Werf. A 72 year old woman with ALCAPA. Heart 2000;83:E2. [3] Purut CM, Sabiston DE. Origin of the left coronary artery from the pulmonary artery in older adults. J Thorac Cardiovasc Surg 1991;102:566 – 70. [4] 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. [5] Neches WH, Mathews RA, Park SC, Lennox CC, Zuberbuhler JR, Siewes RD, Bahnson HT. Anomalous origin of the left coronary artery from the pulmonary artery. A new method of surgical repair. Circulation 1974;50:582 – 7. [6] Reul RM, Cooley DA, Hallman GL, Reul GJ. Surgical treatment of coronary artery anomalies: report of a 37 1/2-year experience at the Texas Heart Institute. Texas Heart Inst J 2002;29:299 – 307. [7] Endoh S, Andoh H, Sonoyama K, Furuse Y, Ohtahara A, Kasahara
B.P.Y. Yan et al. / Cardiovascular Radiation Medicine 5 (2004) 104–107 T. Clinical features of coronary ectasia. J Cardiol 2004;43: 45 – 52. [8] Rigatelli G, Gemelli M, Zamboni A, Docali G, Rossi P, Rossi D, Grazio M, Franco G, Rigatelli G. Are coronary artery anomalies an accelerating factor for coronary atherosclerosis development? Angiology 2004;55:29 – 35. [9] Angelini P. Coronary artery anomalies-current clinical issues:
107
definitions, classification, incidence, clinical relevance, and treatment guidelines. Texas Heart Inst J 2002;29:271 – 8. [10] Seguchi M, Nakanishi T, Nakazawa M, Doi S, Momma K, Takao A, Imai Y, Kondoh C, Hiroe M. Myocardial perfusion after aortic implantation for anomalous origin of the left coronary artery from the pulmonary artery. Eur Heart J 1990;11: 213 – 8.
Primary coronary angioplasty in a patient with prior repair of an anomalous left coronary artery arising from the pulmonary artery BPY Yan a, MC Nguyen a, HM Haqqani a, JL Lefkovits a, N Better a, P Skillington b, LE Grigg a, AE Ajani a,* a
Department of Cardiology, Royal Melbourne Hospital, Parkville, Australia, and Department of Epidemiology and Preventive Medicine, National Health and Medical Research Council, Centre of Clinical Research Excellence in Therapeutics, Monash University, Melbourne, Victoria, Australia b Department of Cardiothoracic Surgery, Royal Melbourne Hospital, Parkville, Australia Received 8 April 2004; accepted 8 April 2004
Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA or Bland-White-Garland syndrome) is a congenital defect that is usually diagnosed in neonates with a 1-year mortality approaching 90% if left untreated [1]. We report on a 56-year-old lady who first came to medical attention in 1993 after presenting with exertional angina. Her cardiac risk factors include hypertension, dyslipidemia (total cholesterol 5.9 mmol/l in 1996), and smoking. Stress perfusion scintigraphy showed inducible anterolateral ischemia. Coronary angiography revealed a giant tortuous dominant right coronary artery (RCA) with extensive collateral filling of the left coronary system up to the left main coronary artery, which drained retrogradely into the pulmonary trunk (Figs. 1 and 2). Left ventriculogram showed moderate anteroapical hypokinesis and no mitral regurgitation. The left coronary artery was reimplanted into the aorta. In 1994 and 1996, coronary angiography showed successful repair, ectatic left and RCA systems, and normal left ventricular function (Fig. 3). The patient remained well until December 2003 when she presented with chest pain and widespread ST depression. Cardiogenic shock rapidly ensued and required high dose inotropic support. She was transferred to the cardiac catheterization laboratory urgently and coronary angiogram revealed the following:
4. RCA was an extremely large vessel with multiple critical stenoses in mid and distal vessel and extensive disease in posterior descending artery (PDA). Coronary angioplasty was undertaken on the LCX artery using a 6 French JL4 guiding catheter. The lesion was crossed with a 0.014 guidewire (Balance Middleweight, Guidant) and predilated with a 4.020-mm balloon at 7 atmosphere (Stormer, Medtronic AVE). The lesion was subsequently stented with a 5.016-mm stent at 12 atmospheres (Express 2, Boston Scientific). Abxicimab (Reopro, Eli Lilly) bolus and infusion were given for extensive thrombus. TIMI III flow was restored (Fig. 5). Creatinine kinase peaked at 1508. Echocardiography showed moderate segmental systolic dysfunction with akinetic posterolateral wall and hypokinetic inferior wall. Ejection fraction was
1. large and ectatic left main coronary artery; 2. tortuous and dilated left anterior descending (LAD) artery with distal 80% stenosis; 3. left circumflex artery (LCX) was totally occluded proximally (Fig. 4); and
* Corresponding author. Tel.: +61-3-934-27000; fax: +61-3-934-72808. E-mail address: [email protected] (A.E. Ajani). 1522-1865/04/$ – see front matter D 2004 Elsevier Inc. All rights reserved. doi:10.1016/j.carrad.2004.04.003
Fig. 1. Coronary angiogram showing anomalous left coronary artery arising from the pulmonary artery (ALCAPA). A large RCA fills the LAD artery and LCX up to left main coronary artery (LMCA) into the pulmonary artery (PA).
