Non ischemic Arrhythmia

1. Cardiomyopathy
1.1. Dilated Cardiomyopathy
1.2. Hipertrophic Cardiomyopathy
1.3. Arrhytmogenic right ventricular
cardiomyopathy
1.4. Infiltratif cardiomyopathy
1.5. Restrictive cardiomyopathy

1.1 Dilated Cardiomyopathy

Dilated cardiomyopathy is a heart muscle disorder
defined by the presence of a dilated and poorly
functioning left ventricle in the absence of
abnormal loading conditions (hypertension,
valve disease) or ischaemic heart disease
sufficient to cause global systolic impairment. (2)
A large number of cardiac and systemic diseases
can cause systolic impairment and left ventricular
dilatation, but in the majority of patients no
identifiable cause is foundhence the term
idiopathic dilated cardiomyopathy (IDC). (2)

Diagnostic criteria for IDC

Ejection fraction < 0.45 and/or a fractional shortening of < 25%,
and a left ventricular end diastolic dimension of > 112%
predicted value corrected for age and body surface area.
Exclusion criteria:
- Absence of systemic hypertension (> 160/100 mm Hg)
- Coronary artery disease (> 50% in one or more major branches)
- Chronic excess alcohol (> 40 g/day female, > 80 g/day male for
more than five years after six month abstinence)
- Systemic disease known to cause IDC
- Pericardial diseases
- Congenital heart disease
- Cor pulmonale (2)

 DCM leads to progressive heart failure and a

decline in LV contractile function, ventricular
and supraventricular arrhythmias,
conduction system abnormalities,
thromboembolism, and sudden or heart failure
related death. (9)

1.2. Hipertrophic
Cardiomyopathy
1. Adults
In an adult, HCM is defined by a wall thickness 15 mm in one or more
LV myocardial segmentsas measured by any imaging technique
(echocardiography, cardiac magnetic resonance imaging (CMR) or computed
tomography (CT))that is not explained solely by loading conditions. (1)
Genetic and non-genetic disorders can present with lesser degrees of wall
thickening (1314 mm); in these cases, the diagnosis of HCM requires
evaluation of other features including family history, non-cardiac symptoms
and signs, electrocardiogram (ECG) abnormalities, laboratory tests and multimodality cardiac imaging. (1)
2. Children
As in adults, the diagnosis of HCM requires an LV wall thickness more than
two standard deviations greater than the predicted mean (z-score > 2,
where a z-score is defined as the number of standard deviations from the
population mean). (1)

 Atrial fibrillation is the most common

arrhythmia in patients with HCM.
Predisposing factors include increased left atrial
pressure and size, caused by diastolic
dysfunction, LVOTO (left ventricular outflow tract
obstruction) and mitral regurgitation. Other
possible predictors include LVOTO, P-wave
duration >140 ms on signal-averaged ECG,
paroxysmal SVT, ST-T changes on baseline
electrocardiography, premature ventricular
contractions, LGE on CMR, and abnormal coronary
flow reserve. (1)

1.3 Arrhytmogenic right ventricular

cardiomyopathy
ARVC is an uncommon form of
inheritable heart muscle disease
(estimated 1:5000) with a relatively recent
description (20 years ago). (9)
ARVC involves predominantly the right
ventricle with progressive loss of
myocytes and fatty or fibrofatty
tissue replacement, resulting in regional
(segmental) or global abnormalities. (9)

 in ARVC it is usually a
monomorphous left bundle branch
block-type ventricular tachycardia.
(8)

Epsilon potential in a patient with arrhythmogenic

right ventricular cardiomyopathy.

1.4. Infiltrative
Cardiomyopathy
Infiltrative cardiomyopathies are
characterized by the deposition of
abnormal substances that cause the
ventricular walls to become
progressively rigid, thereby impeding
ventricular filling. (10)

 Infiltrative disorders, with accumulation of abnormal substances

within myocytes or the myocardial interstitium, may cause
increased wall thickness without actual myocyte hypertrophy.
Consequently, increased wall thickness does not consistently
correlate with an increase in QRS complex amplitude. In fact, QRS
complex amplitude can become decreased (low voltage), a
phenomenon more commonly observed with accumulations
within the interstitium than within myocytes. The absence of
increased voltage on the electrocardiogram (ECG) despite the
appearance of hypertrophy can be the first clue to certain
infiltrative diseases (e.g., cardiac amyloid, Friedreich ataxia). (10)
However, low-voltage QRS complex is not a uniform finding with
the infiltrative cardiomyopathies. Some infiltrative myopathies
have increased voltage (e.g., Danon and Fabry diseases), which
will be seen when there is an increase in the size of cardiac
myocytes, normal intraventricular conduction, and synchronous
activation occurring globally in the myocardium. (10)

