This document summarizes the morning report of a 59-year-old man with a history of kidney transplant, SLE, and prior STEMI managed with thrombus extraction. He presented with recurrent chest pain. Key findings include inferolateral wall perfusion defect on nuclear stress test. The report reviews coronary aneurysms/ectasia classification, etiologies including atherosclerosis, Kawasaki disease, inflammatory disorders. The patient's systemic lupus erythematosus and transplant status suggest an inflammatory cause is possible. Evaluation and management of antiplatelet therapy is discussed.

1. Cardiology Division
Morning Report
Michael G. Katz, M.D.
Fellow in Cardiovascular Disease
University of Rochester
December 13, 2010

2. • 59 year old Nepalese man.
• STEMI -2006
– mechanical thrombus
extraction from RCA and POBA
• Recurrent CP 6 months ago
(details on following slides).

3. 2006 – post MI

4. NSR
LAD
Inferior infarct, age indeterminate

5. 6 months ago

6. NSR
LVH
Inferior infarct, old
Anterolateral infarct, age indeterminate (NEW FINDING)

7. PMH
• 1995 – ESRD requiring HD 2/2
mesangioproliferative
glomerulonephritis
• 1996 - deceased donor kidney
transplant
–Cyclosporin based immunosupression

8. • 2003 – Diagnosis of Clinical SLE
– Dermatologic manefestation: pruritic dermatitis,
post-inflammatory hypopigmentation on the back,
thin, erythematous plaques.
– Bx: epidermal atrophy and interface change c/w
CTD. DIF with IgG deposits on epidermal nuclei
also c/w CTD
– ESR 54
– ANA 1:180
– reduced C4
– Anti-RNP, and Anti-Smith POS
– Pancytopenia

9. – Rash had excellent response to topical steroids
and prednisone taper.
– Started on hydroxychloroquine in an effort to
reduce prednisone over time.
– Plan to reduce prednisone hampered by skin
changes / pain in fingers and hands.
– CellCept added 2007
– Essentially stable with waxing and waning skin
symptoms.

10. Review of LHC from 2006 STEMI

11. • February 2010: several episodes of vague
chest discomfort and palpitations. Several ED
visits, where MI was ruled out.
– Holter: occasional PVCs and PACs but no evidence
of VT or sustained arrhythmia
– SPECT nuclear stress:

14. Perfusion Interpretation:
Mild exercise induced perfusion defect of the
inferolateral wall. Stress induced LV cavity
dilation is notably absent. A slight mild resting of
the basal inferolateral wall regions is noted.
Evidence of low normal stress myocardial
perfusion is noted in the anteroseptal region.
Breast attenuation and splanchnic scatter
artifacts noted on the rotational planar images
likely contribute to the scan appearance. The
calculated ischemia index score = 4%.

15. LHC 2010

16. • At his post cath f/u visit:
– Re-started on clopidogrel (which he had not been taking)
– Continued on low-dose ASA
– Started on TUMS and ranitidine (to avoid potential PPI
interaction with clopidogrel) for empiric treatment of
GERD.
• October 2010
– Dramatic improvement in “chest pressure” with antacid.
– Self-discontinued clopidogrel after easy bruising on arms
and hands.
– TC 112 TG 78 HDL 46 LDL 50 TC/HDL 2.4
– CRP 2

17. Meds
• Amlodipine-
Benazepril
• Aspirin 81
• Cellcept
• Cyclosporine
• Hydroxychloroquine
• Lotrel 5/10 mg
• Metoprolol
• mycophenolate
mofetil
• Nitroglycerin
• Pravastatin
• Prednisone
• Ranitidine
• Sildenafil

18. • Brief summary
• DDx

19. Coronary Aneurysms and Ectasia
• Terminology and Classification
• Etiology and pathogeneisis
• Examples

20. Giovanni Morgagni
1761
Syphilitic aortitis

21. Aneuyrsm

22. Ectasia

27. Ectasia
Markis JE. Am J Cardiol 1976;37(2):217

28. Type I

29. Type III

30. Type IV

31. Epidemiology
• 0.5 – 5% prevalence
• More frequent in men than women
– 2.2% vs. 0.02%
• Age predilection varies by etiology

32. Aneurysmal Ectatic
• Kawasaki disease
• Mycotic
• Trauma / iatrogenic
• Cocaine
• Atherosclerosis • Inflammatory disorders
• “Compensatory dilatation”
• Connective tissue
disorders

