Cardiac involvement in patients with primary antiphospholipid syndrome

Department of Hematology, Rambam Medical Center, Haifa, Israel.

To evaluate cardiac involvement in primary antiphospholipid syndrome, two-dimensional and Doppler echocardiographic studies were performed in 34 consecutive patients with this syndrome. All patients had an increased level of serum anticardiolipin antibodies with no evidence of malignancy or systemic lupus erythematosus. The clinical manifestations of primary antiphospholipid syndrome were arterial thrombosis in 14 patients, venous thrombosis in 6 and recurrent fetal loss in 14. Valvular lesions were observed on two-dimensional echocardiography in 11 patients (32%) (9 women and 2 men), aged 24 to 57 years (mean +/- 1 SD 36 +/- 10). Abnormal echocardiographic findings were observed in 9 (64%) of 14 patients with arterial thrombosis versus 1 (17%) of 6 patients with venous thrombosis and 1 (7%) of 14 patients with recurrent fetal loss. The most common echocardiographic abnormality was mitral leaflet thickening, found in five patients; this was associated with mitral regurgitation in three and with combined mild mitral stenosis and regurgitation in one patient. Localized subvalvular mitral thickening was observed in one patient and calcification of the anulus in another. Aortic valve thickening was observed in two patients, one of whom also had a moderate degree of aortic regurgitation. Vegetation-like lesions on the mitral or aortic valve were found in two patients. It is concluded that valvular lesions are commonly found in primary antiphospholipid syndrome, particularly when the syndrome is manifested by peripheral arterial thrombosis. The location and appearance of valvular lesions in this syndrome are heterogeneous. Most patients have no clinically significant valvular disease. Two-dimensional and Doppler echocardiographic studies are often informative in these patients.

This article has been cited by other articles:

				H. Gorki, V. Malinovski, and R. D.L. Stanbridge The antiphospholipid syndrome and heart valve surgery Eur. J. Cardiothorac. Surg., February 1, 2008; 33(2): 168 - 181. [Abstract] [Full Text] [PDF]

				M. A. Albert, N. Halevy, and E. M. Antman Preoperative Evaluation for Cardiac Surgery Card. Surg. Adult, January 1, 2008; 3(2008): 261 - 280. [Full Text]

				R. J. Mendez, T. F. Cianciulli, C. E. Parisi, H. A. Prezioso, and L. A. Vidal Recurrent systemic embolism caused by thrombosis in a stenotic bicuspid aortic valve Eur J Echocardiogr, January 1, 2008; 9(1): 196 - 198. [Abstract] [Full Text] [PDF]

				D. Erdogan, M. T. Goren, R. Diz-Kucukkaya, and M. Inanc Assessment of Cardiac Structure and Left Atrial Appendage Functions in Primary Antiphospholipid Syndrome: A Transesophageal Echocardiographic Study Stroke, March 1, 2005; 36(3): 592 - 596. [Abstract] [Full Text] [PDF]

				K. M Evans, H. E Robinson, and P. K Chong Thrombotic endocarditis J R Soc Med, January 1, 2005; 98(1): 24 - 25. [Full Text] [PDF]

				L. Barandon, P. Clerc, C. Chauvel, and P. Plagnol Native aortic valve thrombosis: a rare cause of acute ischemia of the lower limb Interactive CardioVascular and Thoracic Surgery, December 1, 2004; 3(4): 675 - 677. [Abstract] [Full Text] [PDF]

				R. I. P. Dornan Acute postoperative biventricular failure associated with antiphospholipid antibody syndrome Br. J. Anaesth., May 1, 2004; 92(5): 748 - 754. [Abstract] [Full Text] [PDF]

				M. A. Albert and E. M. Antman Preoperative Evaluation for Cardiac Surgery Card. Surg. Adult, January 1, 2003; 2(2003): 235 - 248. [Full Text]

				M G Tektonidou, J P A Ioannidis, I Moyssakis, K A Boki, V Vassiliou, P G Vlachoyiannopoulos, M K Kyriakidis, and H M Moutsopoulos Right ventricular diastolic dysfunction in patients with anticardiolipin antibodies and antiphospholipid syndrome Ann Rheum Dis, January 1, 2001; 60(1): 43 - 48. [Abstract] [Full Text]

				M Suguta, Y Hoshino, and S Naito Novel expression of VCAM-1 on the mitral valve in a patient with primary antiphospholipid antibody syndrome Heart, November 1, 2000; 84(5): 10e - 10. [Full Text]

				M Turiel, S Muzzupappa, B Gottardi, C Crema, P Sarzi-Puttini, and E Rossi Evaluation of cardiac abnormalities and embolic sources in primary antiphospholipid syndrome by transesophageal echocardiography Lupus, July 1, 2000; 9(6): 406 - 412. [Abstract] [PDF]

				L. I. Kupferwasser, G. Hafner, S. Mohr-Kahaly, R. Erbel, J.u. Meyer, and H. Darius The presence of infection-related antiphospholipid antibodies in infective endocarditis determines a major risk factor for embolic events J. Am. Coll. Cardiol., April 1, 1999; 33(5): 1365 - 1371. [Abstract] [Full Text] [PDF]

				A. Rangel, C. Lavalle, E. Chavez, M. Jimenez, J. L. Acosta, E. Badui, H. Albarran, and A. Rangel Myocardial Infarction in Patients with Systemic Lupus Erythematosus with Normal Findings from Coronary Arteriography and Without Coronary Vasculitis: Case Reports Angiology, March 1, 1999; 50(3): 245 - 253. [Abstract] [PDF]

				L. Ziporen, I. Goldberg, M. Arad, M. Hojnik, J. Ordi-Ros, A. Afek, M. Blank, Y. Sandbank, M. Vilardell-Tarres, I. de Torres, et al. Libman-Sacks endocarditis in the antiphospholipid syndrome: immunopathologic findings in deformed heart valves Lupus, June 1, 1996; 5(3): 196 - 205. [Abstract] [PDF]

				M. Hojnik, J. George, L. Ziporen, and Y. Shoenfeld Heart Valve Involvement (Libman-Sacks Endocarditis) in the Antiphospholipid Syndrome Circulation, April 15, 1996; 93(8): 1579 - 1587. [Abstract] [Full Text]

				R. Garcia-Torres, M-C. Amigo, A. de la Rosa, A. Moron, and P. Reyes Valvular heart disease in primary antiphospholipid syndrome (PAPS): clinical and morphological findings Lupus, February 1, 1996; 5(1): 56 - 61. [Abstract] [PDF]

				R. A. Asherson and R. Cervera 'Primary', 'Secondary' and Other Variants of the Antiphospholipid Syndrome Lupus, August 1, 1994; 3(4): 293 - 298. [Abstract] [PDF]