Key result
Advanced medical therapy in patients with PAH associated with CHD stabilized clinical condition, with 6-minute walk distance remaining unchanged (increase of 44 +/- 78 m at 1 year, P=0.2).
Why the study?
Does advanced medical therapy stabilize clinical condition and exercise tolerance in adult patients with PAH associated with CHD?
Observational (n=15)
No
Does advanced medical therapy stabilize clinical condition and exercise tolerance in adult patients with PAH associated with CHD?
p-value: p=0.2
Advanced medical therapy in adult patients with PAH associated with CHD appears to stabilize the disease and prevent deterioration in exercise capacity and hemodynamics over medium-term follow-up.
May support stabilization in PAH-CHD; hypothesis-generating and requires randomized confirmation before practice change.
OBJECTIVE: Pulmonary arterial hypertension (PAH) associated with congenital heart defect (CHD), and especially Eisenmenger syndrome, is associated with impaired exercise tolerance and reduced quality of life. In this study, we describe medium-term follow-up of adult patients with PAH associated with CHD, treated in a single center with different types of advanced medication. DESIGN: The treatment and clinical course of 15 patients (11 female, median age 53, range 28-74 years) with PAH associated with CHD is retrospectively described. Data on patient characteristics, exercise test, right-heart catheterization, and type of advanced therapy were collected from medical files. Advanced medical therapy consisted of either intravenous prostacyclin, or endothelin receptor antagonists, or phosphodiesterase-5-inhibitors. Additional therapy was started in case of persistent clinical deterioration or insufficient improvement with monotherapy. RESULTS: All patients (10 patients with Eisenmenger syndrome, 5 patients with a closed defect and PAH) were exposed to different durations of advanced medication. Median period of treatment was 2.5 (range 0.7-6.3) years. Atrial septal defect, type secundum, was the most frequent underlying diagnosis (n = 10). Most patients (n = 9) received a combination of advanced medical therapy. Six-minute walk distance (6-MWD) remained unchanged with an increase of 44 +/- 78 m (P = 0.2) and 41 +/- 80 m (P = 0.3) compared with baseline after respectively 1 and 2 years of treatment. Younger age was associated with better performance (beta = -7 m per year, P < 0.05), patients younger than 45 years showed a greater improvement in 6-MWD after 2 years of treatment (P < 0.05). During a mean follow-up of 23 (range 4-58) months, mean pulmonary arterial pressure (53 +/- 24-49 +/- 17 mmHg, P = 0.3) and pulmonary vascular resistance (770 +/- 1090-650 +/- 444 dynes s/cm5, P = 0.7) showed no deterioration. CONCLUSION: Advanced treatment strategies in patients with PAH associated with CHD are useful. The treatment effect seems to be one of disease stabilization and decreasing the rate of deterioration. Younger age was associated with a greater improvement of 6-MWD.
No takes yet. Share an insight, caveat, or question.
Duffels et al. (2007) conducted an observational in Pulmonary arterial hypertension associated with congenital heart defect (n=15). Advanced medical therapy (prostacyclin, endothelin receptor antagonists, or phosphodiesterase-5-inhibitors) was evaluated on Change in 6-minute walk distance from baseline at 1 year (p=0.2). Advanced medical therapy in patients with PAH associated with CHD stabilized clinical condition, with 6-minute walk distance remaining unchanged (increase of 44 +/- 78 m at 1 year, P=0.2).
Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context: