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679Interv. Cardiol. (2016) 8(1), 679–681 ISSN 1755-5302
Interventional
Cardiology
Case Report
10.2217/ica.15.47 © 2016
We report two cases of an unusual accessory
coronary artery arising from the left coronary
cusp and traveling over the superior epicardial
aspect of the left ventricle parallel to the left
main coronary (LM) and the left anterior de-
scending artery (LAD).
Case 1
A 54 year old male suffered an acute
myocardial infarction and underwent
successful percutaneous coronary intervention
(PCI) with placement of a drug eluting
stent (DES) to the LAD. He was discharged
without complications and returned two
weeks later for a staged PCI to the circumflex
artery. Another DES was placed without
event and the patient was discharged. Three
months later he was screened and enrolled
in an FDA approved stem cell trial for post
myocardial infarction. During a planned
angiogram a 5 French XB 3.0 diagnostic
catheter was advanced to the aortic root. In
the left anterior oblique projection attempts
at cannulation of the LM coronary artery
proved difficult and the catheter tip lodged
into a small vessel that arose from the left
cusp. There was no damping of the catheter
pressures and an angiogram was obtained
using a hand injection (Figure 1).
The vessel was small in both size and
distribution and had a noticeable ostial lesion.
Khan-Malek anomaly–two cases
of a never reported accessory
coronary artery
Christina Tan, Hashim Khan,
Mikhail Malek, Richard A
Schatz*
Richard A Schatz, Scripps Clinic, La Jolla,
San Diego CA, USA,
*Author for correspondence:
Tel.: 85855452487
Fax: +82 42 280 8238
RAS1024@aol.com
We report two cases of an unusual accessory coronary artery arising from the left coronary cusp
and traveling over the superior epicardial aspect of the left ventricle parallel to the left main
coronary (LM) and the left anterior descending artery (LAD).
Submitted: 18 August 2016; Accepted: 25 August 2016; Published online: 31 August 2016
Keywords: Coronary artery anomaly  Congenital coronary artery abnormality
Multiple projections and injections showed
this vessel to originate in the left cusp near
the LM and traveling over the high anterior
wall of the left ventricle (Figure 2).
Case 2
A 62 year old male with history of
hypertension presented with unstable angina,
starting 1 day prior to presentation. Initial
troponin was < 0.1 ng/mL and an exercise
nuclear stress test demonstrated reversible
ischemia with associated stress-induced
diffuse ST depressions on the EKG portion
of the study. The patient was then brought to
the cardiac catheterization lab with findings
of significant LM, LAD and circumflex
disease, as well as incidental demonstration
of an accessory coronary artery arising from
the left coronary cusp and parallel to the LAD
(Figure 3).
One injection resulted in enough reflux in
the cusp to fill the LM coronary artery proving
both its separate ostium and its proximity to
the accessory artery (Figure 4). The patient
was ultimately referred for bypass surgery.
Discussion
Anomalous coronaries occur in about 1%
of the population and are usually benign
and discovered as incidental findings during
680 Interv. Cardiol. (2016) 8(1)
Case Report Tan, Khan, Malek & Schatz
routine coronary angiography [1]. In a series of 1950
angiograms, the most common anomaly identified was
a right coronary artery (RCA) arising from two separate
ostia, which occurs in about 1.23% of identified
anomalies, this was followed by an ectopic RCA from
the right cusp in 1.13% and ectopic RCA from the left
cusp in 0.92% [2]. In a series of 543 subjects studied
with CT coronary angiography, the most common
anomalies were absent left main in 3.3% of total
subjects and 2.6% with rotation of the aortic root with
normal coronary origins [3]. Particular anomalies,
such as coronary artery origin from the wrong aortic
sinus and coronary origin from the pulmonary trunk
(“Bland-White-Garland Syndrome”), are associated
with sudden cardiac death and surgical correction has
often been performed in these cases [4].
Accessory arteries are rarely reported and represent
additional arteries distinct from the usual anomalies
classically described. In fact, in a large series representing
screening of over 120,000 coronary angiograms,
accessory arteries are not mentioned once confirming
that they are strikingly rare [1]. CT coronary
angiography may be better to define these unusual
vessels; however the 2 large series to date totaling 1843
patients, accessory arteries are not mentioned [3].
Thus, it is reportable that two nearly identical accessory
Figure 1. Injection of the left cusp reveals the accessory
artery with ostial disease. The arrow points to previously
placed stents in the LAD which shows that the accessory artery
runs parallel to it.
Figure 3. Right anterior oblique projection of the accessory
artery.
Figure 4. Left anterior oblique view of accessory artery
(solid arrow) with contrast injection concurrently filling L
main. The accessory artery (solid arrow) runs parallel to the
LAD (dashed arrow) in its proximal course.