B.P.Y. Yan et al. / Cardiovascular Radiation Medicine 5 (2004) 104–107
105
Fig. 2. Coronary angiogram (1993) of large tortuous RCA.
estimated to be 40% with moderate mitral regurgitation. A repeat stress perfusion scintigraphy performed 4 weeks postangioplasty showed extensive reversible ischemia in the LAD artery territory. The patient was well for 2 months. She represented to the emergency department three times with chest pain associated with minimal exertion. Coronary angiography performed in March 2004 revealed the LAD artery distal 80% stenosis was unchanged (Fig. 6). The LCX artery stent was widely patent. The RCA was tortuous and ectatic with diffuse severe distal disease. Angioplasty of the distal LAD artery was performed using a 6F XB 3.5-mm guiding catheter (Cordis). A 0.014 guidewire (Balance Middleweight, Guidant) was used to cross the lesion. The lesion was stented with a
Fig. 3. Coronary angiogram showing repaired left coronary artery reimplanted onto the aorta with dilated proximal LAD artery.
Fig. 4. Coronary angiogram showing occluded proximal LCX.
2.2520-mm Taxus Express stent (Boston Scientific). The stent was postdilated with a 2.515-mm balloon (Stormer, Medtronic AVE) (Fig. 6). Medical therapy has been optimized and the patient has had no recurrent symptoms.
Discussion ALCAPA is a condition rarely diagnosed in adults; however, patients may be asymptomatic due to adequate
Fig. 5. Coronary angiogram showing LCX postangioplasty and stent.
106
B.P.Y. Yan et al. / Cardiovascular Radiation Medicine 5 (2004) 104–107
excellent early and late survival as well as long-term functional outcome [4]. The first reported reimplantation of an anomalous LCA from the pulmonary artery into the aorta occurred in 1974 and has since become the preferred operation in patients with ALCAPA [5,6]. Our patient remained stable following surgery until her presentation 10 years later with an acute myocardial infarction and cardiogenic shock. Acute percutaneous intervention for myocardial infarction in repaired ALCAPA has not been reported in the literature. The fact that our patient has such ectatic vessels raises the question of whether anomalous coronary arteries carry an additional risk of atherosclerotic coronary artery disease, over and above conventional risk factors (such as smoking and hypercholesterolemia in our patient). Current evidence suggests a strong relationship between accelerated atherosclerosis and coronary vessel ectasia [7]; however, a relationship between anomalous coronary arteries and coronary artery disease is yet to be established [8,9]. Myocardial perfusion scanning following aortic implantation for ALCAPA often reveals anterolateral reversible ischemia despite normal left ventricular function [10]. This may impact on clinical management since the interpretation of myocardial perfusion scanning may be difficult. Our patient developed ongoing ischemic symptoms following angioplasty and stenting of her LCX with anteroapical wall reversible ischemia on stress sestamibi perfusion scan. She underwent successful stenting to her distal LAD. Percutaneous intervention in anomalous coronary arteries such as repaired ALCAPA poses a new challenge for the interventional community. New technologies such as drugeluting stents are untested in this rare patient subgroup.
References
Fig. 6. Coronary angiogram showing distal LAD artery stenosis preand postintervention.
collateral circulation [2]. The usual presentations in adulthood are myocardial ischemia associated with cardiomyopathy, severe mitral regurgitation, or sudden death [3]. This case demonstrates the challenge of interventional cardiology in patients with anomalous coronary arteries. Anomalous ectatic vessels can often pose technical problems such as adequately sizing stents. Our patient initially presented with symptoms of exertional angina and was diagnosed at coronary angiography. She underwent reimplantation surgery, which carries
[1] 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 – 45. [2] Fierens C, Budts W, Denef B, Van De Werf. A 72 year old woman with ALCAPA. Heart 2000;83:E2. [3] Purut CM, Sabiston DE. Origin of the left coronary artery from the pulmonary artery in older adults. J Thorac Cardiovasc Surg 1991;102:566 – 70. [4] 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. [5] Neches WH, Mathews RA, Park SC, Lennox CC, Zuberbuhler JR, Siewes RD, Bahnson HT. Anomalous origin of the left coronary artery from the pulmonary artery. A new method of surgical repair. Circulation 1974;50:582 – 7. [6] Reul RM, Cooley DA, Hallman GL, Reul GJ. Surgical treatment of coronary artery anomalies: report of a 37 1/2-year experience at the Texas Heart Institute. Texas Heart Inst J 2002;29:299 – 307. [7] Endoh S, Andoh H, Sonoyama K, Furuse Y, Ohtahara A, Kasahara
B.P.Y. Yan et al. / Cardiovascular Radiation Medicine 5 (2004) 104–107 T. Clinical features of coronary ectasia. J Cardiol 2004;43: 45 – 52. [8] Rigatelli G, Gemelli M, Zamboni A, Docali G, Rossi P, Rossi D, Grazio M, Franco G, Rigatelli G. Are coronary artery anomalies an accelerating factor for coronary atherosclerosis development? Angiology 2004;55:29 – 35. [9] Angelini P. Coronary artery anomalies-current clinical issues:
107
definitions, classification, incidence, clinical relevance, and treatment guidelines. Texas Heart Inst J 2002;29:271 – 8. [10] Seguchi M, Nakanishi T, Nakazawa M, Doi S, Momma K, Takao A, Imai Y, Kondoh C, Hiroe M. Myocardial perfusion after aortic implantation for anomalous origin of the left coronary artery from the pulmonary artery. Eur Heart J 1990;11: 213 – 8.