Download Clinical and echocardiogram profile of Cardiomyopathy at tertiary

Original Article
JCDR
Clinical and echocardiogram profile of Cardiomyopathy at tertiary
care centre
Virendra C. Patil1, Neeraj Desai2 and Chetan Galande2
Associate professor Department of medicine; Krishna Institute of Medical, Sciences University (KIMSU)
Resident Department of medicine; Krishna Institute of Medical, Sciences University (KIMSU)
1
2
ABSTRACT
Background: Cardiomyopathies represent a heterogeneous group of diseases that often lead to progressive heart failure
with significant morbidity and mortality. The improved recognition or of other factor, the incidence and prevalence of
heart failure due to cardiomyopathy appears to be increasing. Aims & Objectives: To study the prevalence, clinical profile
and outcome of patients with cardiomyopathies and to study the echocardiographic profile. Material & methods: It is
retrospective observational study of 65 patients, with age >15 years and admitted in medical ICU and ward of KIMSU,
karad. This study was conducted over period of one year. All eligible subjects underwent relevant investigations including
echocardiogram, Doppler study, electrocardiogram, chest radiogram and coronary angiogram. Proforma included age
gender presenting complaints, past history, history of medications, clinical examination and laboratory investigations.
Trans-thoracic 2-dimensional echocardiogram and Doppler study was done according to the standard protocol. Statistical
analysis: Results were given as mean ± SD. Means are compared by unpaired Students t-test. Chi-square was used as
appropriate. The observations and data were analyzed in the statistical package social sciences (SPSS) trial version 11.
The level of significance was set at P<0.05. Results: A total of 65 patients were admitted from Jan 2010 to Dec 2012
who were diagnosed with cardiomyopathy with mean age of 58.76 years (SD±15.98). Out of the total 65 cardiomyopathy
patients admitted 27 (41.53%) had Dilated cardiomyopathy (idiopathic), 15 (23.07%) patients were diagnosed with
Ischemic cardiomyopathy. Total 42 (64.61%) patients had Dilated cardiomyopathy (i.e. idiopathic and ischemic dilated
cardiomyopathy). A total 11 (16.92%) had Hypertrophic cardiomyopathy (HCM), 2 (3.07%) patients had Hypertrophic
obstructive cardiomyopathy (HOCM) with LVOT significant gradient, 5 (7.69%) patients had Restrictive cardiomyopathy
(RCMP), 4 (6.15%) had Peripartum cardiomyopathy (PPCM) and 1 (1.53%) patient had miscellaneous (alcohol induced)
Cardiomyopathy. Out of 27 patients diagnosed with Dilated Cardiomyopathy (DCM), 15 (55.55%) were male patients
and 12 (44.45%) were females. Out of 15 patients diagnosed with Ischemic cardiomyopathy, 7(46.66%) were males and
8(53.33%) were females. Out of 11 patients diagnosed with HCM, 9(81.81%) were males and 2(18.18%) were females.
Out of 2 patients diagnosed with HOCM 1(50%) was a male patient and 1(50%) was a female patient. There were 4
female patients who were diagnosed with PPCM. A total of 5 patients were diagnosed with RCMP, out of which 1(20%)
was a male patient 4(80%) patients were females. There was 1 male patient diagnosed with cardiomyopathy secondary
to chronic alcohol consumption. In present study breathlessness 89.23% was most common presenting symptom and
palpitations (81.53%) and cough (58.46%) were next to it with ‘p’ <0.001. Overall VPC’s (36%) were the most common
ECG abnormality in patients with cardiomyopathy in present study with ‘p’ value <0.01. The LBBB (35.71%) was
more frequently seen in patients with dilated cardiomyopathy with ‘p’ <0.02. The atrial flutter/ fibrillation found more
commonly in patients with restrictive cardiomyopathy (80%) with ‘p’ value < 0.02. Total 74.07% of DCM, 86.67% of
ischemic cardiomyopathy, 50% of PPCM, 81.82% of HCM, 100% of HOCM, RCMP and alcoholic cardiomyopathy
patients had diastolic dysfunction. Idiopathic Dilated cardiomyopathy had 22% (±7.7) of lowest left ventricular ejection
fraction (LVEF). Out of total 65 patients two patients with dilated cardiomyopathy succumbed with overall mortality
3.08% and case fatality rate for DCM was 4.76%. Total 96.92% patient were discharged after treatment from the hospital.
Conclusion: Present study highlights significant burden of Idiopathic dilated cardiomyopathy and next to it was ischemic
cardiomyopathy. A total 6 (9.23%) patients had potentially reversible cardiomyopathy like peripartum, alcoholic and
myocarditis. The cardiomyopathy is asymptomatic in the early stages hence early diagnosis and management is of vital
importance in the form of judicious use of anticoagulant, digoxin, diuretics, ACE inhibitors and betablocker is mandatory
to reduce complications, morbidity and mortality associated cardiomyopathies. Trans-thoracic echocardiogram is an
important, simple and noninvasive modality of investigation which can be reproducible for the diagnosis and fallow up
of most of the cardiomyopathies.
