SESSION TYPE: Cardiovascular Case Report Posters
PRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM
INTRODUCTION: Systemic sclerosis (scleroderma) is a connective tissue disease characterized by vascular dysfunction and fibrosis that can affect multiple organ systems. Cardiac involvement is typically subclinical. It has been estimated that up to 100% of patients with systemic sclerosis have some degree of myocardial involvement.
CASE PRESENTATION: A 43 year old female with anti-Scl-70 positive scleroderma was referred to our pulmonary clinic for evaluation of dyspnea. TTE prior to the visit revealed a left ventricular ejection fraction estimate of 45 % and estimated peak right ventricular systolic pressure was 35 mmHg. HRCT did not reveal any parenchymal lung disease but PFTs showed decreased diffusion. Bronchoscopy was performed to evaluate for alveolitis. BAL as well as biopsy were unremarkable. She continued to be short of breath and repeat echocardiography showed no change while repeat PFT showed decline in diffusion. Chest CT was unrearkable. She was later admitted to the hospital with hypoxia and shortness of breath. TTE revealed an EF of 5-10%. Primary concern was progression of scleroderma with myocardial involvement. Myocardial biopsy was unremarkable. Cardiac MRI (images 1,2) was suggestive of myocardial fibrosis and edema as well as marked bi-ventricular remodeling. Heart failure treatment was optimized and she was discharged home with a Life Vest. Later she developed syncope secondary to ventricular arrhythmia, leading to placement of an ICD. Over next few weeks she experienced multiple ICD shocks secondary to ventricular arrhythmias. Considering her rapid clinical detrioration with poor prognosis she opted for home hospice.
DISCUSSION: Myocardial fibrosis is considered the hallmark of cardiac involvement in systemic sclerosis, tending to have patchy distribution throughout the myocardium. This fibrosis is distinguishable from atherosclerotic coronary disease because CAD fibrosis typically involves the immediate subendocardial layer and is segmental, following a coronary artery distribution while hemosiderin deposits are not observed. Systolic and diastolic dysfunction may occur secondary to fibrosis; however, the time course and susceptibility to ventricular dysfunction are not well understood. Another complication of ongoing myocardial fibrosis is damage to the conduction system and subsequent arrhythmias. Concurrent cardiomyopathy and ventricular arrhythmias is concerning due to the increased likelihood for sudden cardiac death.
CONCLUSIONS: Our case illustrates that once cardiac involvement becomes clinically evident, it is a very poor prognostic factor and can present with rapid, fulminant deterioration. To our knowledge, clinically evident myocardial involvement is a rare entity with scant description in the literature.
1) Kahan A, Coghlan G, McLaughlin V. Cardiac complications of systemic sclerosis. Rheumatology. 2009;48:iii45-iii48
2) Knockaert DC. Cardiac involvement in systemic inflammatory diseases. Eur Heart J. 2007;28:1797-1804
DISCLOSURE: The following authors have nothing to disclose: Robert Stansbury, Robert Biederman, Kashif Hussain
No Product/Research Disclosure InformationWest Virginia University School of Medicine, Morgantown, WV