title: Right ventricular function predicts clinical response to specific vasodilator therapy in patients with pulmonary hypertension.
abstract: BACKGROUND
  We followed patients with pulmonary arterial hypertension (PAH) receiving specific vasodilator therapy and tested for predictors of clinical outcome.
  
  
  METHODS
  Thirty-two patients (mean age 39 ± 15 years, 22 women, diagnosed with pulmonary hypertension; PH): 29 with PAH and 3 patients with inoperable chronic thromboembolic PH received therapy with either bosentan, sildenafil, or both and were evaluated with clinical parameters, biomarkers (B-type natriuretic peptide values), and echocardiography before receiving specific medication and every 3 months thereafter. A right heart catheterization was performed at baseline. A composite endpoint of death, worsening of functional class, or the need of a second vasodilator agent was used to define the clinical nonresponders.
  
  
  RESULTS
  Patients were followed for 14 months (7.5-21). The endpoint was reached by 15 patients: four patients died (two idiopathic PAH and two PAH in context of Eisenmenger syndrome), seven patients showed 1 functional class worsening, and four patients needed to be switched to combination therapy. Patients who remained clinically stable or improved had at baseline a better cardiac output with a less remodeled right ventricle (RV) and better functioning RV (all P < 0.05). A RV fractional area change (RVFAC) lower than 25.7% and a RV global strain value higher than -13.4% predict with 87% sensitivity and 83% specificity (AUC 87.3%, P = 0.001) and 73% sensitivity and 91% specificity (AUC 84.2%, P = 0.003), respectively, patients who will deteriorate clinically under specific vasodilator therapy. A multivariate model showed RVFAC to be the only independent predictor of the endpoint with a HR of 0.87 (0.8-0.96), P = 0.007.
  
  
  CONCLUSIONS
  Over an average period of 1 year, almost half of patients showed signs of clinical deterioration despite specific vasodilator therapy. Parameters of right ventricular morphology and function had prognostic value in these patients.
authors.affiliation: Cardiology Department, Institute for Emergencies in Cardiovascular Diseases C C Iliescu, Bucharest, Romania. soringiusca@gmail.com
authors.forename: Sorin
authors.initials: S
authors.lastname: Giusca
authors.forename: Ruxandra
authors.initials: R
authors.lastname: Jurcut
authors.forename: Ioan Mircea
authors.initials: IM
authors.lastname: Coman
authors.forename: Ioana
authors.initials: I
authors.lastname: Ghiorghiu
authors.forename: Daniela
authors.initials: D
authors.lastname: Catrina
authors.forename: Bogdan A
authors.initials: BA
authors.lastname: Popescu
authors.forename: Laura
authors.initials: L
authors.lastname: Dima
authors.forename: Carmen
authors.initials: C
authors.lastname: Ginghina
mesh_terms: D000328:Adult; D000077300:Bosentan; D004452:Echocardiography / Q000379:methods*; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications / Q000000981:diagnostic imaging* / Q000188:drug therapy*; D008297:Male; D010879:Piperazines / Q000008:administration & dosage; D011687:Purines / Q000008:administration & dosage; D015203:Reproducibility of Results; D012680:Sensitivity and Specificity; D000068677:Sildenafil Citrate; D013449:Sulfonamides / Q000008:administration & dosage; D013450:Sulfones / Q000008:administration & dosage; D016896:Treatment Outcome; D014665:Vasodilator Agents / Q000008:administration & dosage*; D018497:Ventricular Dysfunction, Right / Q000000981:diagnostic imaging* / Q000209:etiology / Q000517:prevention & control*; D016278:Ventricular Function, Right
chemical_list: D010879:Piperazines; D011687:Purines; D013449:Sulfonamides; D013450:Sulfones; D014665:Vasodilator Agents; D000068677:Sildenafil Citrate; D000077300:Bosentan