Systemic sclerosis (SSc) is complicated by pulmonary hypertension and right ventricle (RV) failure in approximately 10% of the patients. Factors influencing the reactivity of pulmonary circulation to vasodilators are not established, while the examination of vasoreactivity is important in determining the treatment, because systemic administration of oral vasodilators can induce severe adverse events in nonresponders. The mechanism of RV failure in SSc is unclear and may result either from increased RV afterload or intrinsic myocardial disease. The aim of the study was to assess the reactivity of pulmonary circulation to inhaled nitric oxide (iNO) and to evaluate its influence on RV function in SSc patients with elevated right ventricle systolic pressure (RVSP). In 60 SSc patients aged 24–73 (58 females, two males; 33 patients with limited SSc and 27 with diffuse SSc), echocardiographic examination with tissue Doppler echocardiography (TDE) was performed. RV function was measured by systolic (S) and early diastolic (E) velocity of tricuspid annulus by TDE. In patients with RVSP >45 mmHg, the reactivity of pulmonary circulation was assessed by iNO test. High-resolution computerized tomography (HRCT) was performed to assess the extent of pulmonary fibrosis. Of 14 SSc subjects with elevated RVSP (13 females, one male; RVSP 47–62 mmHg), positive reaction to iNO was observed in five (RVSP decreased from 51.6 ± 3.7 to 32.24 ± 2.3 mmHg); nine patients were not reactive (RVSP 53.5 ± 5.7 mmHg before iNO vs. 49.6 ± 6.7 mmHg). RV systolic function was decreased in patients with elevated RVSP as compared to the patients with normal pulmonary pressure (S velocity 13.2 ± 1.3 vs. 14.4 ± 1.6 cm/s, respectively, p < 0.05). Significant increase of RV systolic function during iNO test was found in reactive patients only (S velocity before iNO 12.8 ± 1.2 cm/s, during iNO 14.5 ± 1.5 cm/s, p < 0.01). RVSP decrease strongly correlated with S velocity increase (r = 0.95, p < 0.0001). Response to iNO was found only in limited form of SSc; diffuse SSc patients showed no response. Pulmonary fibrosis on HRCT was more frequent in subjects nonreactive to iNO (67% of patients) than in the reactive group (40% of patients). The reactivity of pulmonary circulation to iNO in SSc patients with elevated RVSP was found predominantly in limited form of the disease. Pulmonary fibrosis typical for diffuse SSc was more frequent in nonreactive subjects. Elevated pulmonary pressure plays an important role in RV systolic dysfunction. Pulmonary pressure decrease during iNO test leads to the improvement of RV systolic function. Therapy for right-heart failure in reactive SSc patients should be directed, if possible, at the decrease in pulmonary resistance.
Kowal-Bielecka O, Delcroix M, Vonk-Noordegraaf A et al (2008) Outcome measures in pulmonary arterial hypertension associated with systemic sclerosis. Rheumatology (Oxford) 47(supl. 5):39–41 CrossRef
Sitbon O, Brenot F, Denjean A et al (1995) Inhaled nitric oxide as a screening vasodilator agent in primary pulmonary hypertension: a dose–response study and comparison with prostacyclin. Am J Respir Crit Care Mad 151:384–389
Plazak W, Zabinska-Plazak E, Wojas-Pelc A et al (2002) Heart structure and function in systemic sclerosis. Eur J Dermatol 12:257–262 PubMed
D’Andrea A, Stisi S, Bellissimo S et al (2005) Early impairment of myocardial function in systemic sclerosis: non-invasivasive assessment by Doppler myocardial and strain rate imaging. Eur J Echocardiog 6:407–418 CrossRef
Smolen J, Weisman M (2008) Connective tissue disorders. In: Hochberg M, Silman A, Smolen J, Weinblatt M, Weisman M (eds) Rheumatology. Mosby Elsevier, Philadelphia, pp 1205–1485
Hsu VM, Moreyra AE, Wilson AC et al (2008) Assessment of pulmonary arterial hypertension in patients with systemic sclerosis: comparison of noninvasive tests with results of right-heart catheterization. J Rheumatol 35:458–465 PubMed
Le Pavec J, Humbert M, Mouthon L et al. (201) Systemic sclerosis-associated pulmonary arterial hypertension. Am J Respir Crit Care Med (in press).
Breit SN, Thornton SC, Penny R (1994) Lung involvement in scleroderma. Rev Clin Dermatol 12:243–252 CrossRef
Williamson DJ, Hayward C, Rogers P et al (1996) Acute hemodynamic responses to inhaled nitric oxide in patients with limited scleroderma and isolated pulmonary hypertension. Circulation 94:477–482 PubMed
Allanore Y, Borderie D, Hilliquin P et al (2001) Low levels of nitric oxide (NO) in systemic sclerosis: inducible NO synthase production is decreased in cultured peripheral blood monocyte/macrophage cells. Rheumatology (Oxford) 40:1089–1096 CrossRef
Menkes CJ, Allanore Y, Borderie D et al (2001) Inducible nitric oxide synthase expression and nitric oxide production by monocytes in systemic sclerosis. Bull Acad Natl Med 185:509–522 PubMed
- Reactivity of pulmonary circulation and right ventricle function to inhaled nitric oxide in systemic sclerosis patients
Neu im Fachgebiet Innere Medizin
Meistgelesene Bücher aus der Inneren Medizin
e.Med Kampagnen-Visual, Mail Icon II