Systemic Sclerosis Study Finds Right Ventricular Dysfunction in Range of Patients

The heart’s right ventricle in systemic sclerosis patients is impaired when compared to people without the disease, according to a study titled “Impact of pulmonary fibrosis and elevated pulmonary pressures on right ventricular function in patients with systemic sclerosis,” published in the journal Rheumatology.

Patients with systemic sclerosis, an autoimmune disease characterized by thickening of the skin due to the accumulation of collagen, resulting in vascular damage and fibrosis, are often diagnosed with right ventricle dysfunction. Among the causes are fibrosis or increased right ventricle pressure overload (as a consequence of interstitial lung disease), and pulmonary hypertension. However, how each of these factor contributes to the development of right ventricular dysfunction remains largely unaddressed.

The research team determined the systolic function of the right ventricle in a large cohort of patients with systemic sclerosis using a 2D speckle tracking–derived strain echocardiography. While cardiac function and pulmonary pressures are regularly checked in systemic sclerosis patients via echocardiography, this method is limited to assess right ventricle function due to its complex geometry. The newly developed 2D speckle tracking–derived strain echocardiography bypasses these limitations, and was previously reported to be a both sensitive and accurate strategy for measuring regional and global right ventricle systolic function.

In total, researchers analyzed 102 systemic sclerosis patients who underwent thoracic computed tomography and transthoracic echocardiography, and 2D speckle tracking–derived strain echocardiography for determining right ventricle function. The team found that 50 percent of these patients had no pulmonary fibrosis or high pulmonary pressure, 31 percent had pulmonary fibrosis but no increased pulmonary pressure, and 19 percent had both disorders.

Notably, patients with both pulmonary fibrosis and elevated pulmonary pressure had the higher score, and hence the most impaired value of right ventricle free wall strain at –16.8 percent. Patients with pulmonary fibrosis but not high pulmonary pressure exhibited −21.5 percent, and patients with no pulmonary fibrosis and no increased pulmonary pressure had −24.0 percent. However, in comparison to healthy controls, all three  systemic sclerosis groups presented an impaired right ventricle free wall strain (−28.0 percent).

In conclusion, results showed that when compared to controls, patients with systemic sclerosis have a dysfunctional right ventricle. Moreover, the team found that pulmonary fibrosis and elevated pulmonary pressure are independently associated with right ventricular dysfunction in this population.