Primary Cardiac Involvement in Scleroderma and Role of Cardiac MRI

Kashif Hussain,Robert C Stansbury

doi:10.4236/ijcm.2014.520168

Abstract

Systemic sclerosis (scleroderma) is a connective tissue disease characterized by vascular dysfunction and fibrosis that can affect multiple organ systems. We present case of primary cardiac involvement and the diagnostic role of cardiac MRI. Cardiovascular magnetic resonance imaging (MRI) is an accurate, quantitative method for the non-invasive assessment of myocardial perfusion. The presence of clinically apparent myocardial involvement in scleroderma portends a very poor prognosis. One study of US veterans found that clinical cardiac disease in scleroderma was associated with a 70% mortality rate at five years. Management of heart failure and conduction system abnormalities in scleroderma is similar to other cardiac disease. It includes afterload reduction, beta-blockade, defibrillator placement, etc. Patients with reduced cardiac function and normal coronary arteries may benefit from increased immune suppresion.

Highlights

Cardiac involvement is typically subclinical in scleroderma
Myocardial fibrosis may occur in advanced disease with the fibrotic lesions in a patchy distribution through
magnetic resonance imaging (MRI) may allow non-invasive coronary reserve determination and the evaluation of fibrotic myocardium compared with viable tissue [1]

Summary

Introduction

Cardiac involvement is typically subclinical in scleroderma. Cardiac abnormalities could be more prevalent and severe in the diffuse cutaneous subtype of the disease, which has been the most intensively investigated, there is increasing evidence suggesting that cardiac involvement is a frequent finding in the limited cutaneous subtype. How to cite this paper: Hussain, K. and Stansbury, R.C. (2014) Primary Cardiac Involvement in Scleroderma and Role of Cardiac MRI. Stansbury out both ventricles and not consistent with large coronary artery distribution. MRI may allow non-invasive coronary reserve determination and the evaluation of fibrotic myocardium compared with viable tissue [1]. Of note the fibrosis in cardiac scleroderma can be distinguished from the fibrosis resulting from CAD in that fibrosis secondary to scleroderma can involve the immediate subendocardial layer (typically spared in atherosclerosis) and hemosiderin deposits (commonly observed in atherosclerotic disease) are not appreciated [2]

Presentation

Evaluation

Interim

Work-Up

Conclusion

Discussion