1.5 Restrictive
Cardiomyopathy (3)

2. Inherited Primary Arrhythmia

Syndrome
2.1 Long QT syndrome
2.2 Short QT syndrome
2.3 Brugada syndrome

2.1 Long QT syndrome

1. Long QT Syndrome is diagnosed:
a. In the presence of an LQTS risk score 3.5 in the absence of
a secondary cause for QT prolongation and/or
b. In the presence of an unequivocally pathogenic mutation
in one of the LQTS genes or
c. In the presence of a QT interval corrected for heart rate
using Bazett's formula (QTc) 500 ms in repeated 12-lead
electrocardiogram (ECG) and in the absence of a secondary
cause for QT prolongation. (4)
2. LQTS can be diagnosed in the presence of a QTc between
480499 ms in repeated 12-lead ECGs in a patient with
unexplained syncope in the absence of a secondary cause for
QT prolongation and in the absence of a pathogenic mutation. (4)

 In LQTS, the high-grade arrhythmia underlying

the symptoms is usually a specific polymorphous
ventricular tachycardia (of the torsade de pointes
type in which the QRS complexes seem to spiral
around the isoelectric line). (8)

2.2 Short QT syndrome

1. SQTS is diagnosed in the presence of a
QTc 330 ms.
2. SQTS can be diagnosed in the presence
of a QTc < 360 ms and one or more of
the following: a pathogenic mutation,
family history of SQTS, family history of
sudden death at age 40, survival of a
VT/VF episode in the absence of heart
disease. (4)

Short QT interval, peaked T waves and short ST

segments in Short QT syndrome.

2.3 Brugada syndrome

1. BrS is diagnosed in patients with ST-segment
elevation with type 1 morphology 2 mm in 1
lead among the right precordial leads V1, V2, positioned
in the 2nd, 3rd or 4th intercostal space occurring either
spontaneously or after provocative drug test with
intravenous administration of Class I anti arrhythmic
drugs.
2. BrS is diagnosed in patients with type 2 or type 3
ST-segment elevation in 1 lead among the right
precordial leads V1, V2 positioned in the 2 nd, 3 rd or 4 th
intercostal space when a provocative drug test with
intravenous administration of Class I anti arrhythmic
drugs induces a type I ECG morphology. (4)

 In Brugada syndrome, a right bundle branch block-type deformation of the QRS

complex in the form of a so-called Brugada type 1, 2, or 3 ECG may appear
spontaneously or with pyrexia (8)

Type 1 ECG: Coved-type ST elevation 2 mm with T-wave inversion in at least

two of leads V1 to V3.
Type 2 ECG: Saddle-back ST elevation with increased descent 2 mm and at
least 1 mm, elevation of the ST segment.
Type 3 ECG: Saddle-back or coved-type ST elevation <1 mm. (8)

3. Others
3.1.
3.2.
3.3.
3.4.
3.5.
3.6.

Myocarditis
Endocarditis
Pericarditis
Rheumatoid Heart Disease
Cardiac Sarcoidosis
Valvular Heart Disease

3.1 Myocarditis (7)

 These pathological processes create an

electrically unstable substrate predisposing to the
development of ventricular tachyarrhythmias.
(9)
In both its acute and chronic phase myocarditis
may be associated with severe arrhythmias that
can significantly affect the natural course of the
disease, as they can further contribute to the
deterioration of cardiac systolic and diastolic
function and can be the ultimate cause of death
in these patients.
Mechanisms of arrhythmogenesis in the context
of myocarditis include myocyte necrosis,
replacement fibrosis (favoring re-entry
mechanism), proarrhythmic effects of cytokines

ECG shows repetitive polymorphic ventricular

ectopic beats in myocarditis. (11)

3.2. Endocarditis (6)

3.3. Pericarditis (5)

3.4. Rheumatoid Heart Disease (12)

 Atrial fibrillation (AF) is the most

common sustained arrhythmia, being
present in 0.4% of the overall
population1 and in 3% to 5% of those
> 65 years of age. This incidence is
higher in countries with a high
prevalence of rheumatic heart
disease (RHD).( 16)

3.5. Cardiac Sarcoidosis (14)

Sarcoidosis is a granulomatous disease of
unknown etiology. Noncaseating granulomas
are the pathological hallmark and are most
often associated with pulmonary involvement
but may also involve the heart, liver,
peripheral lymph node, spleen, skin, eyes,
phalangeal bones, parotid gland, or other
organs and tissues.
Recent studies suggest that the disease may
be an immunological response to an
unidentified antigenic trigger. (15)

 There are a number of situations in which

cardiac presentations can be the first
and/or an unrecognized manifestation of
sarcoidosis:
1. Unexplained Mobitz II or Third-Degree AV
Block in Young Patients
2. Sustained Monomorphic VT of Unknown
Etiology
3. Arrhythmogenic Right Ventricular
Cardiomyopathy (15)

3.6. Valvular Heart Disease

Echocardiography is the key technique used to
confirm the diagnosis of VHD, as well as to assess
its severity and prognosis. (13)