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    Abstract
    Introduction
    Case Report
    Discussion
    Conclusion
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CASE REPORT Table of Contents   
Year : 2010  |  Volume : 13  |  Issue : 1  |  Page : 39-43
Anesthetic management of right atrial mass removal and pulmonary artery thrombectomy in a patient with primary antiphospholipid antibody syndrome


1 Department of Anaesthesiology, Critical Care and Cardiovascular Surgery, Indraprastha Apollo Hospitals, New Delhi, India
2 Department of Cardiac Surgery and Radiology, Escorts Heart Institute and Research Centre, New Delhi, India

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Date of Submission17-Oct-2008
Date of Acceptance30-Dec-2008
Date of Web Publication11-Jan-2010
 

   Abstract 

Antiphospholipid antibody syndrome (APLAS) characterises a clinical condition of arterial and venous thrombosis associated with phospholipids directed antibodies. APLAS occurs in 2% of the general population. However, one study demonstrated that 7.1% of hospitalised patients were tested positive for at least one of the three anticardiolipin antibody idiotype. Antiphospholipid antibodies often inhibit phospholipids dependent coagulation in vitro and interfere with laboratory testing of hemostasis. Therefore, the management of anticoagulation during cardiopulmonary bypass can be quite challenging in these patients. Here, we present a case of right atrial mass removal and pulmonary thrombectomy in a patient of APLAS.

Keywords: Anesthesia, antiphospholipid antibody syndrome, pulmonary thrombectomy

How to cite this article:
Rawat S, Mehta Y, Vats M, Mishra Y, Khurana P, Trehan N. Anesthetic management of right atrial mass removal and pulmonary artery thrombectomy in a patient with primary antiphospholipid antibody syndrome. Ann Card Anaesth 2010;13:39-43

How to cite this URL:
Rawat S, Mehta Y, Vats M, Mishra Y, Khurana P, Trehan N. Anesthetic management of right atrial mass removal and pulmonary artery thrombectomy in a patient with primary antiphospholipid antibody syndrome. Ann Card Anaesth [serial online] 2010 [cited 2020 Jul 4];13:39-43. Available from: http://www.annals.in/text.asp?2010/13/1/39/58833



   Introduction Top


For the antiphospholipid syndromes, the term "primary" is used when antiphospholipid (APS) occurs in the absence of any other related disease, whereas the term "secondary" is used when it occurs in the conjunction with other autoimmune diseases i.e., systemic lupus erythematosus (SLE). Antiphospholipid antibody syndrome (APLAS), also known as Hughes syndrome is a well-described clinical entity. The classic syndrome consists of the persistent presence of antiphospholipid antibodies (APLA) (also known as lupus anticoagulant (LA) or anticardiolipin antibodies), with a history of recurrent thromboses (arterial or venous) and/or recurrent foetal loss. More recently, the frequent association of cardiac valvular disease, livedo reticularis and thrombocytopaenia, have also been recognised. [1],[2] It is important to document persistence of the APLA three to six months after initial detection, as their transient presence can accompany other conditions such as infections without any apparent pathogenicity.

The perioperative management of patients with APLAS is complex with an inherent thrombophilia masked by abnormal prolongation of in vitro clotting time, which precludes the use of standard techniques for anticoagulation monitoring during cardiopulmonary bypass (CPB). Hemostatic aspects of APLAS present unique challenge to clinician and laboratory personnel alike, particularly in the perioperative period. These challenges are especially evident in patients requiring cardiac surgery. However, the literature outlining the optimal approach in such patients is limited.

Here, we describe a case of APLAS in which right atrial (RA) mass was removed and pulmonary thrombectomy was done using CPB.


   Case Report Top


A 39-year-old female presented to the hospital with shortness of breath, palpitations, and pleuritic left-sided chest pain since five days. She was admitted to intensive care unit (ICU), where chest x-ray was unremarkable and other routine laboratory investigations were normal. Her past history revealed that she had been diagnosed with internal jugular vein (IJV) thrombosis for which she was treated with oral anticoagulation [with adequate prothrombin time (PT) control], but she herself stopped anticoagulation a year before this illness.