Keywords: Echocardiogram, Dilated cardiomyopathy, Hypertrophic obstructive cardiomyopathy, Peripartum cardiomyopathy,
Restrictive cardiomypathy
Clinical and Echocardiogram Profile of Cardiomyopathy
INTRODUCTION
Cardiomyopathies represent a heterogeneous group of
diseases that often lead to progressive heart failure with
significant morbidity and mortality. The cardiomyopathies
are an important and complex group of heart muscle diseases with multiple etiologies and heterogeneous phenotypic expression. Cardiomyopathy is disease of the heart
muscle. It is estimated that cardiomyopathy accounts for
5–10% of the 5–6 million patients already diagnosed with
heart failure in the United States. This term is intended
to exclude cardiac dysfunction that results from other
structural heart disease, such as coronary artery disease,
primary valve disease, or severe hypertension; however,
in general usage the phrase ischemic cardiomyopathy is sometimes applied to describe diffuse dysfunction occurring in
the presence of multivessel coronary artery disease, and
nonischemic cardiomyopathy to describe cardiomyopathy from
other causes. As of 2006, cardiomyopathies are defined as
“a heterogeneous group of diseases of the myocardium
associated with mechanical and/or electrical dysfunction
that usually (but not invariably) exhibit inappropriate ventricular hypertrophy or dilatation and are due to a variety
of causes that frequently are genetic.” Major types include
dilated cardiomyopathy, hypertrophic cardiomyopathy,
restrictive cardiomyopathy, and arrhythmogenic right
ventricular cardiomyopathy. Although cardiomyopathy is
asymptomatic in the early stages, symptoms are the same
as those characteristically seen in any type of heart failure and may include shortness of breath, fatigue, cough,
orthopnea, paroxysmal nocturnal dyspnea, and edema1–3.
There is very little literature regarding cardiomyopathies
from Asian countries including India.
Materials & Methods
It is retrospective observational study of 65 patients, with age >15
years and admitted in medical ICU and ward of KIMS, karad. This
study was conducted over period of three year (Jan 2010 to Dec
2012). All eligible subjects underwent relevant investigations including
echocardiogram, Doppler study, electrocardiogram, chest radiogram
and coronary angiogram whenever indicated and possible. Proforma
included age gender presenting complaints, past history, history of
medications, clinical examination, and laboratory investigations. Transthoracic 2-dimensional echocardiogram and Doppler study was done
according to the standard protocol.
Aims & Objectives
To study the prevalence, clinical profile and outcome of
patients with cardiomyopathies and to study the echocardiographic profile.
Echocardiography
The echocardiographic examinations were performed in all patients
according to standard techniques while the patients were lying flat or
in the left lateral position by Siemens Accuson 300- X Colour Doppler
ultrasound machine. The primary imaging modality used for diagnosis in
all patients is transthoracic 2-dimensional and Doppler echocardiography
performed by a three operators. Ejection fraction (EF) was calculated by
the formula LVEF % = (LVID)2 - (LVIDS)2 . Systolic dysfunction was
characterized by reduced EF <55%. Diastolic dysfunction was calculated
by measuring E and A velocity across transmitral inflow velocity and tissue Doppler imaging. Diastolic dysfunction was defined as preserved left
ventricle EF with (a) reduced early diastolic filling, (b) prolongation of
isovolumetric relaxation, (c) increased atrial filling, the presence of which
confirms diastolic dysfunction, (d) increased pre-ejection period (PEP)
and shorter left ventricular ejection time (LVET) resulting in increases
PEP/LVET ratio—an evidence of reduced left ventricular distensibility. Left ventricular hypertrophy (LVH) on echocardiogram defined by
LVPW >12 mm and IVS >12 mm by M-mode with an LV mass ≥125
g/m2 for men and ≥110 g/m2 for women4.
Echocadiographic diagnostic criteria for cardiomyopathy Dilated
Cardiomyopathy labeled if enlarged left ventricle with decreased systolic
function as measured by left ventricular ejection fraction characterizes
dilated cardiomyopathy. Systolic failure is more marked than the frequently
accompanying diastolic dysfunction, (Echocardiography criteria: Left ventricular ejection fraction < 45%; Left ventricular end diastolic dimension > 3 cm / body surface area; Global hyokinesia; dilatation of all the
chambers of heart in absence of valvular heart disease and congenital heart disease.) Hypertrophic cardiomyopathy was labeled by features of
marked left ventricular hypertrophy in the absence of other causes, such
as hypertension or valve disease without LVOT obstructive gradient.
