Treatment optimalization in pediatric pulmonary hypertension
Ablonczy László
Clinical Medicine
Dr. Reusz György
SE I. Gyermekgyógyászati Klinika tanterme
2023-06-12 14:00:00
Prevention of Chronic Diseases in Childhood
Dr. Tulassay Tivadar
Dr. Reusz György
Dr. Bohács Anikó egyetemi docens
Dr. Barta Judit egyetemi docens
Dr. Becker Dávid egyetemi tanár
Dr. Ledó Nóra egyetemi tanársegéd
Dr. Faludi Réka egyetemi docens
Three different studies were conducted to answer questions about the optimal treatment of PAH in children. According to international guidelines, treatment is based on risk stratification. We investigated the prognostic value of risk factors in our own patient population, confirming the important role of PVRi among hemodynamic factors. Accordingly, the role of high PVRi (>20 WU*m2) measured during RHC in early risk stratification is of particular importance. Similarly, etiology was found to be important, with better survival demonstrated in CHD-associated PAH than in the iPAH group. Due to study limitations, several risk factors could not be investigated, mainly due to age specificities. Our patients were assessed for both survival and quality of life. Poor WHO FC (II-IV) and low TAPSE were found to be the determinants of worse quality of life. At the same time, we confirmed a strong negative correlation between age and QoL, which is one of the difficulties in treating adolescents. Finally, we demonstrated good tolerability of subcutaneous treprostinil treatment in children, with rapid uptake in our practice. We recommended the initiation of early combination therapy including parenteral treprostinil, even at a good functional stage (WHO FC I-II), especially in the iPAH group. Further studies on quality of life and transplant-free survival in the different PAH patient groups with different etiologies are also recommended to develop an optimal individualised therapy.
