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  1. 1. Killer that claims four young lives each week Daily Express - 31st August 2004 By Hilary Freeman Parents call for action on sudden deaths - Jo Revill Health Editor The Observer - 13th June 2004 Teenager collapses dancing with friends Daily Mail - 17th May 2004 Unexpected tragedies Cycling Weekly - 17th April 2004 Hypertrophic Obstructive Cardiomyopathy Presenter- Dr. Jyotindra Singh
  2. 2. Functional Classification Dilated (Congestive, DCM, IDC) – Ventricular dilation, hypokinetic left ventricle, and systolic dysfunction Hypertrophic (IHSS, HCM, HOCM, ASH) – Inappropriate myocardial hypertrophy, with or without left ventricular obstruction Restrictive (Infiltrative) – Abnormal ventricular filling with diastolic dysfunction Arrhthymogenic Right Ventricular (ARVD) – Fibroadipose replacement of right ventricle
  3. 3. HOW DO WE DEFINE HOCM? IDIOPATHIC HYPERTROPHIC SUBAORTIC STENOSIS A myocardial disease characterized by Asymmetric hypertrophy of IVS & ventricles (LV > RV) Microscopic evidence of myocardial fiber disarray & fibrosis Variable dynamic obstruction that is usually sub aortic & is associated with abnormal SAM of AML Prevalence of HCM in the general population is about 1 in 500 (0.2%). 2
  4. 4. Historical Perspctive HCM was initially described by Teare in 1958 Braunwald was the first to diagnose HCM clinically Goodwin & colleagus- namd it HOCM SAM was described by Fix in 1964 Brock (1957) & Cleland (1958) did Ist myotomy- myectomy Dobell & scott / Lillehi & Levy- Cooley & colleagues- RV approach LA approach
  5. 5. Background Genetic disorder Autosomal dominant Molecular basis beta myosin heavy chain & Myosin binding protein C Myocardial Ca++ kinetics Increase Ca++ intracellular  hypertrophy and cellular disarray Leading cause of sudden death in preadolescent and adolescent
  6. 6. HOCM VARIANTS- Morphology 2
  7. 7. LV CAVITY Sub aortic -Small size Sigmoid shaped Midventricular -Dumbbell shaped – prone for LV apical aneurysm. Apical- Spade shaped Advanced disease-LV dilation
  8. 8. Mitral Valve Dynamic morphology Obstruction in LVOT Positioned closer to ventricular septum Disproportionately elongated & thickened Leaflets -AML PML closes against the body of elongated AML- junction of Middle & free edge
  9. 9. Fate of RV- LA-Coronaries RV RVOTO d/t Distorted IVS shape RVH P HTN d/t long standing LVH LA ----Dilated & thickened ----Reduced LV compliance / MR Coronaries---Large / Prominent muscular bridging (Total systolic occlusion) ---Wall thickening & luminal narrowing of septal branches
  10. 10. Histopathology Increased fibrous tissue Increase muscle cell diameter Increase in number of cell layers Abnormalities in orientation of myofibrils. Avg degree of disarray is 30% ( >5% is diagnostic of HOCM)
  11. 11. Pathophysiology Involves 4 interrelated processes: – LVOT – Diastolic dysfunction – Myocardial ischemia – Mitral regurgitation
  12. 12. Physiological consequences -LVOT Elevated intraventricular pressures Prolongation of ventricular relaxation Increased myocardial wall stress Increased oxygen demand Decrease in forward cardiac output
  13. 13. DIASTOLIC DYSFUNCTION Diastolic Dysfunction - Due to prolongation of isovolumic relaxation time -  LV filling pressure -  Ventricular volume - Atrial contribution to ventricular filling ~ 75% Poor Compliance -  LVEDP for any LVEDV - Subendocardial ischemia
  14. 14. Myocardial Ischemia  Myocardial muscle mass  Myocardial oxygen demand ( wall stress)  Diastolic filling pressures  Coronary capillary density  Vasodilatory reserve Abnormal intramural coronary arteries Small-Vessel Disease and the Morphologic Basis for Myocardial Ischemia in  Systolic compression of coronary arteries HCM(A) Native heart of a patient with end-stage HCM who underwent transplantation. Large areas of gross macroscopic scarring are evident throughout the LV myocardium(white arrows). (B) Intramural coronary artery in cross-section showing thickened intimal and medial layers of the vessel wall associated with small luminal area. (C) Area of myocardium with numerous abnormal intramural coronary arteries within a region of scarring, adjacent to an area of normal myocardium.