Hypertrophic obstructive cardiomyopathy was labeled by features of marked
left ventricular hypertrophy in the absence of other causes, such as
hypertension or valve disease with LVOT obstructive gradient. Restrictive
Cardiomyopathy was labeled if abnormal diastolic function, with mildly
decreased contractility and ejection fraction (usually >30–50%). Both
atria are enlarged, sometimes massively. Peripartum cardiomyopathy was
labeled if features of dilated cardiomyopathy with onset in the third tri-
Corresponding address:
Dr. Virendra. C. Patil
MD. Medicine (Associate Professor),
Department of medicine, KIMSU, Karad
E-mail: [email protected]
DOI: 10.5530/jcdr.2014.1.6
Journal of Cardiovascular Disease Research Vol. 5 / Issue 1 Jan–Mar, 2014
mester of pregnancy or in the first five months postpartum. Alcohol associated cardiomyopathy was labeled if features of dilated cardiomyopathy in
patient with chronic alcoholic with potentially reversible with abstinence
from alcohol use. All above criteria excluded the valvular, congenital and
hepertensive heart disease1–4.
35
Clinical and Echocardiogram Profile of Cardiomyopathy
Statistical analysis
All patients presented with features suggestive of cardiomyopathy
were treated according to the standard protocol. All patients were
Results were given as mean ± SD. Means are compared by unpaired
Students t-test. Chi-square was used as appropriate. The observations
and data were analyzed in the statistical package social sciences (SPSS)
trial version 11. The level of significance was set at P<0.05.
clinically assessed regularly and monitored. In pharmacotherapy
anticoagulant, digoxin, diuretics, ACE inhibitors, inotopic agents,
antiarrhythmic agents and betablockers were used judiciously to
reduce complications, morbidity and mortality associated cardiomyopathies. The regular monitoring of vital organ dysfunction,
Results
drug side effects and electrolyte imbalance was done. In patient
with Complete 3rd degree AV block temporary pacemaking was
A total of 65 patients were admitted from Jan 2010 to Dec 2012 with
diagnosis of cardiomyopathy with mean age of 58.76 years (SD±15.98).
Out of 65 patients 34(52.30%) were males with mean age of 62.41 years
(SD±12.76) and 31(47.70%) were females with mean age of 54.77 years
(SD±18.28). There was no significant statistical difference amongst gender. The total average duration of stay was 6.98 days (SD±3.962) with
average hospital stay among males were 7.02 days (SD±4.427) and of
females were 6.93 days (SD±3.453). (Table 1)
done. Treatment is targeted at relieving the symptoms of heart failure and reducing rates of heart failure. Whenever it was indicated
the implantable cardioverter-defibrillators, cardiac resynchronization therapy, and heart transplantation was advised to get it done
at higher centre. Recommended lifestyle changes include restricting
alcohol consumption, losing weight, exercising, quitting smoking,
and eating a low-sodium diet2,3.
Table 1 Gender distribution in cardiomyopathy patients
Variables
Mean age (n=65)
SD
Mean duration of stay (days)
SD
Male (n=34)
62.41
±12.76
7.03
±4.427
Females (n=31)
54.77
±18.28
6.93
±3.453
Total
58.76
±15.98
6.98
±3.962
Prevalence of patient with cardiomyopathy: Out of the
total 65 cardiomyopathy patients admitted 27 (41.53%) had
Dilated cardiomyopathy (idiopathic) of which one patient
had myocarditis probably viral with complete heart block
(CHB) which was reversible over period of six weeks (figure 1 and 2). Total 15 (23.07%) patients were diagnosed
with Ischemic Cardiomyopathy. Total 42 (64.61%) patients
had Dilated cardiomyopathy (i.e. idiopathic and ischemic
dilated cardiomyopathy (Figure 4). A total 11 (16.92%) had
Hypertrophic cardiomyopathy (HCM), 2 (3.07%) patients
had Hypertrophic Obstructive Cardiomyopathy (HOCM)
(Figure 3). Total 5 (7.69%) patients had Restrictive cardiomyopathy (RCMP) of which one patient had thalasemia with history of recurrent blood transfusions who had
multiform and multifocal VPC’s (Figure 3). Total 4 (6.15%)
patients had peripartum cardiomyopathy (PPCM) and 1
(1.53%) patient had miscellaneous (alcohol induced) Cardiomyopathy. In present study Idiopathic dilated cardiomyopathy was the most common cardiomyopathy (‘p’ < 0.05).
(Table 2)
Mean and standard deviation of patient with cardiomyopathy: Out of 27 patients diagnosed with Dilated
36
Cardiomyopathy, 15 (55.55%) were male patients with
mean age of 61.46 years (SD ±13.61) and 12 (44.45%)
were females with mean age of 53.42 years (SD ±13.47).
Out of 15 patients diagnosed with Ischemic Cardiomyopathy, 7(46.66%) were males with mean age of
70.28 years(SD ±12.89) and 8(53.33%) were females
with mean age of 66.25 years(SD ±11.46). Out of 11
patients diagnosed with Hypertrophic cardiomyopathy
(HCM), 9(81.81%) were males with mean age of 49.33
years (SD ±8.139) and 2(18.18%) were females with
mean age of 60 years (SD ±7.071). Out of 2 patients
diagnosed with Hypertrophic obstructive cardiomyopathy (HOCM)1(50%) was a male patient and 1(50%) was
a female patient with mean age 35 years. There were 4
female patients who were diagnosed with PPCM with
mean age of 27.5 years (SD ±6.24). A total of 5 patients
were diagnosed with RCMP, out of which 1(20%) was
a male patient who’s age was 60 years while 4(80%)
patients were females with mean age of 59.25 years (SD
±30.192). There was 1 35 year old male patient diagnosed with cardiomyopathy secondary to chronic alcohol consumption. (Table 2)
Journal of Cardiovascular Disease Research Vol. 5 / Issue 1 Jan–Mar, 2014
Clinical and Echocardiogram Profile of Cardiomyopathy
Table 2 Gender distribution, mean and standard deviation of patients with cardiomyopathy.
