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Congenital absence of left circumflex coronary artery presented with vasospastic angina and myocardial bridge in single left coronary artery
International Journal of Cardiology 131 (2009) e108 – e111 www.elsevier.com/locate/ijcard
Letter to the Editor
Congenital absence of left circumflex coronary artery presented with vasospastic angina and myocardial bridge in single left coronary artery Young Kyung Yoon, Seung-Woon Rha ⁎, Jin Oh Na, Soon Yong Suh, Cheol Ung Choi, Jin Won Kim, Eung Ju Kim, Chang Gyu Park, Hong Seog Seo, Dong Joo Oh Cardiovascular Center, Korea University Guro Hospital, 80, Guro-dong, Guro-gu, Seoul, 152-703, South Korea Received 11 August 2007; accepted 18 August 2007 Available online 1 November 2007
Abstract Congenital absence of left circumflex coronary artery (LCX) is a very rare congenital anomaly of the coronary circulation, and few cases have been reported in the literature. We report a case of a 48-year-old male who had congenital absence of LCX presented with vasospastic angina. Routine coronary angiography showed congenital absence of the LCX, typical myocardial bridge in the mid left anterior descending coronary artery (LAD) and super-dominant right coronary artery (RCA). The dominant LAD showed a spastic appearance at the baseline coronary angiography and significant spasm was induced by incremental intracoronary acetylcholine (ACh) provocation test at mid LAD where the myocardial bridge site was observed and was extended to the distal LAD. Typical anginal chest pain was documented during the Ach provocation test and the patient was stabilized with conventional anti-anginal medication. Precise morphological and functional evaluation of the anomalous coronary artery is important for selecting the best treatment modality and better prognosis. © 2007 Elsevier Ireland Ltd. All rights reserved. Keywords: Angina pectoris; Congenital; Coronary vessels
1. Introduction Most coronary abnormalities, incidentally diagnosed, are ranged from 0.6% to 1.3% [1]. Although most of the congenital coronary artery anomalies can be found some patients with coronary artery anomalies may present with symptoms such as angina pectoris, acute myocardial infarction (AMI), syncope, cardiac arrhythmias, sudden death, and congestive heart failure [2]. Yamanaka et al. reported that 80% of congenital anomalies of the coronary arteries are benign while 20% are clinically significant [1].
A congenital absence of the left circumflex artery (LCX) is extremely rare with a frequency of only 0.003% in all patients who underwent coronary angiography [1]. In previous case reports of absent LCX, there has been an association with systolic click syndrome, ischemic changes in the zone of hypoperfusion, dilated cardiomyopathy, and AMI [3]. However, there was no reported case associated with typical vasospastic angina and combined myocardial bridge. This case represents a very rare form of absent LCX with super-dominant right coronary artery (RCA) and dominant left anterior descending coronary artery (LAD) presented with vasospastic angina and combined myocardial bridge. 2. Case report
⁎ Corresponding author. Tel.: +82 2 2626 3020; fax: +82 2 864 3062. E-mail address: [email protected] (S.-W. Rha). 0167-5273/$ - see front matter © 2007 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijcard.2007.08.009
A 48-year-old male was presented with typical resting chest pain. Frequently resting chest pain waked him up early
Y.K. Yoon et al. / International Journal of Cardiology 131 (2009) e108–e111
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Fig. 1. Baseline coronary angiogram. Left coronary angiogram in the right anterior oblique (RAO) cranial view (A) and left anterior oblique (LAO) caudal view (B) demonstrating absent circumflex coronary artery. There was a typical focal myocardial bridge in the mid-portion of the left anterior descending artery (LAD) and appears spastic. Right coronary angiogram in RAO cranial view (C) and LAO cranial view (D) demonstrating dominant vessel which gives off large branches supplying the LCX territory including left ventricular posterior-lateral wall.
