Effect of dual pulmonary vasodilator therapy in pulmonary arterial hypertension associated with congenital heart disease: a retrospective analysis.
Patients with pulmonary arterial hypertension (PAH) are managed according to evidence-based treatment guidelines.



In this single-centre retrospective analysis, we examined outcomes of patients with PAH caused by congenital heart disease (PAH-CHD) with respect to exercise capacity and survival of adults treated with either bosentan or sildenafil monotherapy or bosentan-sildenafil dual therapy between January 2007 and January 2014. Of the 82 patients analysed, 29 had Down syndrome; 54 (65.8%) received bosentan monotherapy, 16 (19.5%) sildenafil monotherapy and 12 (14.6%) dual therapy. Mean treatment duration was 2.5 years for all patients and 4.1 years for 38 patients treated for ≥2 years. Pooled patient and treatment data showed initial improvement followed by stabilisation in mean 6 min walk distance (6MWD). For Down and non-Down patients, mean 6MWD increased and then stabilised on bosentan monotherapy. Mean 6MWD of patients on dual therapy at the time of analysis was 246.3 m before PAH-specific therapy initiation, 211.9 m immediately prior to addition of a second therapy and 214.4 m at last visit while on dual therapy. 1, 2 and 3-year survival rates for all patients from time of treatment initiation were 96%, 87% and 80%, respectively.



For the majority of patients, monotherapy with a PAH-specific medication provided improved and sustained exercise benefits. For the small percentage of patients who required it, add-on therapy appeared to prevent further deterioration in exercise capacity but did not improve 6MWD.
University of Manchester, Institute of Cardiovascular Sciences, Manchester, UK; Manchester Heart Centre, Manchester Royal Infirmary, UK; Laboratory of Cardiovascular Science, National Institute on Aging-Intramural Research Program, National Institutes of Health, Baltimore, Maryland, USA.
Oliver
O
Monfredi
University of Manchester, Institute of Cardiovascular Sciences , Manchester , UK.
Elliot
E
Heward
Manchester Heart Centre, Manchester Royal Infirmary , UK.
Linda
L
Griffiths
Sheffield Pulmonary Vascular Disease Unit , Royal Hallamshire Hospital , Sheffield , UK.
Robin
R
Condliffe
University of Manchester, Institute of Cardiovascular Sciences, Manchester, UK; Manchester Heart Centre, Manchester Royal Infirmary, UK; Heart and Vascular Center, University of California, San Francisco, California, USA.
Vaikom S
VS
Mahadevan
CONGENITAL HEART DISEASE; Down syndrome