Welcome to Scribd, the world's digital library. Read, publish, and share books and documents. See more
Download
Standard view
Full view
of .
Save to My Library
Look up keyword
Like this
2Activity
0 of .
Results for:
No results containing your search query
P. 1
alcapa1

alcapa1

Ratings: (0)|Views: 16 |Likes:
Published by qqsham

More info:

Published by: qqsham on Jul 09, 2010
Copyright:Attribution Non-commercial

Availability:

Read on Scribd mobile: iPhone, iPad and Android.
download as PDF, TXT or read online from Scribd
See more
See less

07/22/2013

pdf

text

original

 
326AJR:185, August 2005
AJR 
2005; 185:326–3290361–803X/05/1852–326© American Roentgen Ray Society
Khanna et al.AnomalousOrigin of LeftCoronaryArtery
Cardiovascular ImagingCase Report
Anomalous Origin of theLeftCoronary Artery from thePulmonary Artery inAdulthoodonCT and MRI
Arati Khanna
1
Drew A. Torigian
2
Victor A. Ferrari
3
Robert J. Bross
4
Mark A. Rosen
2
Khanna A, Torigian DA, Ferrari VA, Bross RJ,Rosen MA
Received July 15, 2004; accepted after revisionSeptember27, 2004.
1
Department of Radiology, Drexel University College ofMedicine, Philadelphia, PA 19102.
2
Department of Radiology, Hospital of the University ofPennsylvania, 3400 Spruce St., Philadelphia, PA19104-4283. Address correspondence to D. A. Torigian(Drew.Torigian@uphs.upenn.edu).
3
Department of Medicine, Hospital of the University ofPennsylvania, Philadelphia, PA 19102.
4
Department of Family Practice, Virtua Memorial Hospitalof Burlington County, Mt. Holly, NJ 08060.
e report the clinical, CT, andMRI findings of an intermit-tently symptomatic elderlywoman with the rare congeni-tal syndrome of anomalous origin of the leftcoronary artery from the pulmonary artery(ALCAPA) with marked intercoronary col-lateral arteries and retrograde filling of theleft coronary arterial system. To our knowl-edge, this finding as seen on MRI has notbeen reported in the literature.
Case Report
A 74-year-old woman with a history of hy-pertension, borderline hypercholesterolemia,hypothyroidism, asthma, and a remote historyof near syncopal episodes presented withcough and chronic intermittent nonexertionalchest discomfort that was atypical for angina.No shortness of breath or diaphoresis was re-ported. Physical examination was significantfor mild hypertension but was otherwise un-remarkable.Chest radiography showed mild bibasilaratelectasis or scarring. Stress echocardio-graphy/ECG showed mild left atrial enlarge-ment, normal left and right ventricular size,borderline left ventricular hypertrophy, nor-mal biventricular systolic function, mild leftventricular diastolic dysfunction, mild mitralvalve regurgitation, and trace to mild tricus-pid regurgitation. In addition, a right bundlebranch block, nonsustained supraventriculartachycardia during exercise, a hypertensiveblood pressure response to exercise, transient1-mm horizontal downsloping ST depression just meeting borderline criteria in the V4–V6,II, III, and aVF leads during exercise recov-ery, and moderate–severe hypokinesis of thedistal septum during exercise recovery werenoted, although the patient did not experiencechest pain during the study.Subsequent cardiac catheterization revealedan ejection fraction of 55%, normal left ven-triculography, a patent, large-caliber right cor-onary artery (RCA); anomalous origin of theleft main coronary artery (LMCA) from theright coronary sinus, a large-caliber left ante-rior descending artery (LAD) with functionalocclusion in its proximal portion and reconsti-tution by left-left collaterals, and a large-cali-ber patent left circumflex artery (LCx).Subsequent contrast-enhanced CT of thechest was performed, showing an enlargedLMCA that originated from the posterior as-pect of the main pulmonary artery (PA) alongwith a markedly dilated and tortuous LAD(Fig.1A) and an enlarged tortuous RCA thatoriginated orthotopically from the right coro-nary sinus of the aortic root (Fig.1B), in keep-ing with ALCAPA. The remainder of the coro-nary arterial system appeared markedly dilatedand tortuous (Fig.1B). Confirmatory cardiacMRI revealed similar anatomic findings(Figs.1C–1H). In addition, dynamic gadolin-ium-enhanced images showed late enhance-ment of ALCAPA relative to the PA (Figs.1Eand 1F). Myocardial contractility and thicknessof the ventricles appeared normal.Conservative management rather than sur-gical intervention was implemented, and atclinical follow-up 3years after initial diagno-sis at imaging, the patient did not have newcardiac-related symptomatology.
Discussion
In 1885, Brooks [1] was the first to show thatcoronary arteries may anomalously originatefrom the PA. ALCAPA, otherwise known asBland-White-Garland syndrome, is a rare con-genital defect that accounts for 0.25–0.5% of allcongenital heart defects [2]. It most often pre-sents as an isolated defect, but in 5% of cases itmay be associated with other cardiac anoma-lies, including atrial septal defect, ventricularseptal defect, and aortic coarctation [3].Symptoms usually occur in infants afterthey are 1–2 months old because of left-to-
 W
 
