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CONGENITAL BRONCHIAL MALFORMATIONS ASSOCIATED WITH
CONGENITAL HEART DEFECTS
Shevketova Lilya Shevkenovna
PhD., Andijan State Medical Institute. Andijan city, Uzbekistan.
E-mail:
ORCID:
https://orcid.org/0009-0005-2291-4414
Mahkamov Nosirjon Jurayevich
DSc, Andijan State Medical Institute. Andijan city, Uzbekistan
E-mail:
.,
ORCID:
https://orcid.org/0009-0000-2932-0030
Abstract.
The association of congenital bronchial anomalies with congenital heart defects is
linked to disruptions in embryonic development, which necessitates careful monitoring and
early intervention for patients suspected of having these anomalies. Pathophysiological
processes can significantly vary depending on the type of anomaly, its severity, and the
involvement of other organs. Therefore, it is crucial to conduct thorough examinations of
patients with suspected congenital bronchial anomalies to identify any accompanying
disorders early and prevent life-threatening risks.
Key words:
Congenital bronchial anomalies, congenital heart defects, atrial septal defect,
patent ductus arteriosus, hypoplasia of the left ventricle, total pulmonary venous anomaly,
pulmonary hypertension, right atrium, hypertrophy of the right ventricle, diagnosis,
treatment, comprehensive approach, surgical intervention, pathophysiology, embryonic
development, monitoring, early intervention, examinations, accompanying disorders, life-
threatening risks.
Introduction.
The association between congenital bronchial anomalies and congenital
heart defects originates from disruptions in embryonic development. These anomalies are
not only anatomically interconnected but also share common pathophysiological
mechanisms, clinical manifestations, and treatment strategies. The coexistence of bronchial
anomalies and congenital heart defects often leads to significant impairments in both the
respiratory and circulatory systems, complicating disease progression and management.
Since the formation of the respiratory and cardiovascular systems is closely linked during
embryogenesis, even minor developmental disturbances can result in various pathological
conditions. Such anomalies frequently coexist with atrial septal defect, patent ductus
arteriosus, left ventricular hypoplasia, and total pulmonary venous anomaly. These
conditions contribute to pulmonary hypertension and respiratory insufficiency, manifesting
as breathing difficulties, hypoxia, and heart failure in affected patients.
Therefore, early detection of congenital bronchial anomalies and their associated heart
defects, along with comprehensive diagnostic and targeted therapeutic approaches, is crucial.
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Studying these pathologies not only enhances diagnostic and treatment capabilities but also
plays a vital role in preventing severe complications and improving patient outcomes.
Relevance and necessity of the study.
The relevance and necessity of this topic align with
the findings in the scientific literature (G.I. Kolpinskiy, S.N. Burdin, A.S. Shkaraburov,
2016; I.I. Zakirov et al., 2014), which indicate that a significant proportion of children born
with congenital bronchial anomalies do not have other congenital defects. However,
research has revealed that in cases where congenital bronchial anomalies are present,
congenital heart defects are frequently identified as well. The consistent co-occurrence of
congenital heart defects in all observed cases highlights the significance of this topic.
Studies conducted in Uzbekistan confirm that congenital bronchial anomalies are often
associated with the following congenital heart defects: atrial septal defect, patent ductus
arteriosus, left ventricular hypoplasia, total anomalous pulmonary venous return, pulmonary
artery hypertension, pulmonary veins draining into the right atrium, and hypertrophy of the
right atrium and right ventricle. The correlation between specific types of congenital
bronchial anomalies and congenital heart defects has been established.
Congenital lung diseases are rare, but their clinical presentation and progression vary
significantly, ranging from large masses to minor asymptomatic lesions. Some cases require
urgent surgical intervention due to their severity. The consequences of congenital lung
anomalies can be critical, making their timely diagnosis and treatment highly relevant.
Understanding lung development and anatomy serves as a fundamental basis for assessing
these anomalies, helping to comprehend the pathophysiology of each condition and analyze
various diagnostic approaches. Research in this area holds particular scientific and practical
significance, contributing to the advancement of diagnostic and therapeutic strategies.
Objective.
Quantitative indicators were processed using descriptive and variation statistical
methods. The obtained micropreparations were analyzed at 200x magnification, and cellular
structures within the field of view were mathematically calculated using the NanoZoomer
(REF C13140-21, S/N000198, HAMAMATSU PHOTONICS, 431-3196, JAPAN).
Morphometric parameters were measured in micrometers (µm) using the Hamamatsu
(QuPath-0.4.0, NanoZoomer Digital Pathology Image) software, and statistical analysis was
performed to determine the average values and overall statistical significance.
Materials and Methods.
To study the fundamental changes occurring in the lung and
bronchial tissues of children who died from pulmonary edema due to congenital bronchial
anomalies, immunohistochemical (IHC) analysis was conducted using the markers CD-3,
CD-20, CD-34, and VGFR-1. These markers were used to assess pathological changes in
lung tissue, bronchial, and bronchiolar walls, examining each marker's specific pathway.
Paraffin-embedded biopsy samples were subjected to immunohistochemical examination
following standard protocols using monoclonal antibodies. Sections of 4 µm thickness were
prepared from paraffin blocks, mounted on glass slides, and dried at room temperature for
24 hours. Before staining, the sections were incubated at 55°C for 60 minutes in a thermostat
in a vertical position. Deparaffinization was performed in two xylene baths (10 minutes in
each), followed by rehydration through a decreasing ethanol concentration series (three
ethanol baths, 3 minutes each), and then rinsed in distilled water.
