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A WANDERING CROWN

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

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  1. A WANDERING CROWN ? Rami Khouzam, MD

  2. 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

  3. + 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

  4. Medications: • SL NTG (recently started) • ASA & CaCO3 • Family Hx: • No h / o heart disease • + for HTN, DM • Social Hx: • Remote tobacco user • Denies drugs

  5. 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

  6. Labs: Normal • CXR: Normal • EKG: NSR; flattened T’s in III, aVF and V3-V6

  7. EKG

  8. Echocardiogram • Normal chamber size & dimension • Normal LV Systolic fn. EF: 55-60% • Mild concentric LVH • Trace MR, Mild TR • Normal IVC • No pulmonary HTN

  9. Cardiac catheterization • L Main: Normal • LAD: Normal • L Cx: Normal • RCA: Unsuccessful engagement. Aortic root angiogram: probable anomalous origin from left coronary cusp

  10. Cath

  11. ETT • Exercise time: 7:06 min, Mets: 8.7 • Chest Pain • 1 mm inferolateral ST depression • Clinically & EKG’ly positive

  12. Anatomy of Normal Coronary Arteries • Anomalous origin of Coronary Arteries • Embryology • Pathophysiologic mechanisms • Clinical presentation • Diagnostic modalities • Treatment

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

  14. Corona: (latin) A crown or something like a crown

  15. In Anatomy • “The Upper part of tooth” • “A Skull”

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

  17. In Botany • “The cuplike part of the inner side of the corolla of certain flowers”

  18. In Electricity • “A sometime visible electric discharge around a conductor at high potential”

  19. In my fellowship

  20. And the MOST famous Corona of them all...

  21. 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

  22. 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

  23. 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

  24. 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

  25. 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

  26. 3- Both coronary arteries from right sinus of Valsalva: 0.06-0.19 % • 4- Single coronary artery: 0.06 % • 5- LAD from RCA or right sinus of Valsalva: first reported in 1966 by Jokl et al.

  27. Course is important • - Anterior to pulmonary trunk • - Posterior to aorta • - Within intraventricular septum • - Between aorta & pulmonary trunk

  28. Development of the coronary vessel system Circ. Res. 2002;91:761-768 David E. Reese

  29. Not everyone has coronary vessels • 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

  30. In fish (the last vertebrate class), the presence of coronary vessels is variable • Larger fast-swimming fish have extensive coronary vessels

  31. Most other vertebrates; mammals, reptiles and avians have complete coronary systems • Dependance on pulmonic respiration and the lack of cutaneous respiration

  32. 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

  33. Development of the coronary vessels • 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

  34. Origin of Cells that make up the Heart • Cardiac myocytes • Endocardial endothelium • Fibroblasts • Vascular smooth muscle • Vascular endothelium

  35. 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

  36. The initial phases of coronary vasculogenesis proceed in the absence of blood flow  caliber governed by forces other than blood flow • The final connection to the Aorta involves local apoptotic events that eventually lead to the melding of coronary endothelia with that of the aorta (Unique)

  37. The origins of the coronary arteries to the aorta and the connection of the coronary sinus to the right atrium occur in different regions of the heart

  38. How do these 2 vessel systems run parallel on the surface of the heart but diverge to make connections to the systemic circulation? • Considering the acrobatic nature of this developmental system, it should not be surprising that errors occur

  39. Cellular precursors of the 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

  40. Vascular endothelial growth factor (VEGF) • Both VEGFR-2 and R-3 may play a role in the formation of the coronary artery roots Tomanek RJ - Dev Dyn - 01-NOV-2002;225(3):233-40

  41. Intimal preatherosclerosis thickening of the coronary arteries in human fetuses of smoker mothers J Thromb Haemost. 2003 Oct;1(10):2234-8 Matturri L

  42. Preatherosclerotic intimal alterations of the coronary arteries are already detectable in the prenatal period and are significantly associated with maternal cigarette smoking

  43. Anomalous Coronary Artery from the opposite sinus: Pathophysiologic Mechanisms as documented by IVUS The Journal of Invasive Cardiology, Sept 2003 Paolo Angelini, et al.

  44. 1- 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 %

  45. Young athletes  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

  46. As the aorta and pulmonary artery become larger with late childhood, compression obstructs the blood flow in this aberrant coronary • The expected  in aortic compliance with aging could contribute to ACAOS more benign prognosis in older patients

  47. 2- 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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