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A WANDERING CROWN. ?. Rami Khouzam, MD. Case presentation. 56 yo AAF with no significant PMHx 2-3 months h/o episodic substernal chest pressure to L arm Usually related to activity. Resolves with rest or SL NTG. + DOE One episode at night woke her up

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A wandering crown l.jpg

A WANDERING CROWN

?

Rami Khouzam, MD


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Case presentation

  • 56 yo AAF with no significant PMHx

  • 2-3 months h/o episodic substernal chest pressure to L arm

  • Usually related to activity. Resolves with rest or SL NTG


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  • + DOE

  • One episode at night woke her up

  • Has been told in the past that her EKG is normal and that the pain is d.t. anxiety


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  • Medications:

  • SL NTG (recently started)

  • ASA & CaCO3

  • Family Hx:

  • No h / o heart disease

  • + for HTN, DM

  • Social Hx:

  • Remote tobacco user

  • Denies drugs


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PE

  • Neck: No JVD. No bruits

  • CVS: RR, normal S1 & S2; +S4; no S3 , m or r ; PMI non-displaced

  • Lungs: CTA bilaterally

  • Ext: No e, c, c


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  • Labs: Normal

  • CXR: Normal

  • EKG: NSR; flattened T’s in III, aVF and V3-V6



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Echocardiogram

  • Normal chamber size & dimension

  • Normal LV Systolic fn. EF: 55-60%

  • Mild concentric LVH

  • Trace MR, Mild TR

  • Normal IVC

  • No pulmonary HTN


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Cardiac catheterization

  • L Main: Normal

  • LAD: Normal

  • L Cx: Normal

  • RCA: Unsuccessful engagement. Aortic root angiogram: probable anomalous origin from left coronary cusp



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ETT

  • Exercise time: 7:06 min, Mets: 8.7

  • Chest Pain

  • 1 mm inferolateral ST depression

  • Clinically & EKG’ly positive


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Definitions of the word corona in webster s dictionary l.jpg

Definitions of the word “Corona” in Webster’s Dictionary



In anatomy l.jpg
In Anatomy

  • “The Upper part of tooth”

  • “A Skull”


In astronomy l.jpg
In Astronomy

  • “A circle of light around the sun or the moon; the halo around the sun during a total eclipse”


In botany l.jpg
In Botany

  • “The cuplike part of the inner side of the corolla of certain flowers”


In electricity l.jpg
In Electricity

  • “A sometime visible electric discharge around a conductor at high potential”




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Number and size of coronary ostia

  • Normally an individual has 2 or sometimes 3 coronary ostia

  • Conal branch of the RCA may arise separately from the right sinus

  • The Cx or LAD may, on occasion, arise directly from the aortic root/ Absent Lt. Main


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  • Anomalous pulmonary origins of the coronaries (APOC)

  • Anomalous aortic origins of the coronaries (AAOC)

  • Congenital atresia of the left main coronary artery (CALM)

  • Coronary arteriovenous fistulae (CAVF)

  • Coronary artery bridging (CB)

  • Coronary artery aneurysm

  • Coronary stenosis


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Frequency

  • In the US: Coronary arteries anomalies are observed in:

    • 0.3-1.3% of patients undergoing diagnostic coronary angiography

    • 1% of routine autopsy examinations

    • in 4-15% of young people who experience sudden death


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  • Coronary Artery Anomalies

  • A review of more than 10,000 patients from the Clayton Cardiovascular Labs

  • Charles Wilkins, et al. Texas Heart Institute Journal 1988;15:166-73


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  • Aanomalous Origin & Course…

  • Incidence

  • 1- Cx from RCA or right sinus of Valsalva: the most common: 0.48 %

  • 2- Both coronary arteries from left sinus of Valsalva: 0.28 %: reported in 1982 by Roberts et al


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Course is important 0.06-0.19 %

  • - Anterior to pulmonary trunk

  • - Posterior to aorta

  • - Within intraventricular septum

  • - Between aorta & pulmonary trunk


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Development of the coronary vessel system 0.06-0.19 %

Circ. Res. 2002;91:761-768

David E. Reese


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Not everyone has coronary vessels 0.06-0.19 %

  • Not all organisms with a heart have coronary vessels

  • Invertebrates DO NOT have coronaries (seastar, jellyfish, worms…)

  • Among the vertebrates: Most amphibians; newts, salamanders and bullfrogs have no coronary vessels


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  • Complete anatomical separation of left (oxygenated) and right (deoxygenated) sides of the heart 

    • The use of circulating luminal blood to deliver oxygen to the right ventricle is impossible

    • Thick-walled ventricle that can’t be served by simple gas diffusion from the myocardium to the heart lumen

  • An alternative method of delivery, presumably the coronary system was necessitated


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Development of the coronary vessels right (deoxygenated) sides of the heart

  • Complex vasculogenic process that begins after heart looping

  • Coronary vasculogenesis: regulated by myocardium, but also dependent on epicardium and its precursor, the proepicardial organ, for the provision of coronary vascular progenitor cells


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Origin of Cells that make up the Heart right (deoxygenated) sides of the heart

  • Cardiac myocytes

  • Endocardial endothelium

  • Fibroblasts

  • Vascular smooth muscle

  • Vascular endothelium


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The Decision to make Arteries & Veins, Remodeling, and Making the final connection to systemic circulation

