| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
|
|
|
|||||||
|
|||||||||
Case Report |
1 Department of Radiology, Drexel University College of Medicine, Philadelphia,
PA 19102.
2 Department of Radiology, Hospital of the University of Pennsylvania, 3400
Spruce St., Philadelphia, PA 19104-4283.
3 Department of Medicine, Hospital of the University of Pennsylvania,
Philadelphia, PA 19102.
4 Department of Family Practice, Virtua Memorial Hospital of Burlington County,
Mt. Holly, NJ 08060.
Received July 15, 2004;
accepted after revision September 27, 2004.
Address correspondence to D. A. Torigian
(Drew.Torigian{at}uphs.upenn.edu).
Introduction
 Top Introduction Case ReportDiscussionReferences |
|---|
|
TopIntroductionCase ReportDiscussionReferences |
|---|
Chest radiography showed mild bibasilar atelectasis or scarring. Stress echocardiography/ECG showed mild left atrial enlargement, normal left and right ventricular size, borderline left ventricular hypertrophy, normal biventricular systolic function, mild left ventricular diastolic dysfunction, mild mitral valve regurgitation, and trace to mild tricuspid regurgitation. In addition, a right bundle branch block, nonsustained supraventricular tachycardia during exercise, a hypertensive blood pressure response to exercise, transient 1-mm horizontal downsloping ST depression just meeting borderline criteria in the V4–V6, II, III, and aVF leads during exercise recovery, and moderate–severe hypokinesis of the distal septum during exercise recovery were noted, although the patient did not experience chest pain during the study.
Subsequent cardiac catheterization revealed an ejection fraction of 55%, normal left ventriculography, a patent, large-caliber right coronary artery (RCA); anomalous origin of the left main coronary artery (LMCA) from the right coronary sinus, a large-caliber left anterior descending artery (LAD) with functional occlusion in its proximal portion and reconstitution by left-left collaterals, and a large-caliber patent left circumflex artery (LCx).
Subsequent contrast-enhanced CT of the chest was performed, showing an enlarged LMCA that originated from the posterior aspect of the main pulmonary artery (PA) along with a markedly dilated and tortuous LAD (Fig. 1A) and an enlarged tortuous RCA that originated orthotopically from the right coronary sinus of the aortic root (Fig. 1B), in keeping with ALCAPA. The remainder of the coronary arterial system appeared markedly dilated and tortuous (Fig. 1B). Confirmatory cardiac MRI revealed similar anatomic findings (Figs. 1C, 1D, 1E, 1F, 1G, 1H). In addition, dynamic gadolinium-enhanced images showed late enhancement of ALCAPA relative to the PA (Figs. 1E and 1F). Myocardial contractility and thickness of the ventricles appeared normal.
|
|
|
|
|
|
|
|
|
TopIntroductionCase ReportDiscussionReferences |
|---|
Symptoms usually occur in infants after they are 1–2 months old because of left-to-right shunting from the higher pressure left coronary arterial system to the lower pressure pulmonary arterial system. This most often results in death due to circulatory insufficiency from left ventricular dysfunction or mitral valve incompetence, myocardial infarction, or life-threatening cardiac dysrhythmias. Before they are 1 month old, however, physiologic pulmonary arterial hypertension tends to preserve antegrade blood flow within the left coronary artery (LCA), accounting for the usual lack of symptomatology in this age group [4]. Without treatment, approximately 90% of infants die within the first year of life [5]. Rarely, however, they survive into adulthood with clinical presentations varying from symptomatic chronic mitral insufficiency or global ischemic cardiomyopathy to little or no symptomatology. Furthermore, the risk for sudden cardiac death due to ischemic malignant ventricular dysrhythmias exists even in asymptomatic adult patients [5, 6]. Factors that may lead to survival beyond infancy include the development of abundant intercoronary collateral arteries, an alteration in hemodynamics that encourages antegrade blood flow into the left coronary arterial tree, and a reduction in the area of the left ventricular myocardium supplied by the LCA [5].
In general, once the diagnosis of ALCAPA is established, early surgical repair is undertaken to prevent potential future complications. Surgical repair procedures that can be implemented include aortocoronary saphenous vein or internal mammary artery grafting, aortic root reimplantation with or without a pulmonary flap, or intrapulmonary baffling depending on the anatomic location of the ostium of ALCAPA [6]. In the present case, surgical intervention was not performed and the patient has remained asymptomatic for the last 3 years since initial diagnosis with conservative management.
Conventional angiography can detect and depict the course of anomalous coronary arteries, although this sometimes can be difficult to perform or interpret, as in the present case. For example, the LMCA that was reported to arise from the right coronary sinus on the conventional angiographic study likely represented an enlarged tortuous collateral arterial branch of the RCA. MRI is highly accurate in the detection of and delineation of the proximal course of anomalous coronary arteries, and CT may also accurately depict the origin and proximal course of anomalous coronary arteries [7–10].
On CT and MRI, previously described imaging findings of ALCAPA in adults include direct visualization of the origin of the LMCA from the posterior aspect of the PA, dilatation of the RCA, and visualization of dilated intercoronary collateral arteries along the external surface of the heart or within the interventricular septum [7, 11]. Other differential diagnostic considerations for dilatation of the coronary arteries include Kawasaki's disease, coronary arterial fistulas, atherosclerosis, vasculitis (polyarteritis nodosa or Takayasu's arteritis), scleroderma, Ehlers-Danlos syndrome, hereditary hemorrhagic telangiectasia, trauma, and hyperlipidemia [12, 13]. In the present case, anomalous origin of a dilated and tortuous LMCA from the posterior aspect of the main PA, along with dilatation and tortuosity of the RCA and external intercoronary collateral arteries, were well shown on enhanced CT and MRI and were diagnostic for ALCAPA. In addition, dynamically enhanced MR images showed a lack of early enhancement of ALCAPA followed by late enhancement, in keeping with retrograde flow of blood from the RCA to ALCAPA via intercoronary collateral arteries, which to our knowledge has not been reported on MRI. Furthermore, the age of clinical presentation of ALCAPA in the present case is to our knowledge the oldest reported age in adults.
|
TopIntroductionCase ReportDiscussionReferences |
|---|
CiteULike 
Complore 
Connotea 
Del.icio.us 
Digg 
Reddit 
Technorati What's this?
This article has been cited by other articles:
|
|
 |
|
  E. Pena, E. T. Nguyen, N. Merchant, and C. Dennie ALCAPA Syndrome: Not Just a Pediatric Disease RadioGraphics, March 1, 2009; 29(2): 553 - 565. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 A. Korach, P. Menon, M. Dhadly, K. E. Raby, R. J. Shemin, and O. M. Shapira Anomalous Left Main Coronary Artery From the Main Pulmonary Artery in an Elderly Patient Ann. Thorac. Surg., December 1, 2006; 82(6): 2285 - 2287. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 B. Mesurolle, S. D. Qanadli, F. Mignon, and P. Lacombe Anomalous Origin of the Left Coronary Artery Arising From the Pulmonary Trunk Am. J. Roentgenol., April 1, 2006; 186(4): 1202 - 1202. [Full Text] [PDF]
|
|
|
|
|
|
D. A. Torigian Reply Am. J. Roentgenol., April 1, 2006; 186(4): 1202 - 1202. [Full Text] [PDF]
|
|
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
