1. Dr Tarun Bhatnagar
Consultant Cardiac Anaesthesiologist
dr.tarunbhatnagar@yahoo.co.in

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)

4.  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

5. Medslides.com 5
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48

6.  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

7.  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

8.  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

11.  Aim of treatment
-Manage the symptoms
-Reduce the progression of disease
-Prevent Complications
 Mainstay of Therapy
Vasodilators
+
Digoxin
+
Diuretics

12.  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

13.  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

14.  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

15.  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%

16.  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

17.  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

18.  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

19.  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

20.  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

21. Massive left ventricular
hypertrophy, mainly
confined to the septum
Histopathology showing
significant myofiber
disarray and interstitial
fibrosis

22.  Causes:
Inherited, acquired,
unknown
 Autosomal
dominant
inheritance pattern
 >450 mutations in 13
cardiac sarcomere &
myofilament-related
genes identified
 ?? Role for
environmental
factors

23. Hypertrophic cardiomyopathy: variants
Hypertrophic cardiomyopathy morphology exhibits heterogeneity.
The mostcommon variant is assymetric septal hypertrophy involving
the entire septum

24.  Subaortic Obstruction
 Diastolic Dysfunction
 Myocardial Ischemia
 Mitral Regurgitation
 Arrythmias

25.  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

26.  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

28.  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

29. Hypertrophied & disorganized myocytes with ↑CoT
Impaired relaxation+ ↑ Chamber stiffness
Diastolic Dysfunction→↓ rate of rapid ventricular filling
↑ atrial systolic filling
↑ Filling pressures & pulmonary congestion

30.  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

31.  Dyspnea on exertion (90%)
 Angina (70-80%)
 Syncope (20%)
 Sudden cardiac death

32.  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

36.  Pharmacological
 Surgical
 Non Surgical Alternatives
 Implantable Cardioverter Defibrillator(ICD)
 Cardiac Transplantation

37.  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

38.  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

39. Nishimura RA et al. NEJM. 2004. 350(13):1320.

40.  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

41. .

42. Before After

43.  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

44.  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

45.  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

46. 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

47.  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

48.  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

51.  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

53. Characteristic RCM Constrictive Pericarditis
Jugular venous ↑ with more rapid y descent ↑ with less rapid y descent
waveform
Paradoxical Pulse Rare Frequent
Auscultation Late S3, low pitched, S4 occ Early S3,highpitched, No S4
Heart size N to ↑ N to ↑
MR/TR Frequently present Frequently absent
CXR Pericardial calcification rare common
ECG Conduction abn common rare
ECHO Major enlargmt of Atria Slight enlargmt of Atria
LAP>RAP Always Absent
RVP waveform Square root pattern, Dip & Square root pattern
Plateau less prominent
RVEDP/LVEDP
LVEDP>RVEDP by ↑ ↑ & Equal
5mmHg
CT/MRI Rarely thickened Thickened pericardium>3mm
pericardium
Endomycardial Non specific abn Normal
Biopsy

54.  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

55.  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

56.  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

57.  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