Adhantoro Rahadyan

Esnawan Antariksa Hospital

European Society of Cardiology (ESC):

Diseases with abnormal myocardial

structure or function in the absence of
ischemic insult or loading conditions
capable of causing such disorder
(CAD, hypertension, valvular disease,
congenital heart disease).
WHO Classification
anatomy & physiology of the LV
1. Dilated
• Enlarged
• Systolic dysfunction
2. Hypertrophic
• Thickened
• Diastolic dysfunction
3. Restrictive
• Diastolic dysfunction
4. Arrhythmogenic RV dysplasia
• Fibrofatty replacement
5. Unclassified
• Fibroelastosis
• LV noncompaction

Circ 93:841, 1996

 Mutation Exogenous
insult
Contraction and relaxation
disorder
Ineffective energy utilization
Altered Ca ions handling
Activation of compensatory
neurohumoral mechanisms
Apoptosis
Fibrosis
Hypertrophy

Heart failure Arrhythmia, sudden Thromboembolic

death complication
Left ventricular hypertrophy not due to pressure overload
Hypertrpohy is variable in both severity and location:
-asymmetric septal hypertrophy
-symmetric (non-obstructive)
-apical hypertrophy

Vigorous systolic function, but impaired diastolic function

impaired relaxation of ventricles
elevated diastolic pressures

prevalence as high as 1/500 in general population

mortality in selected populations 4-6% (institutional)
probably more favorable (1%)
 Most common cause of death in young people.

 The magnitude of left ventricular hypertrophy is

directly correlated to the risk of SCD.

 Young pts with extreme hypertrophy and few or

no symptoms are at substantial long-term risk of
SCD.

.
Spirito P. N Engl J Med. 1997;336:775-785.
Maron BJ. N Engl J Med. 2000;342:365-373.
 Sudden Cardiac Death of Athletes

Maron BJ et al, JAMA 1996 ; 276 : 199 - 203

 20
Incidence of Sudden Death

18.2
18
(per 1,000 person/yr)

16
14
12 11.0
10
7.4
8
6
4 2.6
2 0
0
< 15 16-19 20-24 25-29 > 30
Maximum Left-Ventricular-Wall Thickness (mm)

Spirito P. N Engl J Med. 2000;342:1778-

1785.
 Symptoms include :
◦ Shortness of breath with exercise
◦ chest pain (usually with exercise)
◦ Diziness (at rest or with exercise)
◦ blackouts
◦ Palpitations
◦ No symptoms

 Risk of sudden death ~ 1% per year

 Intensive exercise can increase risk
 No cure, but can prevent complications
 Manage symptoms
◦ Medications (Beta-blocker tablets)
◦ Modify lifestyle
◦ Surgery (only in very limited circumstances)
 Ensure family members checked
 Assess risk of sudden death
◦ Low-risk, reassure, but still avoid intense exercise
◦ High-risk, recommend implantable defibrillator (ICD
•Dilation and impaired contraction of ventricles:
•Reduced systolic function with or without heart failure
•Characterized by myocyte damage
•Multiple etiologies with similar resultant pathophysiology

•Majority of cases are idiopathic

•incidence of idiopathic dilated CM 5-8/100,000
•incidence likely higher due to mild, asymptomatic cases
•3X more prevalent among males and African-Americans
Ischemic
Valvular
Hypertensive
Familial
Idiopathic
Inflammatory
Infectious
Viral – picornovirus, Cox B, CMV, HIV
Ricketsial - Lyme Disease
Parasitic - Chagas’ Disease, Toxoplasmosis
Non-infectious
Collagen Vascular Disease (SLE, RA)
Peripartum
Toxic
Alcohol, Anthracyclins (adriamycin), Cocaine
Metabolic
Endocrine –thyroid dz, pheochromocytoma, DM, acromegaly,
Nutritional
Thiamine, selenium, carnitine
Neuromuscular (Duchene’s Muscular Dystrophy--x-linked)
• Sudden death prevention
Based on symptoms and severe systolic
dysfunction

