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Table of Contents
Year : 2019  |  Volume : 16  |  Issue : 3  |  Page : 199-201

A rare case of mitral valve replacement in antiphospholipid antibody syndrome

1 Department of Rheumatology, Apollo Hospitals, Indore, Madhya Pradesh, India
2 Department of Cardiothoracic Surgery, Apollo Hospitals, Indore, Madhya Pradesh, India
3 Department of Cardiology, Apollo Hospitals, Indore, Madhya Pradesh, India

Date of Submission31-Jan-2019
Date of Acceptance03-Apr-2019
Date of Web Publication11-Sep-2019

Correspondence Address:
Akshat Pandey
Apollo Hospitals, Scheme No.74-C, Sector-D, Vijaynagar, Indore - 452 010, Madhya Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/am.am_6_19

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Antiphospholipid antibody syndrome (APLS) is commonly associated with cardiac valvular abnormalities with the involvement of the mitral valve. The mitral valve replacement (MVR) is a high-risk procedure owing to postoperative complications. The major challenge in the management of these patients is to maintain anticoagulation in pre-, intra-, and postoperative conditions. The optimal choice of prosthesis valve (bioprosthetic or mechanical valve) for MVR depends on the individual patient's clinical condition. Previous reports indicate no clear guidance on the ideal prosthesis for MVR in patients with APLS. In the present case report, we performed MVR using bioprosthetic valve in a 42-year-old female diagnosed with APLS with persistent dyspnea secondary to severe mitral regurgitation.

Keywords: Anticoagulation, antiphospholipid syndrome, bioprosthetic valve, mitral valve

How to cite this article:
Pandey A, Ali M, Jain S. A rare case of mitral valve replacement in antiphospholipid antibody syndrome. Apollo Med 2019;16:199-201

How to cite this URL:
Pandey A, Ali M, Jain S. A rare case of mitral valve replacement in antiphospholipid antibody syndrome. Apollo Med [serial online] 2019 [cited 2020 Feb 28];16:199-201. Available from: http://www.apollomedicine.org/text.asp?2019/16/3/199/266789

  Introduction Top

Antiphospholipid syndrome (APLS), a prevalent autoimmune disorder, is characterized by the presence of arterial or venous thrombosis, thrombocytopenia, increased levels of antiphospholipid antibodies including positive lupus anticoagulant, and/or anticardiolipin antibodies and β2-glycoprotein I antibodies. The lower limbs and the cerebral arterial circulation are the typical sites of venous and arterial thrombosis.

Aortic and mitral valve abnormalities constitute the typical manifestations associated with APLS.[1] Therefore, patients with APLS are considered to be associated with cardiovascular system abnormalities. There is a probable risk of the development of vasculo-occlusive complications in pre-, intra-, and postoperative conditions. A study by Erdozain et al. reported a high rate of mortality (12.5%), 14 patients experienced 20 complications, and only 50% of patients had an uneventful outcome among 32 patients with APLS undergoing valvular replacements.[2]

Observations of several previous studies indicate that approximately 30%–40% of patients with primary APLS and about 50%–60% of patients with systemic lupus erythematosus (SLE) and APLS affect one or more valves most often on the left side, suggesting the frequent occurrence of heart valve lesions in patients with APLS.[3],[4],[5],[6] In the literature, only few valve operations in APLS patients have been reported. However, a recent report by Arif et al. which included 15 patients with SLE and APLS undergoing cardiac valve surgery concluded that these patients can be operated on for cardiac valve disease, with favorable early results and acceptable long-term outcomes.[7]

Here, we present a rare case of mitral valve replacement (MVR) in a 42-year-old female with a history of APLS and recurrent episodes of thrombosis in both the upper and lower limbs for the past 10 years.

  Case Report Top

A 42-year-old female presented to our institution on October 30, 2017, with a complaint of shortness of breath on exertion for 1 month (New York Heart Association functional Class III) which had increased during the past 10 days. The patient had a history of recurrent upper- and lower-limb thrombosis for 10 years and was on oral anticoagulants. Furthermore, the patient had a history of intermittent lower-limb pain particularly while walking and reported blue/green patches and prominent veins over both the thighs and calf regions.

On February 20, 2016, she had an episode of myocardial infarction, post which she went into cardiorespiratory arrest and was on mechanical ventilator support for 4 days and recovered. After her discharge from the hospital, her coronary angiography was done, which suggested normal coronaries. Two-dimensional echocardiography was performed simultaneously which reported a slight increase in the left ventricle wall thickness suggestive of concentric left ventricular (LV) hypertrophy. LV cavity size was increased. Basal inferolateral and lateral segments were hypokinetic. Overall, LV ejection fraction was 45%–50% suggestive of mild LV dysfunction [Table 1]. Moderate-to-severe mitral regurgitation (MR) was observed with an MR jet area of 7.8 cm 2 and mild tricuspid regurgitation (TR) was observed with a TR gradient of 48 mmHg. Aortic and pulmonary valves were normal.
Table 1: Two-dimensional echocardiography

