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Two Cardiology Zebras
ERIC MARTIN MD
Disclosures
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Bayer
Gilead Sciences
NIH
Vascular Dynamics, In.
Employer—Iowa Heart Center/Mercy
Des Moines
Zebra # 1
History
•  CC: 52-year-old man seen in
consultation for a 3 year history of
heart-failure
•  FHx: Father had “MI” at 43 years of age
died of "second heart attack" 6 months
later. Paternal grandfather and one
uncle both died in their 40s of
"myocardial infarction“. His son had
patent ductus arteriosus.
http://www.theheart.org/viewArticle.do?primaryKey=117699
Physical Examination
•  Middle-aged WM in no acute distress
•  Heart rate: 60 bpm with frequent
ectopic beats BP: 120/80 mm Hg, pulse
96
•  Lungs clear
•  Mild cardiomegally, no murmur but loud
S4 gallop was present. No peripheral
edema noted. No jugular-vein
distention or hepato-jugular reflux.
Routine Studies
•  ECG: normal sinus rhythm, 1° AV
block, frequent PVCs in an
intermittent bigeminal pattern,
non-specific ST and T-wave
changes
•  Chest x-ray: minimally enlarged
cardiac silhouette with clear lungs
ECHO Cardiogram
Contrast Enhanced ECHO
Cardiogram
Differential Diagnoses
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Mural thrombi
False Tendon
Apical Hypertrophic Cardiomyopathy
Cardiac Fibroma
Cardiac Metastases
Loeffler Endocarditis
LV Noncompaction
Left Ventricular Non-Compaction
(LVNC)
•  Synonyms
–  Noncompaction of the LV Myocardium
–  Left Ventricular Hyper Trabeculation
–  Spongy Myocardium
Left Ventricular Non-Compaction
(LVNC)
•  Incidence or prevalence is uncertain
–  Estimates vary between 0.12 and 2.2/100,000
•  May be isolated or associated with other
congenital cardiac and non-cardiac
abnormalities
•  Autosomal dominant form of transmission
•  Multiple genetic defects have been
documented
Proposed Etiology
•  A congenital disorder of endomyocardial
embryogenesis.
•  The postulated but unproven cause:
–  Arrest of the normal compaction of the loose
interwoven mesh of myocardial fibers en utero
during days 32 to 70 of fetal development.
•  There is little direct evidence to support this
proposition
Location of Lesions in 7 patients
with LVNC
The basal septum and basal inferior wall are spared
Heart 2001;86:666-671
Multiple Phenotypes:
Gross Appearance
•  A: Anastomosing
broad trabeculae
•  B :Coarse trabeculae
resembling multiple
papillary muscles
Virmani R et al Hum Pathol. 2005 Apr;36(4):403-11
LVNC: Gross Appearance
•  C: interlacing smaller
muscle bundles
resembling a sponge
•  D: The trabeculae can
only be appreciated
viewed en face
Virmani R et al Hum Pathol. 2005 Apr;36(4):403-11
Histopathology
Note the thin compacted normal outer layer of myocardium and the
endocardial (non-compacted) layer. There is scar tissue within the
trabeculations (asterisks) and in the subendocardial area but not in the
epicardial zone.
Heart 2001;86:666-671
Clinical Classification
•  Isolated LVNC
–  Typically presents in adulthood
–  No communication between coronary arteries
and LV chamber.
Clinical Classification
•  Complex LVNC-Reported with various forms of
congenital heart disease. Often seen in children
–  PDA
–  Bicuspid aortic valve disease
–  Multiple type of complex congenital heart disease
particularly with RV outflow tract problems
•  Trabeculations may communicate with coronary
arteries creating a coronary-cameral shunt (LV)
Clinical Imaging in LVNC
•  Left heart cath contrast
LV angiogram
•  TTE and TTE with
Doppler
•  Cardiac Magnetic
Resonance Imaging
Contrast Left Ventriculogram
•  Left ventricular
angiography RAO
projection.
•  Left ventricular
angiography LAO
projections.
Criteria for the Dx Isolated LVNC
•  A 2 layer structure
–  Compacted thin epicardial
band and a much thicker
noncompacted endocardial
layer
–  Deep endomyocardial
spaces
–  Maximal end-systolic ratio
of noncompacted to
compacted layers (>2:1
ratio by echo and 2.3:1 by
CMR).
•  CMR or color Doppler evidence
of deeply perfused
intertrabecular recesses.
Jenni, R et al Heart 86 (2001)666
Trans Esophageal Echocardiogram
•  On transgastric two
chamber view, the
anteriomedial papillary
muscle is poorly defined
and characterized by the
presence of numerous
separated bands (arrows)
inserting in to the anterior
wall near the apex.
•  The absence of a well
defined papillary muscle
is common in LVNC
Color Doppler in LVNC
•  Color Doppler study shows typical flow away from the
ventricular cavity into the deep spaces between the
prominent trabeculation during diastole (in A represented
by a red signal) with flow back into the ventricle during
systole (in B, blue signal).
