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Cardiomyopathies

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Cardiomyopathies

  1. 1. Dr Tarun BhatnagarConsultant Cardiac Anaesthesiologist dr.tarunbhatnagar@yahoo.co.in
  2. 2.  WHO in 1995 defined Cardiomyopathies as diseases of myocardium associated with cardiac dysfunction Types Dilated Cardiomyopathy(DCM) Hypertrophic Cardiomyopathy(HCM) Restrictive Cardiomyopathy(RCM) Arrythmogenic RVCardiomyopathy(ARVC)
  3. 3.  DCM is most common of all CMs(60%) Aetiology -Idiopathic (50%) -Myocarditis (9%) -Ischemic (7%) -Others-Viral, Peripartum, Substance abuse etc Morphologically Enlargement of RV & LV cavities without an increase in ventricular septal or free wall thickness → spherical shape & dilatation of heart → Displacement of papillary muscles → Regurgitant lesions despite valve leaflets being normal
  4. 4. Medslides.com 5 of 48
  5. 5.  Microscopically –Patchy & diffuse loss of tissue with interstistial fibrosis & scarring Systolic Dysfunction>>> Diastolic dysfunction SV is initially maintained by ↑↑ EDV With disease progression→Marked LV dilatation with normal or thin wall →↑ Wall stress + Valvular Regurgitation →Overt Circulatory Failure
  6. 6.  Symptoms -Typically pts c/o months of fatigue, weakness, reduced exercise tolerance due to CHF -May also present as a Stroke, Arrythmia or Sudden Death Physical Signs -Tachycardia -pulsus alternans -Jugular venous distension -Murmurs of AV valve regurgitation -Gallop heart sounds
  7. 7.  CXR- Cardiomegaly , Pulmonary venous congestion ECG- Normal or low QRS voltage , abn axis, non specific ST seg abnormalities, LV hypertrophy, conduction defects, AF, Non sustained VT 2D Echo Coronary Angiography -usually normal coronaries -coronary vasodilatation is impaired by ↑ LV filling pressures -distinguishes b/w Ischemic & Idiopathic DCM Endomyocardial Biopsy rarely valuable to identify the aetiology
  8. 8.  Aim of treatment -Manage the symptoms -Reduce the progression of disease -Prevent Complications Mainstay of Therapy Vasodilators + Digoxin + Diuretics
  9. 9.  ACE Inhibitors -Indicated for all patients - Reduce symptoms & improve effort tolerance - Suppress ventricular remodelling & endothelial dysfunction -Reduce CV mortality Milrinone -Selective PDE-3 inhibitor -may improve quality of life but doesn’t affect mortality -rarely adm in chronic situations
  10. 10.  Spironolactone used along with ACE Inhibitors has shown to reduce mortality by 30% in a large double blind randomized trial Digoxin clinically beneficial as reaffirmed by two large trials in adults β Blockers untill recently contraindicated but recent studies show that they not only provide symptomatic improvement but substantial reduction in sudden death in NYHA class II & III HF pts
  11. 11.  Amiodarone -High grade ventricular arrythmias (Sustained VT or VF) are common in DCM→↑ risk of SCD -Preferred antiarrythmic agent as it has least negative inotropic effect & proarrythmogenic potential -Implantable Defibrillators are used for refractory arrythmias Anticoagulants -indicated for pts with moderate ventricular dilatation+mod-severe systolic dysfunction -H/O stroke , AF or evidence of Intracardiac thrombus
  12. 12.  Dual Chamber Pacing Cardiomyoplasty LV Assist Devices improved pts sufficiently to avoid transplant or enable later transplant Cardiac Transplantation has substantially prolonged survival in DCM pts with 5 yr survival rate of 78%
  13. 13.  Cardiac procedures which DCM pts undergo -Correction of AV valve insufficiency -Placement of ICD device -LV Assist device placement -Allograft Transplantation Goals of Anaesthetic Mx - Reduction of afterload -Optimizing preload -Minimize myocardial depression DCM pts are extremely sensitive to cardiac depressant anaesthetic drugs