Variables
Total (n)
Mean age
SD
Male
15
61.46
±13.61
Female
12
53.42
±13.47
Male
7
70.28
±12.89
Female
8
66.25
±11.46
Male
9
49.33
±8.139
Female
2
60
±7.071
Hypertrophic obstructive (HOCM) [M:1; F:1]
2
35
± 6.5
4
27.5
±6.244
Male
1
60
Female
4
59.25
±30.192
1
50
Dilated Cardiomyopathy
Ischemic Cardiomyopathy
Hypertrophic cardiomyopathy
Peripartum cardiomyopathy
Female
Restrictive cardiomyopathy
Alcoholic cardiomyopathy
Male
Figure 1: Echocardiogram and Doppler study of Dilated
cardiomyopathy & myocarditis with MR &TR
Figure 2: Echocardiogram and Doppler study of Dilated
cardiomyopathy, myocarditis and PPCM with MR & TR
Journal of Cardiovascular Disease Research Vol. 5 / Issue 1 Jan–Mar, 2014
Figure 3: Echocardiogram and Doppler study of HOCM
with MR and SAM and LVOT gradient with restrictive
cardiomyopathy with biatrial enlagement ad TR
Figure 4: Echocardiogram and Doppler study of Isch-
emic dilated cardiomyopathy showing MR & TR
37
Clinical and Echocardiogram Profile of Cardiomyopathy
Clinical presentation of patients with cardiomyopathy: Total 40 (95.23%) patient with DCM, 6(54.54%)
with HCM, 2 (100%), with HOCM, 5(100%) with restrictive cardiomyopathy, 4(100%) with peripartum cardiomyopathy and 1(100%) with alcoholic cardiomyopathy
had breathlessness at the time of admission. A total
58(89.23%) of patients had breathlessness as a presenting
complaint at the time of admission the hospital. Total 1
(2.38%) patient with DCM, 1(20%) with restrictive cardiomyopathy, 2(50%) with PPCM had hemoptysis at the time
of admission. A total 4(6.15%) of patients had hemoptysis as a presenting complaint at the time of admission the
hospital. Total 37 (88.09%) patient with DCM, 5(45.45%)
with HCM, 2 (100%), with HOCM, 4(80%) with restrictive cardiomyopathy, 4(100%) with peripartum cardiomyopathy and 1(100%) with alcoholic cardiomyopathy had
palpitations at the time of admission. A total 53(81.53%)
of patients had palpitations as a presenting complaint at
the time of admission the hospital. Total 29 (69.04%)
patient with DCM, 2(18.18%) with HCM, 2(40%) with
restrictive cardiomyopathy, 4(100%) with PPCM and
1(100%) with alcoholic cardiomyopathy had cough at the
time of admission. A total 58(89.23%) of patients had
cough as a presenting complaint at the time of admission
the hospital. Total 21 (50%) patient with DCM, 1(9.09%)
with HCM, 5(100%) with RCMP, 2(50%) with PPCM and
1(100%) with alcoholic cardiomyopathy had pedal edema
at the time of admission. A total 30(46.15%) of patients
had pedal edema as a presenting complaint at the time
of admission the hospital. Total 15 (35.71%) patient with
DCM, 1(9.09%) with HCM, 1 (50%) with HOCM and
1(20%) with restrictive cardiomyopathy, had chest pain at
the time of admission. A total 18(27.69%) of patients had
chest pain as a presenting complaint at the time of admission the hospital. Total 3 (7.14%) patient with DCM,
2(18.18%) with HCM, 1 (50%) with HOCM and 1(25%)
with PPCM had syncope at the time of admission. A
total 7(10.76%) of patients had chest pain as a presenting complaint at the time of admission the hospital. In
present study breathlessness 89.23% was most common
presenting symptom and palpitations (81.53%) and cough
(58.46%) were next to it with ‘p’ <0.001. (Table 3)
Table 3 Clinical presentation at the time of admission:
Variable
Total
DCM
HCM
HOCM
RCM
PPCM
ACM
Total
(n=42)
%
(n=11)
%
(n=2)
%
(n=5)
%
(n=4)
%
(n=1)
%
Breathlessness
40
95.23
6
54.54
2
100
5
100
4
100
1
100
58
89.23
Hemoptysis
1
2.38
0
0
0
0
1
20
2
50
0
0
4
6.15
Pedal edema
21
50
1
9.09
0
0
5
100
2
50
1
100
30
46.15
Palpitations
37
88.09
5
45.45
2
100
4
80
4
100
1
100
53
81.53
Syncope
3
7.14
2
18.18
1
50
0
0
1
25
0
0
7
10.76
Cough
29
69.04
2
18.18
0
0
2
40
4
100
1
100
38
58.46
Chest pain
15
35.71
1
9.09
1
50
1
20
0
0
0
0
18
27.69
Electrocardiogram findings in patients with
cardiomyopathy: Overall VPC’s (36%) were the
most common ECG abnormality in patients with
cardiomyopathy in present study with ‘p’ value <0.01. The
LBBB (35.71%) was more frequently seen in patients with
dilated cardiomyopathy with ‘p’ <0.02. The ‘QS’ pattern
in chest leads was more frequently seen in patients with
38
%
Ischemic cardiomyopathy (73.33%) with ‘p’ value< 0.001.
