A 55-year-old male presented with shortness of breath for 7 days and chest pain for 6 days. Examination revealed a systolic murmur, cardiomegaly on chest x-ray, and ECG findings consistent with hypertrophic cardiomyopathy. The patient was diagnosed with hypertrophic cardiomyopathy, an inherited heart condition characterized by thickened heart muscle and small ventricle size. Risks include sudden cardiac death, heart failure, and embolic events. Treatment focuses on symptom management through medication and potentially surgery to reduce risks.

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