The heart in the primary antiphospholipid syndrome

Twenty consecutive female patients (mean age 30.1 years) with Primary Antiphospholipid Syndrome (PAPS) were studied cardiologically through noninvasive methods and compared with 20 age-and sex-matched healthy subjects. On physical examination 13/20 patients (65%) with PAPS had a valvular abnormality. In 12/20 (60%) patients with PAPS the ECG was abnormal, mainly due to sinus tachycardia in 5 (25%) and acute myocardial infarction in 3 (15%). In 7/20 patients with PAPS (35%) abnormal pulmonary findings were detected by X-ray and in 6 (30%) they were related to dilated pulmonary arch and pulmonary hypertension. In 14/20 cases (70%) with PAPS, abnormal echocardiographic findings were present; 13/20 patients (65%) had valvular complications attributable to PAPS: mitral insufficiency in six cases; mitral valve prolapse in three and aortic insufficiency in three. Two had pulmonary artery hypertension and two, tricuspid regurgitation (one attributable to PAPS). All valve diseases were regurgitant with mild to moderate hemodynamic repercussion. No stenotic lesions were detected. The mean mitral thickness in patients with mitral valve involvement was 7.0 +/- 1.6 mm, compared to 2.8 +/- 0.7 mm in patients with normal valves and 3.1 +/- 0.9 mm in the control group (p < 0.001). The mean aortic valve thickness in patients with aortic valve involvement was 3.6 +/- 0.5 mm compared to 1.5 +/- 0.3 mm in patients with normal valves and 1.4 +/- 0.4 in the control group (p < 0.001). None of the patients from the control group had valve disease (p < 0.0001). Three cases (15%) had pericardial effusion diagnosed by echo. Two patients with PAPS died during the 4.7 +/- 1.2 years of cardiological follow up, due to acute myocardial infarction and embolic cerebrovascular accident, respectively. In conclusion, cardiologic complications are common in PAPS, including left side regurgitant lesions that might be hemodynamically significant, acute myocardial infarction, pericardial effusion and pulmonary hypertension.