2.  A 55yr old Male, c/o shortness of breath –
7 days; chest pain – 6days
 No h/o syncope ; no h/o oliguria; no h/o
fatigue
 Not a known Diabetic; known hypertensive
for 5yrs
 No h/o similar illness in the family

3.  O/E Patient Conscious
 Mildly dyspneic,tachypneic
 Mild pallor – I0/PE0/L0/CL0
 CVS S1 S2+ S4+; systolic murmur+ not
radiating to carotid
 RS NVBS heard
 P/A soft
 BP 130/80 mm hg
 PR 76/min

4.  Blood Sugar, Urea Creatinine levels are
within normal limits
 CBC with normal limits
 Chest X-Ray
 Mild cardiomegaly
 Lung fields clear

6.  ECG shows:
 Rate : 75/min
 Normal axis of 15 degree
 Normal ST segment
 PR interval normal
 normal sinus beat followed by atrial ectopic
 Atrial bigeminal rhythm
 Tall R waves with deep symmetrical sharply
pointed inverted T waves in the mid precordial
leads
 The initial horizontality of ST Segment with well
developed ST T angle – ST Segment shelf is seen

7.  Hypertrophic cardiomyopathy
 lV SYSTOLIC OVERLOAD like hypertension
 Myocardial ischemia
 CVA with qrs st pattern
 Valvular aortic stenosis

8.  Inheritable autosomal dominant disease of
heart muscle d/tmutation in beta mhc of
chr14
 characterized by thickened but non dilated
left ventricle in the absence of another
cardiac or systemic condition capable of
producing magnitude of the hypertrophy
evident
 Small ventricular cavity and marked
hypertrophy of myocardium with myofibril
disarray w/wo dynamic outflow tract
obstruction

11.  Most common cause of sudden cardiac death
in young people including trained athelets
 WHO designated with HCM to describe this
unique process of primary muscle
hypertrophy
 M mode echo define ASH
 Myocardial disarray of muscle fibre result in
WHORLING characteristic of HCM

12.  Diastolic dysfunction
 LV Out flow tract obstruction
 Mitral regurgitation due to elongated mitral
leaflets and chordae
 Intramyocardial ischaemia due to partially
obliterated intra mural coronary arteries
 Arrythymias
 Autonomic dysfunction – systolic BP↓ on
exercise

13.  MANEUVER PHYSIOL EFFECT HCM AS MR
Valsalva vr,svr,co
squat&hand vr,svr,co
 Grip &phenyl
ephrine
 Amylnitrite vr, dec svr

14.  Parameters associated with sudden death
 survivor of cardiac arrest
 Sustained VT
 Family history of premature sudden death
 Massive degree of ventricular hypertrophy
 Hypotensive response to exercise
 Myocardial bridging
 Septal thickness > 30mm
Troponin t mutation
Courtesy Braunwald heart diseases & Harrison Medicine

15.  LV hypertrophy with septum >1.3times
posterior LV wall thickness
 Ground glass appearance of septum
 Spade shaped LV Cavity
 small lv cavity
 SAM of mitral valve
 septal immobility
 premature closure of aortic valve
 resting gradient>30mm
 provocable gradient>50mm

16.  TYPE 1 ..ANT SEPTUM 10%
 TYPE 2…ANT AND POST SEPTUM 20%
 TYPE 3 ..ANT AND POST SEPTUM INCLUDING
LAT.FREE WALL 52%
 TYPE 4 ..REGION OTHER THAN SEPTUM AND
POST FREE WALL 18%

18.  Sudden death
 Infective endocarditis
 Systemic embolism
 Atrial fibrillation
 High incidence of SVT 46%, PVC 43%, VT 26%
AF 25-30%

19.  Screening Echo for first degree relatives
 Avoid strenuous exercise
 IE prophylaxis
 Keep well hydrated
 Medical therapy like Beta blockers, calcium
channel blockers, diisopyramide
 Surgical options include septal myectomy.
Dual chamber pacing, septal ablation in
patients not responding to surgery
 AICD for prevention of sudden death