  14. 14.  Fentanyl-(30u/kg )provides excellent anaesthesia & hemodynamics in pts with EF<0.3 Remifentanyl- assoc with severe hypotension & bradycardia ,therefore unsuitable in low EF pts Etomidate-least effect on cardiac contractility in pts undergoing cardiac transplant Ketamine-excellent choice in combination with fentanyl for induction in pts with severe myocardial depression Propofol-causes CV depression due to inhibition of sympathomimetic activity & vasodilatation Volatile agents-Desflurane with lowest BG partition coefficient may allow some benefit for rapid induction, rapid recovery from anaesthesia & early extubation
  15. 15.  Invasive hemodynamic monitoring -Mandatory in pts with DCM undergoing surgery -Physical s/s may not accurately reflect physiological parameters -Pts with Implanted defibrillators have severely depressed cardiac function but are routinely managed without a PAC Hemodynamic Instability -managed by low dose of inotrope & vasodilator
  16. 16.  Afterload reduction -improves regional & global indices of ventricular relaxation & EF during anaesthesia when myocardial depression may be significant -it also reduces valvular regurgitation & volumes Patients on Amiodarone on long term basis can interact with anaesthetic agents & further reduce contractility & conduction-requires careful titration Arrythmogenic factors – Hypokalemia, Hypomagnesemia, & Sympathatic activation should be monitored & corrected
  17. 17.  Hypertrophic Obstructive Cardiomyopathy(HOCM) Idiopathic Hypertrophic subaortic stenosis(IHSS) Assymetric Septal Hypertrophy M/c genetic cardiac disease Prevalance in adults-0.2% Primary myocardial abnormality with sarcomeric disarray & assymetric LV hypertrophy
  18. 18. Massive left ventricularhypertrophy, mainlyconfined to the septumHistopathology showingsignificant myofiberdisarray and interstitialfibrosis
  19. 19.  Causes: Inherited, acquired, unknown Autosomal dominant inheritance pattern >450 mutations in 13 cardiac sarcomere & myofilament-related genes identified ?? Role for environmental factors
  20. 20. Hypertrophic cardiomyopathy: variantsHypertrophic cardiomyopathy morphology exhibits heterogeneity.The mostcommon variant is assymetric septal hypertrophy involvingthe entire septum
  21. 21.  Subaortic Obstruction Diastolic Dysfunction Myocardial Ischemia Mitral Regurgitation Arrythmias
  22. 22.  Cause -Assymetrical Septal Myocardial Hypertrophy Unlike Aortic stenosis hypertrophy begets pressure gradient , not the other way around Wide spectrum of severity of obstruction ch by Variability- absent to critically severe Dynamic nature - depends on contractility & loading conditions Timing - begins early & peaks variably Location -subaortic
  23. 23.  Subaortic Obstruction Cause-Hypertrophic septum encroaching on the systolic outflow tract Bounded-Anteriorly by IVS & Posteriorly by AML Effect-Systolic anterior motion(SAM) of AML → accentuating obstruction Mechanism of SAM Thickened IVS→Restricted LVOT → ejection of blood at a higher velocity closer to the AML → Drawing of AML closure towards the hypertrophied septum due to the venturi effect during LV systole→ Dynamic LVOT obstruction
  24. 24.  Factors aggravating SAM & producing Dynamic Obstruction- -↑ Contractility -↓ Afterload (Aortic outflow resistance) -↓ Preload (End diastolic volume) Therapeutically Myocardial depression, Vasoconstriction & Volume overloading should minimize obstruction & augment forward flow LVOT gradient ≥ 30mmHg assoc with physiologic & prognostic importance LVOTO is assoc with ↑ wall stress, myocardial ischemia, cell death & eventually fibrosis→VT /VF Dynamic LVOTO may also occur in Cardiac tamponade or Acute MI
  25. 25. Hypertrophied & disorganized myocytes with ↑CoT Impaired relaxation+ ↑ Chamber stiffnessDiastolic Dysfunction→↓ rate of rapid ventricular filling ↑ atrial systolic filling↑ Filling pressures & pulmonary congestion
  26. 26.  Myocardial Ischemia ◦ Often occurs without atherosclerotic coronary artery disease ◦ Postulated mechanisms  Abnormally small and partially obliterated intramural coronary arteries as a result of hypertrophy  Inadequate number of capillaries for the degree of LV mass
  27. 27.  Dyspnea on exertion (90%) Angina (70-80%) Syncope (20%) Sudden cardiac death