Figure 2. Right anterior oblique view of the accessory artery.
681
arteries were discovered within weeks of each other in
two different patients.
In neither of these cases were the arteries of any
clinical significance despite one having an ostial lesion,
presumably atherosclerotic in origin. Their size and
distribution were nearly identical about 2 mm in
diameter, running parallel to the left main coronary
artery and terminating along the high anterior wall of
the ventricle. It is presumed that they run posterior to
the pulmonary artery as well due to the short distance
travelled and absence of tortuosity.
Due to the striking rarity of this finding we propose
the nomenclature “Khan-Malek Anomaly” after the
trainee who discovered it and in memory of all trainees
whose countless errant catheters passes usually draw
the ire of the attending. Particularly relevant is the fact
that several other experienced operators performed
angiography on one of the patients and did not find this
anomaly making it particular meaningful and fitting it
be named after him. This is one time an errant catheter
pass resulted in a significant finding.
References
1.	 Yamanaka O, Hobbs RE. Coronary Artery Anomalies in 126,
595 Patients Undergoing Coronary Arteriography. Catheter.
Cardiovasc. Diag. 21, 28-40 (1990).
2.	 Angelini P, Villason S, Chan AV, et al. Normal and anomalous
coronary arteries in humans. In: Angelini P ed. Coronary
Artery Anomalies: A Comprehensive Approach. Philadelphia,
PA: Lippincott Williams & Wilkins, 27-150 (1990).
3.	 Cademartiri F, La Grutta L, Malago R, et al. Prevalence
of Anatomical Variants and Coronary Anomalies in 543
Consecutive Patients Studied with 64-slice CT Coronary
Angiography. Eur. Radiol. 18, 781-791 (2008).
4.	 Basso C, Maron, BJ, Corrado D, et al. Clinical Profile of
Congenital Coronary Artery Anomalies with Origin from
the Wrong Aortic Sinus Leading to Sudden Death in Young
Competitive Athletes. J. Am. Coll. Cardiol. 36, 1494-1501
(2000).
Executive summary
•	 Accessory coronary arteries are an exceedingly rare and may be underdiagnosed due to difficulty identifying such
vessels.
•	 In two cases of an accessory coronary artery parallel to the Left anterior descending artery, the prognosis
associated with these findings was benign.
Case ReportKhan-Malek Anomaly–Two Cases of a Never Reported Accessory Coronary Artery

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1755-5302-8-585

  • 1. 679Interv. Cardiol. (2016) 8(1), 679–681 ISSN 1755-5302 Interventional Cardiology Case Report 10.2217/ica.15.47 © 2016 We report two cases of an unusual accessory coronary artery arising from the left coronary cusp and traveling over the superior epicardial aspect of the left ventricle parallel to the left main coronary (LM) and the left anterior de- scending artery (LAD). Case 1 A 54 year old male suffered an acute myocardial infarction and underwent successful percutaneous coronary intervention (PCI) with placement of a drug eluting stent (DES) to the LAD. He was discharged without complications and returned two weeks later for a staged PCI to the circumflex artery. Another DES was placed without event and the patient was discharged. Three months later he was screened and enrolled in an FDA approved stem cell trial for post myocardial infarction. During a planned angiogram a 5 French XB 3.0 diagnostic catheter was advanced to the aortic root. In the left anterior oblique projection attempts at cannulation of the LM coronary artery proved difficult and the catheter tip lodged into a small vessel that arose from the left cusp. There was no damping of the catheter pressures and an angiogram was obtained using a hand injection (Figure 1). The vessel was small in both size and distribution and had a noticeable ostial lesion. Khan-Malek anomaly–two cases of a never reported accessory coronary artery Christina Tan, Hashim Khan, Mikhail Malek, Richard A Schatz* Richard A Schatz, Scripps Clinic, La Jolla, San Diego CA, USA, *Author for correspondence: Tel.: 85855452487 Fax: +82 42 280 8238 RAS1024@aol.com We report two cases of an unusual accessory coronary artery arising from the left coronary cusp and traveling over the superior epicardial aspect of the left ventricle parallel to the left main coronary (LM) and the left anterior descending artery (LAD). Submitted: 18 August 2016; Accepted: 25 August 2016; Published online: 31 August 2016 Keywords: Coronary artery anomaly  Congenital coronary artery abnormality Multiple projections and injections showed this vessel to originate in the left cusp near the LM and traveling over the high anterior wall of the left ventricle (Figure 2). Case 2 A 62 year old male with history of hypertension presented with unstable angina, starting 1 day prior to presentation. Initial troponin was < 0.1 ng/mL and an exercise nuclear stress test demonstrated reversible ischemia with associated stress-induced diffuse ST depressions on the EKG portion of the study. The patient was then brought to the cardiac catheterization lab with findings of significant LM, LAD and circumflex disease, as well as incidental demonstration of an accessory coronary artery arising from the left coronary cusp and parallel to the LAD (Figure 3). One injection resulted in enough reflux in the cusp to fill the LM coronary artery proving both its separate ostium and its proximity to the accessory artery (Figure 4). The patient was ultimately referred for bypass surgery. Discussion Anomalous coronaries occur in about 1% of the population and are usually benign and discovered as incidental findings during