33. A/E Atherosclerosis
• Most common is western countries (50%)
• Abnormal tunica media  enlargement,
remodeling
• Saccular aneurysms and post-stenotic
dilatation
• Turbulent and reduced flow  endothelial
damage and wall stress
• ? predisposition

34. • Usually multiple and involve more than one
coronary artery
– As opposed to congenital, traumatic, or dissection
• RCA (40%–61%), LAD (15%–32%), and LCx
(15%–23%). LM involvement rare (0.1%–3.5%)

35. Kawasaki Disease
A
• first described in Japan in 1967 by Tomisaku
Kawasaki
• Etiology unknown – thought to be infectious
vs. autoimmune
• Aneurysm or ectasia develops in 15%–25% of
untreated children within 3–6 months
• Cardiac sequelae may develop as many as 10–
21 years after the acute phase of the disease

36. • By 2 years after the onset of Kawasaki disease,
49% of the patients have spontaneous
regression of aneurysms
• LM (12%), RCA (3%), both arteries in 8%

38. Misc.
• Iatrogenic
– Trauma from oversized balloon or high inflation pressures, coronary
dissection; compounded by inadequate healing because of antiproliferative
treatment with cortisone, colchicine, and antiinflammatory drugs
• Mycotic
– Infection with S.A., P.A., syphilis, Lyme disease
– Microembolization to vasa vasorum, direct pathogen invasion of arterial wall,
immune com-
– plex deposition
• Cocaine
– Direct endothelial damage from severe episodic hypertension,
vasoconstriction, and underlying atherosclerosis
A

39. Inflammatory
Disorders
• Takayasu arteritis, SLE, RA, Wegener
granulomatosis, GCA, Churg-Strauss
syndrome, MPA, APS, Behçet syndrome,
sarcoid, PAN, CREST syndrome, AS, Reiter
syndrome, psoriatic arthritis
• Inflammatory mediators: VCAM-1, ICAM-1, E
selectin
E

40. Takayasu arteritis
• large-vessel vasculitis
• young women
• marked thinning of the tunica media
• of the vessel, disruption of the elastic fibers, and
thickening of the tunica adventitia and intima.
• Coronary involvement: 12%
• Signs and symptoms are due to ischemia secondary
to arterial stenosis or occlusion
• Aneurysms and ectatic collateral vessels as a
compensatory mechanism

43. Compensatory
dilatation
• Fistula
– Compensatory dilatation secondary to high-flow
E
state
– originate from the RCA in 52% of cases; LAD 30%,
and LCx 18%.
– drainage is to right chambers, direct volume
overload to the pulmonary vascular bed, the left
atrium, and left ventricle; whereas if drainage to
left cardiac chambers overload spares the
pulmonary vasculature

44. • Coronary Artery Anomalies – eg. anomalous
origin of the left coronary artery from the
pulmonary artery (ALCAPA) syndrome (or
Bland-White-Garland syndrome)
– 1/300,000 live births
– LM from PA  Ectatic RCA (due to elevate
pressures)  R to L collaterals and steal
phenomena in RCA terrirory
E

47. • Connective Tissue Disorders
– Ehlers-Danlos syndrome, Marfan syndrome, cystic
medial necrosis
– IL-6, C-reactive protein MMP-2, MMP-9
E

48. • What is the most likely Dx?
• W/u?
• Antiplatelet therapy?
• Anticoagulation?

54. • Group A –Ectasia and obstructive CAD
• Group B – Ectasia, but no stenosis
• Group C – Obstructive CAD, but no ectasia

55. Group A –Ectasia and obstructive
CAD

56. In patients with ectasia, was it
specifically associated with
stenosis? – No.

57. MI in Group B (Ectasia, but no
stenosis)
• 31 pts
– 12 pts
• 3 were non-QW MI
– 0 had HK on ventriculography
– 2 had culprit vessel localization by EKG
» In both, culprit artery was ectatic
• 9 were QW MI
– In all 9, culprit vessel was ectatic
– In 7, it was the only ectatic vessel

58. Follow-up at 10-14 months
State-of-the-art 1997 therapy: “Most of these patients were on
triple anti-ischaemictreatment (nitrates, B blockers, and calcium
antagonists) and aspirin.”