Low voltage and poor ‘r’ wave progression was found
more frequently in PPCM (100%) with ‘p’ value < 0.001.
The atrial flutter/ fibrillation found more commonly in
patients with restrictive cardiomyopathy (80%) with ‘p’
value < 0.02. [Table 4]
Journal of Cardiovascular Disease Research Vol. 5 / Issue 1 Jan–Mar, 2014
Clinical and Echocardiogram Profile of Cardiomyopathy
Table 4 Electrocardiogram findings in patients with cardiomyopathy
DCM
(n=42)
HCM
(n=11)
HOCM
(n=2)
RCM
(n=5)
PPCM
(n=4)
ACM
(n=1)
Total
%
AVNRT
2
0
0
0
0
1
3
4.61
Atrial / flutter fibrillation
1
1
0
4
0
0
6
9.23
LBBB
15
1
0
0
0
0
16
24.61
RBBB
1
0
0
1
1
0
3
4.61
VPC’s
15
5
1
1
2
0
24
36.92
ST- depression
3
9
0
0
0
0
12
18.46
CHB
1
0
0
0
0
0
1
1.53
I deg. AV block
1
0
0
0
1
0
2
3.07
II deg AV block
1
0
0
0
0
0
1
1.53
LAHB
3
1
1
1
0
0
6
9.23
LPHB
1
1
0
0
0
0
2
3.076
Low voltage & poor ‘r’ wave
progression
4
0
0
3
4
1
12
18.46
QS pattern
11
0
0
0
0
0
11
16.92
LVH with strain pattern
0
10
2
0
0
0
12
18.46
Total
59
28
4
10
8
2
111
‘p’< 0.02
Variable
Echocardiographic findings in patients with cardiomyopathy: Total 74.07% of DCM, 86.67% of ischemic
cardiomyopathy, 50% of PPCM, 81.82% of HCM, 100%
of HOCM, RCMP and alcoholic cardiomyopathy patients
had diastolic dysfunction. Idiopathic Dilated cardiomyopathy had 22% (±7.7), Ischemic cardiomyopathy had
26% (±9.5), HCM had, 65% (±12.5), HOCM had 68 %(
±11.3), RCMP had 58% (±7.9), PPCM had 21 %( ±8.7)
and alcoholic cardiomyopathy had 32% left ventricular ejection fraction (LVEF) (Figure 1). Total 19% patients with
DCM and 13% with ischemic cardiomyopathy had clot in
left ventricle. Total 37.03% with DCM, 40% with Ischemic
cardiomyopathy, 9.09% with HCM, 40% with RCMP, 50%
with PPCM had LV echo-contrast on echocardiogram.
Total 74.07% with DCM, 73.33% with Ischemic cardiomyopathy, 27.27% with HCM and 100% with HOCM, RCM,
PPCM and alcoholic cardiomyopathy had mitral incompetence/ regurgitation. Total 55.55% with DCM, 46.66%
with Ischemic cardiomyopathy, 18.18% with HCM and
100% with RCM, PPCM and alcoholic cardiomyopathy and
50% with HOCM had tricuspid incompetence/ regurgitation. The mean of pulmonary artery pressure inpatient with
DCM was 52(±7.8), 48(±5.7) with ischemic cardiomyopathy, 30(±5.8) with HCM, 19(±7.9) with HOCM, 38(±6.4)
with RCM, 37(±3.8) with PPCM and 35 with alcoholic cardiomyopathy. The mean of PAP was significantly high in
patient with DCM with ‘p’ < 0.001. (Table 5) (Figure 1–4)
One patient had myocarditis causing LV systolic dysfunction (LVEF:28%) with moderate MR & TR probably viral
with complete heart block (CHB) which was reversible over
period of six weeks and was treated with temporary pacemaker and treatment of CCF. (Figure 5)
Table 5 Echocardiographic findings in patients with cardiomyopathy
Types of
cardiomyopathy
Total
(n=65)
%
LVEF %
DD
%
PAP
Clot
%
Echocontrast
%
MR
%
TR
%
DCM
27
41.53
22 (±7.7)
20
74.07
52(±7.8)
5
19
10
37.03
20
74.07
15
55.55
IDCM
15
23.07
26(±9.5)
13
86.67
48(±5.7)
2
13
6
40
11
73.33
7
46.66
HCM
11
16.92
65(±12.5)
9
81.82
30(±5.8)
0
0
1
9.09
3
27.27
2
18.18
HOCM
2
3.07
68(±11.3)
2
100
19(±7.9)
0
0
0
0
2
100
1
50
RCMP
5
7.69
58(±7.9)
5
100
38(±6.4)
0
0
2
40
5
100
5
100
PPCM
4
6.15
21(±8.7)
2
50
37(±3.8)
0
0
2
50
4
100
4
100
ALCOHOLIC
1
1.53
32
1
100
35
0
0
0
0
1
100
1
100
Total
65
100
Journal of Cardiovascular Disease Research Vol. 5 / Issue 1 Jan–Mar, 2014
39
Clinical and Echocardiogram Profile of Cardiomyopathy
Discussion
We compared our results with various other studies from India and
abroad. The dilated cardiomyopathy in adults is most commonly
caused by CAD (ischemic cardiomyopathy) and hypertension1. In our
study patients 41.53% had DCM (idiopathic) and 15 (23.07%) patients
had Ischemic cardiomyopathy. Total 64.61% patients had DCM (i.e.
