Ankylosing Spondylitis and Anti–Phospholipid Syndrome Resulting in Non–Bacterial Thrombotic Endocarditis: Challenges in Valvular Management

Abstract

We present a difficult management case of a 39-year-old men with controlled ankylosing spondylitis who developed non–bacterial thrombotic endocarditis. He was initially admitted with pneumonia, when thoracic auscultation unveiled a pan–systolic murmur. Transthoracic echocardiogram showed moderate aortic stenosis (AS) and aortic regurgitation (AR). Subsequent transoesophageal echocardiogram (TOE) showed thickened right and left coronary cusps with moderate eccentric AR. This was thought to be secondary to valvulitis from ankylosing spondylitis and was planned for observant management. Repeat TOE at 6 months showed that his aortic valve pathology progressed to severe AS and AR, with a large mass on the anterior aspect of the valve measuring 1.3 × 1.2 cm (Figure 1). Soon after, he was admitted with an ischaemic right arm, from a thromboembolism, and underwent an uncomplicated thrombectomy. During this admission, TOE showed persistence of the mass structure and severe AS and AR. Serology was positive for primary antiphospholipid syndrome. A literature review showed that patients with antiphospholipid syndrome undergoing cardiac surgery had a perioperative morbidity and mortality of up to 50%. Owing to his extreme surgical risk, a consensus decision, from multiple specialities, was obtained to postpone surgery until he was anticoagulated for 3 months. With meticulous coordination with anaesthetics and haematology, he underwent an uncomplicated surgical mechanical aortic valve replacement. Histology showed myxoid degenerative changes of the valve without thrombus. At the 3-month review, he remained well and has planned life-long anticoagulation with international normalised ratio between 2.5 and 3.5. This case exemplifies the importance of multidisciplinary management of complex, systemic disorders where evidenced-based treatment and guidelines are deficit.