in the morning. He had a history of hypertension and heavy habitual drinking but denied history of diabetes mellitus, hyperlipidemia and smoking. The initial electrocardiogram (ECG) showed sinus bradycardia (54/minute) and left ventricular hypertrophy without obvious ST-T change. The chest X-ray brought forward no pathologic finding. Echocardiography showed left ventricular hypertrophy only with normal left ventricular function. He subsequently underwent coronary angiography from standard right femoral Judkins technique. A left coronary angiogram showed one artery arose from the left sinus of Valsalva and continued as a single LAD which gave off a large diagonal branch to supply the left ventricular lateral wall. No obvious LCX was demonstrated even after taking several different views with repeated coronary angiograms (Fig. 1. Panel A, B). The angiogram showed the characteristic focal myocardial bridge in the mid LAD, especially clearly demonstrated at the right anterior oblique (RAO) cranial view after the intracoronary nitroglycerine injection.
The RCA was normally originated from the right sinus of Valsalva. It was a super-dominant RCA ascended the posterior atrioventricular free groove beyond the crux. Its large branches were going up to the area of proximal LCX territories such as the left ventricular inferior and posterolateral walls like good collateral circulation from RCA to the LCX (Fig. 1, Panel C, D). No significant fixed stenosis was observed in both LAD and RCA. There was no angiographic evidence of separate ostium of LCX after the aortography. Acetylcholine (ACh) was injected in doses of 50 μg into the left coronary artery for the intracoronary ACh provocation test. Diffuse significant spasm was induced at midportion of the LAD where the myocardial bridge was observed and this was extended to the distal LAD with reproducing typical chest pain and the ST elevation in the ECG (Fig. 2). The patient was diagnosed as having congenital absence of the LCX and characteristic vasospastic angina with myocardial bridge in the LAD. Non-surgical management was primarily considered for this situation and the patient
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Y.K. Yoon et al. / International Journal of Cardiology 131 (2009) e108–e111
Fig. 2. Intracoronary provocation test using acetylcholine (ACh) in doses of 50 μg (A2) into the left coronary artery showed significant diffuse spastic response from mid to distal of the left anterior descending artery (LAD) where the myocardial bridge was observed (A, B) and the spasm was relieved with 200 μg of intracoronary nitroglycerine (N) injection (C, D).
was followed with conventional anti-anginal and antihypertensive medications. He has been stable and asymptomatic for the past 2 years of clinical follow-up. 3. Discussion Congenital absence of the LCX is extremely rare occurring in 4 of 126,595 patients (0.003%) undergoing cardiac catheterization according to the largest study in the literature [1]. The absence of LCX is usually compensated with the large super-dominant RCA, crossing the crus of the heart that ascends to the left atrioventricular groove and perfusing the zone extending to the LCX territories [2]. The dominant LAD with more developed branches also may help the absent LCX territories from this anomaly. Because of this anatomical and functional compensating mechanisms, this condition is generally thought to have a benign outcome unless atherosclerotic coronary artery disease superimposed [4,5]. In our case, considering the typical ischemic resting chest pain, especially during the night and dawn time suggestive of vasospastic angina history, typical myocardial bridge on
coronary angiography and subsequent combined significant diffuse vasospasm induced by ACh provocation test, confirming the characteristic vasospastic angina in single left coronary artery. Absence of effort-induced anginal pain and no inducible ischemia on non-invasive study might be caused from the well-developed super-dominant RCA and relatively LAD without significant atherosclerotic fixed lesions to cover the territories of the absent LCX. Our unpublished preliminary data shows the higher incidence (greater than 70%) of significant ACh-induced endothelial dysfunction in patients with focal or diffuse myocardial bridge and this angiographic milieu of this patient support higher possibility of clinically overt vasospastic angina. Repetitive episodes of severe vasospasm in this compensatory single dominant left coronary artery may induce more significant ischemic insult toward the LCX territories when compared with that of ordinary co-dominant coronary arteries. Although we didn't perform detailed further non-invasive stress tests because of the angiographically insignificant myocardial bridge and confirmed Ach-induced spasm, we should consider non-invasive stress tests including radionuclide scan or stress echocardiography to confirm whether
