Assessment of Quality of Life in Children With Pulmonary Hypertension Using Parent and Self-report Questionnaires
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Materials and Methods
Data of 25 patients (aged 2-18 years, 17 male) with a diagnosis of PAH requiring medical treatment were reviewed retrospectively in this cross-sectional study. Basic patient characteristics are shown in Table 1.
Of 25 patients with PAH according to etiologic classification [8], 1 patient had idiopathic pulmonary hypertension (group 1.1), 15 patients had PAH associated with congenital heart disease (group 1.4.4), 2 patients had PAH associated with lung disease (group 3), and 7 patients had
Results
Twenty-five patients with pediatric PAH were enrolled in the study: 7 aged 2 to 4 years, 4 aged 5 to 7 years, 8 aged 8 to 12 years and 6 aged 13 to 18 years. Three patients (1 aged 5 to 7 years and 2 aged 13 to 18 years) did not complete any part of patient self-report survey because of mental disability.
Growth retardation defined as height less than −2.0 z score was observed in 5 of 25 patients (20%), weight less than −2.0 z score in 6 of 25 (24%) patients, and body mass index less than −2.0 z
Discussion
The treatment and prognosis of pediatric PAH depends on the presence of disease-specific risk factors [8] Echocardiographic parameters used for risk stratification PAH are left and right ventricular diameter, left and right ventricular diameter ratio, and TAPSE [11]. TAPSE represents basal right ventricular longitudinal function, which may be preserved in pediatric patients with PAH despite the already elevated hemodynamic parameters [12]. According to the recent pediatric PAH risk
Conclusions
In conclusion, the HRQOL of patients with PAH is impaired in almost every domain compared with healthy children and is similar to the group of patients with the most severe chronic heart disease with great complexity. We were also able to establish an association between the reduced systolic function of the right ventricle, as assessed by TAPSE, and the significantly lower HRQOL in all core domains.
These results point to the need for psychosocial rehabilitation in addition to somatic care to
Acknowledgements
We thank Gyöngyi Mate and Katalin Kiralyne Jene for specialty nursing and patient education.
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Funding Sources: G.S.R. was supported by NKFI grant no. 124549.