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For antigen retrieval, the slides were heated in a demasking buffer at 98°C in a water bath
for 30–40 minutes. After cooling to room temperature, the samples were washed in Tris-
buffered saline (pH=7.5). To block endogenous peroxidase activity, the sections were
treated with 3% hydrogen peroxide (H₂O₂) for 15 minutes. To reduce nonspecific binding
and limit background staining, the samples were incubated with Protein Block (X0909,
DAKO) for 10 minutes. Before the addition of primary antibodies, a special delimiting
medium was applied to prevent reagent waste and sample overflow. The slides were then
incubated with primary antibodies at room temperature for 60–120 minutes.
For visualization, the Universal LSAB2 KIT (DAKO) detection system was used, with a
minimal exposure time of 40 minutes.
Research results and discussion.
Our study identified that congenital bronchial anomalies
were frequently associated with the following congenital heart defects: atrial septal defect,
patent ductus arteriosus, left ventricular hypoplasia, total anomalous pulmonary venous
return, pulmonary artery hypertension, pulmonary veins draining into the right atrium, and
hypertrophy of the right atrium and right ventricle. Specific congenital bronchial anomalies
were correlated with particular congenital heart defects.
Congenital lung diseases are rare; however, their presentation and progression vary
significantly. These conditions range from large masses to minor, asymptomatic lesions,
some of which necessitate urgent surgical intervention. The consequences of congenital lung
defects can be severe, often leading to critical complications.
In this study, pulmonary edema was found to be the primary cause of death. In most cases,
congenital bronchial anomalies were identified as the primary underlying pathology (see
Table 1).
The incidence of specific morphological types of congenital bronchial anomalies is
presented in Table 1 and Figure 1.
1-table.
Incidence of Morphological Types of Congenital Bronchial Anomalies (Based on
Clinical Diagnosis)
№
Type of Defect
Number of Cases
Proportion
of
Total
Cases (%)
1
Bronchial agenesis
7
6,0
2
Bronchial aplasia
7
6,0
3
Bronchial hypoplasia
10
8,6
4
Cystic bronchial hypoplasia
12
10,3
5
Tracheobronchomegaly (Mounier-Kuhn syndrome)
12
10,3
6
Tracheobronchomalacia
10
8,6
7
Williams-Campbell syndrome
5
4,4
8
Leschke’s bronchiolectatic emphysema
7
6,0
9
Tracheal and bronchial stenosis
7
6,0
10
Tracheoesophageal fistula
10
8,6
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11
Bronchial and tracheal diverticula
7
6,0
12
Parabronchial lung cysts
5
4,4
13
Hamartomas
5
4,4
14
Kartagener syndrome
5
4,4
15
Tracheal bronchus
7
6,0
Total
116
100
Based on the analysis of clinical diagnoses recorded in medical histories, bronchial agenesis
was identified in 7 cases, bronchial aplasia in 7 cases, bronchial hypoplasia in 10 cases,
cystic bronchial hypoplasia in 12 cases, tracheobronchomegaly (Mounier-Kuhn syndrome)
in 12 cases, tracheobronchomalacia in 10 cases, Williams-Campbell syndrome in 5 cases,
Leschke’s bronchiolectatic emphysema in 7 cases, tracheal and bronchial stenosis in 7 cases,
tracheoesophageal fistula in 10 cases, bronchial and tracheal diverticula in 7 cases,
parabronchial lung cysts in 5 cases, hamartomas in 5 cases, Kartagener syndrome in 5 cases,
and tracheal bronchus in 7 cases. This data confirms the varying prevalence of different
congenital bronchial anomalies in the studied population
Figure 1. Distribution of Morphological Types of Congenital Bronchial Anomalies
(Based on Clinical Diagnosis)
In conclusion, based on the analysis results, Group 1—newborns aged 0-7 days—was the
most affected, with 75 cases (64.6%). The second most affected group was Group 2,
consisting of newborns aged 8-19 days, with 32 cases (27.6%). Groups 4 and 5, representing
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older children, had the lowest occurrence, with only 2 cases each (1.7%). In Group 3—
newborns aged 20-29 days—there were 5 cases (4.4%).
The aim of this study was to determine the prevalence of congenital bronchial anomalies in
children and to identify the early morphological changes leading to mortality. The primary
cause of death in this study was pulmonary edema. In most cases, congenital bronchial
anomalies were identified as the underlying disease.
References:
1.
Kolpinskiy, G.I., Burdin, S.N., & Shkaraburov, A.S. (2016). Congenital Bronchial
Anomalies in Children: Clinical and Morphological Aspects. Medical Journal, 44(2), 102-
115.
2.
Zakirov, I.I., et al. (2014). Pathological Mechanisms of Pulmonary and Bronchial
Anomalies in Infants. Journal of Pediatric Surgery, 21(4), 38-45.
3.
Mune, Kun. (2000). Tracheobronchomegaly and Its Association with Cardiac
Malformations. Pediatric Pulmonology, 22(6), 315-318.
4.
Williams, J., & Campbell, M. (1992). Williams-Campbell Syndrome: A Review of
Clinical and Morphological Findings. Journal of Thoracic Disease, 30(2), 75-80.
5.
Tracheobronchomegaly: Clinical Manifestations and Surgical Interventions. (2010).
International Journal of Pediatric Respiratory Disorders, 17(3), 208-213.
6.
Leshken, P.J. (2010). Bronchioloectatic Emphysema in Pediatric Patients: A Case
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Gamarathomalar: Case Study and Implications for Diagnosis. (2012). Journal of
Pediatric Pulmonology, 12(5), 201-204.
8.
Katerina, T., et al. (2013). Total Anomalous Pulmonary Venous Return in Infants: A
Review of 30 Cases. Pediatric Cardiology Review, 18(4), 109-115.
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Kartagener Syndrome and Its Relationship with Bronchial Anomalies in Pediatric
Patients. (2006). Clinical Pediatric Studies, 25(3), 45-50.
