UNMCRC# CARDIAC ANAESTHESIA

1. CARDIOMYOPATHY
Presentor Dr jeevraj rajawat
Moderator Dr jigisha pujara
Dr nirav parikh

2. Cardiomyopathy
WHO Classification
anatomy & physiology of the LV
1. Dilated
• Enlarged
• Systolic dysfunction
2. Hypertrophic
• Thickened
• Diastolic dysfunction
3. Restrictive
• Myocardial stiffness
• Diastolic dysfunction
4. Arrhythmogenic RV dysplasia
• Fibrofatty replacement
5. Unclassified
• Fibroelastosis
• LV noncompaction

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. • 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
Pathophysiology

6. Pressure volume loops IN dilated
cardiomyopathy
• .. The slope of the end-systolic
pressure volume relation (ESPVR,
arrow) is an excellent load-
independent index of myocardial
contractility.
• EDV and ESV increase
• Decrease ESP and ESPVR
• Compared with the normal heart
(A), the dilated cardiomyopathy
heart is enlarged, and has
depressed contractility and
diastolic dysfunction. Dashed line
in B represents normal ESPVR. LV
= left ventricular.
• (From Hare JM: Etiologic basis of
congestive heart failure

7. Clinical presentation of DCM
• The clinical picture of DCM typically
includes signs and symptoms of CHF
often corresponding to months of
fatigue, weakness, and reduced
exercise tolerance before diagnosis.
• One-third of individuals report chest
pain. However, the first indication of
DCM may be a stroke, arrhythmia, or
even sudden death.
• Physical signs of DCM, depending on
the disease's progression, include
pulsus alternans, jugular venous
distention, murmurs of
atrioventricular valvular
regurgitation, tachycardia, and gallop
heart sounds

8. Prognosis in various dilated
cardiomyopathy

9. Diagnosis
• chest roentgenogram demonstrates variable
degrees of cardiomegaly and pulmonary venous
congestion
• An ECG may be surprisingly normal or depict low
QRS voltage, abnormal axis, nonspecific ST-
segment abnormalities, left ventricular
hypertrophy, conduction defects, and evidence of
atrial enlargement.
• Atrial fibrillation is common, and about one
fourth of patients have nonsustained ventricular
tachycardia.

10. ECHO
• dilated mitral annulus with incomplete mitral leaflet
coaptation causing functional MR, evidence of low
cardiac output (decreased excursion of mitral leaflets),
enlarged atrial cavities, RV enlargement, and
occasionally apical mural thrombus.
• Intraventricular mechanical dyssynchrony caused by
left or right bundle branch block is common and
contributes to further cardiac dysfunction.
• In view of the incidence (20% to 50%) of familial
dilated cardiomyopathy, echocardiographic screening
of immediate family members is recommended

11. • In dilated cardiomyopathy, the LV
cavity is enlarged and global
systolic function is decreased]
• End-diastolic and end-systolic
dimensions and volumes are
increased,
• With gradual dilation, the LV
cavity becomes more spherical,
with a sphericity index (short-axis
dimension/long-axis dimension)
nearing the value of 1 (normally,
≥1.5).
• LV mass is uniformly increased
(eccentric hypertrophy), and wall
thickness is typically within
normal limits.

12. • Apical four-chamber view (left) and pulsed-
wave Doppler velocity recording of mitral
valve inflow (right) in a patient with dilated
ischemic cardiomyopathy showing a
relaxation abnormality pattern with
increased A velocity. Patients with this type
of diastolic filling pattern usually have
minimal to mild symptoms, despite severe LV
systolic dysfunction.
• B, In this patient with dilated
cardiomyopathy, the MV inflow velocity
pattern shows restrictive physiology, with a
markedly decreased A velocity and an
increased E/A ratio. Deceleration time (DT) of
mitral E velocity is shortened. Patients with
this type of diastolic filling have increased
filling pressure and symptomatic congestive
heart failure

