Isolated right ventricular dysfunction in systemic sclerosis: latent pulmonary hypertension?

• Published in: The European respiratory journal Vol. 30; no. 5; pp. 928 - 936
• Main Authors: Huez, S, Roufosse, F, Vachiery, J-L, Pavelescu, A, Derumeaux, G, Wautrecht, J-C, Cogan, E, Naeije, R
• Format: Journal Article
• Language: English
• Published: Leeds Eur Respiratory Soc 01.11.2007; Maney
• Subjects: Biological and medical sciences; Cardiology. Vascular system; Case-Control Studies; Echocardiography, Doppler; Female; Heart; Heart failure, cardiogenic pulmonary edema, cardiac enlargement; Humans; Hypertension, Pulmonary - complications; Hypertension, Pulmonary - diagnostic imaging; Hypertension, Pulmonary - physiopathology; Linear Models; Male; Medical sciences; Middle Aged; Pneumology; Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases; Respiratory Function Tests; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Scleroderma, Systemic - complications; Scleroderma, Systemic - diagnostic imaging; Scleroderma, Systemic - physiopathology; Ventricular Dysfunction, Right - diagnostic imaging; Ventricular Dysfunction, Right - etiology; Ventricular Dysfunction, Right - physiopathology; Sonography; Heart failure; Immunopathology; Connective tissue disease; Skin disease; Echocardiography; Respiratory disease; Right ventricle; Tissue doppler; Cardiovascular disease; Autoimmune disease; Heart ventricle; Right ventricular failure; tissue Doppler imaging; Pulmonary hypertension; Latent; systemic sclerosis; Heart disease; Systemic disease; Scleroderma; Pneumology
• ISSN: 0903-1936; 1399-3003
• DOI: 10.1183/09031936.00025607

Right ventricular function is frequently abnormal in patients with systemic sclerosis, but whether this is related to pulmonary vascular complications of the disease is unclear. Standard echocardiography with tissue Doppler imaging was performed at rest and during exercise for the study of right ventricular function and pulmonary circulation in 25 consecutive systemic sclerosis patients and in 13 age-matched healthy controls. When compared with the controls, the patients had no difference in systolic right ventricular pressure gradient, but a decreased pulmonary flow acceleration time, and increased right ventricular free wall thickness and end-diastolic dimensions. At the tricuspid annulus, the E maximal velocity was decreased (8.9 +/- 4 versus 11.7 +/- 2.3 cm.s(-1)) and the isovolumic relaxation time corrected to RR interval was increased (6.5 +/- 2.9 versus 4.5 +/- 2.5%). The tissue Doppler imaging profile at the mitral annulus was similar in both groups. At exercise, 18 patients had a decreased maximum workload and cardiac output, no change in systolic right ventricular pressure gradient, but an increase in the slope of pulmonary artery pressure/flow relationships. These results suggest that patients with systemic sclerosis may present with latent pulmonary hypertension as a likely cause of right ventricular diastolic dysfunction, as revealed by stress echocardiography and tissue Doppler imaging.