Trans Esophageal Echocardiogram
•  Note the reduced left
ventricular function
and the appearance of
left ventricular
hypertrophy.
•  The fingers of
myocardium extend
into the cavity
approximately 2 to 3
cm.
Contrast Enhanced CMR in LVNC
White Blood
Technique
LVNC on CMR
Black Blood Technique
CMR Cine “4 Chamber View”
Detection of Thrombi in NCLV
Heart 2005;91:e4
Detection of Thrombi in LVNC
Heart 2005;91:e4
CMR with Gadolinium Delayed
Enhancement
•  A-Single frame from a 4 chamber cine view
•  B-Delayed enhancement consistent with edema,
scaring or fibrosis of the LV septum
Heart 2005;91:582
Largest Published Series
•  34 Patients Followed for ~3 Years
–  Clinical features:
•  Heart failure 53%
•  Heart transplantation 12%
•  Thromboembolic events 24%
•  Ventricular tachycardia 41%
JACC 2000;36:493-5001
34 Patients Followed for 3 Years
•  Many of these patients who presented with
congestive heart failure had dyspnea and
profound pulmonary edema with relatively
preserved LVEFs and Doppler changes
which were indicative of diastolic
dysfunction.
Zebra # 2
Zebra # 2
Zebra # 2
TTE, ECG and LV-gram
From 58 YO Woman with Head
Injury
Differential Diagnosis
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Acute Myocardial Infarction
Cocaine-related ACS
Myocarditis
Pheochromocytoma
Stress Cardiomyopathy
Stress Cardiomyopathy
•  This pathological process has many names
–  Stress Cardiomyopathy
–  Apical Dilation in the Absence of CAD
–  Tako Tsubo Syndrome
–  “Broken Heart Syndrome”
Transient LV Apical Dilation in the
Absence of CAD
•  Initially described in the Japanese literature.
–  Tako Tsubo Syndrome = Octopus trap
•  The syndrome consists of chest pain associated
with ST-T abnormalities, moderate increases in
cardiac markers, and a reversible apical wall
motion abnormality in the absence of coronary
artery disease.
•  It is typically associated with emotional or mental
stress.
Bybee et al: Ann Intern Med 2004;141:858-865
Classic Japanese Octopus Trap:
Tako Tsubo
Transient LV Apical Dilation in the
Absence of CAD
•  The syndrome more often affects
postmenopausal women.
•  The in-hospital mortality rate seems to be
low, as does the risk for recurrence.
Left Ventricular Apical Ballooning
Cardiac MRI
Clinical Characteristics of 19 Patients with Stress Cardiomyopathy on Admission
Wittstein, I. S. et al. N Engl J Med 2005;352:539-548
Typical Electrocardiograms Obtained 24 to 48 Hours after Presentation in Four Patients with
Stress Cardiomyopathy
Wittstein, I. S. et al. N Engl J Med 2005;352:539-548
Serial Echocardiographic Assessment of the Ejection Fraction in 19 Patients with Stress
Cardiomyopathy
Wittstein, I. S. et al. N Engl J Med 2005;352:539-548
Ventriculographic Assessment of Cardiac Function and MRI Assessment of Myocardial Viability
at Admission in a Patient with Stress Cardiomyopathy
Wittstein, I. S. et al. N Engl J Med 2005;352:539-548
Transient LV Apical Dilation in the
Absence of CAD
•  Reversible balloon-like
asynergy at the apex with
hypercontraction of the
basal segment of the
ventricle was observed
during the acute phase
•  This disappeared during
the chronic phase.
•  The interval between these
left ventriculograms from
acute to chronic phase was
51 days.
Plasma Catecholamine and Neuropeptide Levels
Wittstein, I. S. et al. N Engl J Med 2005;352:539-548
Transient LV Apical Dilation in the
Absence of CAD
•  The majority of patients survive this problem
•  The only autopsy report is that of a patient who
died of multiple organs system failure who also
developed Takotsubo’s Syndrome.
–  The patient had no macroscopic signs of recent
myocardial infarction or scars.
–  Microscopic examination revealed normal myocardial
tissue, except for some fatty infiltration.
•  This observation suggests that acute myocarditis does not
contribute to the etiology of this syndrome.
Diagnostic Criteria for Primary Transient
Left Ventricular Apical Ballooning
•  Major criteria
1. Reversible balloon-like left ventricular wall motion
abnormality at the apex with hypercontraction of the basal
segment
2. ST-T segment abnormalities on ECGs mimicking
acute myocardial infarction
•  Minor criteria
1. Physical or emotional stress as triggering factors
2. Limited elevation of cardiac markers
3. Chest pain
Modified Mayo Clinic Criteria
•  Transient regional wall motion abnormality w/ or
w/o apical involvement and a stressful trigger
often but not always present.
•  Absence of obstructive CAD or plaque rupture
•  New ECG abnormalities or cardiac enzyme
elevation
•  Absence of pheochromocytoma or myocarditis