  28. 28.  ECG-↑ QRS voltage, ST-T changes, Axis deviation, LV Hypertrophy +strain pattern CXR-Lt atrial enlargement or normal Echo Invasive Cardiac Cath- indicated for suspected CAD or Severe mitral valve disease - shows LV pressure gradient,↓ ventricular volume, ↑ LVEDP
  29. 29.  Pharmacological Surgical Non Surgical Alternatives Implantable Cardioverter Defibrillator(ICD) Cardiac Transplantation
  30. 30.  β Blockers- mainstay of therapy relieves symptoms of exercise intolerance & dyspnoea assoc with CHF by- negative inotropic effect -HR reduction -lower myocardial O2 demand - longer diastolic filling times CCB-Verapamil is indicated if β Blockers not tolerated or ineffective -it improves diastolc function & ventricular relaxation causing improved filling decreased obstructive features in 50% pts -CCBs with strong vasodilatory effect are C/I in pts with obstructive symptoms Disopyramide- has negative inotropic & vasoconstrictive effects -most effective agent to reduce LVOTO , gradient & relieving the symptoms
  31. 31.  Indications Subaortic gradients≥ 50mmHg frequently assoc with CHF & are refractory to medication Septal Myotomy +Partial Mymectomy thru a transaortic approach relieves the obstruction, reduces the LVOTO gradient, SAM & MR Complications –CHB or septal perforation (0-2%) Mortality rate-1to 3% Intraop guidance & Evaluation of surgical result by an experienced echocardiographer are essential for the success of the procedure
  32. 32. Nishimura RA et al. NEJM. 2004. 350(13):1320.
  33. 33.  Septal Ablation with Ethanol -Non surgical septal reduction therapy -2-5 ml of Ethanol is adm thru an angioplasty balloon catheter lumen to the first major septal perforator of the LAD - reduce LVOT grad in 85-90% pts immediately -Further ↓in grad & sympt improvement seen over next 3-6mths - Permanent heart blocks ( 5-10%) Dual Chamber or AV Sequential Pacing(DDD) -Exact mechanism unkn -Possible mech: Excitation of the septum of LV contracts it away from apposing wall which may reduce the LVOT gradient -now rarely recommended since symptoms actually worsen despite gradient reductions
  34. 34. .
  35. 35. Before After
  36. 36.  HCM is the most common cause of SCD in otherwise healthy young individuals VT /VF is primarily responsible for SCD Identification of High Risk Individuals is very important -Pts < 30yrs at the time of diagnosis -Prior cardiac arrest -Symptomatic VT on Holter monitor -Family H/O SCD or Syncope The only effective modality to prevent SCD in HCM pts is an ICD Pharmacological therapy for prevention of SCD in these pts has been abandoned
  37. 37.  Aim of Anaesthetic management - Avoid aggravating the subaortic gradient Anaesthetic goals for a patient with HCM are same for cardiac or non cardiac surgery : Preload- Increased Afterload-Increased Contractility-Depressed Avoid tachycardia, Inotropes, Vasodilators To achieve these, -Maintain adequate volume status -Avoid direct or reflex increase in HR or contractility by heavy premedication & maintaining adequate anaesthesia & analgesia -Continuation of β blockers or CCBs upto the day of sx & restart immediately after sx -Use of vasoconstrictors to maintain MAP or CPP instead of Inotropes
  38. 38.  Induction- IV Narcotics/ Propofol in carefully titrated doses can be used Maintenance-Halothane is advantageous because of its negative inotropic & chronotropic effect Intraop Hypotension- Trendelenburg position, Volume replacement, & Vasoconstrictors Arrythmia management -Asymptomatic Nonsustained VT-benign -Pts with ICD device needs to be suspended in presence of Electrocautery -Chronic AF :B Blocker+Verapamil -Amiodarone is effectve in restoring NSR in pts with HCM Monitoring ECG-closely monitor for arrythmias CVP/PAC/TEE- for volume status, Hemodynamic monitoring Avoid Inotropes, B agonists & Calcium
  39. 39. HCM Athletic heart Can be asymmetric  Concentric & regresses with deconditioning Wall thickness: > 15 mm  < 15 mm LA: > 40 mm  < 40 mm LVEDD : < 45 mm  > 45 mm Diastolic function: always  Normal abnormal  Occurs in about 2% of elite althetes – typical sports, rowing, cycling, canoeing 46 of 48