Anomalous Origin of Left Coronary Artery
AJR:185, August 2005327
right shunting from the higher pressure leftcoronary arterial system to the lower pressurepulmonary arterial system.This most often re-sults in death due to circulatory insufficiencyfrom left ventricular dysfunction or mitralvalve incompetence, myocardial infarction,or life-threatening cardiac dysrhythmias. Be-fore they are 1month old, however, physio-logic pulmonary arterial hypertension tendsto preserve antegrade blood flow within theleft coronary artery (LCA), accounting for theusual lack of symptomatology in this agegroup [4]. Without treatment, approximately90% of infants die within the first year of life[5]. Rarely, however, they survive into adult-hood with clinical presentations varying fromsymptomatic chronic mitral insufficiency orglobal ischemic cardiomyopathy to little or
ABCD
Fig.1—
74-year-old woman with cough and chronic intermittent nonexertional chest discomfort atypical for angina.
A,
Axial enhanced CT shows dilated left main coronary artery (LMCA) (
black arrow 
) originating from posterior main pulmonary artery (P) leading to dilated and tortuous leftanterior descending (LAD) artery (
white arrow 
).
B,
Axial enhanced CT at level of aortic root shows enlarged tortuous right coronary artery (RCA) and intercoronary collateral arteries (
arrows 
) along epicardial surface ofheart.
C
and
D,
Confirmatory cardiac MRI shows anomalous origin of left coronary artery from pulmonary artery (ALCAPA). Axial thin-section and oblique coronal maximal-inten-sity-projection (MIP) postgadolinium fat-suppressed T1-weighted gradient-echo images (TR/TE, 150/1.6/flip angle [FA] 90° and TR/TE, 3.8/0.9/FA 20°, respectively) show dilatedLMCA (
black arrow 
,
C
) originating anomalously from posterior main P, leading to dilated and tortuous LAD (
arrows 
).
(Fig.1 continues on next page)
 
Khanna et al.
328AJR:185, August 2005
no symptomatology. Furthermore, the risk forsudden cardiac death due to ischemic malig-nant ventricular dysrhythmias exists even inasymptomatic adult patients [5, 6]. Factorsthat may lead to survival beyond infancy in-clude the development of abundant intercoro-nary collateral arteries, an alteration in hemo-dynamics that encourages antegrade bloodflow into the left coronary arterial tree, and a
EFGH
Fig.1(continued)—
74-year-old woman with cough and chronic intermittent nonexertional chest discomfort atypical for angina.
E
and
F,
Oblique sagittal postgadolinium fat-suppressed T1-weighted gradient-echo MIP images (TR/TE, 3.8/0.9/FA 20°) during early and late phases of enhancement revealearly lack of enhancement of ALCAPA (
arrows 
) during enhancement of P with subsequent late enhancement of ALCAPA, implying retrograde flow of blood within ALCAPAfrom RCA.
G,
Oblique coronal postgadolinium fat-suppressed T1-weighted gradient-echo MIP image (TR/TE, 3.8/0.9/FA 20°) shows orthotopic dilated and tortuous RCA (
arrow 
).
H,
Axial T1-weighted cine gradient-echo image (TR/TE, 36/3.3/FA 30°) through ventricles shows high-signal-intensity flow in enlarged intercoronary collateral arteries (
arrow 
).

You're Reading a Free Preview

Download
/*********** DO NOT ALTER ANYTHING BELOW THIS LINE ! ************/ var s_code=s.t();if(s_code)document.write(s_code)//-->