  • 2. 680 Interv. Cardiol. (2016) 8(1) Case Report Tan, Khan, Malek & Schatz routine coronary angiography [1]. In a series of 1950 angiograms, the most common anomaly identified was a right coronary artery (RCA) arising from two separate ostia, which occurs in about 1.23% of identified anomalies, this was followed by an ectopic RCA from the right cusp in 1.13% and ectopic RCA from the left cusp in 0.92% [2]. In a series of 543 subjects studied with CT coronary angiography, the most common anomalies were absent left main in 3.3% of total subjects and 2.6% with rotation of the aortic root with normal coronary origins [3]. Particular anomalies, such as coronary artery origin from the wrong aortic sinus and coronary origin from the pulmonary trunk (“Bland-White-Garland Syndrome”), are associated with sudden cardiac death and surgical correction has often been performed in these cases [4]. Accessory arteries are rarely reported and represent additional arteries distinct from the usual anomalies classically described. In fact, in a large series representing screening of over 120,000 coronary angiograms, accessory arteries are not mentioned once confirming that they are strikingly rare [1]. CT coronary angiography may be better to define these unusual vessels; however the 2 large series to date totaling 1843 patients, accessory arteries are not mentioned [3]. Thus, it is reportable that two nearly identical accessory Figure 1. Injection of the left cusp reveals the accessory artery with ostial disease. The arrow points to previously placed stents in the LAD which shows that the accessory artery runs parallel to it. Figure 3. Right anterior oblique projection of the accessory artery. Figure 4. Left anterior oblique view of accessory artery (solid arrow) with contrast injection concurrently filling L main. The accessory artery (solid arrow) runs parallel to the LAD (dashed arrow) in its proximal course. Figure 2. Right anterior oblique view of the accessory artery.
  • 3. 681 arteries were discovered within weeks of each other in two different patients. In neither of these cases were the arteries of any clinical significance despite one having an ostial lesion, presumably atherosclerotic in origin. Their size and distribution were nearly identical about 2 mm in diameter, running parallel to the left main coronary artery and terminating along the high anterior wall of the ventricle. It is presumed that they run posterior to the pulmonary artery as well due to the short distance travelled and absence of tortuosity. Due to the striking rarity of this finding we propose the nomenclature “Khan-Malek Anomaly” after the trainee who discovered it and in memory of all trainees whose countless errant catheters passes usually draw the ire of the attending. Particularly relevant is the fact that several other experienced operators performed angiography on one of the patients and did not find this anomaly making it particular meaningful and fitting it be named after him. This is one time an errant catheter pass resulted in a significant finding. References 1. Yamanaka O, Hobbs RE. Coronary Artery Anomalies in 126, 595 Patients Undergoing Coronary Arteriography. Catheter. Cardiovasc. Diag. 21, 28-40 (1990). 2. Angelini P, Villason S, Chan AV, et al. Normal and anomalous coronary arteries in humans. In: Angelini P ed. Coronary Artery Anomalies: A Comprehensive Approach. Philadelphia, PA: Lippincott Williams & Wilkins, 27-150 (1990). 3. Cademartiri F, La Grutta L, Malago R, et al. Prevalence of Anatomical Variants and Coronary Anomalies in 543 Consecutive Patients Studied with 64-slice CT Coronary Angiography. Eur. Radiol. 18, 781-791 (2008). 4. Basso C, Maron, BJ, Corrado D, et al. Clinical Profile of Congenital Coronary Artery Anomalies with Origin from the Wrong Aortic Sinus Leading to Sudden Death in Young Competitive Athletes. J. Am. Coll. Cardiol. 36, 1494-1501 (2000). Executive summary • Accessory coronary arteries are an exceedingly rare and may be underdiagnosed due to difficulty identifying such vessels. • In two cases of an accessory coronary artery parallel to the Left anterior descending artery, the prognosis associated with these findings was benign. Case ReportKhan-Malek Anomaly–Two Cases of a Never Reported Accessory Coronary Artery