Her obstetric history was normal and she gave uneventful birth to a child eight years ago. Computerised tomographic (CT) angiograms revealed large acute hypodense thrombus in left main pulmonary artery (LMPA) with extension into arterial branches and minimal flow distally. Right lower lobe pulmonary artery (RLPA) also showed thrombus. However, the main PA was normal (26 mm) in diameter. A large filling defect with specks of calcification was also present in RA. Transesophageal echocardiogram (TEE) revealed a pedunculated mass in RA attached to interatrial septum. Other findings of TEE were normal. On further investigation she was tested for antinuclear factor and antidouble stranded DNA (anti Ds-DNA), antiphospholipid antibody (APLA), and LA; for which, results were positive. Urine analysis revealed significant proteinurea and granular casts. She was treated with intravenous heparin sulphate. Subsequent CT angiogram revealed partial lysis of the thrombus occluding the left upper lobe PA with reperfusion of the lung. Repeated ECHO and TEE revealed no resolution of RA clot, therefore she was referred to the author's institution for RA clot removal and pulmonary thrombectomy. CT chest performed at the author's institute revealed calcific foci in the right atrium, LMPA and superior vena cava. CT pulmonary angiogram showing intra-luminal filling defect with foccai of calcification in the LMPA with extension in to left lower lobe artery and in the right atrium bulging through tricuspid valve [Figure 1],[Figure 2],[Figure 3].

After preoperative evaluation, she was scheduled for RA mass removal and pulmonary thrombectomy. She was premedicated with lorazepam (2 mg) orally, and parenteral sulphate (5 mg). Under local analgesia, left peripheral venous line and left radial artery catheter (20G) were inserted. Monitoring included-ECG, direct arterial pressure, central venous pressure (CVP), pulse oximetry, nasal temperature, ETCO 2 , arterial blood gases, blood sugar, urine output, TEE, and BI-spectral index (BIS). Her preinduction arterial blood pressure was 107/103 mmHg and SPO 2 was 97%. Pulmonary arterial pressure was 56/32 mmHg, CO = 4.2 L/min, CI = 2.6 L/min, SVR = 1750 dg/cm 5 and PVR = 238 dg/cm 5 . Anesthesia was induced with intravenous fentanyl citrate (3 µg/kg), sleep dose of thiopentone (150 mg) and midazolam (2 mg). Orotracheal intubation was facilitated with 0.1 mg/kg of pancuronium bromide. Anesthesia was maintained with isoflurane in oxygen and air and incremental doses of fentanyl citrate. Mechanical ventilation was given through a closed circuit with tidal volume of 7 ml/kg. Her arterial blood gases at that time showed pH = 7.39, partial pressure of carbon dioxide (PCO 2 ) = 39 mmHg, partial pressure of oxygen (PO 2 ) = 275 mmHg, bicarbonate (HCO 3 ) = 24.9 mmol/L, base excess (BE) = -1.1 on 0.6 fractional inspired concentration of oxygen (FiO 2 ). Her arterial blood pressure was 114/71 mmHg. After heparinization (3 mg/kg) and achieving an activated clotting time (ACT) of 789 seconds, CPB was instituted by aorto-caval bypass. On hypothermic {core temperature (nasal)} CPB, her ACT was 892 sec, ABG were normal, and mean arterial pressure (MAP) was 78 mmHg. After cross-clamping the aorta and achieving antegrade root blood cardioplegic arrest, RA was opened and RA mass was removed. Then patient was cooled down to 18°C. During this period, ice packs were kept on the head and neck of the patient and incremental doses of relaxant and fentanyl were added to the CPB venous reservoir. Also, 1 gm of thiopantone was slowly added on the CPB and 2 gm of methyl prednisolone was administered in the pump after reaching the core temperature of 18°C. Pulmonary arteriotomy was performed and thrombectomy was done. The total deep hypothermic circulatory arrest (DHCA) time was 13 minutes. After closing the pulmonary artery and RA, CPB was weaned off slowly when adequate core temperature was reached. Her ABG after weaning from CPB were pH = 7.48, PCO 2 = 34 mmHg, PO 2 = 529 mmHg, PCO 2 = 22.6 mmHg, BE = − 0.25 at FiO 2 of 1. Later, FiO 2 was reduced to 0.6. Her arterial pressure at this time was 117/85 mmHg, PA = 39/14 mmHg, CO = 4.1 L/min, CI = 2.5 L/min, SVR = 1230 dynes.sec/cm 5 , PVR = 127 dynes.sec/cm 5 . After decannulation, chest was closed and heparin reversal was achieved by equivalent (1:1) dose of protamine sulphate. Inj N-acetylcysteine infusion was started at the dose of 20 mg/kg/hr. Patient was hemodynamically stable (BP = 117/85 mmHg, PA= 37/14, CO = 4.0, CI = 2.1) and shifted to ICU with ionotropic support of dobutamine 5 µg/kg/hr. Trachea was extubated after 8 hours. On second postoperative day anticoagulation and antiplatelet drugs were started. On third day tab Sildenafil citrate was started with the dose of 12.5 mg thrice daily dose and was transferred to ward with stable condition. Her postoperative echocardiogram was normal with no residual mass or PA thrombus. Repeat CT angiography revealed normal PA and no RA mass [Figure 4]. She was discharged from hospital on seventh postoperative day.