• Heart failure therapy

Drug therapy
Mechanical assist devices
Heart transplant

• Prevention of thromboembolic complications of atrial

fibrillation
 Myocardium of RV free wall replaced:
◦ Fibrofatty tissue
◦ Regional wall motion/function is reduced
 Ventricular arrhythmias
◦ SCD in young
Histologic specimen showing combination of fatty
deposits (white) and interstitial fibrosis (blue)
incorporating atrophic myocytes (red).
 ARVD/C usually is characterized by the occurrence of symptomatic RV
arrhythmias during exercise.
 Islands of fibrofatty tissue may form the arrhythmogenic substrate
underlying these arrhythmias that typically are induced by adrenergic
stimulation.
 During exercise testing, 50% to 60% of patients with ARVD/C show
ventricular arrhythmias: characteristically monomorphic with a
predominant LBBB pattern in 96% (Figure 3).
 Inferior axis: RVOT is site of origin, superior axis: RV inferior wall.
 The occurrence of arrhythmic cardiac arrest due to ARVD/C is significantly
increased in athletes. Particularly in certain regions in Italy, ARVD/C has
been shown to be the most frequent disease (22%) leading to exercise-
induced cardiac death in athletes.
  diagnosis of ARVD/C is considered incompatible with competitive
sports and/or moderate-to-high intensity level recreational activities.
 Natural history of ARVD/C: cardiac electrical instability and
progressive RV dys.
 Mortality rates ranged from 4% to 20%.
 Both sexes have similar mortality risk, with a peak of risk
during the fourth decade.
 May account for up to 5% of SDs in young adults in the US
and 25% of exercise-related deaths in the Veneto region of
Italy.
 Unless SCD occurs, progressive impairment of cardiac
function may result in right or biventricular HF late in the
evolution of ARVD/C, usually in a time course of 4 to 8 years
after typical development of complete RBBB.
 Fontaine et al evaluated the natural history of 130
ARVD/C patients who were followed from 1977 to
2000:
◦ 21 cardiac deaths, of which 14 were due to progressive HF
and 7 to SCD,  an annual mortality of 2.3%.
 In most patients, the mechanism of SD in ARVD/C is
acceleration of VT with ultimate degeneration into
VF.
 Generally, RV failure and LV dysfunction were
independently associated with cardiovascular
mortality.
 There are five therapeutic options in
patients with ARVD/C:
◦ (1) antiarrhythmic agents,
◦ (2) radiofrequency ablation,
◦ (3) ICD therapy,
◦ (4) HF treatment, and
◦ (5) surgical treatment.
 is indicated in case of serious risk of SD.
 Patients at highest risk are
◦ those who have been resuscitated,
◦ those who are unresponsive or intolerant of antiarrhythmic therapy
(secondary prevention), and
◦ those with the disease who have a family history of cardiac arrest in
first-degree relatives (primary prevention).
 There is no evidence that patients who have a positive family
history but no evidence of the disease are at exceptional high
risk.
 Although an ICD is the most effective safeguard against
sudden arrhythmic death in large patient populations with
ARVD/C, its precise role still needs to be Defined.
  458 patients with NYHA Class I to III, NICM, LVEF ≤ 36%
and premature ventricular contractions (> 10/h) or NSVT

 Randomized to standard medical rx alone or in

combination with single-chamber ICD

 Strong trend toward ↓ all-cause mortality with ICD

therapy, although not statistically significant (p=0.08)

Kadish A, Dyer A, Daubert JP, et al. Prophylactic defibrillator implantation in patients with nonischemic dilated cardiomyopathy. N Engl J
Med 2004;350:2151-8.
Kaplan–Meier Estimates of Death from Any Cause (Panel A)
Sudden Death from Arrhythmia
Patients Who Received Standard Therapy and Those Who
Received an Implantable Cardioverter–Defibrillator (ICD)(panel B)
2521 patients with NYHA Class II or III HF, ICM, or NICM and
LVEF ≤ 35% •
Randomized to
1) conventional rx for HF + placebo;
2) 2) conventional rx + amiodarone; or
3) 3) conventional rx + conservatively programmed
shockonly single lead ICD •

• No survival benefit for amiodarone •

• 23% ↓ in overall mortality with ICD therapy •
• Absolute ↓ in mortality of 7.2% after 5 y in the overall
population

Bardy GH, Lee KL, Mark DB, et al. Amiodarone or an implantable cardioverter-defibrillator for
congestive heart failure. N Engl J Med
 Recent onset DCM (9/12) and LVEF <30%
 ICD V/S no ICD
 The trial was terminated after the inclusion of 104 pts
because all-cause mortality rate at 1 year did not reach
expected 30% in control
 Mean follow-up 5.5yrs- 30 deaths (13-ICD, 17-control)
 Cumulative survival was not significantly different
between the two groups (93% and 80% in control V/S
92% and 86% in ICD after 2 and 4 yrs, resp)
 Recent onset DCM (9/12) and LVEF <30% ICD V/S no
ICD
 The trial was terminated after the inclusion of 104
pts because all-cause mortality rate at 1 year did not
reach expected 30% in control
 Mean follow-up 5.5yrs- 30 deaths (13-ICD, 17-
control)
 Cumulative survival was not significantly different
between the two groups (93% and 80% in control
V/S 92% and 86% in ICD after 2 and 4 yrs, resp)
 Multicenter registry study of implanted ICDs in 506 unrelated
patients with HCM @ high risk for SCD (family hx of SCD, [septal
thickness ≥ 30 mm], NSVT, syncope)

 • Mean patient age 42 years (SD=17) and 87% had no or only

mildly limiting symptoms
 ICD interventions appropriately terminated ventricular
tachycardia/fibrillation in 103 patients (20%)

 • Appropriate ICD discharge rates were 11% per year for

secondary prevention and 4% per year for primary prevention

 • For primary prevention, 35% of patients with appropriate ICD

interventions had undergone implant for only a single risk factor.

