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 Table of Contents  
Year : 2016  |  Volume : 2  |  Issue : 1  |  Page : 112-114

Hypoplastic left anterior descending coronary artery as the cause of inducible myocardial ischemia in a 46-year-old man

Department of Cardiology, St. Luke's University Health Network, Bethlehem, Pennsylvania, USA

Date of Submission14-Oct-2015
Date of Acceptance03-Nov-2015
Date of Web Publication2-Jun-2016

Correspondence Address:
Jamshid Shirani
Department of Cardiology, St. Luke's University Health Network, 801 Ostrum Street, Bethlehem, Pennsylvania 18015
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2455-5568.183319

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Hypoplasia of the left anterior descending (LAD) coronary artery in the absence of other congenital cardiac anomalies is quite rare and presents a real therapeutic challenge. Few cases of this type coronary anomaly have been reported in children and young adults. We present a middle-aged man with clinical, electrocardiographic, and dobutamine stress echocardiographic evidence of myocardial ischemia caused by a hypoplastic distal LAD coronary artery originating from a septal perforator. Diagnostic and therapeutic challenges posed by this rare condition are discussed.
The following core competencies are addressed in this article: Medical knowledge, Patient care

Keywords: Angiography, anomalous coronary artery, congenital heart disease in the adult

How to cite this article:
Acharya Y, Chebrolu LH, Shirani J. Hypoplastic left anterior descending coronary artery as the cause of inducible myocardial ischemia in a 46-year-old man. Int J Acad Med 2016;2:112-4

How to cite this URL:
Acharya Y, Chebrolu LH, Shirani J. Hypoplastic left anterior descending coronary artery as the cause of inducible myocardial ischemia in a 46-year-old man. Int J Acad Med [serial online] 2016 [cited 2022 Nov 29];2:112-4. Available from: https://www.ijam-web.org/text.asp?2016/2/1/112/183319

  Introduction Top

Hypoplasia of the coronary arteries, defined as either shorter than the normal length or smaller than the normal diameter of a major epicardial coronary artery, is an uncommon anomaly and has predominantly involved the right and left circumflex coronary arteries.[1] Although unexplained myocardial scar and sudden death have been reported in association with hypoplastic right and left circumflex coronary arteries, a relationship between this type anomaly and clinical evidence of myocardial ischemia has not been firmly established. Hypoplasia of the left anterior descending (LAD) coronary artery has been rarely reported as an isolated coronary anomaly.[2],[3],[4] Hereby, an unusual case of hypoplastic distal LAD is reported in an adult who presented with clinical evidence of exertional and inducible myocardial ischemia.

  Case Report Top

A 46-year-old African American man with a clinical history of asthma, bipolar disorder and epilepsy was admitted for evaluation of recurrent and progressive chest pain. Admission electrocardiogram showed sinus bradycardia, incomplete right bundle branch block, and no ischemic changes. Cardiac biomarkers were normal. Resting echocardiogram showed normal left ventricular size, systolic function and segmental wall motion. During dobutamine stress echocardiography and at a heart rate of 90 beats/min, ST segment depression was noted in multiple electrocardiographic leads [Figure 1] and was associated with frequent ventricular ectopy [Figure 1], chest pain, and anteroseptal as well as apical left ventricular wall motion abnormality [Figure 2]. Invasive coronary angiography showed no significant obstructive disease. However, the distal portion of the LAD coronary artery originated deep within the ventricular septum from a large septal perforator, was hypoplastic (<1 mm in diameter) and coursed within the anterior interventricular sulcus to reach the apex [Figure 3]. Interestingly, the distal LAD coronary artery had no septal perforator branches.
Figure 1: Serial electrocardiographic tracings obtained at rest (a) and at peak (b and c) as well as immediate recovery period (d) showing ST segment depression at peak (b) and appearance of multifocal and consecutive ventricular premature beats (c and d) during dobutamine stress echocardiography

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Figure 2: Representative end-systolic single frame images of the left ventricle at baseline (a) and at peak stress (b) during dobutamine stress echocardiography demonstrating left ventricular cavity dilation due to inducible anteroseptal and apical ischemia

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Figure 3: Anteroposterior (a) and left anterior oblique (b) projections of the left coronary artery during selective angiography demonstrating the origin of the hypoplastic distal left anterior descending coronary artery from the septal perforator with initial intramuscular course before reaching the interventricular sulcus superficially. The reference catheter is 2 mm in diameter. Most of the anterior ventricular septum is supplied by the solitary septal perforator originating from the proximal left anterior descending. D: Diagonal, LC: Left circumflex, LM: Left main

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  Discussion Top

The coronary anomaly described in this report is unique and presents evidence for the clinical relevance of coronary artery hypoplasia as an etiology for myocardial ischemia in an adult patient. Hypoplastic LAD coronary artery, defined as lumen diameter <1.5 mm, has been reported at necropsy in seven adults (26–48-year-old) who died suddenly and unexpectedly.[2] In three of the latter seven no other potential explanation for sudden death was found. Myocardial ischemia and sudden death have been also reported in a 15-year-old boy with known hypoplastic LAD coronary artery since birth.[3] More recently, anteroapical left ventricular infarct has been reported in a 20-year-old man in association with a hypoplastic LAD coronary artery.[4] To the best of our knowledge, this is the first reported case of inducible myocardial ischemia during pharmacologic stress echocardiography in an adult patient with congenital hypoplasia of the coronary artery. The prevalence of this condition is estimated to be quite low. As previously mentioned, we have only found few cases of this condition in literature despite more widespread use of noninvasive coronary computed tomography angiography in recent years. Currently, there are no specific guidelines or recommendations regarding treatment of symptomatic coronary artery hypoplasia. As coronary intervention or surgery is not an option, medical treatment and lifestyle modification appear to be logical choices in management. Medical therapy should be aimed at reducing myocardial oxygen consumption by lowering heart rate and reducing systemic vascular resistance. Patients should also be advised to refrain from resistance and strenuous exercise. Our patient had significant improvement in his symptoms on a regimen of beta-blocker and angiotensin converting enzyme inhibitor and after modest weight loss and quitting heavy weight lifting.

  Conclusion Top

This report provides evidence for congenital hypoplasia of a major epicardial coronary artery as an uncommon cause of myocardial ischemia in the adult patient. Management of active ischemia in adult patients with this type of coronary artery anomaly should focus on reducing myocardial oxygen demand.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Roberts WC, Glick BN. Congenital hypoplasia of both right and left circumflex coronary arteries. Am J Cardiol 1992;70:121-3.  Back to cited text no. 1
Zugibe FT, Zugibe FT Jr, Costello JT, Breithaupt MK. Hypoplastic coronary artery disease within the spectrum of sudden unexpected death in young and middle age adults. Am J Forensic Med Pathol 1993;14:276-83.  Back to cited text no. 2
Sim DS, Jeong MH, Choi S, Yoon NS, Yoon HJ, Moon JY, et al. Myocardial infarction in a young man due to a hypoplastic coronary artery. Korean Circ J 2009;39:163-7.  Back to cited text no. 3
Amabile N, Fraisse A, Quilici J. Hypoplastic coronary artery disease: Report of one case. Heart 2005;91:e12.  Back to cited text no. 4


  [Figure 1], [Figure 2], [Figure 3]


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