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(ISSN
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ABSTRACT
The article examines aspects of the clinical course, diagnostics and treatment of various forms of cardiomyopathy
(dilated, hypertrophic, restrictive) in children. Due to the relevance of the problem, the obtained data on the features
of the clinical course, diagnostics and tactics of patient management are of interest to cardiologists, general
practitioners and pediatricians of family clinics.
A total of 37 children aged 0-17 years were examined. Among those examined, the frequency of various forms of
cardiomyopathy was: dilated - 62.1%, hypertrophic - 32.4%, restrictive - 5.4%. Anamnestic data, severity of the course,
features of ECG, echocardiography, radiography for each form of cardiomyopathy were studied.
Latent, mild, but more often moderate and severe course of the disease was noted. Patients with DCM and HCM often
have various rhythm and conduction disorders. Outpatient observation and timely (continuous) therapy of CHF are
indicated for children with cardiomyopathies. In severe cases of HCM with obstructive forms of HCM, the issue of
surgical treatment is considered.
Research Article
CARDIOMYOPATHIES IN CHILDHOOD: CLINICAL COURSE, DIAGNOSIS
AND CARE TACTICS
Submission Date:
October 29, 2024,
Accepted Date:
November 03, 2024,
Published Date:
November 08, 2024
Crossref doi:
https://doi.org/10.37547/ijmscr/Volume04Issue11-03
Shadieva Khalima Nuridinovna
Associate Professor of the Department of Propaedeutics of Children's Diseases, Samarkand Medical University,
Uzbekistan
Qodirova Marxabo Miyassarovna
Assistant of the Department of Propaedeutics of Children's Diseases, Samarkand Medical University,
Uzbekistan
Journal
Website:
https://theusajournals.
com/index.php/ijmscr
Copyright:
Original
content from this work
may be used under the
terms of the creative
commons
attributes
4.0 licence.
Volume 04 Issue 11-2024
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International Journal of Medical Sciences And Clinical Research
(ISSN
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2771-2265)
VOLUME
04
ISSUE
11
P
AGES
:
14-22
OCLC
–
1121105677
Publisher:
Oscar Publishing Services
Servi
KEYWORDS
Dilated, hypertrophic, restrictive cardiomyopathy, children, chronic heart failure.
INTRODUCTION
Cardiovascular pathology in childhood is considered a
serious problem due to its prevalence, difficulties in
recognition, features of the course, treatment and
outcomes
[1-9].
Cardiomyopathy
in
pediatric
cardiology
remains
the
least
studied
and,
unfortunately, untreatable heart pathology. High
disability and mortality, non-specificity of clinical and
laboratory markers, lack of effective treatment
methods, difficulties in heart transplantation in
childhood determine the relevance of the problem. In
recent years, an increase in the incidence of various
forms of cardiomyopathy in children has been noted,
and this is primarily due to improved diagnostics. A true
increase in the incidence of cardiomyopathy is also
possible [7, 10-18].
Dilated cardiomyopathy (DCM) is the most common
form of cardiomyopathy in both adults and children.
DCM in children is a primary myocardial disease of
unknown
etiology,
characterized
by
dilation
predominantly of the left ventricle with a decrease in
the contractile ability of the myocardium. Most likely,
DCM has a polyetiological heterogeneous nature,
including viral persistence, genetic determination, and
autoimmune factors [10-16,18,20]. The incidence in
childhood is 0.5%. Genetic forms of DCM account for
approximately 30%. The incidence does not depend on
gender or age. The average age of children at the time
of diagnosis is 6-13 years [11,16,19].
Hypertrophic cardiomyopathy (HCM) is a genetically
determined disease of the heart muscle, characterized
by massive hypertrophy of the myocardium, most
often the left ventricle, with the obligatory
involvement of the interventricular septum. The
population frequency among children is 2.5 per
100,000. According to numerous studies in different
geographical locations around the globe, the
frequency of HCM is 0.2-1.1%. Symptoms of obstruction
are detected in only 20% of individuals with HCM [10-
12,16].
