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Published online before print January 23, 2008
Eur Respir J 2008, doi:10.1183/09031936.00135407
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ORIGINAL ARTICLE

Different right ventricular contractility in limited cutaneous systemic sclerosis-associated pulmonary arterial hypertension and idiopathic pulmonary arterial hypertension

M.J. Overbeek 1, J-W. Lankhaar 2, N. Westerhof 3, A.E. Voskuyl 4, A. Boonstra 1, J.G.F. Bronzwaer 5, K.M.J. Marques 5, E.F. Smit 1, B.A.C. Dijkmans 4, A.V. Noordegraaf 1*

1 Depts of Pulmonary Diseases
2 Depts of Pulmonary Diseases; and Physics and Medical Technology
3 Depts of Pulmonary Diseases; and Physiology
4 Rheumatology
5 Cardiology, Institute for Cardiovascular Research. VU University Medical Center, Amsterdam

* To whom correspondence should be addressed. E-mail: A.Vonk{at}VUmc.nl.


  Abstract

Since systemic sclerosis (SSc) also involves the heart, we evaluated possible differences in right ventricular (RV) pump function between SSc-associated pulmonary arterial hypertension (SScPAH) and idiopathic PAH (IPAH).

In 13 limited cutaneous SScPAH and 17 IPAH-patients, RV pump function was described by the pump function graph, which relates mean RV pressure (mPrv) and stroke volume index (SVI). Differences in pump function result in shift or rotation of the pump function graph. Mean Prv and SVI were measured by standard catheterization. The mean of the hypothetical RV isovolumic pressure (mPrviso) was estimated using a single-beat method. The pump function graph was approximated by a parabola: mPrv=mPrviso [1 – (SVI/SVImax)2], enabling calculation of SVImax, the hypothetical maximal SVI at zero mPrv.

There were no differences in SVI and SVImax. Both mPrv and mPrviso were significantly lower in SScPAH than in IPAH (mPrv: 30.7±8.5 vs. 41.2±9.4 mmHg, p=0.006; mPrviso: 43.1±12.4 vs. 53.5±10.0 mmHg, p=0.04). Since higher pressures were found at similar SVI, the difference in pump function graph results from a lower contractility in SScPAH than in IPAH.

RV contractility is lower in SScPAH than in IPAH.

Keywords:  Myocardial contraction, pump function graph, right ventricular function, right ventricular pressure, stroke volume




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