idiopathic and ischemic dilated cardiomyopathy. B. Ushasree, et al5
studied one hundred seven DCM patients and 105 healthy individuals,
they reported that, epidemiological factors like gender, age at onset
and vegetarian diet in conjunction with sarcomere gene mutations may
play a role in the disease expression. Pacheco OE et al6 studied 91 new
patients with the diagnosis of dilated cardiomyopathy. Ischemic
cardiomyopathy was identified as the most common cause of dilated
cardiomyopathy, representing 37% of the patient and an idiopathic
etiology was found in 22% of the patients. Similarly in our study
23.07% patients had Ischemic cardiomyopathy. Non ischemic dilated
cardiomyopathy (NIDCM) is a disorder of myocardium. It has varying
etiologies. Albeit the varying etiologies of this heart muscle disorder, it
presents with symptoms of heart failure, and rarely as sudden cardiac
death (SCD). Manifestations of this disorder are in many ways similar
to its counterpart, ischemic dilated cardiomyopathy (IDCM). A
proportion of patients with NIDCM carries a grave prognosis and is
prone to sudden cardiac death from sustained ventricular arrhythmias7.
In present study the VPC’s (36%) were the most common ECG
abnormality in patients with cardiomyopathy in present study with ‘p’
value <0.01. The LBBB (35.71%) was more frequently seen in patients
with dilated cardiomyopathy with ‘p’ <0.02. The ‘QS’ pattern in chest
leads was more frequently seen in patients with Ischemic
cardiomyopathy (73.33%) with ‘p’ value< 0.001. Taliercio CP, et al8
concluded in their study 24 patients with idiopathic dilated
cardiomyopathy the most common presentation was congestive heart
failure (92% of patients). Echocardiography (22 patients) generally
revealed a dilated left ventricle with reduced fractional shortening
(mean 14%) and ejection fraction (mean 26%). Two-dimensional
echocardiographic evidence of left ventricular thrombus was present
in 3 (23%) of 13 patients. These finding are comparable with our
study. M B Cod et al9 reported 45 new cases of idiopathic dilated
cardiomyopathy (DCM) and 19 new cases of hypertrophic
Figure 5: Chest radiogram (cardiomegaly and bilateral
pleral effusion) before and after treatment in patient with
myocarditis with electrocardiogram showing pacemaker
rhythum with temporary pacemaker for III degree AV
block (CHB).
Outcome of patients with cardiomyopathy: Out of
two patients who succumbed one was male and one was
female. Out of total 65 patients two patients with dilated
cardiomyopathy succumbed with overall mortality 3.08%
and case fatality rate for DCM was 4.76%. Total 96.92%
patient were discharged after treatment from the hospital.
(Table 6)
Table 6 Outcome in cardiomyopathy patients
Variables
Total (n=65)
%
Discharged
63
96.92
Death
2
3.08
2 (n=42)
4.76%(case fatality rate)
Death due to DCM
40
Journal of Cardiovascular Disease Research Vol. 5 / Issue 1 Jan–Mar, 2014
Clinical and Echocardiogram Profile of Cardiomyopathy
cardiomyopathy (HCM) with overall age- and sex-adjusted incidence
rates were 6.0/100,000 and 2.5/100,000 person-years, respectively.
These findings are comparable with our study in which 27 (41.53%)
had DCM (idiopathic), 15 (23.07%) had Ischemic cardiomyopathy, 11
(16.92%) had HCM and 2 (3.07%) patients had HOCM. Ntusi, Ntobeko
B A et al10 in their retrospective study of familial and idiopathic DCM
of Eighty patients with idiopathic DCM and 40 familial cases reported
that, ECG T-wave inversion was significantly more frequent in familial
DCM (87.5%) than in idiopathic cases (68.8%) (‘p’=0.014), whereas
idiopathic patients had a higher prevalence of pathological Q waves
(32.5%) than familial cases (12.5%) (p=0.028). In our study the LBBB
(35.71%) was more frequently seen in patients with dilated
cardiomyopathy with ‘p’ <0.02. The ‘QS’ pattern in chest leads was
more frequently seen in patients with Ischemic cardiomyopathy
(73.33%) with ‘p’ value< 0.001. Ntobeko B A Ntusi, et al11 in their study
of 109 unrelated patients with DCM, 29 (26.6%) had familial disease.
