Effect of dual pulmonary vasodilator therapy in pulmonary arterial hypertension associated with congenital heart disease: a retrospective analysis

Abstract

BackgroundPatients with pulmonary arterial hypertension (PAH) are managed according to evidence-based treatment guidelines.Methods and resultsIn 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.ConclusionsFor 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.

Comparison of short-term outcome between monotherapy versus combination therapy in the treatment of pulmonary hypertension associated with congenital heart disease

“…However, upper respiratory tract infection and vomiting had a higher prevalence in group A participants. A retrospective analytical study also showed similar 17 However, a study with patients suffering from Eisenmenger syndrome showed that upfront combination therapy with bosentan and sildenafil was not superior to bosentan monotherapy concerning changes in 6MWD. 18 As such, the general approach regarding treatment is to observe and record clinical worsening, to guide the decision to escalate treatment only when necessary.…”

Comparison of adverse effects in the treatment of pulmonary hypertension with monotherapy and combination therapy

“…During the study period, 70 patients were enrolled: 35 were assigned to sildenafil monotherapy (Group A), and 17 However, a study with Eisenmenger syndrome patients found that upfront combination therapy with Bosentan and sildenafil was not superior to Bosentan monotherapy in terms of changes in 6MWD. 18 As a result, the general treatment approach is to observe and record clinical worsening in order to guide the decision to escalate treatment only when necessary.…”

Prognostic Value of Pulmonary Artery Compliance in Patients with Pulmonary Arterial Hypertension Associated with Adult Congenital Heart Disease

“…There is established evidence that PAH-specific therapeutic drugs have efficacy in APAH-CHD patients. [20][21][22] In general, the determination of the timing of initiation of proactive or aggressive PAH-specific therapy depended on worsening of cardiac function, such as WHO function class and 6-MWD. Tan, et al 23) considered that rather than the consequence of distal proliferative vasculopathy, decreased Cp contributed to the progressive damage of microvasculature.…”