13. • Tissue Doppler recording
of the septal mitral
annulus in a patient with
dilated cardiomyopathy.
Showing decreased
systolic velocity (S′), early
diastolic velocity (E′), and
prominent bidirectional
velocities during
isovolumic relaxation

14. MANAGEMENT OF dilated
cardiomyopathy
• angiotensinconverting enzyme inhibitors suppress
ventricular remodeling and endothelial dysfunction,
accounting for the improvement in mortality noted with
this medication in DCM.
• Other afterload-reducing agents, such as selective
phosphodiesterase-3 inhibitors like milrinone, may improve
quality of life but do not affect mortality, and thus are
rarely administered in chronic situations.
• More recently, spironolactone has assumed a greater role
in treatment as mortality rate was reduced by 30% from all
causes in patients receiving standard angiotensin-
converting enzyme inhibitors for DCM with the addition of
spironolactone in a large, double-blind, randomized trial.

15. • However, compared with ischemic CHF, patients
with nonischemic DCM show greater
improvement in symptoms, left ventricular
function, and remodeling
• Treatment revolves around management of
symptoms and progression of DCM, whereas
other measures are designed to prevent
complications such as pulmonary
thromboembolism and arrhythmias.
• The mainstay of therapy for DCM is vasodilators
combined with digoxin and diuretics.

16. Anaesthestic management of DCM Pt
undergoing cardiac surgery
• Individuals with DCM are extremely sensitive to
cardiodepressant anesthetic drugs.
• Intravenously administered anesthetic agents such as
fentanyl (30 μg/kg) provide excellent anesthesia and
hemodynamics in patients with ejection fractions less
than 30% but contribute to prolonged respiratory
depression delaying extubation.
• Shorter-acting narcotics such as remifentanil may be
unsuitable for patients with poor left ventricular
function undergoing cardiac surgery because of high
incidences of bradycardia and severe hypotension.
• .

17. • Ketamine has been recommended for induction in critically ill
Patients. because of its cardiovascular actions attributed mainly to a
sympathomimetic effect from the central nervous system.
• This makes ketamine (less than 0.5 mg/kg) an excellent choice to
use in combination with fentanyl for induction in patients with
severe myocardial dysfunction secondary to cardiomyopathy.
• The use of propofol with cardiomyopathy may be a concern
because cardiovascular depression has been observed possibly
because of inhibition of sympathetic activity and a vasodilatory
property.
• However, in a cardiomyopathic hamster model, there was no direct
effect on myocardial contractility with propofol

18. • Volatile agents have long been a concern in
persons with failing hearts because of their
known depressant effects on myocardial
contractility.
• The effect of currently used volatile anesthetics
on intrinsic myocardial contractility is difficult to
project clinically.
• Animal data indicate halogenated volatile agents
may have more profound Negative inotropic
effects in cardiomyopathic muscle than healthy
cardiac muscle.

19. Hemodynamic management during
intraop
• During induction if Hemodynamic instability occur with DCM managed by
with a low dose of inotrope and vasodilator.
• Phosphodiesterase-3 inhibitors are useful if combined with another β-
adrenergic agonist, such as milrinone and epinephrine, for short-term
hemodynamic support.
• Afterload reduction in DCM is important because it improves regional and
global indices of ventricular relaxation and ejection fraction during
anesthesia when myocardial depression may be significant.
• It also may reduce valvular regurgitation and atrial volumes.
• The use of levosimendan, an effective calcium-sensitizing agent with
vasodilatory and inotropic features, may be an excellent agent to maintain
hemodynamics because it also increases myocardial performance without
major changes in the oxygen consumption or effects on the diastolic
function
• Arrhythmogenic factors such as hypokalemia, hypomagnesemia, and
sympathetic activation should be monitored and corrected

20. • Dual Chamber Pacing
• Cardiomyoplasty LIKE DOR AND BARISTA
PROCEDURE
• 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%
Pts refractory to Pharmacological therapy for
CHF