Y.K. Yoon et al. / International Journal of Cardiology 131 (2009) e108–e111
there are inducible myocardial ischemia due to underlying coronary steal phenomenon through the anomalous coronary artery, myocardial bridge or microvascular dysfunction unless if there are significant ACh-induced vasospasm. Recently a coronary multidetector-row computed tomography (MDCT) can be a useful tool to understand the coronary artery anomalies further in addition to the conventional coronary angiography [6]. Because further detailed information of coronary artery anomalies including the anomalous origin of coronary ostium may not be confirmed only by the conventional coronary angiography although it has been regarded as the gold standard for confirming a variety of coronary artery disease. Despites of the absence of LCX can be considered mostly as a benign condition, detailed angiographic and functional evaluation including ACh provocation test of coronary anomaly may be crucial both for understanding future clinical course such as chronic stable angina, variant angina, myocardial infarction and sudden cardiac death and for deciding best treatment option. Furthermore, sometimes in patients with
e111
atypical chest pain with low conventional coronary risk factors may need screening test for this kind of unexpected coronary artery anomaly with functional impairment. References [1] Yamanaka O, Hobbs RE. Coronary artery anomalies in 126,595 patients undergoing coronary arteriography. Catheter Cardiovasc Diagn 1990;21:28–40. [2] Attila K, Laszlo B, Laszlo R, et al. Epidemiology of congenital coronary artery anomalies. Catheter Cardiovasc Diagn 1997;42:270–5. [3] Hashimoto N, Nagashima J, Miyazu O, et al. Congenital absence of the left circumflex coronary artery associated with acute myocardial infarction: a case report. Circ J 2004;68:91–3. [4] Vijayvergiya R, Kumar Jaswal R. Anomalous left anterior descending absent circumflex and unusual dominant course of right coronary artery: a case report-R1. Int J Cardiol 2005;102:147–8. [5] Lin TC, Lee WS, Kong CW, Chan WL. Congenital absence of the left circumflex coronary artery. Jpn Heart J 2003;44:1015–20. [6] Sato Y, Matsumoto N, Ichikawa M, et al. MDCT detection of congenital absence of the left circumflex artery associated with atherosclerotic coronary artery disease. Int J Cardiol 2006;113:408–9.
Letter to the Editor
Congenital absence of left circumflex coronary artery presented with vasospastic angina and myocardial bridge in single left coronary artery Young Kyung Yoon, Seung-Woon Rha ⁎, Jin Oh Na, Soon Yong Suh, Cheol Ung Choi, Jin Won Kim, Eung Ju Kim, Chang Gyu Park, Hong Seog Seo, Dong Joo Oh Cardiovascular Center, Korea University Guro Hospital, 80, Guro-dong, Guro-gu, Seoul, 152-703, South Korea Received 11 August 2007; accepted 18 August 2007 Available online 1 November 2007
Abstract Congenital absence of left circumflex coronary artery (LCX) is a very rare congenital anomaly of the coronary circulation, and few cases have been reported in the literature. We report a case of a 48-year-old male who had congenital absence of LCX presented with vasospastic angina. Routine coronary angiography showed congenital absence of the LCX, typical myocardial bridge in the mid left anterior descending coronary artery (LAD) and super-dominant right coronary artery (RCA). The dominant LAD showed a spastic appearance at the baseline coronary angiography and significant spasm was induced by incremental intracoronary acetylcholine (ACh) provocation test at mid LAD where the myocardial bridge site was observed and was extended to the distal LAD. Typical anginal chest pain was documented during the Ach provocation test and the patient was stabilized with conventional anti-anginal medication. Precise morphological and functional evaluation of the anomalous coronary artery is important for selecting the best treatment modality and better prognosis. © 2007 Elsevier Ireland Ltd. All rights reserved. Keywords: Angina pectoris; Congenital; Coronary vessels
1. Introduction Most coronary abnormalities, incidentally diagnosed, are ranged from 0.6% to 1.3% [1]. Although most of the congenital coronary artery anomalies can be found some patients with coronary artery anomalies may present with symptoms such as angina pectoris, acute myocardial infarction (AMI), syncope, cardiac arrhythmias, sudden death, and congestive heart failure [2]. Yamanaka et al. reported that 80% of congenital anomalies of the coronary arteries are benign while 20% are clinically significant [1].