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APLA profile was done [Table 2]. Other investigations were within normal limits. Factor V Leiden mutant detection and paroxysmal nocturnal hemoglobinuria confirmation test were negative. She was prescribed treatment on discharge and was advised to undergo MVR. After a year, the patient again presented with severe dyspnea on rest (for 10 days). She was admitted and was taken for MVR.
Table 2: Antiphospholipid syndrome profile

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The standard protocol using opioid-based induction and maintenance was followed in the procedure. The patient was mechanically ventilated with a tidal volume of 8 ml/kg with respiratory rate adjusted to obtain end-expiratory carbon dioxide tension of 35–40 mmHg. Anesthesia was maintained with isoflurane (0.6%–1.0%) in oxygen–air mixture with an inspired oxygen fraction of 0.5 and intermittent doses of midazolam, fentanyl, and vecuronium. The MVR was done with a bioprosthetic valve on cardiopulmonary bypass. Activated clotting time was kept around 800 using 400 U/kg of heparin. She was cooled to 32°C. Cold del Nido cardioplegia was used to arrest the heart. Only half dose of protamine was used for heparin reversal. Heparin infusion was started at 500 U/h after 4 h of chest closure (after making sure that the drains were not draining much). Acenocoumarol and aspirin were started. Heparin infusion was stopped when international normalized ratio (INR) was above 2. The patient was kept under observation for 4 days and discharged when stable.

  Discussion Top

Cardiac involvement is common in cases of APLS. The challenge is to maintain anticoagulation and prevent pre-, intra-, and postoperative complications in such patients when they undergo cardiac surgery. The present case was one of the very unusual cases of APLS undergoing MVR surgery.

There are very few long-term studies to decide which valve is suitable for MVR surgery. The choice of valve prosthesis should always be individualized and patients younger than 60 years of age must carefully choose their valve prosthesis. Bioprosthetic valve doesn't require lifelong anticoagulation therapy, but there is a high risk of structural valve degeneration, which increases the likelihood of reoperation and its associated risks.[8],[9] On the contrary, the use of mechanical valve avoids the risk of structural valve degeneration at the cost of lifelong anticoagulation.[10] In the present case, bioprosthetic valve was used for MVR surgery and heparin infusion was discontinued after achieving postoperative INR above 2.

There is a scarcity of reports in regard to the best option of prosthetic valve selection for MVR surgery (bioprosthetic or mechanical valves) and associated complications. The largest case series reported involving 32 patients with APLS, indicating that cardiac valve surgery is a very high-risk procedure in patients with APLS.[2]

In conclusion, a detailed evaluation of the patient's clinical condition and therapeutic alternatives should be considered before surgery which will be beneficial in better management of postoperative complications.


Written informed consent was taken from the patient for publication.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Cervera R, Piette JC, Font J, Khamashta MA, Shoenfeld Y, Camps MT, et al. Antiphospholipid syndrome: Clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients. Arthritis Rheum 2002;46:1019-27.  Back to cited text no. 1
Erdozain JG, Ruiz-Irastorza G, Segura MI, Amigo MC, Espinosa G, Pomar JL, et al. Cardiac valve replacement in patients with antiphospholipid syndrome. Arthritis Care Res (Hoboken) 2012;64:1256-60.  Back to cited text no. 2
Brenner B, Blumenfeld Z, Markiewicz W, Reisner SA. Cardiac involvement in patients with primary antiphospholipid syndrome. J Am Coll Cardiol 1991;18:931-6.  Back to cited text no. 3
Hojnik M, George J, Ziporen L, Shoenfeld Y. Heart valve involvement (Libman-Sacks endocarditis) in the antiphospholipid syndrome. Circulation 1996;93:1579-87.  Back to cited text no. 4
Galve E, Ordi J, Barquinero J, Evangelista A, Vilardell M, Soler-Soler J. Valvular heart disease in the primary antiphospholipid syndrome. Ann Intern Med 1992;116:293-8.  Back to cited text no. 5
Nesher G, Ilany J, Rosenmann D, Abraham AS. Valvular dysfunction in antiphospholipid syndrome: Prevalence, clinical features, and treatment. Semin Arthritis Rheum 1997;27:27-35.  Back to cited text no. 6
Arif R, Farag M, Seppelt P, Beller CJ, Ruhparwar A, Karck M, et al. Patients with systemic lupus erythematosus and antiphospholipid syndrome undergoing cardiac valve surgery. J Heart Valve Dis 2015;24:228-35.  Back to cited text no. 7
Ruel M, Kulik A, Rubens FD, Bédard P, Masters RG, Pipe AL, et al. Late incidence and determinants of reoperation in patients with prosthetic heart valves. Eur J Cardiothorac Surg 2004;25:364-70.  Back to cited text no. 8
Johnston DR, Soltesz EG, Vakil N, Rajeswaran J, Roselli EE, Sabik JF 3rd, et al. Long-term durability of bioprosthetic aortic valves: Implications from 12,569 implants. Ann Thorac Surg 2015;99:1239-47.  Back to cited text no. 9
Jaffer IH, Whitlock RP. A mechanical heart valve is the best choice. Heart Asia 2016;8:62-4.  Back to cited text no. 10


  [Table 1], [Table 2]


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