Right ventricular function mirrors clinical improvement with use of prostacyclin analogues in pediatric pulmonary hypertension
Pulmonary hypertension (PH) causes significant morbidity and mortality in children due to right ventricular (RV) failure. We sought to determine the effect of prostacyclin analogues on RV function assessed by echocardiography in children with PH. We conducted a retrospective cohort study of children...
| Main Authors: | , , , , , , , |
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| Format: | Article |
| Language: | English |
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Wiley
2018-02-01
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| Series: | Pulmonary Circulation |
| Online Access: | https://doi.org/10.1177/2045894018759247 |
| _version_ | 1818114992329719808 |
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| author | Rachel K. Hopper Yan Wang Valerie DeMatteo Ashley Santo Steven M. Kawut Okan U. Elci Brian D. Hanna Laura Mercer-Rosa |
| author_facet | Rachel K. Hopper Yan Wang Valerie DeMatteo Ashley Santo Steven M. Kawut Okan U. Elci Brian D. Hanna Laura Mercer-Rosa |
| author_sort | Rachel K. Hopper |
| collection | DOAJ |
| description | Pulmonary hypertension (PH) causes significant morbidity and mortality in children due to right ventricular (RV) failure. We sought to determine the effect of prostacyclin analogues on RV function assessed by echocardiography in children with PH. We conducted a retrospective cohort study of children with PH treated with a prostacyclin analogue (epoprostenol or treprostinil) between January 2001 and August 2015 at our center. Data were collected before initiation of treatment (baseline) and at 1–3 and 6–12 months after. Protocolized echocardiogram measurements including tricuspid annular plane systolic excursion (TAPSE) and RV global longitudinal strain were made with blinding to clinical information. Forty-nine individuals (65% female), aged 0–29 years at the time of prostacyclin initiation were included. Disease types included pulmonary arterial hypertension (idiopathic [35%], heritable [2%], and congenital heart disease-associated [18%]), developmental lung disease (43%), and chronic thromboembolic PH (2%). Participants received intravenous (IV) epoprostenol (14%) and IV/subcutaneous (SQ) (67%) or inhaled (18%) treprostinil. Over the study period, prostacyclin analogues were associated with improvement in TAPSE ( P = 0.007), RV strain ( P < 0.001), and qualitative RV function ( P = 0.037) by echocardiogram, and BNP ( P < 0.001), functional class ( P = 0.047) and 6-min walk distance ( P = 0.001). TAPSE and strain improved at early follow up ( P = 0.05 and P = 0.002, respectively) despite minimal RV pressure change. In children with PH, prostacyclin analogues are associated with an early and sustained improvement in RV function measured as TAPSE and strain as well as clinical markers of PH severity. RV strain may be a sensitive marker of RV function in this population. |
| first_indexed | 2024-12-11T03:59:32Z |
| format | Article |
| id | doaj.art-04d802c5840044a98d1e903321076dc4 |
| institution | Directory Open Access Journal |
| issn | 2045-8940 |
| language | English |
| last_indexed | 2024-12-11T03:59:32Z |
| publishDate | 2018-02-01 |
| publisher | Wiley |
| record_format | Article |
| series | Pulmonary Circulation |
| spelling | doaj.art-04d802c5840044a98d1e903321076dc42022-12-22T01:21:41ZengWileyPulmonary Circulation2045-89402018-02-01810.1177/2045894018759247Right ventricular function mirrors clinical improvement with use of prostacyclin analogues in pediatric pulmonary hypertensionRachel K. HopperYan WangValerie DeMatteoAshley SantoSteven M. Kawut0Okan U. ElciBrian D. HannaLaura Mercer-RosaDepartment of Medicine and the Center for Clinical Epidemiology and Biostatistics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USAPulmonary hypertension (PH) causes significant morbidity and mortality in children due to right ventricular (RV) failure. We sought to determine the effect of prostacyclin analogues on RV function assessed by echocardiography in children with PH. We conducted a retrospective cohort study of children with PH treated with a prostacyclin analogue (epoprostenol or treprostinil) between January 2001 and August 2015 at our center. Data were collected before initiation of treatment (baseline) and at 1–3 and 6–12 months after. Protocolized echocardiogram measurements including tricuspid annular plane systolic excursion (TAPSE) and RV global longitudinal strain were made with blinding to clinical information. Forty-nine individuals (65% female), aged 0–29 years at the time of prostacyclin initiation were included. Disease types included pulmonary arterial hypertension (idiopathic [35%], heritable [2%], and congenital heart disease-associated [18%]), developmental lung disease (43%), and chronic thromboembolic PH (2%). Participants received intravenous (IV) epoprostenol (14%) and IV/subcutaneous (SQ) (67%) or inhaled (18%) treprostinil. Over the study period, prostacyclin analogues were associated with improvement in TAPSE ( P = 0.007), RV strain ( P < 0.001), and qualitative RV function ( P = 0.037) by echocardiogram, and BNP ( P < 0.001), functional class ( P = 0.047) and 6-min walk distance ( P = 0.001). TAPSE and strain improved at early follow up ( P = 0.05 and P = 0.002, respectively) despite minimal RV pressure change. In children with PH, prostacyclin analogues are associated with an early and sustained improvement in RV function measured as TAPSE and strain as well as clinical markers of PH severity. RV strain may be a sensitive marker of RV function in this population.https://doi.org/10.1177/2045894018759247 |
| spellingShingle | Rachel K. Hopper Yan Wang Valerie DeMatteo Ashley Santo Steven M. Kawut Okan U. Elci Brian D. Hanna Laura Mercer-Rosa Right ventricular function mirrors clinical improvement with use of prostacyclin analogues in pediatric pulmonary hypertension Pulmonary Circulation |
| title | Right ventricular function mirrors clinical improvement with use of prostacyclin analogues in pediatric pulmonary hypertension |
| title_full | Right ventricular function mirrors clinical improvement with use of prostacyclin analogues in pediatric pulmonary hypertension |
| title_fullStr | Right ventricular function mirrors clinical improvement with use of prostacyclin analogues in pediatric pulmonary hypertension |
| title_full_unstemmed | Right ventricular function mirrors clinical improvement with use of prostacyclin analogues in pediatric pulmonary hypertension |
| title_short | Right ventricular function mirrors clinical improvement with use of prostacyclin analogues in pediatric pulmonary hypertension |
| title_sort | right ventricular function mirrors clinical improvement with use of prostacyclin analogues in pediatric pulmonary hypertension |
| url | https://doi.org/10.1177/2045894018759247 |
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