699 - Myocardial Involvement in Systemic Sclerosis As Assessed by Ultrasonography and Scintigraphic Perfusion Imaging

Sunday, November 6, 2011: 9:00 AM-6:00 PM
Hall F2 - Poster Hall (McCormick Place West)
Niki Tsifetaki, Registrar in Rheumatology, Ioannina, Greece, Charalampos Papagoras, Fellow in Rheumatology, Ioannina, Greece, Kersten Achenbach, Cardiologist, Ioannina, Greece, Athanasia Georgiou, Fellow in Nuclear Medicine, Ioannina, Greece, Spyridon Tsiouris, Registrar of Nuclear Medicine, Greece, Andreas Fotopoulos, Associate Professor of Nuclear Medicine, Greece and Alexandros A. Drosos, Professor of Medicine/Rheumatology, Ioannina, Greece
Presentation Number: 699

Background/Purpose: Systemic sclerosis (SSc) is an autoimmune disease with two cardinal features: vascular involvement with intense vasoconstriction and smooth muscle proliferation, resulting in increased vascular resistance and tissue ischemia, together with excessive extra-cellular collagen deposition resulting in fibrosis. Both features affect multiple organs such as the skin and the lung. However, limited data are available on heart involvement.

Method: We conducted a cross-sectional study of subclinical heart involvement in patients with SSc followed in a single tertiary rheumatology department. Enrolled patients underwent transthoracic echocardiography (GE Vivid 7 equipment), which assessed left ventricular hypertrophy (LVH) (defined as LV wall thickness >11mm), LV diastolic dysfunction, LV ejection fraction (EF) and pulmonary systolic arterial pressure (PASP). Stress echocardiography with dobutamine was performed in some cases. We also performed stress-rest myocardial perfusion imaging (MPI) scintigraphy by 99mTc-tetrofosmin single-photon emission computed tomography (SPECT). LV wall was divided in 20 segments to quantify ischemia as follows: mild (1-2), medium (3-4) & significant (≥5).

Result: Thirty-seven patients with SSc were enrolled (33 females, 4 males; median age 56 years, range 30-75). Eighteen of them had limited and 19 diffuse SSc, with median disease duration 15 years (range 2-57). Eleven patients had a history of arterial hypertension (AH), 2 of pulmonary arterial hypertension (PAH), 1 of myocardial infarction and 1 of diabetes mellitus. Two patients were on endothelin receptor antagonists due to PAH, while 14 received bosentan alone or in combination with sildenafil for digital ulcers without PAH. Echocardiography revealed LVH in 9 cases (24.3%); diastolic dysfunction (up to grade 1) was found in 17 (45.9%), though only one had EF<55% (EF median 67%, range 50-80). After excluding patients with a history of AH, LVH was still found in 6 (23.1%) and diastolic dysfunction in 10 (38.5%). PASP>30 mmHg was found in 13 patients (35.1%), 11 of whom had no history of PAH, while 3 were already on bosentan for digital ulcers. Stress echocardiography was performed in 12 patients and was negative in all. MPI by 99mTc-tetrofosmin SPECT was performed in 35 patients. Twenty-one (60%) exhibited reversible LV perfusion defects consistent with ischemia. Their median age was 54 years (range 30-75); 4 patients were less than 40 years and 8 patients less than 50 years old. In the majority of cases (20) ischemia was graded as mild and in one it was moderate.

Conclusion: Subclinical heart involvement is common in SSc, even in patients in the younger age groups. Diastolic dysfunction without compromised systolic performance, increased PASP and ischemia on MPI SPECT are found in a significant proportion of SSc cases. Although the precise underlying pathology need be elucidated, careful screening of SSc patients for potential heart involvement and consultation by a cardiologist may often be warranted.

Keywords: systemic sclerosis

Disclosure: N. Tsifetaki, None; C. Papagoras, None; K. Achenbach, None; A. Georgiou, None; S. Tsiouris, None; A. Fotopoulos, None; A. A. Drosos, None.