Restrictive cardiomyopathy (RCM) is the least rare of
all cardiomyopathies. The disease is based on
widespread interstitial fibrosis, combined with a sharp
thickening of the endocardium. With this type of
cardiomyopathy, diastolic function of the myocardium
suffers with little change in systolic function. The
etiology of the disease is unknown. It can occur in
children at any age, with a predominance in girls. The
incidence is about 5% among all forms of
cardiomyopathy. The mortality rate in children from
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the time of diagnosis is 63-75% from 3-6 years,
respectively, from the onset of the disease [10,11,16].
All forms of cardiomyopathy, given the increase in the
frequency of recognition, severity of the disease, lack
of effective methods of therapy, and high mortality,
require further study.
The goalto study the course, diagnostic criteria and
treatment tactics to improve the quality of life and
optimize the prognosis.
METHODS
The study included 37 patients who were inpatients in
the cardiology department and subsequently followed
up as outpatients at the ODMMC from 2019 to 2024.
The observed children were aged 0-17 years, including
19 boys (51.4%) and 18 girls (48.6%). All children
underwent echocardiography, ECG, chest X-ray,
ultrasound of the abdominal organs, and general
clinical tests. The main criteria for their determination
according to echocardiography data (in 4 patients,
additionally, MSCT of the heart and vessels with
contrast) are: the presence of dilation of the cavity or
hypertrophy of the myocardium of the left (in some
cases, the right) ventricle, the predominance of atrial
lesions (in patients with RCM) or ventricles, the degree
of change in the functional state of the heart
(decreased ejection fraction).
RESULTS AND DISCUSSION
When studying the anamnesis, a family history of
cardiomyopathy was found in 8 (21.6%) children, all of
whom had HCM, 2 (5.4%) had intrauterine TORCH
infection, and 12 (33.3%) children were frequently ill. In
our observation, we did not aim to conduct a genetic
examination; according to the anamnesis of our
patients with DCM, no familial cases were identified.
However, according to the literature, about 30-40% of
cases of familial isolated DCM have an established
genetic origin; more than 50 associated genes have
been identified, and this number is constantly
increasing as new genes are discovered [4,11,13,16]. In
all
examined
patients,
the
diagnosis
of
cardiomyopathy was made at least several months
after the onset of the first symptoms, after ineffective
therapy for other diseases (acute and recurrent upper
respiratory tract infections, pneumonia, carditis).
According to the severity of the disease, severe course
was observed in 24 patients (64.9%), moderate course
- 8 (21.6%), mild and asymptomatic - 5 (13.9%). Severe
course was observed mainly in patients with DCM, mild
and asymptomatic - in 5 children with HCM.
Cardiomyopathies in children were accompanied by
circulatory failure of HC I degree (FC1) - 7 (18.9%), HC II
degree (FC2-3) - 20 (54.1%), HC III degree (FC4) - 5
(13.5%). After clinical and instrumental examination, the
following forms of cardiomyopathy were revealed:
DCM - 23 (62.2%), RCM - 2 (5.4%) and HCM - 12 (32.4%),
among the latter, the obstructive form - in 5 children
(13.9%). These data are consistent with the literature on
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the incidence of various forms of cardiomyopathy
[10,11,15,16]. The distribution by gender was as follows:
among patients with DCM, there were 10 boys (43.5%),
13 girls (56.5%), 8 boys (66.7%) and 4 girls (33.3%) with
HCM, 1 boy (50%) and 1 girl (50%) with RCM.
ECG of patients with DCM reveals signs of left
ventricular hypertrophy (enlargement) (100%), atrial
overload (69.6%), and repolarization disorders, often
of ischemic origin (56.5%). Rhythm disorders include
sinus tachycardia (78.3%), supraventricular tachycardia
(17.4%), pacemaker migration (4.8%), ventricular
extrasystole (39.1%), and polytopic extrasystole in one
patient (4.3%), and atrial flutter (4.3%) (Fig. 1). In ECG in
HCM we found signs of left ventricular hypertrophy
with its overload (100%), repolarization disorders in the
form of ST depression and deep negative T waves (Fig.
