Hocm

2. Hypertrophic Obstructive
cardiomyopathy(HOCM)
• Definition: HOCM is a genetic myocardial disorder that is
characterized by left and/or right ventricular hypertrophy that is usually
but not always asymmetric and is associated with microscopic
evidence of myocardial fiber disarray and fibrosis
• Hypertrophy frequently involves the IVS,resulting in LVOT
obstruction
• Transmitted as AD trait with variable penetrance and expressivity
• Mutation in β myosin HC gene in chr. 14 is MC

3. PATHOPHYSIOLOGY
• Left ventricular outflow
obstruction
• Diastolic dysfunction
• Myocardial ischemia

4. Clinical features
• Dyspnea on exertion(90%),Orthopnoea,PND
• Palpitations(PAC,PVC,Sinus pauses,AF, A flutter VT,SVT)
• Congestive heart failure
• Angina
• Syncope and Pre syncope

5. • Sudden cardiac death
• HCM is the MC cause in young people including athletes
• Can be the first manifestation
• Most common cause is arrhythmias esp. VF either de novo or AF
degenerated into VF due to 20 accessory pathway
• D/D’s
• Athletes Heart: Kinking of coronary artery
• Commotio cordis: Blunt trauma to chest causing V.FIB
• Holiday Heart Syndrome: A.Fib

6. Signs
• Pulse: Pulsus bisrifiens
• Prominent a wave in JVP: ↓ RV compliance
• Double apical impulse: Forceful LA contraction against a non
compliant LV
• Heart sounds
• S1 normal
• S2 is usually split and in severe cases paradoxically split
• S3 indicates HF
• S4 present due to LAH

7. • Medium pitch crescendo decrescendo Ejection systolic murmur along
LLSB and apex and radiates to suprasternal notch
• Dynamic maneuvers
• Murmur intensity increases with decreased preload (i.e valsalva,
nitrates,standing,diuretics)
• Murmur intensity decreases with increased preload(i.e hand grip,
squatting)

8. Diagnostic evaluation
• ECG
• Echocardiography
• Catheterization
• Cardiac MRI

9. ECG
• LVH with non specific ST/T wave abnormalities
• Left or Right axis deviation, LAE, Conduction abnormalities
• Abnormal and prominent Q waves In precordial and lateral lead
• A fib wit prexcitation indicates poor prognosis
• Findings on holter monitoring includes APC’s,VPC’s,sinus
pauses, wandering atrial pacemaker,atrial
flutter/fibrillation/tachycardia and non sustained ventricular
tachycardia

10. 2D ECHO
• Abnormal systolic anterior leaflet motion of mitral valve
• LV hypertrophy
• Left Atrial enlargement
• Diastolic Dysfunction
• Small ventricular chamber size
• Septal hypertrophy with septal to free wall ratio greater than 1.4:1
(Absolute septal wall thickness >15 mm)
• Decreased Mid aortic flow
• Partial systolic closure of the aortic valve in mid systole

11. 2D ECHO Diagnostic criteria
• Unexplained maximal wall thickness (measured at end diastole)≥
15mm or >2 SD for age, sex , height in any myocardial segment
• Septal/posterior wall thickness ratio of >1.3 in a non dilated ventricle
and >1.5 in the setting of systemic hypertension

12. CARDIAC MRI
• Useful particularly when ECHO is questionable, particularly with
Apical hypertrophy
• SAM of mitral valve is clearly seen
• Improvement in obstruction after septal ablation or myomectomy can
be demonstrated

13. MANAGEMENT
• Prevention of SCD:ICD
• Medical Rx of Heart failure and AF
• Surgery
• Dual chamber pacing
• Alcohol septal ablation

14. Implantable cardioverter
defibrillator
• Recent unexplained Syncope
• LV hypertrophy with a maximal wall thickness>30mm
• H/o SCD in family
• Multiple episodes of Non Sustained VT
• Hypotensive response to exercise

