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36 INNOSC Theranostics and Pharmacological Sciences, 2023, Vol. 6, No. 1
also been reported to be a severe complication of
SCA leading to an accelerated death [7]. Hence,
PHT occurring secondary to SCA is classified as
a separate condition as the mortality due to SCA-
PHT can be 10 times higher than SCA patients
without PHT [8,9].
A diagnosis of PHT can be established by the
right ventricular catheterization with end systolic
pressure value ≥25 mmHg. However, there are
certain non-invasive methods available to diagnose
PHT such as Doppler echocardiography. It can
be used to assess the tricuspid valve regurgitant
velocity (TRV). TRV ≥2.5 m/s among SCA patients
has been established as a surrogate marker for
PHT [10-12]. Several studies have made efforts to
determine the burden of PHT using TRV among
children and adolescents with SCA [13-16].
However, studies have reported wide variation in
the prevalence of PHT among SCA children and
adolescents. However, there is scarcity in pooling
the knowledge regarding the prevalence of PHT in
the pediatric SCA patients. Hence, this study was
conducted to pool the evidence reporting the PHT
among SCA children and adolescents and report a
pooled estimate.
2. Materials and methods
2.1. Design
We conducted a systematic review and meta-
analysis of observational studies. We followed the
Preferred Reporting Items for Systematic Reviews
and Meta-analyses (PRISMA) checklist for
reporting systematic reviews incorporating meta- text of the articles that might meet the eligibility
analyses for reporting our review [17].
2.2. Eligibility criteria
2.2.1. Type of studies
For the present study, articles describing the
prevalence of PHT among SCA patients were
considered. There were no limitations based on
the site or design of the study. Unpublished data
and grey literature were discarded, but studies with
complete texts or abstracts were included.
2.2.2. Type of participants
Studies conducted among children and adolescents
(aged up to 19 years), irrespective of their ethnicity,
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comorbid status, or severity of the condition were
included in the study.
2.2.3. Type of outcome measure
Studies reporting the prevalence of PHT
and diagnosed it using either the right heart
catheterization or transthoracic Doppler
echocardiography were included in the study.
2.3. Search strategy
To find relevant information, we created a
thorough search strategy and conducted thorough,
methodical searches in databases and search
engines such as MEDLINE, SCOPUS, Web
of Science, ScienceDirect, Cochrane library,
and Google Scholar. To conduct the search, we
employed free-text headings and MeSH—selected
medical subject headings. The phrases “SCA,”
“PHT,” “Pulmonary Arterial Hypertension,”
“Children,” “Adolescent,” “Epidemiology,”
“Prevalence,” and “Burden” were used in different
combinations in PubMed and other search engines.
Without regard to language, the search period was
set from the database’s establishment through
January 2021. In addition, we manually searched
for any articles meeting the inclusion criteria and
cross-checked the bibliographies of the research
we have retrieved.
2.4. Study selection
Title, keyword, and abstract screening — or primary
screening — was carried out initially. The complete
requirements was obtained. The retrieved full
texts were then subjected to a further screening to
determine their eligibility based on pre-established
standards.
2.5. Data extraction
A pre-defined template was used as a data
extraction form to obtain the following set of data:
authors, title of study, year of publication, study
period, study design, setting, country/region, total
sample size, sampling criteria, diagnostic tool
and measures, cutoff, statistical tests, outcome
assessment details, average age, non-response
rate, and burden of PHT.