   Discussion Top


APLAs were initially described 50 years ago because of the interference with phospholipid dependent clotting time assays. [3],[4] They were identified in patients with SLE and initially were believed to cause hemorragic disorder; therefore, receiving the misnomer LAs. In 1963, Bowie et al., noted association between these inhibitors and thrombotic diseases. [5] They described four patients with SLE, who had prolonged, inhibited clotting times, and thrombotic manifestations. It was subsequently realised that many individuals existed with these finding in the absence of SLE. Further investigation revealed that there were a wide variety of APLAs with varying spectrum of in vitro activity. Many were found to have activity directed against phospholipid complex and proteins such as â2 glycoprotein I or prothrombin factor II. [6]

The biologic diversity of APLAs led to variety of assays for detection and quantification. These include functional assays of LA activity and immunological-based assays i.e., Enzyme Linked Immunosorbent Assay (ELISA) to detect anticardiolipin antibodies. Assay of LA activity are based on in-vitro prolongation of clotting time by the inhibition of phospholipid required by these techniques. [7] Both types of assays (functional and immunological) are important for optimal diagnosis of APLAs and many laboratories rely on more than one functional assay technique to optimise sensitivity of detection of LA.

APLAs is considered to be more than an epi-phenomenon in many patients with evidence to suggest a causal role for some of the manifestations of the disease. Breanner et al. performed a prospective echocardiographic evaluation on 34 consecutive patients with primary antiphospholipid syndrome; valvular abnormality were observed in 32% of these and were more frequent in patients suffering from arterial thrombosis (64%) compared to those with venous thrombosis (17%) or recurrent foetal loss (7%). [8] In our case, there was PA and RA thrombus and all cardiac valves were normal.

Intraoperative heparin monitoring during CPB may be an issue in patient with APLAs. The ACT may be markedly affected and thus may be not suitable as a guide to the adequacy of heparinization, although anecdotal successes have been reported with empiric heparin management during CPB. [9],[10]

Therefore, we used heparin (3 mg/kg) in our case to achieve the appropriate clotting time during CPB. A blind approach could lead to insufficient heparin-dosing resulting in potentially life threatening hemorrhagic or thrombotic complications.

DHCA is a standard technique for pulmonary thrombectomy. We used methyl predenisolone, which is useful for reducing brain injury by free radical release. Thiopentone directly scavenges reactive oxygen species and inhibit lipid peroxidation. Anesthetic agents may prevent the elevation of extra-cellular glutamate concentration and inhibit the activation of excitatory glutaminergic receptor that augments oxidative stress after ischemia. A survey on the current practice of pharmacological agents as cerebral protecting agent during DHCA revealed that 59% respondents used thiopentone. [11] It is also known that barbiturates produces EEG burst suppression and reduces cerebral metabolic rate and glucose and oxygen requirement. We used BIS for monitoring cerebral activity. We also used TEE intraoperatively to identifying the extent of RA mass, to exclude mass embolization if any, and to visualise the residual RA mass.

N-acetylcysteine was started just coming off CPB because the antioxidant treatment of this drug just before and at the beginning of hypoxia should be more effective in reducing hypoxic pulmonary hypertension. [12] Sidlenafil is a selective Phosphodiesterase-5 (PDE5) inhibitor that has been reported to be a potent pulmonary vasodilator. Sidlenafil selectively inhibits PDE5 which is abundant in pulmonary tissue. This leads to stabilization of cyclic guanosine monophosphate (cGMP), which is a second messenger of nitric oxide (NO). Stabilization of cGMP results in increasing NO at the tissue level leading to pulmonary vasodilation. [13],[14] We started dobutamine infusion which helps in improving the right heart function. The patient was excubated within 8 hours to improve the pulmonary perfusion, which may be reduced due to high intrathoracic pressures and PEEP.

A specific complication that occurs in most of the patients to some degree is localised pulmonary edema, or the "reperfusion syndrome." Reperfusion injury is defined as a radiologic opacity seen in the lungs within 72 hrs of pulmonary endarterectomy. Reperfusion injury adversely impacts the patient's clinical course, occurs only in 10% of the patients. In its most dramatic form, it occurs soon after operation and is associated with profound desaturation. Edema like fluid sometimes with bloody tinge, is suctioned from the endotracheal tube. Frank blood from the endotracheal tube, however, signifies a mechanical violation of the airway barrier that has occurred at operation and stems from a technical error. [15] This complication was not found in our case.


