RT Journal Article
SR Electronic
T1 Triple vasodilator therapy in pulmonary arterial hypertension associated with congenital heart disease
JF Heart
JO Heart
FD BMJ Publishing Group Ltd and British Cardiovascular Society
SP 346
OP 352
DO 10.1136/heartjnl-2023-323015
VO 110
IS 5
A1 Luna-Lopez, Raquel
A1 Segura de la Cal, Teresa
A1 Sarnago Cebada, Fernando
A1 Martin de Miguel, Irene
A1 Hinojosa, Williams
A1 Cruz-Utrilla, Alejandro
A1 Velazquez, Maria Teresa
A1 Delgado, Juan F
A1 Mendoza, Alberto
A1 Arribas Ynsaurriaga, Fernando
A1 Escribano-Subías, Pilar
YR 2024
UL http://heart.bmj.com/content/110/5/346.abstract
AB Objective This study assessed the long-term effects of triple therapy with prostanoids on patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD), as there is limited information on the safety and efficacy of this treatment approach.Methods A retrospective cohort study was conducted on patients with PAH-CHD who were actively followed up at our centre. All patients were already receiving dual combination therapy at maximum doses. Clinical characteristics, including functional class (FC), 6-minute walking test distance (6MWTD) and N-terminal pro-brain natriuretic peptide (NT-proBNP) levels, were documented before initiating triple therapy and annually for a 2-year follow-up period.Results A total of 60 patients were included in the study, with a median age of 41 years and 68% being women. Of these, 32 had Eisenmenger syndrome, 9 had coincidental shunts, 18 had postoperative PAH and 1 had a significant left-to-right shunt. After 1 year of triple combination initiation, a significant improvement in 6MWTD was observed (406 vs 450; p=0.0027), which was maintained at the 2-year follow-up. FC improved in 79% of patients at 1 year and remained stable in 76% at 2 years. NT-proBNP levels decreased significantly by 2 years, with an average reduction of 199 ng/L. Side effects were experienced by 33.3% of patients but were mostly mild and manageable. Subgroup analysis showed greater benefits in patients without Eisenmenger syndrome and those with pre-tricuspid defects.Conclusions Triple therapy with prostanoids is safe and effective for patients with PAH-CHD, improving FC, 6MWTD and NT-proBNP levels over 2 years. The treatment is particularly beneficial for patients with pre-tricuspid defects and non-Eisenmenger PAH-CHD.All data relevant to the study are included in the article or uploaded as supplemental information.