21. Stress (Tako-tsubo or Broken Heart
Syndrome)
• Takotsubo cardiomyopathy (TCM) is a transient cardiac
syndrome that involves left ventricular apical akinesis and
mimics acute coronary syndrome. It was first described in
Japan in 1990 by Sato et al.
• An acute cardiomyopathy can be provoked by a stressful or
emotional situation or exposure to high doses of
catecholamines (sympathomimetic drugs
• Patients often present with chest pain, have ST-segment
elevation on electrocardiogram, and elevated cardiac
enzyme levels consistent with a myocardial infarction.
• This cardiomyopathy is most common among middle-aged
women, appears to be related to catecholamine release,
and in most cases is fully reversible with supportive care

22. HOCM
• Hypertrophic cardiomyopathy (HCM), the most common of
the genetic cardiovascular diseases, is caused by a
multitude of mutations in genes encoding proteins of the
cardiac sarcomere.
• HCM is characterized by heterogeneous clinical expression,
unique pathophysiology, and diverse natural history.
• The first contemporary reports of HCM in 1958 are from
Brock in the cardiac catheterization laboratory and from
Teare, who described at autopsy “asymmetrical
hypertrophy of the heart” as responsible for sudden cardiac
death in a small group of young people
• However, since obstruction to LV outflow is not invariable
and about 30% of patients have the nonobstructive form

23. Massive left ventricular
hypertrophy, mainly
confined to the septum
Histopathology showing
significant myofiber
disarray and interstitial
fibrosis
Increased connective tissue, combined with markedly
disorganized and hypertrophied myocytes, contributes to the
diastolic abnormalities of HCM that manifest as increased
chamber stiffness, impaired and prolonged relaxation, and an
unstable EP substrate that causes complex
arrhythmias and sudden death

24. Etiology
Familial in ~ 55% of cases with autosomal dominant transmission
Mutations in one of 4 genes encoding proteins of cardiac sarcomere
account for majority of familial cases
-MHC
cardiac troponin T
myosin binding protein C
-tropomyosin
Remainder are
spontaneous
mutations.

25. Pathophsiology of hocm

26. ECG IN HOCM
• Most common abnormalities include
increased voltages consistent with LV
hypertrophy, ST-T changes including marked T
wave inversion in the lateral precordial leads,
left atrial enlargement, deep and narrow Q
waves, and diminished R waves in the lateral
precordial leads.

27. Patterns of LV hypertrophy
in HCM. Heterogeneous
distribution
. A, Massive asymmetric hypertrophy
of ventricular septum (VS) with
thickness >50 mm
. B, Septal hypertrophy with distal
portion considerably thicker than
proximal region.
C, Hypertrophy confined to proximal
septum just below aortic valve
(arrows).
D, Hypertrophy localized to LV apex
(asterisk), that is, apical HCM.
E, Relatively mild hypertrophy in
symmetric pattern showing similar or
identical thicknesses within each
segment (paired arrows).
F, Inverted pattern with posterior free
wall (PW) thicker (40 mm) than
anterior VS. Calibration marks = 1 cm.
Ao = aorta;

28. Role of cardiac MRI IN HOCM
DIAGNOSIS
• CMR is complementary to echocardiography by
clarifying technically ambiguous LV wall
thicknesses, by visualizing abnormalities often
not identifiable with echocardiography (e.g.,
areas of segmental hypertrophy in the
anterolateral free wall, or by depicting pathologic
changes in the apical region including
hypertrophy and aneurysm formation that may
clarify diagnosis or in some patients alter
management strategies