15. MEDICAL THERAPY
• Beta blockers
• Increased ventricular diastolic filling/relaxation
• Decreased myocardial O2 consumption
• Verapamil
• Augments Ventricular diastolic filling/relaxation
• Disopyramide
• Used along with beta blockers in presence of persistent symptoms
• Negative inotrope
• Low dose diuretics

16. Surgery
• Severe medical refractory symptoms develops in 5% patient for
whom surgery is indicated
• Septal Myectomy and alcohol septal ablation are used
• Neither procedure has been shown to improve outcomes other than
symptoms
• Complete Heart block is the MC complication associated with it

18. RESTRICTIVE CARDIOMYOPATHY
• RCM is characterized by Rigid Heart with poor ventricular filling but
generally a non dilated LV and normal LVEF
• RHF symptoms are common
• Can be Primary or secondary to infiltrative conditions

20. MECHANISM

21. Symptoms
• Volume overload
• Fatigue
• Dyspnoea
• Orthopnoea
• Nocturnal Dyspnoea
• Arryhthmias
• Palpitations,Syncope,Exercise
intolerance
• Reduced cardiac
output
• Exercise intolerance
• Cognitive difficulties
• Angina,Dyspnoea/Sy
ncope on exertion
• Sudden Cardiac death

22. Signs
• Pulse: Tachycardia, Bradycardia, irregular weak peripheral pulse
• BP: Low
• Raised JVP
• S3 and/ S4
• Pulmonary crackles
• Kussmual sign: Paradoxical rise in RA pressure during inspiration
• Findings of Rt. Heart failure such as Hepatomegaly, ascites and
pitting pedal edema

23. WORK UP
• ECG
• ECHO
• Cardiac MRI
• Catheterization
• Lab diagnosis of Amyloid protein
• Endo myocardial Biopsy

24. • ECG:
• Low QRS voltage in amyloidosis
• Large P waves indicating bi atrial enlargement
• Conduction delays and various ST/T wave abnormalities
• Atrial fibrillation and tacharryhtmias
• ECHO
• Non dilated,non hypertrophied ventricles
• Unless infiltrative or storage disease
• Mod. to marked Biatrial enlargement
• Doppler is required to assess impaired ventricular filling

25. • Cardiac Catheterization
• Square root wave sign
• Seen in constrictive pericarditis as well
• Cardiac MRI:
• Gold standard for non invasive diagnosis of Hemochromatosis
• Helps in differentiating from constrictive pericarditis
• Endo myocardial Biopsy
• Gold Standard
• Not commonly used
• Indicated for treatable systemic disease involving myocardium
such as Amyloidosis, eosinophillia,Sarcoidosis

26. Management
• Treat underlying disease in secondary causes
• Attempt to maintain sinus rhythm , atrial fibrillation is poorly tolerated
• Amiodarone
• Treat HF symptoms
• Diuretics and ACE inhibitors
• Most are irreversible and requires cardiac transplant
• Pacemakers for conduction defects
• Anticoagulation for thrombus/emboli prevention

27. Cardiac Amyloid
• Usually ineffective and generally consist of supportive therapy
• Autologous stem cell transplantation in conjunction with melphalan
therapy
• Heart transplantation: used only if the patient has isolated cardiac
amyloid
• ICD placement: Controversial give most SCD is related to
electromechanical dissociation and not ventricular arryhthmias

28. CARDIAC SARCOID
• Glucocorticoid to slow the progression of inflammation and
fibrosis
• Dose unclear
• Relapses are common after tapering
• Chloroquine,HCQ,Cyclosporine and methotrexate can be used
• ICD placement

29. Treatment for other causes
• Hemochromatoisis
• Treatment with serial phelbotomy and chelation therapy
• Endomyocardial fibrosis
• Poor prognosis with medical therapy
• Prednisone in acute carditis
• Endomyocardial resection with valve replacement/repair
• Fabry’s
• Recombinant α-galactosidase A enzyme replacement