Maron BJ, Spirito P, Shen WK, et al. Implantable cardioverter-defibrillators and prevention of
sudden cardiac death in hypertrophic cardiomyopathy. JAMA 2007;298:405-12.
 137 patients were enrolled in the ARVC registry.
 Fifty-two of the 108 (48%) patients with ICDs had
spontaneous SMVT or sustained polymorphic
ventricular fibrillation (SPVF) before ICD implantation
 Of the 108 patients who received an ICD, 48 had
ventricular arrhythmias treated by the ICD during
follow-up
 In a multivariate analysis, the only 2 predictors of ICD
treatment of ventricular arrhythmias were pre-
implantation SMVT or SPVF (p = 0.0029) and T-wave
inversions inferiorly (p = 0.0159).
 no sudden deaths

J Am Coll Cardiol. 2014 July 15; 64(2): 119–125

 CLASS IIa
I IIaIIbIII
ICD implantation is reasonable for patients with unexplained
syncope, significant LV dysfunction, and non ischemic DCM.

I IIIIAa
IIbIII
ICD implantation is reasonable for patients with HCM who
have 1 or more major† risk factors for SCD.

I IIaIIbIII
ICD implantation is reasonable for the prevention of SCD in
patients with arrhythmogenic right ventricular
dysplasia/cardiomyopathy (ARVD/C) who have 1 or more
risk factors for SCD.

All primary SCD prevention ICD recommendations apply only to patients who are receiving optimal medical therapy and have reasonable expectation of
survival with good functional capacity for more than 1 year.

† See Section 3.2.4, “Hypertrophic Cardiomyopathy,” in the full-text guidelines for definition of major risk factors.
 ICD therapy is indicated in patients with nonischemic
DCM who have an LVEF ≤35% and who are NYHA Class
II or III.*

 ICD therapy is recommended for primary prevention of

SCD to reduce total mortality in selected patients with
nonischemic DCM or ischemic heart disease at least 40
days post-MI with LVEF of 35% or less and NYHA Class II
or III symptoms on chronic GDMT, who have reasonable
expectation of meaningful survival for more than 1
year.**
*2008 ACC/AHA/HRSGuidelines forDevice-Based Therapy
**2013 ACC/AHA Guidelinefor the Management of Heart Failure
 Primary prevention ICD therapy in patients
with symptomatic heart failure of non-
ischaemic aetiology has a class I B
recommendation in the ESC guidelines1
and a class I A recommendation in the AHA
guidelines.
 An ICD should be implanted in patients with
nonischemic DCM and significant LV dysfunction
who have sustained VT or VF, are receiving chronic
optimal medical therapy, and who have reasonable
expectation of survival with good functional status
for more than 1 year*

*2006 ACC/AHA/ESC Guidelines for Management of Patients with Ventricular Arrhythmias

and thePrevention of Sudden Cardiac Death
 ICD therapy is indicated in patients who are
survivors of cardiac arrest due to VF or
hemodynamically unstable sustained VT after
evaluation to define the cause of the event and to
exclude any completely reversible causes.

 ICD therapy is indicated in patients with clinically

relevant, hemodynamically significant sustained VT
or VF induced at electrophysiologic study.

2008 ACC/AHA/HRSGuidelines for Device-Based Therapy

 Ambulatory ECG is indicated when there is a need to
clarify the diagnosis by detecting arrhythmias, QT-interval
changes, T-wave alternans (TWA), or ST changes to
evaluate risk, or to judge therapy. (Class I level A)

 Event monitors are indicated when symptoms sporadic to

establish whether or not they are caused by transient
arrhythmias. (Class I level B)

 Implantable recorders are useful in patients sporadic

symptoms suspected to be related to arrhythmias such as
syncope when a symptom-rhythm correlation cannot be
established by conventional diagnostic techniques. (Class
I level B)
Echocardiography is recommended in patients
 with ventricular arrhythmias who are suspected
of having structural heart disease. (Class I level B)

 at high risk for the development of serious

ventricular arrhythmias or SCD, such as those
with dilated, hypertrophic, or RV
cardiomyopathies, AMI survivors, or relatives of
patients with inherited disorders associated with
SCD. (Class I level B)
 It is reasonable to use T-wave alternans to improve the
diagnosis and risk stratification of patients with ventricular
arrhythmias or who are at risk for developing life-threatening
ventricular arrhythmias. (Class IIa level A)

 ECG techniques such as signal-averaged ECG(SAECG), heart

rate variability (HRV), baroreceptor reflex sensitivity, and
heart rate turbulence may be useful to improve the diagnosis
and risk stratification of patients with ventricular arrhythmias
or who are at risk of developing life-threatening ventricular
arrhythmias.
(Class IIb level B)