A congenital absence of the left circumflex artery (LCX) is extremely rare with a frequency of only 0.003% in all patients who underwent coronary angiography [1]. In previous case reports of absent LCX, there has been an association with systolic click syndrome, ischemic changes in the zone of hypoperfusion, dilated cardiomyopathy, and AMI [3]. However, there was no reported case associated with typical vasospastic angina and combined myocardial bridge. This case represents a very rare form of absent LCX with super-dominant right coronary artery (RCA) and dominant left anterior descending coronary artery (LAD) presented with vasospastic angina and combined myocardial bridge. 2. Case report
⁎ Corresponding author. Tel.: +82 2 2626 3020; fax: +82 2 864 3062. E-mail address: [email protected] (S.-W. Rha). 0167-5273/$ - see front matter © 2007 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijcard.2007.08.009
A 48-year-old male was presented with typical resting chest pain. Frequently resting chest pain waked him up early
Y.K. Yoon et al. / International Journal of Cardiology 131 (2009) e108–e111
e109
Fig. 1. Baseline coronary angiogram. Left coronary angiogram in the right anterior oblique (RAO) cranial view (A) and left anterior oblique (LAO) caudal view (B) demonstrating absent circumflex coronary artery. There was a typical focal myocardial bridge in the mid-portion of the left anterior descending artery (LAD) and appears spastic. Right coronary angiogram in RAO cranial view (C) and LAO cranial view (D) demonstrating dominant vessel which gives off large branches supplying the LCX territory including left ventricular posterior-lateral wall.
in the morning. He had a history of hypertension and heavy habitual drinking but denied history of diabetes mellitus, hyperlipidemia and smoking. The initial electrocardiogram (ECG) showed sinus bradycardia (54/minute) and left ventricular hypertrophy without obvious ST-T change. The chest X-ray brought forward no pathologic finding. Echocardiography showed left ventricular hypertrophy only with normal left ventricular function. He subsequently underwent coronary angiography from standard right femoral Judkins technique. A left coronary angiogram showed one artery arose from the left sinus of Valsalva and continued as a single LAD which gave off a large diagonal branch to supply the left ventricular lateral wall. No obvious LCX was demonstrated even after taking several different views with repeated coronary angiograms (Fig. 1. Panel A, B). The angiogram showed the characteristic focal myocardial bridge in the mid LAD, especially clearly demonstrated at the right anterior oblique (RAO) cranial view after the intracoronary nitroglycerine injection.
The RCA was normally originated from the right sinus of Valsalva. It was a super-dominant RCA ascended the posterior atrioventricular free groove beyond the crux. Its large branches were going up to the area of proximal LCX territories such as the left ventricular inferior and posterolateral walls like good collateral circulation from RCA to the LCX (Fig. 1, Panel C, D). No significant fixed stenosis was observed in both LAD and RCA. There was no angiographic evidence of separate ostium of LCX after the aortography. Acetylcholine (ACh) was injected in doses of 50 μg into the left coronary artery for the intracoronary ACh provocation test. Diffuse significant spasm was induced at midportion of the LAD where the myocardial bridge was observed and this was extended to the distal LAD with reproducing typical chest pain and the ST elevation in the ECG (Fig. 2). The patient was diagnosed as having congenital absence of the LCX and characteristic vasospastic angina with myocardial bridge in the LAD. Non-surgical management was primarily considered for this situation and the patient
e110
Y.K. Yoon et al. / International Journal of Cardiology 131 (2009) e108–e111
Fig. 2. Intracoronary provocation test using acetylcholine (ACh) in doses of 50 μg (A2) into the left coronary artery showed significant diffuse spastic response from mid to distal of the left anterior descending artery (LAD) where the myocardial bridge was observed (A, B) and the spasm was relieved with 200 μg of intracoronary nitroglycerine (N) injection (C, D).