  15. 15. MITRAL REGURGITATION/SAM SAM: (Bernoulli effect) dynamic pressure gradient across LV outflow tract midsystolic intraventricular obstruction of the flow SAM - Septal Contact dynamic obstruction increased by:  afterload  contractility
  16. 16. Mitral Regurgitation SAM (Classical of HOCM) At systole -PML closes against mid part of AML (Not the free edge) On AML venturi effect of high velocity of blood stream (D/t rapid & early ventricular ejection) Free edge hinges on rest of leaflet & angulates towards aortic valve & contacts with IVS LVOTO MR – Results from the systolic anterior motion of the mitral valve – Usually mid / late systolic – Jet is directed posteriorly – Severity of MR directly proportional to LV outflow obstruction Usually relieved after relief of LVOTO. – Results in symptoms of dyspnea, orthopnea
  17. 17. Electrical disturbances - Paroxysmal supraventricular arrhythmias (30-50%) - result in shorter diastolic filling time; patients have palpitations, shortness of breath, syncope - - Atrial fibrillation (15-20%) - poorly tolerated – the time for diastolic filling decreased Non-sustained ventricular tachycardia (25% ) - occurs during ambulatory monitoring - Sustained ventricular tachycardia/ventricular fibrillation
  18. 18. SYMPTOMS Dizziness:  by Exertion Hypovolemia Maneuver (rapid standing or valsalva) Medication (diuretics, NTG and Vasodilator Meds) Arrhythmia hypotension decrease cerebral perfusion Dyspnea: – Most common symptom, 90% –  Lt Ventricular Diastolic filling pressure   PAP Orthopnea and Paroxysmal Nocturnal Dyspnea: – Pulmonary venous congestion – Early signs of CHF 19
  19. 19. SYMPTOMS Angina: – Common with no CAD – Impaired diastolic relaxation +  MVO2 Sub-endocardial ischemia –  Capillary density leads to  flow to hypertrophic muscle – Extramural compression of coronaries –  Systolic ejection time leads to  diastolic interval for coronaries perfusion Syncope and pre-syncope: – – – – Very common  CO with exertion or arrhythmia High risk of sudden death Urgent work-up and aggressive treatment 20
  20. 20. SYMPTOMS Palpitation: – Ventricular Arrhythmia75% – SVT 25% – A- fib 5-10% Sudden cardiac death (SCD): – 6 % in children – Related to extreme exertion – MCC of SCD is arrhythmia 80 % ~ V-fib 21
  21. 21. PHYSICAL SIGNS Double apical impulse: – Forceful left atrial contraction against non-compliant ventricle S1: normal S2: normal or paradoxical split S3 gallop: decompensated Lt. ventricle S4: atrial systole against hypertrophic ventricle Jugular venous pulse: prominent a- wave Double carotid arterial pulse: declines in mid systole as gradient develop 22
  22. 22. MURMURS Systolic Ejection Murmur: Crescendo - Decrescendo – Between apex and left sternal border – Radiate to suprasternal notch –  by  Preload (volume loading)  Afterload (vasopressor) –  by  Preload (nitrates, diuretic, standing)  Afterload (vasodilator) 23
  23. 23. MURMURS Holosystolic Murmur of MR: – Retrograde ejection of blood flow into low pressure left atrium – Best heard at apex and axilla – Pt. with SAM* and significant LV outflow gradients Diastolic Decrescendo Murmur of AR: 10% of Pt. * Murmur intensity increases ( reduced LV size ---- raises level of obstruction) --Reduced Preload – valsalva/ standing/ tachycardia --Reduced after load -- nitro vasodilators --Raised contractility – Ionotropes, exercise Murmur intensity decreases --Increased preload -- squatting, hand grip --Increased after load --Reduced contractility – B blockers, CCB 24
  24. 24. DIAGNOSTIC EVALUATION-ECG LVH - increased precordial voltages and nonspecific ST segment and T-wave abnormalities. Asymmetrical septal hypertrophy produces deep, narrow (“dagger-like”) Q waves infarction Q waves are typically > 40 ms duration while septal Q waves in HCM are < 40 ms. Lateral Q waves are more common than inferior Q waves in HCM. compensatory left atrial hypertrophy, with signs of left atrial enlargement (“P mitrale”) on the ECG. Atrial fibrillation and supraventricular tachycardias are common. Ventricular dysrhythmias (e.g. VT) also occur and may be a cause of sudden death.
  25. 25. (A) Parasternal long-axis view depicting severe asymmetric septal hypertrophy and systolic anterior : mitral valve motion (arrowhead); (B) M-mode across the mitral leaflets depicting prominent systolic anterior motion (thick arrows) of the anterior mitral leaflet (SAM); (C) M-mode tracing across the aortic valve demonstrating partial closure of aortic leaflets Date of download: (arrowheads); and (D) accentuationCopyright © The American Collegeleft ventricular outflow tract obstruction of late-peaking dynamic of Cardiology. 3/28/2013 All rights reserved. after the Valsalva maneuver.
