The document discusses various types of cardiomyopathies including dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), and restrictive cardiomyopathy (RCM). It provides details on the definition, causes, symptoms, diagnostic criteria and treatment options for each type. DCM is the most common cardiomyopathy, usually caused by idiopathic or viral factors, leading to ventricular dilation and dysfunction. HCM is a genetic disease causing left ventricular hypertrophy that can lead to obstruction of blood flow. RCM involves restrictive filling of the ventricles with normal wall thickness.
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.
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
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
9.
10.
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
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
27.
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
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
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
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
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
49.
50.
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
52.
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