was followed with conventional anti-anginal and antihypertensive medications. He has been stable and asymptomatic for the past 2 years of clinical follow-up. 3. Discussion Congenital absence of the LCX is extremely rare occurring in 4 of 126,595 patients (0.003%) undergoing cardiac catheterization according to the largest study in the literature [1]. The absence of LCX is usually compensated with the large super-dominant RCA, crossing the crus of the heart that ascends to the left atrioventricular groove and perfusing the zone extending to the LCX territories [2]. The dominant LAD with more developed branches also may help the absent LCX territories from this anomaly. Because of this anatomical and functional compensating mechanisms, this condition is generally thought to have a benign outcome unless atherosclerotic coronary artery disease superimposed [4,5]. In our case, considering the typical ischemic resting chest pain, especially during the night and dawn time suggestive of vasospastic angina history, typical myocardial bridge on
coronary angiography and subsequent combined significant diffuse vasospasm induced by ACh provocation test, confirming the characteristic vasospastic angina in single left coronary artery. Absence of effort-induced anginal pain and no inducible ischemia on non-invasive study might be caused from the well-developed super-dominant RCA and relatively LAD without significant atherosclerotic fixed lesions to cover the territories of the absent LCX. Our unpublished preliminary data shows the higher incidence (greater than 70%) of significant ACh-induced endothelial dysfunction in patients with focal or diffuse myocardial bridge and this angiographic milieu of this patient support higher possibility of clinically overt vasospastic angina. Repetitive episodes of severe vasospasm in this compensatory single dominant left coronary artery may induce more significant ischemic insult toward the LCX territories when compared with that of ordinary co-dominant coronary arteries. Although we didn't perform detailed further non-invasive stress tests because of the angiographically insignificant myocardial bridge and confirmed Ach-induced spasm, we should consider non-invasive stress tests including radionuclide scan or stress echocardiography to confirm whether
Y.K. Yoon et al. / International Journal of Cardiology 131 (2009) e108–e111
there are inducible myocardial ischemia due to underlying coronary steal phenomenon through the anomalous coronary artery, myocardial bridge or microvascular dysfunction unless if there are significant ACh-induced vasospasm. Recently a coronary multidetector-row computed tomography (MDCT) can be a useful tool to understand the coronary artery anomalies further in addition to the conventional coronary angiography [6]. Because further detailed information of coronary artery anomalies including the anomalous origin of coronary ostium may not be confirmed only by the conventional coronary angiography although it has been regarded as the gold standard for confirming a variety of coronary artery disease. Despites of the absence of LCX can be considered mostly as a benign condition, detailed angiographic and functional evaluation including ACh provocation test of coronary anomaly may be crucial both for understanding future clinical course such as chronic stable angina, variant angina, myocardial infarction and sudden cardiac death and for deciding best treatment option. Furthermore, sometimes in patients with
e111
atypical chest pain with low conventional coronary risk factors may need screening test for this kind of unexpected coronary artery anomaly with functional impairment. References [1] Yamanaka O, Hobbs RE. Coronary artery anomalies in 126,595 patients undergoing coronary arteriography. Catheter Cardiovasc Diagn 1990;21:28–40. [2] Attila K, Laszlo B, Laszlo R, et al. Epidemiology of congenital coronary artery anomalies. Catheter Cardiovasc Diagn 1997;42:270–5. [3] Hashimoto N, Nagashima J, Miyazu O, et al. Congenital absence of the left circumflex coronary artery associated with acute myocardial infarction: a case report. Circ J 2004;68:91–3. [4] Vijayvergiya R, Kumar Jaswal R. Anomalous left anterior descending absent circumflex and unusual dominant course of right coronary artery: a case report-R1. Int J Cardiol 2005;102:147–8. [5] Lin TC, Lee WS, Kong CW, Chan WL. Congenital absence of the left circumflex coronary artery. Jpn Heart J 2003;44:1015–20. [6] Sato Y, Matsumoto N, Ichikawa M, et al. MDCT detection of congenital absence of the left circumflex artery associated with atherosclerotic coronary artery disease. Int J Cardiol 2006;113:408–9.