title: Pulmonary arterial hypertension associated with a congenital heart defect: advanced medium-term medical treatment stabilizes clinical condition.
abstract: 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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors: Academic Medical Center-Department of Cardiology, Amsterdam, The Netherlands.
authors: Mariëlle
authors: M
authors: Duffels
authors: Laura
authors: L
authors: van Loon
authors: Rolf
authors: R
authors: Berger
authors: Anco
authors: A
authors: Boonstra
authors: Anton
authors: A
authors: Vonk-Noordegraaf
authors: Anton
authors: A
authors: Vonk-Noordergraaf
authors: Barbara
authors: B
authors: Mulder
mesh_terms: D000328:Adult; D000367:Age Factors; D000368:Aged; D000959:Antihypertensive Agents / Q000627:therapeutic use*; D001794:Blood Pressure; D006328:Cardiac Catheterization; D004359:Drug Therapy, Combination; D004541:Eisenmenger Complex / Q000150:complications; D065128:Endothelin Receptor Antagonists; D005080:Exercise Test / Q000379:methods; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000150:complications*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D010726:Phosphodiesterase Inhibitors / Q000627:therapeutic use*; D011651:Pulmonary Artery / Q000503:physiopathology; D012189:Retrospective Studies; D012720:Severity of Illness Index; D013997:Time Factors; D016896:Treatment Outcome; D014655:Vascular Resistance; D016138:Walking
chemical_list: D000959:Antihypertensive Agents; D065128:Endothelin Receptor Antagonists; D010726:Phosphodiesterase Inhibitors