P1690 Dynamic mitral stenosis and Liebman-Sacks endocarditis associated with primary antiphospholipid syndrome
Authors: Rauber M , Jese R , Gaspersic N , Tomsic M , Bervar M , Toplisek J 
Published: January 2020

Primary antiphospholipid syndrome (PAPS) is a rare immune-mediated acquired thrombophilia defined by vascular thrombosis, and/or pregnancy morbidity associated with the presence of persistent antiphospholipid antibodies (aPL) in the absence of any other related disease. Although common cardiac involvement predominantly with heart valve disease is clearly defined in secondary APS associated with systemic lupus erythematosus (SLE), its prevalence in PAPS is still a matter of debate.

We report a clinical case of a 33 year old female with PAPS. She was diagnosed at her age of 23 after suffering from severe preeclampsia with HELLP syndrome and stillbirth in the 26th week of pregnancy. She was treated with aspirin and was later able to carry 2 full-term pregnancies. Subsequently, some features of SLE: proteinuria, high aPL titers, presence of antinuclear antibodies, hepatosplenomegaly and thrombocytopenia, were observed and treatment with chloroquine and perindopril were added. At 32 years of age she developed symptomatic epilepsy due to chronic ischemic changes of deep white brain matter (visible on MRI). Because of an audible heart murmur an echocardiogram was performed which revealed a thickened mitral valve (MV) annulus and distal free margins of mitral leaflets. A small (5 x 6 mm) verrucous vegetation on the ventricular side of the MV was also discerned. These changes resulted in mild to moderate mitral stenosis (MS) (MV area 1.8 cm2, mean pressure gradient - PG 8 mmHg), mild valvular regurgitation and mild postcapillary pulmonary hypertension (right ventricular systolic pressure - RVSP 40 mmHg). With negative blood cultures vegetations were attributed to Liebmann Sacks endocarditis (LSE). The patient refused a transesophageal echocardiogram, however a stress echocardiogram was performed which revealed moderately decreased physical capacity (90 W; 58 % of expected). At 25 W we noted a disproportional increase of mean trans-mitral PG from 11 mmHg to 25 mmHg and RVSP rise from 40 mmHg to 60 mmHg, indicating dynamic, possibly clinically significant MS.

While, according to European and American guidelines, interventional treatment (valve repair or replacement) is reserved for severe symptomatic MS there are no recommendations for borderline severe/dynamic MS with concomitant, possibly symptomatic LSE. In our patient discerning the cause of ischemic brain lesions remains a challenge since they might be of cardiac origin or due to vascular thrombogenicity of the PAPS itself. Nonetheless with clear evidence of disease progression in a patient who is currently refusing surgical treatment, a combined treatment with coumarin and aspirin in combination with statin, chloroquine, perindopril and an uptitratable dose of β-blocker was started. We decided for initial monthly echocardiographic follow-up to assess disease dynamics and to ensure possible timely surgical treatment.

Significant dynamic MS due to LSE is a rare and challenging complication of PAPS.