Aortic valve surgery for aortic regurgitation caused by Libman-Sacks endocarditis in a patient with primary antiphospholipid syndrome: a case report


  • Y. L. Ho Department of Cardiothoracic Surgery, Penang General Hospital, Georgetown, Malaysia
  • Nurul A. Zaidi Department of Pathology, Penang General Hospital, Georgetown, Malaysia
  • Ahmadi Salleh Department of Cardiothoracic Surgery, Penang General Hospital, Georgetown, Malaysia
  • Basheer A. Kareem Department of Cardiothoracic Surgery, Penang General Hospital, Georgetown, Malaysia



Antiphospholipid syndrome, Libman-Sacks endocarditis, Cardiac manifestations, Aortic regurgitation, Valve replacement surgery


Antiphospholipid syndrome is an antibody mediated pro-thrombotic state leading to various arterial and venous thromboses. The syndrome can be either primary or secondary to other autoimmune diseases. Cardiac involvement, in particular valvular disease is common in patients with antiphospholipid syndrome (APS) but it is frequently underestimated as most clinicians do not routinely screen for valvular lesion in patients with APS unless they are symptomatic. Valvular disease associated with antiphospholipid syndrome often occurs as valve thickening and non-bacterial vegetation or Libman-Sacks endocarditis, with a higher propensity towards mitral valve although haemodynamically significant valvular dysfunction is rare. Valve surgery in patients with APS carries considerable early and late morbidity and mortality, usually caused by thromboembolic and bleeding events. The perioperative anticoagulation management and haemostatic aspect of APS present exceptional challenges to clinicians, surgeons, anaesthetists and laboratory personnel. Thus, the indication of valve surgery and the choice of valve remains a critical consideration in these patients. We present a successful isolated aortic valve replacement with cardiopulmonary bypass in a 48 year old lady with newly diagnosed antiphospholipid syndrome, who has severe aortic regurgitation as a result of Libman-Sacks endocarditis. Antiphospholipid antibodies were positive and the clinical data showed both negative cultures and infective parameters. Surgically resected vegetations revealed sterile fibrin fibrinous and verrucous vegetations on aortic valve. Valve replacement and the course of cardiopulmonary bypass were uneventful, and patient was discharged well.


Brenner B, Blumenfeld Z, Markiewicz W, Reisner SA. Cardiac involvement in patients with primary antiphospholipid syndrome. J Am Coll Cardiolog. 1991;18(4):931-6.

Zuily S, Regnault V, Selton-Suty C, Eschwege V, Bruntz JF, Bode-Dotto E, et al. Increased risk for heart valve disease associated with antiphospholipid antibodies in patients with systemic lupus erythematosus: meta-analysis of echocardiographic studies. Circul. 2011;124(2):215–24.

Skyrmc-Joncs RA, Wardrop CA, Wiles CM, Fraser AG. Transoesophageal echocardiographic demon-stration of resolution of mitral vegetations after warfarin in a patient with the primary antiphospholipid syndrome. J Am Soc Echocardiogr. 1995;8(3):251-6.

Blank M, Shani A, Goldberg I, Kopolovic J, Amigo MC, Magrini L, et al. Libman-Sacks endocarditis associated with antiphospholipid syndrome and infection. Thromb Res. 2004;114(5,6):589-92.

Shearn MA. The heart in systemic lupus eryth-ematosus: a review. Am Heart J. 1959;58(3):452-66.

Nesher G, Ilany J, Rosenmann D, Abraham AS. Valvular dysfunction in antiphospholipid syndrome: prevalence, clinical features, and treatment. Semin Arthrit Rheum. 1997;27(1):27-35.

Roldan CA, Sibbitt WL, Qualls CR, Jung RE, Greene ER, Gasparovic CM, et al. Libman-Sacks endocarditis and embolic cerebrovascular disease. J Am Coll Cardiol Img. 2013;6:973-83.

Colli A, Carlos AM, Espinosa G, Plasín MA, Pomar JL, Font J, et al. Heart valve surgery in patients with the antiphospholipid syndrome: analysis of a series of nine cases. Euro J Cardio-Thorac Surg. 2010;37(1):154-8.

Gorki H, Malinovski V, Stanbridge RDL. The anti-phospholipid syndrome and heart valve surgery. Eur J Cardiothorac Surg. 2008;33(2):168-81.

Koniari I, Siminelakis SN, Baikoussis NG, Papadopoulos G, Goudevenos J, Apostolakis E. Antiphospholipid syndrome; its implication in cardiovascular diseases: a review. J Cardiothorac Surg. 2010;3(5):101.






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