2) (58.3%) - a characteristic sign of HCM, bundle branch
block in the form of left anterior block (25%), left
posterior block (8.3%), we found right bundle branch
block in (25%), extrasystoles - in (16.7%). In RCM, signs
of severe right atrium hypertrophy are the most
characteristic sign (100%), in our observations, patients
had rhythm disturbances only in the form of sinus
tachycardia (100%). The frequency of arrhythmias in
children with cardiomyopathy is also shown in the
works of foreign authors [11,13-17]. Arrhythmias are the
most
common
cause
of
sudden
death
in
cardiomyopathy [11,16,19,20].
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Fig. 1 ECG of patient M. with DCM. Sharp deviation of the EAH to the right, ventricular extrasystoles,
signs of overload of both parts of the heart, changes in the ventricular myocardium, decreased voltage
Fig. 2. ECG of patient A. with RCM. Deviation of the EAH to the right. Signs of severe RA overload.
Imcomplete blockade of the right bundle branch.
According to X-ray data, in DCM the heart is
significantly enlarged in cross-section due to the left or
both ventricles (Fig. 3). CTI was 0.60-0.71. The heart is
of a characteristic spherical or trapezoidal shape. In
(52%) patients, venous congestion was observed. In
HCM the heart shadow is enlarged in cross-section
mainly due to the left ventricle. CTI up to 0.66. In RCM
the pulmonary pattern is not enriched on the X-ray. The
heart shadow is slightly enlarged in cross-section. CTI -
up to 0.60. The arches are smoothed.
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Fig. 3. X-ray of patient M. with RCM.
In patients with all three forms, regardless of the
severity of the patient's condition, chronic heart failure
of varying degrees was noted. In 3 (25%) patients with
HCM, chronic heart failure was absent during the
survey and examination, in 2 (16.7%) it corresponded to
FC1. All patients with DCM had chronic heart failure.
Upon admission to the hospital FC3-4, upon discharge
- FC2. Patients with RCM were admitted to the hospital
with CHF FC3, and later treated outpatients with CHF
FC2.
In the hospital, children with DCM received cardiotonic
therapy with cardiac glycosides, dopamine if indicated,
ACE inhibitors, beta-blockers (carvedilol), diuretic,
cardiotrophic and symptomatic therapy. Positive
dynamics were noted against the background of the
therapy in the hospital. However, after discharge,
despite
outpatient
treatment,
CHF
gradually
progressed again. 2 (9.1%) girls with DCM died. Their
disease duration was more than 5 years. The remaining
children in the follow-up are under dispensary
registration at the place of residence and receive
symptomatic therapy for CHF. Patients with HCM
received treatment with beta-blockers (egilok) on an
outpatient basis. 1 child was operated on in the cardiac
surgery department of the ODMMC, the condition
after the operation improved significantly, continues
to receive egilok. 2 children with RCM received therapy
for CHF, but without the use of inotropic drugs; they
were treated jointly with a gastroenterologist, since
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congestion in the liver and ascites led to
gastrointestinal dysfunction. All patients with DCM and
RCM received anticoagulant therapy.
Thus,
the
frequency
of
various
forms
of
cardiomyopathy in children in the overall structure of
cardiovascular diseases is currently increasing.
Diagnostics has improved, and, consequently, the
detection of all forms. Protocols for the treatment of
cardiomyopathy have been developed. Most patients
with severe cardiomyopathy, with proper monitoring
and treatment, have a disease history of more than 5
years. However, a large number of complications
requires that this pathology be classified as a disease
with an unfavorable prognosis.
CONCLUSIONS
1. Among those examined, the frequency of various
forms of cardiomyopathy was: DCM - 61.1%, HCM -
33.3%, RCM - 5.6%.
2. Almost all forms of cardiomyopathy (with the
exception of 3 patients with HCM) were accompanied
by moderate and severe persistent CHF.
3. Often, late detection of the disease in the majority of
patients dictates the need for at least an annual
examination of all children using the echocardiography
method.
4. Most patients with DCM and HCM exhibit various
rhythm and conduction disturbances.
5. Dispensary observation and timely (continuous)
therapy of CHF in children with cardiomyopathy are
indicated.
6. For patients with obstructive forms of HCM, the
issue of surgical treatment is considered.
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