HYPERTROPHIC

CARDIOMYOPATH
Y
MARKO VUKOVI
4TH YEAR

 Asymmetric septal hypertrophy (ASH)

Idiopathic hypertrophic subaortic stenosis (IHSS)
Muscle subaortic stenosis

Hypertrophic obstructive cardiomyopathy (HOCM).

Autosomal dominant disease of the heart muscle,
characterized by a small left ventricular cavity and
marked hypertrophy of the myocardium with myofibril
disarray.

Overall prevalence is 1:500 to 1:1000

Disease of the myofilaments, with alteration in
structure and function
Most common cause of sudden cardiac death in young
people.

ETIOLOGY
Most common genetic cardiovascular disease
Mutation in genes encoding proteins of the cardiac
sarcomere myofilaments
Beta-myosin heavy chain, myosin-binding protein C,
cardiac troponin T and I,alpha -tropomyosin, actin, titin,
and myosin light chains
Other genes outside of the sarcomere myofilaments
have also been recently implicated
Deletions, insertions, missense, and splice site
mutations.
Hypothesis is that sarcomeric dysfunction leads to
compensatory hypertrophy

PATHOLOGY

Asymmetrical hypertrophy with a small LV cavity

Mural endocardium thickened by fibrous tissue
Elongation of chordae and ant. displacement of the
hypertrophied papillary muscles
LA is usually dilated

HISTOLOGY

Short runs of severely hypertrophied fibers interrupted

by connective tissue
Large and bizarre nuclei and fibrosis , with degenerating
muscle fibers.
Whorling of muscle fibers is characteristic of HCM

PATHOPHYSIOLOGY

Diastolic dysfunction
Impaired ventricular relaxation and chamber stiffness
Compensatory increase in late diastolic filling by atrial
systole.
During exercise or any other type of catecholamine
stimulation, diastolic filling period decreases as well as
myocardial ischemia occurs
Leads to severe diastolic dysfunction- dyspnoea

LVOT obstruction
Multifactorial
During systolic contraction the hypertrophied basal
septum encroaches to the outflow tract
Mitral leaflets are pulled in by the venturi force
In midcavitary region hypertrophied pappillary muscles
pressing against septum may cause obstruction
LVOT obstruction is dynamic
Increased myocardial contractility, decreased ventricular
volume, or decreased afterload

Autonomic dysfunction
Either a failure of systolic blood pressure to rise >20
mmHg or a fall in systolic blood pressure during
excercise.
Speculated that there is high degree of abnormal
autonomic tone in HCM
Assoc. with poor prognosis
Mitral regurgitation
Secondary phenomenon due to systolic anterior motion
of leaflets due to LVOT obstruction
During mid and late systole- jet directed post. and
laterally
Myocardial ischemia
Supply-demand mismatch
compromised coronary blood flow to the LV myocardium
-- abnormally small and partially obliterated intramural
coronary arteries

CLINICAL PRESENTATION

Most are asymptomatic

Dyspnoea-diastolic dysfunction and elevated LV filling
pressures
Angina epicardial coronary disease may be absent
Syncope or presyncope- LV baroreceptors causes reflex
vasodilatation
Atrial fibrillation occurs more in elderly

PHYSICAL EXAMINATION

Carotid pulse- spike and dome pattern

rapid rise (percussion wave)
midsystolic drop
secondary wave (tidal wave)
Jugular venous pressure- usually normal; sometimes
prominent a wave (hypertrophy and lack of compliant RV
and powerful Rt atrial contraction)

 Apical impulse- forceful, sustained, diffuse

double or triple apical impulse
(outward thrust of ventricular contraction, a presystolic
accentuated atrial contraction, and expansion of early
diastolic filling)
Cardiac sounds- usually split sometimes paradoxically
split
S4 if severe hypertrophy

 Murmurs =crescendodecrescendo murmur located

primarily at the left sternal border. (usually ends before
S2)

May radiate to the base or apex, but seldom radiation

to the carotid arteries.