  26. 26. CARDIAC CATH
  27. 27. Disease Progression J Am Coll Cardiol. 2003;42(9):1693.
  28. 28. Sudden Cardiac Death  Most frequent in young     adults <30-35 years old Primary VF/VT Tend to die during or just following vigorous physical activity Often is 1st clinical manifestation of disease HCM is most common cause of SCD among young competitive athletes J Am Coll Cardiol. 2003;42(9):1693.
  29. 29. COMPLICATIONS Complications Atrial Fibrillation Heart Failure – Only 10-15% progress to NYHA III-IV – Prevalent in up to 30% of older patients – CO decreases by 40% if AF present Endocarditis Autonomic Dysfunction – 4-5% of HCM patients – Usually mitral valve affected Embolisation – 25% of HCM patients – Associated with poor prognosis
  30. 30. TREATMENT Goals: –  Ventricular contractility Myocardial depression –  Ventricular volume Volume loading –  Ventricular compliance and outflow tract dimensions Medical therapy Device therapy Surgical Alcohol septal ablation Transplantation –  Pressure gradient across the LVOT – Vasoconstriction 31
  31. 31. Medical Therapy in HOCM Goals Exercise-induced gradient Beta-blockers Verapamil Disopyramide Oxygen-demand Prolong Diastolic Filling Period If maximum dose fails Pacing Ablation Myectomy
  32. 32. TREATMENT How Beta blockers work?  Pressure gradient across LVOT –  Inotropic state of left ventricle. –  Diastolic dysfunction –  Lt. Ventricle compliance  HR –  Myocardial oxygen consumption –  Myocardial ischemia potential CONTRAINDIACATION Inotropic Sympathomimetic Nitrates Except in patients with CAD Digitalis Except with uncontrolled Afib. Diuretics  Preload and ventricular volume  Outflow gradient 33
  33. 33. SURGICAL MANAGEMENT Septal myectomy Trans aortic / Left ventriculotomy Extended septal myectomy myectomy with Plication of Aml Modified konno operation Mitral valve replacement
  34. 34. Surgical septal myectomy •In patients that remain symptomatic despite maximal medical therapy with •SAM • Septum thickness more than 18 mm • Resting gradient more than 50 mmHg • Provoked gradient more than 50 mmHg . Occurrence of AF . High risk for sudden death • Asymptomatic younger patients with . Gradient > 100 mmHg . High risk of sudden death
  35. 35. TRANS AORTIC APPROACH Median sternotomy/ CPB estsblished Transverse aortotomy/ stay sutures Right aortic cusp –retracted anteriorly Narrow ribbon retractor-placed in LVOT 1st -Incision below right coronary cusp & parallel to LVOT 2nd parallel incision- as far leftward as possible- careful about MV Both incision deepened –toward LV apex Two parallel vertical incision connected by transverse incision.- rectangular piece of septum has been excised. Patients with left anterolateral free wall hypertrophy- third incision below the commissure between left and right coronary cusp & directed towards base of anterolateral papillary muscle.
  38. 38. TRANS AORTIC APPROACH This picture demonstrates the hypertrophied septum protruding into the left ventricular outflow tract. The leaflets of the aortic valve are pulled aside. Looking through the annulus, the anterior leaflet of the mitral valve is noted inferiorly, the bulging septum superiorly.
  39. 39. TRANS AORTIC APPROACH Opening of the outflow tract is much larger after the fibrious tissue has been removed, along with muscle; that the chords of the anterior leaflet of the mitral valve are now visible
  40. 40. ADJUNCTS TO MYECTOMY-TEE Location & thickness Adequacy of myectomy Correction of MR Identifying complications Gradient/SAM/MR IF residual gradient > 10 to 15 mm hg- additional muscle resection.
  41. 41. APICAL MYECTOMY Apical HCM /Midventricular obstruction Incision in the apex of the left ventricle lateral to the LAD Excision of the ventricular muscle at the apex and midventricular level is performed. Objective- increasing LV end diastolic volume / Improve LV compliance Post op – significant decrease in LV end diastolic pressure ,increase in LV diastolic volume index,increase in stroke volume.
  42. 42. Modified konno operation
  43. 43. Modified Konno Operation Localized subaortic stenosis or tunnel stenosis-when aortic annulus and valve are normal. Transverse aortotomy. RV opened –transverse incision- 2cm inferior to the level of pulmonary valve cusp. Right angle clamp –passed from aortotomy through aortic valve –into left side of LVOT Tip of the clamp palpated from septum-incision made through RV sideIncision extended inferiorly about 1 cm parallel to LVOT – at an angle to RVOT. Patch used to widen- LVOT
  44. 44. Extended myectomy with reconstruction of subvalvular apparatus -- Messmer Septal myectomy extended into the LV cavity- wide toward the apex Providing access to both the papillary musclemobilized down to the apex All hypertrophied portions and muscle trabeculae are resected. Mobilization of malpositioned papillary musclepermits mitral leaflets to deflect from LVOT during
  45. 45. Myectomy with plication of AML -- McIntosh Plication can be performed through the aortic valve. Horizontal/vertical direction Polypropylene sutures
  46. 46. ANTERIOR LEAFLET EXTENSION Insertion of a pericardial patch. Increases leaflet stiffness. Causes lateral displacement of the secondary chordae tendinae. Functions haemodynamically as a spinnakar sail to eliminate SAM.