  • Several molecules play a role in the diversification of vessels

  • Further growth ; complex process of angiogenesis, vascular remodeling, and arterialization of specific branches gives rise to the definitive coronary system


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Cellular precursors of the the heart but diverge to make connections to the systemic circulation?coronary arteries

  • EPDCs (Epicardial derived cells) might be found to have a competence similar to that shown by the recently discovered bipotential vascular progenitors cells, which are able to differentiate into endothelium or smooth muscle depending on their exposure to VEGF or PDGF-BB

Tex Heart Inst J. 2002; 29 (4): 243-249


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Tomanek RJ - Dev Dyn - 01-NOV-2002;225(3):233-40


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Intimal preatherosclerosis thickening of the coronary arteries in human fetuses of smoker mothers

J Thromb Haemost. 2003 Oct;1(10):2234-8

Matturri L


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Anomalous Coronary Artery from the opposite sinus: Pathophysiologic Mechanisms as documented by IVUS

The Journal of Invasive Cardiology, Sept 2003

Paolo Angelini, et al.


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  • 1- Pathophysiologic Mechanisms as documented by IVUS Tangential origination with a proximal intramural course  lateral compression of the lumen  outward displacement of the inner (more than the outer) layer of the aortic wall.

  • Phasically accentuated during late systole and early diastole.

  • On IVUS: intramural segment: area reduction of 22 % to 69 %


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  • Young athletes Pathophysiologic Mechanisms as documented by IVUS CO from 5 L/min at rest (HR 70, SV 71)

  • to ~ 25 L/min during exercise

  • (HR 200, SV 125)

  •  significantly  aortic wall stress while expanding aortic dimensions


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  • 2- childhood, compression obstructs the blood flow in this aberrant coronary Spasm of ostium and/or proximal ectopic coronary artery (slit-like or hypoplastic ostium)

  • 3- Clot formation

  • 4- Aortopulmonary scissors effect:

  • Influence exerted entirely by aorta ? (pulmonary a. pressure much lower than c.a pressure, & less or not likely to cause coronary luminal collapse)

  • 5- Aortic hinge effect (acute angle of take-off)


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Symptoms & Signs: more vs. )

  • Asymptomatic

  • Symptoms in < 30 %:

  • - Palpitation

  • - DOE

  • - Angina ( in older patients)

  • - Syncope

  • - Fatigue

  • - Sudden death (in younger patients)


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Imaging Studies more vs. )(non-invasive)

  • Transthoracic echocardiography TTE

  • Transesophageal echocardiography TEE

  • Electron beam computed tomography EBCT

  • Multidetector computer tomography MDCT

  • Magnetic resonance imaging MRI


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TEE more vs. )

  • Intermediate course between the aorta & PA:

  • - Predominant systolic flow pattern

  • Anomalous LM coursed anterior to the pulmonary trunk:

  • - Predominant diastolic flow pattern

J Am Soc Echocardiogr 2003;16(12)


The american journal of cardiology volume 87 number 2 jan 15 2001 dieter ropers md germany l.jpg

Visualization of coronary artery anomalies and their anatomic course by contrast-enhanced electron beam tomography and three-dimensional reconstruction

The American Journal of Cardiology

(Volume 87. Number 2. Jan.15, 2001)

Dieter Ropers, MD

Germany


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EBCT anatomic course by contrast-enhanced electron beam tomography and three-dimensional reconstruction

  • Very high temporal resolution

  • No mechanical parts are involved in image acquisition

  • Instead, X-ray are created by an electron beam, which sweeps across fixed tungesten targets arranged in a semicircular manner around the patient

Dieter Ropers,Germany Am J Cardiology 2001;87(2)


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  • One high resolution image acquired in 50 to 100 ms anatomic course by contrast-enhanced electron beam tomography and three-dimensional reconstruction

  • Slice thickness is 1.5 or 3.0 mm, (triggered by the EKG images acquired up to 5 times within one cardiac cycle) 7 to 9 line pairs per cm


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MDCT anatomic course by contrast-enhanced electron beam tomography and three-dimensional reconstruction

  • X-ray gantry rotation time of 500 ms or less

  • Images free of motion artifact

  • Stimultaneous data acquisition in 16 parallel cross-sections with collimations of less than 1 mm

  • 9 line pairs per cm


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Treatment anatomic course by contrast-enhanced electron beam tomography and three-dimensional reconstruction

  • Surgery is the only definitive treatment of coronary artery anomalies


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  • ) RCA from the left sinus of Valsalva anatomic course by contrast-enhanced electron beam tomography and three-dimensional reconstruction

  • Reimplantatoin of the anomalous coronary artery to the ipsilateral sinus of Valsalva

  • Ligation of the anomalous artery and bypass with a saphenous vein graft

  • (RIMA) without ligation of the RCA


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  • Many efforts have been made to non-invasively image the coronary arteries using magnetic resonance, electron beam computed tomography, and recently multidetector computed tomography (MDCT)

  • A new generation of MDCT scanners with arrays of detectors, a higher temporal an spatial resolution


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Monarchies of the World coronary arteries using magnetic resonance, electron beam computed tomography, and recently multidetector computed tomography (MDCT)


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Moral of the Story world

  • Well-deserved Royalty:

  • Not just to have a crown….but to have a well-fitting ONE!


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