Summary
A new imaging technique reveals evidence of heart dysfunction in Scleroderma patients with undiagnosed heart disease. In a team effort between the Johns Hopkins Divisions of Rheumatology, Cardiology, CPR and Pulmonary/Critical Care Medicine, led by Monica Mukherjee, M.D. and Ami A. Shah, M.D., researchers coupled traditional echocardiography (echo) with a new technique called “speckle-tracking” to reveal the presence of right heart dysfunction that was not detected by traditional methods. This new method may identify patients with Scleroderma who are at high risk for developing disability and even death due to severe heart disease.
Why was this study done?
Heart disease in Systemic Sclerosis (Scleroderma) is associated with a high rate of disability and death, typically due to right-sided heart failure and the development of pulmonary hypertension. Despite close monitoring by routine clinical exams and a yearly echo, heart disease in people with scleroderma remains difficult to detect until late in the disease course when patients become symptomatic. A traditional two-dimensional (2D) echo uses sound waves to create an image of the heart which doctors can use to look at the heart beating and detect heart disease. In the present study, we sought to use a new echocardiographic technique to detect abnormalities in right heart structures.
How was this study done?
For people with Scleroderma who were receiving traditional echos for routine monitoring of their disease, we used a new technique called “speckle-tracking” echocardiography (STE). STE is a special software program that uses 2D images, analyzes them, and looks for regional abnormalities in how the heart is contracting, known as strain. 138 volunteers with Scleroderma seen at the Johns Hopkins Scleroderma Center participated in this study.
What were the major findings?
We found that even when the right heart was normal in shape and function by traditional echo, STE strain analysis revealed that there was a unique pattern of how the right heart contracts, indicating dysfunction, in patients with Scleroderma. We observed this pattern in all Scleroderma patients, regardless of whether or not they already had symptoms of heart failure, or pulmonary hypertension.
What is the impact of this work?
Early detection of right heart dysfunction, prior to the onset of symptoms of heart failure and pulmonary hypertension, may allow physicians to identify, monitor, and more aggressively treat patients with early stages of heart failure. In the future, this early detection and treatment may reduce disability and death due to heart disease in patients with Scleroderma
This research was supported by:
The Donald B. and Dorothy L. Stabler Foundation, the Scleroderma Research Foundation, the Catherine Keilty Memorial Fund for Scleroderma Research, the Martha McCrory Professorship, and the National Institutes of Health (NIH/NIAMS K23 AR061439).
Link to original research article:
Editorial: http://circimaging.ahajournals.org.ezp.welch.jhmi.edu/content/9/6/e005009.long
Unique Abnormalities in Right Ventricular Longitudinal Strain in Systemic Sclerosis Patients. Mukherjee M, Chung SE, Ton VK, Tedford RJ, Hummers LK, Wigley FM, Abraham TP, Shah AA. Circ Cardiovasc Imaging. 2016 Jun;9(6).