  47. 47. MITRAL VALVE REPLACEMENT/REPAIR Mitral valve replacement- Reserved for severely symptomatic individuals with gradient > 50 mmHg in special situations like Thin septum < 18 mm Small aortic annulus Unusual morphology of septum Inability to achieve adequate resection Not amenable to repair- Myxomatous/ degenerative. Residual or recurrent obstruction Annuloplasty rings should be avoided to prevent SAM If necessary – flexible or rigid bands on the posterior leaflet preferred.
  48. 48. Postoperative care Maintain adequate preload- LA pressure of 16 to 18 mm hg may be required Avoid digitalis & isoproterenol – increase residual outflow gradient Avoid hypovolemia & NTG AF is poorly tolerated - Use of amiodarone
  49. 49. Complications Complete heart block (2.5% to 10 %) / LBBB (50%) Perioperative MI VSD (3%) > if septal thickness < 18 mm D/t iatrogenic / septal infarctionb AR (5%) Progressively increasing Risk - Small aortic annulus (<21 mm), Low mitral septal contact lesion D/t iatrogenic/ loss of support to right cusp/ Hemodynamic changes LV aneurysm
  50. 50. Surgical Myectomy Operative mortality 0.8% Gradient reduction 67 ----3 Post-op NYHA 1-2 1.0 94% Obstructive Post-myectomy 0.9 0.8 NYHA Pre Post I 2 24 III 48 7 IV 0.6 30 II 0.7 1 14 0 Obstructive Operative mortality 0.8% 0.5 0 2 4 6 Ommen S et al. J Am Coll Cardiol 2005 8 10
  51. 51. Hospital mortality 0- 6% 5 yr survival 93-84% 10 yr survival 88-71 %
  52. 52. ALCOHOL Ablation Alcohol SeptalSEPTAL ABLATION - performed percutaneously - 100% alcohol is injected into a septal perforator - results in infarction of the injected area  Successful short-term outcomes  LVOT gradient reduced from a mean of 60-70 mmHg to <20 mmHg  Symptomatic improvements, increased exercise tolerance  Long-term data not available yet  Complications spill over Complete heart block Large myocardial infarctions Ventricular arrhythmia & ECG changes
  53. 53. ALCOHOL SEPTAL Ablation Alcohol Septal ABLATION Before After
  54. 54. DUAL CHAMBER PACING Dual-Chamber Pacing Proposed benefit: pacing the RV apex will Decrease the outflow tract gradient By – Decreased septal motion Reduced SAM of the AML Late activation of base of septum Decreased LV contractility used in patients with significant symptoms who would not tolerate surgical therapy Objective measures such as exercise capacity and oxygen consumption are not improved No correlation has been found between pacing and reduction of LVOT gradient
  55. 55. OTHER MEASURES Dual-Chamber Pacing CARDIOVERTER – DEFIBRILLATOR in combination with myectomy - pts. With history of cardiac arrest/ unexplained syncope. CARDIAC TRANSPLANT not responding to maximal medical/surgical theraphy intractable heart failure with dilated ventricular cavities LEFT VENTRICULAR- AORTIC CONDUIT valved conduit from apex of LV to the thoracic or abdominal aorta
  56. 56. Ablation vs. Myectomy 279 patients Procedural Mortality (%) 1.4 80 1.3 1.2 60 1 0.8 70 75 71 50 0.9 40 30 0.6 20 10 0.4 0 15 7 Myectomy 0.2 0 Myectomy Ablation Ablation
  57. 57. Efficacy of Therapeutic Strategies Nishimura et al. NEJM. 2004. 350(13):1323.
  58. 58. HCM vs. Athletic Heart HCM Can be asymmetric Wall thickness: > 15 mm LA: > 40 mm LVEDD : < 45 mm Diastolic function: always abnormal 60 of 48 Athletic heart Concentric & regresses < 15 mm < 40 mm > 45 mm Normal Occurs in about 2% of elite althetes – typical sports, rowing, cycling, canoeing Former athletes & weekend warriors do NOT develop athletic heart Elite female athletes do NOT develop athletic heart
  59. 59. X ray Cardiomegaly LA enlargement Small aorta Pulmonary edema