29. MANAGEMENT IMPLICATIONS: ECHOCARDIOGRAM (C) AND COMPARATIVE CMR IMAGE (D) FROM 46-YEAR-
OLD MAN WITH HCM. C, ECHOCARDIOGRAPHIC SHORT-AXIS IMAGE SHOWS ANTEROLATERAL FREE WALL
THICKNESS OF 18 MM. D, CMR SHOWS FOCAL AREA OF MASSIVE LV HYPERTROPHY (WALL THICKNESS, 35 MM)
IN THE SAME REGION OF LV, SIGNIFICANTLY UNDERESTIMATED BY TWO-DIMENSIONAL ECHOCARDIOGRAPHY.
THIS FINDING DEFINED HIGH-RISK STATUS, PROMPTING ALTERED MANAGEMENT STRATEGY WITH AN ICD
RECOMMENDATION FOR PRIMARY PREVENTION OF SUDDEN DEATH

30. SAM IN HOCM
• The narrowing of the LVOT by septal
hypertrophy necessitates an increase
in flow velocity through the LVOT.
• This increase in flow velocity draws
the anterior leaflet of the mitral valve
into the LVOT, called the Venturi effect.
The mitral leaflet and apparatus
• subsequently cause obstruction of
flow through the LVOT, as well
• as mitral regurgitation.
• Temporally, mitral regurgitation
occurs after LVOT obstruction:
ejection → obstruction to left
ventricular outflow
• → mitral regurgitation. The jet of
mitral regurgitation typically
• is directed posterolaterally

32. MR IN HOCM
• A further consequence of SAM is
production of mitral regurgitation in
mid- or late systole as the anterior
leaflet moves forward.
• Mitral regurgitation occurs
independently of SAM in about 20%
of patients with HOCM.
• It can result from mitral valve
prolapse, chordal rupture, anomalous
attachment of a papillary muscle to
the anterior leaflet, extensive
anterior leaflet fibrosis resulting from
repeated mitral leaflet–septal
contact, congenital abnormalities,
rheumatic disease,
• Mitral anular calcification is
frequently present in older patients
with HOCM.

33. SUDDEN DEATH AND HOCM
• Sudden death in HCM may occur at a
wide range of ages but most
commonly in adolescents and young
adults <30 to 35 years of age (
• These events are arrhythmia based,
caused by primary ventricular
tachycardia and ventricular
fibrillation.[
• Sudden death is often the initial
clinical manifestation of HCM,
occurring commonly in asymptomatic
individuals, many of whom are
undiagnosed during life.
• HCM is the most common
cardiovascular cause of athletic field
deaths

35. • 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

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

37. Signs
• The three cardinal signs of HOCM are
• (1) late-onset systolic ejection murmur
between the left sternal edge and apex,
• (2) bifid arterial pulse, and
• (3) palpable left atrial contraction.

39. Cardiac Catheterization
LV pullback
Brockenbrough-Braunwald Sign
failure of aortic pulse pressure to rise post PVC
Provocative maneuvers:
Valsalva
amyl nitrate inhalation

40. Diastolic Filling Pattern and Tissue
Doppler Imaging
• Markedly impaired
myocardial relaxation is the
predominant diastolic
abnormality in HCM.
• Atrial contraction makes an
important contribution to
LV filling,
• As compliance decreases
and LA pressure increases,
early filling gradually
increases and DT decreases.
• .

41. • Mitral annulus velocity,
Ea, reflects the status of
myocardial relaxation
and is reduced in most
patients with HCM

42. • Continuous-wave Doppler
spectra obtained from the
apex showing dynamic LVOT
obstruction in hypertrophic
cardiomyopathy. Note the
typical late-peaking
configuration resembling a
dagger or ski slope (arrow).
The baseline (left) velocity is
2.8 m/sec, corresponding to
the peak LVOT gradient of 31
mm Hg (= 4 ? 2.82). With the
Valsalva maneuver (right), the
velocity increased to 3.5
m/sec, corresponding to the
gradient of 50 mm Hg

43. MANAGEMENT OF HOCM

44. Treatment options
• Surgical like septal myectomy
• Dual Camber (DDD pacemaker
• ETOH septal ablation
• AICD to prevent sudden death