Their mean age of onset of cardiomyopathy (28.01 (standard deviation
(SD) 15.33) years) was significantly younger than that for non-familial
cases (39.1 (SD 12.6) years) (p=0.001). Male predominance was present
(72.4%). Similarly in present study 34(52.30%) were males slightly
more with mean age of 62.41 years (SD±12.76) and 31(47.70%) were
females with mean age of 54.77 years (SD±18.28). There was no
significant statistical difference amongst gender. Haq Nawaz et al12
reported the mean age was 47.12±17.9 year with male predominance
(males=34, females=16). Mean ejection fraction was 30.6±6.9%.
Complications revealed on echocardiography were intracardiac
thrombi (5, 10%), spontaneous echo contrast (5, 10%), pericardial
effusion (6, 12%), mitral regurgitation (46, 92%), tricuspid (25, 50%),
aortic (5, 10%), pulmonary (2, 4%) multi-valvular regurgitation (28,
56%), and left atrial dilatation (36, 72%). LV systolic dysfunction,
cardiac thrombi, spontaneous echo contrast, mitral and tricuspid
regurgitation and left atrial enlargement are important complications
of dilated cardiomyopathy. Echocardiography is important tool
towards identification of these complications. Phadke R S et al13 stated
that, the incidence of HCM amongst the autopsied primary
cardiomyopathies (N = 101) was 13.9% (n=14). Males were affected
more. In present study total 11 (16.92%) had HCM, 2 (3.07%) patients
had HOCM. Common presenting symptoms were exertional dyspnoea,
angina and palpitations. Concentric and asymmetric hypertrophy was
equally seen. Cardiac failure was the commonest cause of death. In our
study 6(54.54%) with HCM, 2 (100%), with HOCM had breathlessness,
5(45.45%) with HCM, 2 (100%), with HOCM had palpitations,
2(18.18%) with HCM had cough, 1(9.09%) with HCM, had pedal
edema, 1(9.09%) with HCM, 1 (50%) with HOCM, chest pain
2(18.18%) with HCM and 1 (50%) with HOCM syncope at the time
of admission. Tanjore RR et al 14 studied the 127 HCM cases were
divided into subtypes based on pattern of hypertrophy. They suggests
that modes of inheritance other than autosomal dominant may be
encountered in a subset of HCM especially in asymmetric septal
hypertrophy, apical, concentric and mid cavity obstruction subsets.
Hypertrophic cardiomyopathy is caused by 11 mutant genes with more
than 500 individual transmutations. The most common variation
Journal of Cardiovascular Disease Research Vol. 5 / Issue 1 Jan–Mar, 2014
involves the beta-myosin heavy chain and myosin-binding protein C.
Not all persons with a hypertrophic cardiomyopathy genetic defect are
symptomatic. High prevalence of Arginine to Glutamine Substitution
at 98, 141 and 162 positions in Troponin I (TNNI3) associated with
hypertrophic cardiomyopathy among Indians15. Fundamental
knowledge about HCM and sarcomere biology has emerged recently16.
In present study genetic study was not possible due to resources
limitations. Seth S et al17 in their study of 52 patients with RCMP had
heart failure with normal or near normal left ventricular size and
function. Similarly in present study total 5 (7.69%) patients had
(RCMP) had near normal LVEF 58% (±7.9) with bi-atrial enlagement,
functional MR and TR with more prevalence of atrial flutter/
fibrillation (80%) with ‘p’ value <0.02. Total 100% of RCMP patients
had diastolic dysfunction. Naser M. Ammash et al18 studied 94 patients
with echocardiographic criteria of idiopathic restrictive cardiomyopathy,
mainly dilated atria with nonhypertrophied, nondilated ventricles.
Atrial fibrillation was noted in 74% of patients and systolic dysfunction
in 16%. The endomyocardial fibrosis is the most common restrictive
cardiomyopathy observed world wide. The disease occurs in its classical
endemic form in the selected coastal belt in Kerala, India, affecting
young people of lower socio- economic families, and attracting
worldwide attention19. The Thalassemia associated with the restrictive
phenotype, with restrictive left ventricular filling, pulmonary
hypertension, and right heart failure. The pathophysiology of the
disorder is multifactorial, with a central role of myocardial iron
overload and the significant contribution of immuno inflammatory
mechanisms20. In our study one female patient with age 20 years had
Restrictive cardiomyopathy with Thalassemia with history of recurrent
blood transfusions who had multiform and multifocal VPC’s. Jahan
Ara Hasan et al21 studied 32 patients with peripartum cardiomyopathy of
them total 20 (62.57%) patients presented in late post partum period.