  40. 40.  WHO in 1995 defined RCM as restrictive filling & reduced diastolic volume of either or both ventricles with normal or near normal systolic function & wall thickness Classification of RCM Myocardial Nonifiltrative – Idiopathic, Familial, HCM, Diabetic Infiltrative- Amyloidosis, Sarcoidosis Storage diseases- Haemochromatosis, Glycogen storage diseases Endomyocardial Endomyocardial fibrosis Carcinoid Radiation Drug Induced –Serotonin, Methysergide, Busulfan, Ergotamine
  41. 41.  Hallmark –Abnormal Diastolic Dysfunction Impaired ventricular relaxation & abnormal Compliance causes rapid filling in early diastole & impeded filling during rest of diastole Characteristic -Ventricular diast waveform of Dip & Plateau (Square root sign) -RA pressure waveform-M or W shaped due to rapid y descent Pressure in the ventricle rises precipitously in response to small volume Both ventricles appear thick with small cavities in contrast to corresponding dilated atria Lt sided Pulmonary venous pressure >Rt sided venous pressure by 5mmHg PASP↑↑ upto 50mmHg Either RVF or LVF or BVF
  42. 42.  Symptoms of Rt &/or Lt heart failure Kussmaul’s sign- ↑ JVP during inspiration Pulsus paradoxus- infrquent CXR- pulmonary congestion, small heart size ECG- BBBs, low voltage, QR or QS complexes 2D Echo
  43. 43. Characteristic RCM Constrictive PericarditisJugular venous ↑ with more rapid y descent ↑ with less rapid y descentwaveformParadoxical Pulse Rare FrequentAuscultation Late S3, low pitched, S4 occ Early S3,highpitched, No S4Heart size N to ↑ N to ↑MR/TR Frequently present Frequently absentCXR Pericardial calcification rare commonECG Conduction abn common rareECHO Major enlargmt of Atria Slight enlargmt of AtriaLAP>RAP Always AbsentRVP waveform Square root pattern, Dip & Square root pattern Plateau less prominentRVEDP/LVEDP LVEDP>RVEDP by ↑ ↑ & Equal 5mmHgCT/MRI Rarely thickened Thickened pericardium>3mm pericardiumEndomycardial Non specific abn NormalBiopsy
  44. 44.  Idiopathic Diuretics-To relieve congestion B-blockers, Amiodarne, CCBs- Control of HR Long term anticoagulation CCBs, ACEI- To enhance myocardial relaxation Dual Chamber Pacing- AV block Cardiac Transplantation- Refractory Heart Failure Amyloidosis- Melphelan, prednisone, H+L transplant Haemochromatosis- Phlebotomy, Desferrioxamine Carcinoid- Somatostatin analogs, Valvuloplasty/Valve replacement Sarcoidosis –Steroids , Pacing, ICD, Transplantation EMF with eosinophilic cardiomyopathy: Endocardiectomy +TV/MV replacement
  45. 45.  Adults with RCM present for CT or MVR/TVR Diastolic dysfunction + filling abn- Poor CO & systemic perfusion Aggresive preop diuretic tharapy- Severe hypovolemia Pulmonary Congestion leads to ↑ Airway pressures Induction-Avoid drugs causing ↓ venous return, bradycardia & myocardial depression Fentanyl (30u/kg), Sufentanyl, Etomidate , Ketamine provide stable hemodynamics for induction Remifentanil, Propofol –unsuitable Invasive hemodynamic monitoring & TEE Inotropic support to maintain CO Diuretics / Vasodilators may be deleterious because higher filling pressures are needed to maintain the CO
  46. 46.  Progressive replacement of RV myocardium with fat & fibrous tissue creating an excellent envt of fatal arrythmias Typical involves regional RV→Global RV→Partial LV involvement with sparing of septum Familial Inheritance, adolescents Presentation -Onset of Arrythmias from RV range fromVPCs-VF -SCD 75% due to VT/VF in sports related exercise -CHF 25% -Progressive RV & LV Dysfunction Diagnosis- Genetics, ECG, Serial Echo, EM Biopsy ECG-Inverted T waves (Rt precordial leads) QRS >110ms Extrasystoles +LBBB
  47. 47.  Any Family H/O SCD or syncope at an early age must alert the anaesthesiologist Arrythmias are more likely in the periop period Intraop /Postop Avoid any noxious stimuli Light anaesthesia Inadequate analgesia Hypercarbia Hypovolemia Acidosis-detrimental due to its effect on arrythmia generation & myocardial function GA perse doesn’t appear to be arrythmogenic Propofol , Midazolam, fentanyl-successfully used Amiodarone- Antiarrythmic of choice during Anaesthesia

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