45. Surgical septal myectomy
• 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
• Does not prevent Sudden Cardiac Death
• Myomyectomy
• removal of small portion of upper IV
septum
• +/- mitral valve replacement
• Intraop guidance & Evaluation of surgical result
by an experienced echocardiographer are
essential for the success of the procedure
• 5 year symptomatic benefit in ~ 70% of patients

46. GOAL OF ANAESTHETIC
MANAGEMENT
• Anaesthetic technique and the perioperative
management of these patients must aim to
maintain haemodynamic stability, maintaining
adequate preload and afterload.
• Avoiding vasodilators and avoiding agents that
increase contractility are pivotal in the
management of these patients.
• Intra-operative goals include maintaining sinus
rhythm, minimum stressful stimuli and minimize
or prevent dynamic LV outflow tract obstruction

47. • The characteristic diastolic dysfunction makes
the heart sensitive to changes in volume,
contractility, and SVR.
• Because of this diastolic dysfunction, an acute
increase in the pulmonary artery pressure may
warn of the rapid onset of pulmonary
congestion and edema.
•

48. ANAESTHESIA MANAGEMENT
• This patient was adequately premedicated to reduce
perioperative anxiety.
• Such patients should be given adequate premedication,
to reduce stress response (like benzodiazepines,
opioids).
• Anaesthesia was induced with low doses of propofol
and fentanyl.
• Rapid inj of such drugs can lead to hypotension and
tachycardia which are not desirable in such patients..
• Vecuronium is muscle relaxant that can be used as it
decreases heart rate and thus avoids LV outflow tract
obstruction

49. HEMODYANAMIC MANAGEMENT
• Episodes of hypotension can be treated with trendelenburg
position, volume replacement and/or vasoconstrictors like
norepinephrine or phenylephrine.
• Dobutamine was used in minimal dose under-TOE monitoring after
relieving obstruction during weaning.
• Dobutamine should be used cautiously in preoperative period as it
can cause tachycardia and decrease in afterload, aggravating the
obstruction.
• b-receptor antagonists, in contrast, decrease the pressure gradient
and improve cardiac output by decreasing outflow obstruction.
• Episodes of tachycardia in the perioperative period may be treated
with b-receptor antagonist like esmolol or metoprolol
• Arrhythmogenic factors such as hypokalemia, hypomagnesemia,
and sympathetic activation should be monitored and corrected

50. • Adequate preload should be the goal by use CVP and
TOE monitoring.
• CVP should be kept high as adequate preload is
necessary to maintain optimal cardiac output and
avoid any undue increase in contractility because of
hypovolemia.
• TOE helps to assess filling conditions.
• A pulmonary artery catheter may be desirable in
patients to optimize filling conditions particularly in
patients with compromised myocardial function but
carries the risk of arrhythmia and adds cost to patient

51. TEE IN MYOMECTY
• Before incision, TEE can identify the location of hypertrophy in
relation to the aortic annulus, as well as the thickness of the
ventricular septum, which assists the surgeon in planning the
location and depth of the myectomy
• The mitral valve should be closely evaluated for abnormalities that
may contribute to mitral regurgitation, particularly if the regurgitant
jet is directed centrally or anterior.
• After the myectomy, TEE is used to assess the degree of residual
mitral regurgitation and evidence of continued systolic anterior
motion (SAM) and LVOT obstruction
• TOE also helps in assessing volume status of patients.