   Conclusion Top


Anesthetic management of APLAs requires detailed preoperative knowledge regarding the biochemical and pathological abnormality of the patient. Vigilant monitoring of heparin dose, CPB machine, and standard DHCA protocol are crucial for intraoperative management. The role of TEE has also been stressed in present case report.

 
   References Top

1.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. 1      
2.García-Torres R, Amigo MC, de la Rosa A, Morón A, Reyes PA. Valvular heart disease in the primary antiphospholipid syndrome(PAPS)clinical and morphological findings. Lupus1996;5:56-61.  Back to cited text no. 2      
3.Conley CL, Rathbun HK, Morse Will, Robinson JE Jr. Circulating anti-coagulant as cause of hemorrhagic diathesis in man. Bull Johns Hopkins Hosp 1948;83:288-96.  Back to cited text no. 3      
4.Conley CL, Hartmann RC. A haemorrhagic disorder caused by circulating anticoagulant in patients with disseminated lupus erythematosus. J Clin Invest 1952;31:621-2.  Back to cited text no. 4      
5.Bowie EJ, Thompson JH Jr, Pascuzzi CA, Owen CA Jr. Thrombosis in systemic lupus erythematosus despite circulating anticoagulants. J Lab Clin Med 1963;62:416-30.  Back to cited text no. 5      
6.Bevers EM, Galli M, Barbui T, Comfurius P, Zwaal RF. Lupus anticoagulant IgG's (LA) are not directed to phospholipids only, but to a complex of lipid-bound human prothrombin. Thromb Haemost 1991;66:629-32.  Back to cited text no. 6      
7.Brandt JT, Triplett DA, Alving B, Scharrer I. Criteria for the diagnosis of lupus anticoagulants: an update. On behalf of the Subcommittee on Lupus Anticoagulant/Antiphospholipid Antibody of the Scientific and Standardisation Committee of the ISTH. Thromb Haemost 1995;74:1185-90.  Back to cited text no. 7      
8.Brenner B, Blumenfeld Z, Markiewicz W, Reisner SA. Cardiac involvement in patients with primary antiphopholipid syndrome. J Am Coll Cardiol 1991;18:931-6.  Back to cited text no. 8      
9.Nakayama M, Kumon K, Yahagi N, Haruna M, Watanabe Y, Hayashi H. Antiphospholipid antibody syndrome in case with redo coronary artery bypass grafting under CPB. Surg Today 1998;28:423-6.  Back to cited text no. 9      
10.Ando M, Takamoto S, Okita Y, Matsukawa R, Nakanishi N, Kyotani S, et al. Operation for chronic pulmonary thromboembolism accompanied by thrombophilia in 8 patients. Ann Thorac Surg 1998;66:1919-24.  Back to cited text no. 10      
11.Dewhurst AT, Moore SJ, Liban JB. Pharmacological agents as cerebral protectants during deep hypothermic circulatory arrests in arrests in adults thoracic aortic surgery. A survey of current practice. Anaesthesia 2002;57:1016-21.  Back to cited text no. 11      
12.Lachmanová V, Hnilicková O, Povýsilová V, Hampl V, Herget J. N-acetyicysteine inhibits hypoxic pulmonary hypertension most effectively in the initial phase of chronic hypoxia. Life Sci 2005;77:175-82.  Back to cited text no. 12      
13.Leuchte HH, Schwaiblmair M, Baumgartner RA, Neurohr CF, Kolbe T, Behr J. Hemodynamic response to sildenafil, nitric oxide, and iliprost in primary pulmonary hypertension. Chest 2004;125:580-6.  Back to cited text no. 13      
14.Chockalingam A, Gnanavelu G, Venkatesan S, Elangovan S, Jaggannath V. Efficacy and optimal dose of sildenafil in primary pulmonary hypertension. Int J Cardiol 2005;99:91-5.  Back to cited text no. 14      
15.Mi MM, Wi JS. Pulmonary Thromboendarterectomy. In: Cohn LH, Edmunds LH Jr, edu. Cardiac Surgery in the Adult. New York: McGraw-Hill; 2003. p. 1205-28.  Back to cited text no. 15      

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Correspondence Address:
Yatin Mehta
Department of Cardiac Anesthesia and Critical Care, Indraprastha Apollo Hospital, Sarita Vihar, New Delhi - 110 076
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-9784.58833

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