CTSN -

Hypertrophic Cardiomyopathy

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1. Definition
Unknown etiology
Genetically determined
Primary cardiac muscle hypertrophy
Dynamic LVOT obstruction
Myocardial fiber disarray

2. Morphology
Asymmetric septal hypertrophy
Systolic anterior motion (SAM) mitral valve
Small LV cavity
Myocardial fiber disarray
Left atrial enlargement
Distortion right ventricle
Large diameter coronary arteries
Impaired coronary perfusion


3. Clinical features
Symptoms
Dyspnea Diastolic dysfunction
Syncope <===== Arrhythmias
Angina Myocardial ischemia
Outflow obstruction

Signs
Rapid pulse upstroke
Jugular a-wave
Double LV impulse (S4)
S3 at apex
Mid-systolic precordial murmur

4. Diagnostic Criteria
Electrocardiography
LV strain
RBBB, LBBB, left anterior hemiblock
Chest radiography
Cardiomegaly
Increased pulmonary vascular markings
Echocardiography
LVOT obstruction
Early aortic valve closure
Small LV cavity
SAM
5. Cardiac catheterization
Right sided pressures - normal or increased
LVOT obstruction
Increased LVEDP
Post-PVC gradient potentiation (Brockenbrough)
Dynamic gradient (provocative)
Mitral regurgitation
Coronary arteriography

6. Natural History
Asymptomatic ASH ===?===> clinical HOCM
Ages 20-30 most common
Obstruction in only 20%
Progressive symptomatic course
Mortality without operation
15% at 5 years
25% at 10 years

7. Operative Treatment
Indications for Operation
NYHA class III or IV
Symptoms not relieved by medical treatment
Gradient (rest or provocative) > 50 mmHg
Goals of Operation
Relieve gradient
Abolish SAM
Enlarge LVOT
Abolish MR

8. Technique
Myotomy - Myectomy
Aortic root
Aortic root and LV
Modified Konno
LV-Ao conduit
MVR
Myectomy and MVR
DDD pacemaker

9. Complications
Complete heart block (3-5%)
Myocardial infarction (3-4%)
Embolism
Iatrogenic
VSD (3%)
Aortic valve injury
Mitral valve injury
LV pseudoaneurysm
Results
Early death (5-8%)
Late death - CHF, arrhythmias, stroke