title: Clinical significance of dynamic pulmonary vascular resistance in two populations at risk of pulmonary arterial hypertension.
abstract: OBJECTIVE
  Patients at risk of pulmonary arterial hypertension (PAH) may present with abnormal dynamic pulmonary vascular resistance (PVR) during exercise. However, its clinical significance remains unclear. The present study aimed at analysing the meaning of dynamic PVR in two populations at risk of PAH: secundum atrial septal defect (ASD) and systemic sclerosis (SSc).
  
  
  RESULTS
  Adult patients with corrected ASD were consecutively selected from the database of Pediatric and Congenital Heart Disease of the University Hospitals Leuven. Patients with SSc were consecutively selected from the rheumatology database of the University Hospital Liège. At inclusion, all underwent a rest and bicycle stress echocardiography to obtain baseline right heart characteristics and dynamic PVR. Routine follow-up echocardiography was performed. Twenty-eight patients with corrected ASD (mean age 41 ± 17 years, 79% female) were followed for a median time of 3.7 [inter-quartile range (IQR) 2.9-4.1] years. No patient developed PAH. Dynamic PVR was significantly associated with right atrial dilatation at latest follow-up (Spearman's ρ 0.51, P = 0.013). Forty-five SSc patients (mean age 54 ± 13 years, 76% female) were followed for a median time of 2.4 (IQR 0.8-2.9) years. Thirteen patients (30%) developed PAH. Dynamic PVR was the only independent predictor of PAH (hazards ratio 1.22, 95% confidence interval 1.01-1.47). No significant right heart morphometric changes occurred.
  
  
  CONCLUSIONS
  Dynamic PVR predicted PAH development in patients with SSc, whereas dynamic PVR was associated with right heart morphometric changes after ASD closure. The predictive role of dynamic PVR might depend on the underlying disease type. Larger studies are needed to confirm this hypothesis.
authors.affiliation: Department of Cardiology, University Hospitals Leuven, Herestraat 49, Leuven B-3000, Belgium.
authors.forename: Charlien
authors.initials: C
authors.lastname: Gabriels
authors.affiliation: Department of Cardiology, GIGA Cardiovascular Sciences, University Hospital Sart Tilman Liège, Liège B-4000, Belgium.
authors.forename: Patrizio
authors.initials: P
authors.lastname: Lancellotti
authors.affiliation: Department of Cardiology, University Hospitals Leuven, Herestraat 49, Leuven B-3000, Belgium.
authors.forename: Alexander
authors.initials: A
authors.lastname: Van De Bruaene
authors.affiliation: Department of Cardiology, GIGA Cardiovascular Sciences, University Hospital Sart Tilman Liège, Liège B-4000, Belgium.
authors.forename: Damien
authors.initials: D
authors.lastname: Voilliot
authors.affiliation: Department of Cardiology, University Hospitals Leuven, Herestraat 49, Leuven B-3000, Belgium.
authors.forename: Pieter
authors.initials: P
authors.lastname: De Meester
authors.affiliation: Department of Rehabilitation Sciences, University Hospitals Leuven, Leuven, Belgium.
authors.forename: Roselien
authors.initials: R
authors.lastname: Buys
authors.affiliation: Department of Pneumology, University Hospitals Leuven, Leuven, Belgium.
authors.forename: Marion
authors.initials: M
authors.lastname: Delcroix
authors.affiliation: Department of Cardiology, University Hospitals Leuven, Herestraat 49, Leuven B-3000, Belgium werner.budts@uzleuven.be.
authors.forename: Werner
authors.initials: W
authors.lastname: Budts
mesh_terms: D000328:Adult; D025401:Echocardiography, Stress; D005260:Female; D006344:Heart Septal Defects, Atrial / Q000503:physiopathology*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D012306:Risk; D012595:Scleroderma, Systemic / Q000503:physiopathology*; D014655:Vascular Resistance / Q000502:physiology*
keywords: atrial septal defect; pulmonary vascular disease; stress echocardiography; systemic sclerosis