52. • these jets of blood flow are not
confused with a ventricular septal
defect.
• The ventricular septum also must be
closely evaluated for evidence of
shunting via an iatrogenic ventricular
septaldefect (VSD).
Doppler imaging reveals two jets of blood
flow into the left ventricular outflow
(arrows) tract from small coronary
perforators that were transected during the
myectomy

53. ARRYTHMIA IN WEANING OR POST OP
• Atrial fibrillation and junctional rhythm are poorly
tolerated in HOCM patients due to the loss of atrial kick.
• Arrhythmias, particularly atrial fibrillation require prompt
treatment.
• Beta blockers and amiodarone are drugs to control
arrhythmias.
• Digoxin is unsuitable because of its positive inotropic
effects.
• Calcium channel blockers should be used with caution
because of the potential for systemic vasodilation. b-
blockers like esmolol are helpfuL
• Amiodarone is especially effective in restoring normal sinus
rhythm in HCM and reducing recurrences

54. Septal ablation with alcohol
• first attempted in 1994 based on interruption
of blood supply to the interventricular
septum, causing infarction.
• This resulted in reduced septal thickness and
LVOT modeling with resolution of symptoms
in HCM.
• Alcohol is administered through an
angioplasty balloon to the first major septal
perforator of the left anterior descending
coronary artery.
• Reduce LVOT grad in 85-90% pts
immediately
-Further ↓in grad & sympt improvement
seen over next 3-6mths
- Permanent heart blocks ( 5-10%)
• The study showed effective gradient
reduction and improvement in NYHA class
comparable with myectomy 12 months after
ablation

55. ICD

56. Athlete's Heart Versus Hypertrophic
Cardiomyopathy
• LV hypertrophy occurs after years of intense athletic training, and the two-
dimensional echocardiographic findings of athlete's heart are similar to those of
HCM (The following characteristics of athlete's heart can differentiate it from HCM:
•
• • Symmetric hypertrophy
•
• • Hypertrophy is rarely greater than 17 mm.
•
• • LV cavity dimension is increased, whereas it is decreased in HCM.
•
• • Diastolic function is normal (Ea >7 cm/sec).
•
• • Tissue Doppler velocities and strain values are normal.
•

57. DDD PACING IN HOCM
• Preexcitation of the right ventricular apex alters the septal
activation sequence, reducing the LVOT gradient and mitral
regurgitation severity and possibly attenuating long-term
LV remodeling.
• This therapy has shown a small benefit in randomized
clinical trials, particularly in patients older than 65 years.
• The diastolic function of patients with obstructive HCM
may show long-term effects from continued DDD pacing.
• Additionally, prolonged ventricular pacing can negatively
impact LV systolic function, leading to long-term clinical
decline.

58. RESTRICTIVE CARDIOMYOPATHY
• 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
• infiltration of myocardium by abnormal
substance cause fibrosis or scarring of
endocardium

59. Amyloid infiltrative CM

60. Pathophysiology of Restriction
Elevated systemic and pulmonary venous pressures
right and left sided congestion
reduced ventricular cavity size with SV and CO

61. • Symptoms of Rt &/or Lt heart failure BUT Right > Left heart
failure
• Dyspnea
• Orthopnea/PND
• Peripheral edema
• Ascites/Hepatomegaly
• Kussmaul’s sign- ↑ JVP during inspiration
• Pulsus paradoxus- infrquent
• CXR- pulmonary congestion, small heart size
• ECG- show BBBs, low voltage, QR or QS complexes
Clinical Features/Diagnosis

62. • Early in the disease, LV
SIZE AND systolic
function is usually
preserved, but diastolic
function is abnormal,
which causes increased
atrial pressures and
marked biatrial
enlargement

63. • Restrictive cardiomyopathy is accompanied
by restrictive diastolic filling,
• . The increase in LA pressure causes (1) the
mitral valve to open at a higher pressure,
resulting in a decrease in IVRT; (2) an
increased transmitral pressure gradient with
associated increased mitral E velocity; and
(3) a decrease in pulmonary venous flow
velocity during systole and an increase during
diastole
• With high LVEDP, the atrial contribution to
ventricular filling is minimal, and A velocity is
usually decreased , causing the E/A ratio to
increase markedly (>2).
• In contrast to constrictive pericarditis,
hepatic vein diastolic flow reversal is greater
during inspiration .
• Myocardial relaxation is universally impaired
so that mitral annulus E′ velocity obtained
from the septal annulus is usually less than 7
cm/sec

