Resolution of Recurrent GI Bleed Following Alcohol Septal Ablation for

Resolution of Recurrent GI Bleed
Following Alcohol Septal Ablation for
Hypertrophic Obstructive Cardiomyopathy
Oral A. Waldo, MD, Pragnesh P. Parikh, MD, Joseph L. Blackshear, MD.
March 2nd 2013
American College of Physicians
PGY-2 Internal Medicine
©2012 MFMER | slide-1
Learning Objecti
Objective
e
• To understand the recognition, diagnosis,
and management of acquired von
Willebrand syndrome (AVWS) in the
g of Hypertrophic
yp
p
Obstructive
setting
Cardiomyopathy (HOCM)
©2012 MFMER | slide-2
Hi t
History
off presentt Ill
Illness
• A 71 year old female chief complaint of recurrent melena
• Transfusion dependent GI bleed for 4 years baseline hemoglobin 7
mg/dl
• Multiple endoscopies/deep enteroscopies:
• Small bowel arteriovenous malformations (AVM)
• Ablation of four AVMs in the jejunum.
• She ultimately underwent resection of the ileocolonic region, but
continued to require frequent transfusions as often as every 2 to 3
weeks.
• Review of Systems:
• Dyspnea on exertion, chest discomfort. Few presyncopal episodes,
y
p
but no syncope.
©2012 MFMER | slide-3
• Past Medical History
• Mitral regurgitation
• Exploratory laparotomy with partial small bowel
resection
• Hysterectomy
• Repair of left hip fracture
• Social History
• Does not Drink. Smoke 3 cigarettes daily
• Family History
• She had 5 siblings
• 1 sister who died suddenly in her 40s unknown
cause.
• 5 sons and 1 daughter, no history of heart disease
©2012 MFMER | slide-4
Ph i l E
Physical
Exam
•
•
•
•
•
•
•
•
VS: Afebrile BP= 140/72 P=82 RR =18
General: feeble female
female, poor functional status
HEENT: No oral mucosal bleeding
Cardiovascular:
• S1S2,, RRR,, Bifid carotid pulse
p
and bifid apical
p
beat.
• Harsh, 3/6 systolic murmur at left parasternal edge which decreased
with hand grip.
• Occasional extrasystoles, with an increase in the murmur to grade 4
following the extrasystole
Chest: Clear to auscultation
Abdomen: Soft with mild epigastric tenderness
Extremity: Without edema
I t
Integument:
t No
N bruising
b i i or suspicious
i i
llesions
i
©2012 MFMER | slide-5
L b t
Laboratory
fifindings
di
• Hb= 7.3, Plt = 129
• BMP normal
• PT/PTT normal
©2012 MFMER | slide-6
Echocardiogram Showing HCM
©2012 MFMER | slide-7
Peak velocity = 4m/s, Peak gradient = 64mm Hg
“Severe Obstruction”
©2012 MFMER | slide-8
F th Laboratory
Further
L b t
A
Analysis
l i
• Platelet Functional Assayy ((PFA))
• Collagen EPI >300 (50- 175)
• Collagen
g ADP = 182 ((57-121))
• VW ag = 165%
• VW activity = 98%
• Ratio, Act/Ag =0.59 (normal > 0.8) =AVWD
• VWF multimeric analysis: Loss of highest
molecular weight multimers
©2012 MFMER | slide-9
Von Willebrand Disease Background
• Most common inherited
bleeding disorder ~1%
of the population
• Made by Endothelial
cells and
megakaryocytes
• Carries factor VIII
• High molecular weight
multimers (HMWM) are
the most active forms
• Can
C be qualitative vs
quantitative defects
• Leo et al. Haleo Orthopedics 2008
©2012 MFMER | slide-10
V Willebrand
Von
Will b d Disease,
Di
Bleeding
Bl di
• Trauma/wounds/surgery,
g y, p
post-partum,
p
, menorrhagia,
g ,
epistaxis/gingival, GI
• Often exacerbated by aspirin
• Inherited types:
• Type I, partial Quantitative deficiency
• Type 2, qualitative abnormalities
• Type 3, severe, undetectable levels of VWF
• Acquired von Willebrand syndrome:
• Cardiac disorders with turbulent flow, ie aortic
stenosis,
ste
os s, hypertrophic
ype t op c ca
cardiomyopathy,
d o yopat y,
ventricular assist devices
• Hematologic disorders, ie monocolonal
gammopathy
• Drugs,
D
h
hypothyroidism
th idi
©2012 MFMER | slide-11
AVWS in Aortic Stenosis
VWF multimers and Model of Pathophysiology
Blackshear et al
Blackshear,
al., 2012
Sadler NEJM
Sadler,
NEJM, 2003
Casonato, et al., Thrombo and Haemo, 2011: Evidence against proteolysis by
ADAMTS13 in all cases. Decrease in HMWM more p
pronounced
with rheumatic than degenerative, dystrophic AV disease.
©2012 MFMER | slide-12
AVWS testing and treatment
Coordination with Reference Laboratory is Critical
• Many VWF tests associate with severity of AS
• PFA-100
PFA 100 – sensitive
• VWF collagen binding assays -sensitive
• VWF multimer analysis - sensitive
• Ristocen Cofactor activity – insensitive
MULTIMERIC ANALYSIS
Patient
Control
HMWM
LMWM
©2012 MFMER | slide-13
E l ti off th
Evolution
the Case
C
• Multiple
p comorbidities, p
poor surgical
g
candidate.
• Alcohol septal ablation was performed to two
septal branches of the left anterior
d
descending
di coronary artery.
t
• Alcohol Septal Ablation is a performed in the
cardiac
di catheterization
th t i ti lab
l b
• Echo contrast injected to show target area
for infarction: creates localized damage with
thinning of septum, and reduced gradient
• Alcohol injected slowly to damage muscle
©2012 MFMER | slide-14
Al h l S
Alcohol
Septal
t l Abl
Ablation
ti
Echo contrast injected
Position of microcatheter
Showing Septal perforator
©2012 MFMER | slide-15
Four Months After Septal Ablation
©2012 MFMER | slide-16
Follow-up
• Six months following the procedure
• Did not required a transfusion
• VWF multimers normalized
• Baseline hemoglobin now 14 mg/dL (the highest in four years),
• Left ventricular outflow tract g
gradient reduced from 64 mm Hg
g
previously to 14 mm Hg.
Peak velocity 1.8 m/s
Peak gradient 14 mm Hg
©2012 MFMER | slide-17
Summary
• Consider VWS in p
patients with recurrent GI
Bleed and cardiac murmurs.
• Coordinate with laboratoryy directoryy to assist
with lab orders of VWS
• Correction of the cardiac abnormalityy resolves
bleeding in 80% of cases
©2012 MFMER | slide-18
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