Similarly all 4 patients in present study presented in postpartum
periods with features of PPCM. The main complication was congestive
cardiac failure in 20 (62.57) patients and 3 maternal deaths occurred. In
our study all 4 patients with PPCM were discharged with no mortality.
The incidence of PPCM was 1 case per 1374 live births. Major risk
factors for the development of PCM were multiparity and advanced
maternal age. Echocardiography is useful in early diagnosis and for
follow-up assessment of left ventricular function. Most of the patients
noticed good symptomatic improvement at time of discharge and
during the follow-up with standard therapy22. The incidence of PPCM
was one case per 1374 live births in an Indian study23. Similarly in our
study total 4 (6.15%) patients had PPCM with mean age of 27.5
years(±6.24). total 4(100%) patients with PPCM 4 (100%) had
breathlessness, 2(50%) had hemoptysis had palpitations at the time of
admission. 4(100%) had cough 2(50%) had pedal edema and 1(25%)
had syncope. Low voltage & poor ‘r’ wave progression on ECG was
present in 4(100%) patients with ‘p’ value < 0.001. Total 2 (50%)
patients had diastolic dysfunction and the mean left ventricular ejection
fraction (LVEF) was 21% (±8.7). Total 2 (50%) patients had LV echocontrast on echocardiogram, 4 (100%) had mitral incompetence and
the mean of pulmonary artery pressure was 37(±3.8) mmHg. All four
41
Clinical and Echocardiogram Profile of Cardiomyopathy
patients with PPCM were discharged with improved LVEF with no
mortality. The abstinence from alcohol has been associated with
improvement in left ventricular function. Conversely, patients with
ongoing alcohol abuse and dilated cardiomyopathy have a poor
prognosis, with progressive biventricular failure and, ultimately,
death24. Alcoholic cardiomyopathy (ACM) causes myocyte dysfunction,
due to abnormalities in calcium homeostasis, and cause elevated levels
of norepinephrine. Increasing doses of ethanol have been associated
with a negative inotropic effect on myocytes in animal experiments25.
ACM is characterized by an increase in myocardial mass, dilation of
the ventricles, and wall thinning and associated with diastolic
dysfunction, whereas systolic dysfunction is a common finding in
symptomatic ACM patients. ACM remains an important cause of a
dilated cardiomyopathy, and in latter stages can lead to heart failure26.
Alcoholic cardiomyopathy represents about 3.8% of all cardiomyopathy
cases and it is a result of long-term (>5 years) alcohol consumption
(>90 g of alcohol per day). It occurs twice as frequently in men as in
women. Alcoholism is one of the main causes of myocardial damage27.
In our study one 45 years male with chronic alcoholic presented with
AVNRT with CCF with LV systolic dysfunction with LVEF 30%
(dilated cardiomyopathy) was improved with medical line of treatment
and was discharged with improved with LV systolic dysfunction and
was advised alcohol abstinence. The myocarditis is an inflammatory
disease of the heart frequently resulting from viral infections and/or
post-viral immune-mediated responses. It is one of the important
causes of dilated cardiomyopathy worldwide28. In our study one
patient had myocarditis probably viral with complete heart block
(CHB) which was reversible over period of six weeks and was treated
with temporary pacemaker and treatment of CCF.
Conclusion
Present study highlights significant burden of Idiopathic dilated cardiomyopathy and next to it was ischemic cardiomyopathy. A total 6
(9.23%) of patients had reversible cardiomyopathy like peripartum,
alcoholic and myocarditis. Total two patients with dilated cardiomyopathy succumbed with overall mortality 3.08% and case fatality rate
for DCM was 4.76%. Although cardiomyopathy is asymptomatic
in the early stages, symptoms are the same as those characteristically seen in any type of heart failure and may include shortness of
breath, fatigue, cough, orthopnea, paroxysmal nocturnal dyspnea, and
edema. Finding a potentially reversible cause (alcohol induced, peripartum cardiomyopathy, hyperthyroidism, ischemic cardiomyopathy,
tokotsubo cardiomyopathy and myocarditis etc.) of cardiomyopathy
is equally important. Trans-thoracic echocardiogram is an important,
simple and noninvasive modality of investigation which can be reproducible for the diagnosis and fallow up of most of the cardiomyopathies. Treatment is targeted at relieving the symptoms of heart failure
and reducing rates of heart failure–related hospitalization and mortality. Treatment options include pharmacotherapy, implantable cardioverter-defibrillators, cardiac resynchronization therapy, and heart
42
transplantation. Recommended lifestyle changes include restricting
alcohol consumption, losing weight, exercising, quitting smoking, and
eating a low-sodium diet. Judicious and proper use of anticoagulant,
digoxin, diuretics, ACE inhibitors and beta blocker is mandatory to
reduce complications, morbidity and mortality associated cardiomyopathies. The regular watch over vital organ dysfunction, drug side
effects and electrolyte imbalance is strongly recomonded.
Limitations of study: Present study was retrospective catering the patients
from rural area single centre study with resource limitations for doing
coronary angiogram, cardiovascular MRI, endomyocardial biopsy and
genetic study.
Conflicts of interest: We have no conflicts of interest to declare.
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