title: Right ventricular contractility in systemic sclerosis-associated and idiopathic pulmonary arterial hypertension.
abstract: Since systemic sclerosis (SSc) also involves the heart, the aim of the present study was to evaluate possible differences in right ventricular (RV) pump function between SSc-associated pulmonary arterial hypertension (PAH; SScPAH) and idiopathic PAH (IPAH). In 13 limited cutaneous SScPAH and 17 IPAH patients, RV pump function was described using the pump function graph, which relates mean RV pressure ((RV)) and stroke volume index (SVI). Differences in pump function result in shift or rotation of the pump function graph. (RV) and SVI were measured using standard catheterisation. The hypothetical isovolumic (RV) ((RV,iso)) was estimated using a single-beat method. The pump function graph was approximated by a parabola: (RV) = (RV,iso)[1-(SVI/SVI(max))(2)], where SVI(max )is the hypothetical maximal SVI at zero (RV), enabling calculation of SVI(max). There were no differences in SVI and SVI(max). Both (RV) and (RV,iso) were significantly lower in SScPAH than in IPAH ((RV) 30.7+/-8.5 versus 41.2+/-9.4 mmHg; (RV,iso) 43.1+/-12.4 versus 53.5+/-10.0 mmHg). Since higher pressures were found at similar SVI, the difference in the pump function graph results from lower contractility in SScPAH than in IPAH. Right ventricular contractility is lower in systemic sclerosis-associated pulmonary arterial hypertension than in idiopathic pulmonary arterial hypertension.
authors.affiliation: Dept of Pulmonary Diseases, VU University Medical Centre, Vrije University, Amsterdam, The Netherlands.
authors.forename: M J
authors.initials: MJ
authors.lastname: Overbeek
authors.forename: J-W
authors.initials: JW
authors.lastname: Lankhaar
authors.forename: N
authors.initials: N
authors.lastname: Westerhof
authors.forename: A E
authors.initials: AE
authors.lastname: Voskuyl
authors.forename: A
authors.initials: A
authors.lastname: Boonstra
authors.forename: J G F
authors.initials: JG
authors.lastname: Bronzwaer
authors.forename: K M J
authors.initials: KM
authors.lastname: Marques
authors.forename: E F
authors.initials: EF
authors.lastname: Smit
authors.forename: B A C
authors.initials: BA
authors.lastname: Dijkmans
authors.forename: A
authors.initials: A
authors.lastname: Vonk-Noordegraaf
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015331:Cohort Studies; D005260:Female; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D009200:Myocardial Contraction; D012595:Scleroderma, Systemic / Q000150:complications / Q000503:physiopathology*; D013318:Stroke Volume; D018497:Ventricular Dysfunction, Right / Q000175:diagnosis / Q000503:physiopathology*