In SSc Study, Pulmonary Fibrosis and Hypertension Linked to Right Ventricular Dysfunction

Patients with systemic sclerosis (SSc) frequently develop problems in the heart’s right ventricle. A new study from the Leiden University Medical Center, in the Netherlands, found that both pulmonary fibrosis and pulmonary hypertension were independently linked to the disruption of right ventricular function.

Researchers know that several mechanisms might contribute to the development of right ventricle dysfunction in SSc patients. Fibrosis of the heart muscle and high pressure in the right ventricle, due to fibrotic lung disease and pulmonary hypertension, are suspected to promote the heart problem, but so far, few studies have explored the potential link.

The study, “Impact of pulmonary fibrosis and elevated pulmonary pressures on right ventricular function in patients with systemic sclerosis,“ set out to investigate these factors. Of the 102 patients included in the study, 50 percent did not have either lung fibrosis or pulmonary hypertension, 31 percent had lung fibrosis but not hypertension, and the remaining 19 percent had both pulmonary fibrosis and pulmonary hypertension.

Using traditional echocardiography and a new technique, called 2-D speckle tracking strain echocardiography, the research team measured the properties of the right ventricle. The findings, published in the journal Rheumatology, showed that while traditional echocardiography produced similar results among the three groups of patients, the speckle tracking technique distinguished among patients with the associated conditions.

Patients with both pulmonary fibrosis and pulmonary hypertension were most impaired in right ventricle function compared to those in the other two groups. All three groups also had more right ventricle impairment than controls. Moreover, an analysis showed that both pulmonary fibrosis and measurements of pulmonary hypertension were associated with right ventricle impairment.

The association between lung fibrosis and hypertension found in the study, however, does not prove that these conditions cause the heart dysfunction. Future prospective studies, along with exploration of the mechanisms involved, are needed to establish such a fact.

This study did show that speckle tracking echocardiography is a sensitive technique that could detect right ventricle dysfunction in patients before it was symptomatic, even when traditional echocardiography failed to produce similar results. This observation has important implications for the clinical management and monitoring of heart function in SSc patients.