64. Cardiac Catheterization
Prominent y descent “dip and plateau”Sqaure root sign
rapid atrial emptying rapid ventricular filling
then abrupt cessation of blood flow due to non-compliant myocardium

65. Constriction vs. Restrictive CM

67. • Adults with RCM present for CT or MVR/TVR
• Aggresive preop diuretic tharapy- Severe hypovolemia
• Induction-Avoid drugs causing ↓ venous return, bradycardia & myocardial depression
• Ketamine is an excellent choice to use with fentanyl for induction in patients with
severe myocardial dysfunction and low cardiac output due to cardiomyopathy
• Invasive hemodynamic monitoring Invasive hemodynamic monitoring is important
because both left and right filling pressures do not accurately reflect ventricular
volumes, so small volume shifts may greatly impact CO.
• Inotropic support may be indicated to support hemodynamics because the risk for
death from low CO is high
• Patients may be chronically taking amiodarone, which has a negative inotropic effect
that interacts with volatile agents to further reduce contractility and conduction,
requiring careful titration of these anesthetic agent
• Diuretics / Vasodilators may be deleterious because higher filling pressures are
needed to maintain the CO
Anaesthetic Considerations

68. • ARVC is familial in 30% to 50% of cases, with primarily autosomal dominant
inheritance
• The disease is now known to proceed through three phases: (1) concealed phase
without symptoms but some EP changes that place patients at risk for sudden
death; (2) overt arrhythmias; and (3) advanced stage with myocardial loss,
biventricular involvement with CHF
• Progressive replacement of RV myocardium with fat & fibrous tissue creating an
excellent cUSE of fatal arrythmias
• Diagnosis is based on ECG, structure, genetic studies, and arrhythmias.
• The characteristic ECG includes inverted T waves in the right
• precordial leads, QRS prolongation > 110 milliseconds, and extrasystoles with left
bundle branch block.
• Sudden death occurs most often during sports-related exercise, primarily from
ventricular tachycardia/ fibrillation.Although a rare disease, it accounts for 20% of
sudden deaths in the young
• Diagnosis may depend on endomyocardial biopsy to reveal the distinctive changes of
ARVC
Arrythmogenic RV Cardiomyopathy (ARVC)

69. Arrhythmogenic Right Ventricular
Dysplasia/Cardiomyopathy
• The systolic and diastolic RVOT and RV inflow
tract dimensions are increased, with diastolic
RVOT enlargement being most common
(RVOT diameter was >25 mm in all patients).
An RVOT dimension greater than 30 mm
from the parasternal long-axis view had the
highest sensitivity (89%) and specificity (86%)
for the diagnosis of arrhythmogenic RV
dysplasia.
• RV function was abnormal in and RV
regional wall motion abnormalities were
frequent, most commonly affecting the apex
and anterior wall.
• RV trabecular derangements, hyperreflective
moderator band, and sacculations are
present
• In some patients, LV dilation and dysfunction
also occur
• .

70. TREATMENT
• ICD therapy,
• Antiarrhythmic agents,
• Radiofrequency ablation,
• HF treatment,
• Surgical treatment / cardiac
transplantation

71. Anaesthetic Considerations
• Because arrhythmias are more likely in the perioperative period,
noxious stimuli, hypovolemia, hypercarbia, and light anesthesia
must be minimized intraoperatively and during recovery.
• However, general anesthesia alone does not appear to be
arrhythmogenic.
• . Anesthesia has been conducted successfully with propofol,
midazolam, and fentanyl.
• Acidosis may be especially detrimental because of its effect on
arrhythmia generation and myocardial function.
• Currently, there are no guidelines for arrhythmia prophylaxis.
• Amiodarone is the first line of antiarrhythmic medication during
• anesthesia.
• Empiric administration of antiarrhythmia agents is ineffective
• at preventing arrhythmias or sudden death in these patients.

72. • THANK YOU