Any condition which increases the LVOT outflow gradient

more obstruction murmur is louder longer and peak
late in systole

LVOT OBSTRUCTION AND SYSTOLIC

MURMUR INCREASED BY.
A reduction in LV cavity volume and pressure ( less
preload)
A decrease in systemic vascular resistance / arterial
pressure ( less after load )
An increase in LV contractility
Converse is also true

Anterior Mitral
Leaflet

Myocardial Contractility
Exercise
Cathecolamines
Digitalis

Ventricular Volume
Valsalva
Standing
Nitroglycerine
Tachycardia

Gradient / Murmur

Per Arterial Pressure

Methoxamine
Sustained Handgrip
Passive Leg Raise
BV Expansion

Gradient / Murmur

ECG

Abnormal in 90-95 % patients

LVH

LAE

Deep and narrow q waves in infer lateral leads

Diminished R waves in lateral precordial leads

 Large negative precordial T waves

Left axis deviation
No ECG finding is predictive of future events
Tall R and deep S waves weakly corelated with magnitude of
LV hypertrophy
SVT 46%
PVCs 43%
Non sustained VT 26%
AF can occur in 25-30% of elderly

CHEST X-RAY

Mild to moderate enlargement of the cardiac silhouette.

The left ventricular contour is rounded consistent with
concentric LVH.
Enlargement of the left atrium
Right-sided chambers are usually normal.

ECHOCARDIOGRAPHY
Gold standard for the diagnosis of HCM
Increased wall thickness in the absence of other etiology
Septum>=1.3 times LV post-free wall
Subaortic Gradient > 30mm Hg ( rest) >50(provocation)
Vigorous motion of post. Free wall with reduced septal
excursion
Avg. LV wall thickness in HCM is 20- 22mm
ECG and echo has to be correlated.
Low voltages in ECG in the presence of increased wall
thickness is seen in infiltrative disorder

 Small LV cavity/ Septal immobility

Primary tool for defining the presence and severity of
LVO tract obstruction
Systolic anterior motion of mitral valve
Presence and severity of MR
(laterally and posteriorly directed jet in mid to late
systole)
Cardiac MRI

 Subaortic gradient >30 is a determinant of heart failure

but risk of sudden death
Elderly patients are not targeted for risk
stratification(SCD is uncommon and survival to old age
itself suggests low risk)
Beta myosin heavy chain and trop T mutations are
assoc. with higher risk for premature death.

MANAGEMENT

Prevention of SCD
ICD implantation as primary prevention following
cardiac arrest
-as secondary prevention if one or
more high risk factors
Empirical pharmacological therapy with amiodarone is
now obsolete

MEDICAL TREATMENT
Heart failure
Beta blockers (slows heart rate, reduce force of LV
contraction, augments ventricular filling and relaxation,
decreases myocardial O2 demand, blunts outflow
gradient triggered by sympathetic activity)
Verapamil (improves LV relaxation and filling)
Disopyramide
End stage failure ACE ,ARBs, ICD to prevent SCD

 Atrial fibrillation- most common sustained arrhythmia in

HCM
Paroxysmal AF occurs in 20%
Increases with age and is related to LA dilatation
Acute decompensation- cardioversion
Amiodarone prevents recurrences
Rate control with beta blockers or verapamil
Started on anticoagulation

DIURETICS

Can be use alone judiciously or with betablockers /

verapamil to lessen pulmonary congestion

NON MEDICAL TREATMENT

Surgery
Severe drug refractory heart failure symptoms
Severe functional disability (NYHA class III , IV)
Associated with LV outflow obstruction at rest or with
physiological exercise (>50mm gradient)
Transaortic ventricular septal myectomy (morrow
procedure)-small portion from basal septum

ALCOHOL ABLATION
96-98 % ethanol (1-3 ml)introduced into major septal
perforator vessel
Necrosis and permanent MI in proximal septum is
created
Thinning and reduced excursion of septum, Outflow
tract enlarges- reduction of subaortic obstruction and MR
alternative treatment strategy for selected patients
(advanced age, significant comorbidity and increased
operative risk, without access to expert surgical centers,
or pt.against surgical intervention)

ALCOHOL ABLATION

INFECTIVE ENDOCARDITIS

Vegetations most commonly seen on anterior mitral

leaflet or septal endocardium at the site of mitral valve
septum contact
standard antimicrobial prophylaxis is indicated prior to
dental or surgical procedures

Thank you