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- 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 硫 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/Ds 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 APCs,VPCs,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 Fabrys Recombinant 留-galactosidase A enzyme replacement