cold agglutinin disease in cardiac surgery

case management of cold agglutinin disease in patient undergoing cadiac surgery

1. COLD AGGLUTININS IN CARDIAC SURGERY PATIENT- FOCUSED ANESTHESIA MANAGEMENT APPROACH PRESENTOR –DR AKSHI GOEL DrNB RESIDENT MODERATOR- DR ARUN MAHESHWARI HEAD OF THE DEPARTMENT DEPARTMENT OF CARDIAC ANESTHESIA, DHARAMAVIRA HEART CENTRE, SGRH,NEW DELHI

2. 52 year old female, diabetic, hypertensive presented to the hospital with chief complaints of Chest pain on exertion - 6 months Breathlessness on exertion - 3 months On evaluation, patient was diagnosed with significant LEFT MAIN TRIPLE VESSEL coronary artery disease with EF- 35% with moderate MR and was advised coronary artery bypass grafting

3. All his routine blood investigations were sent Detailed history and examination was done. No significant medical history was present apart from diabetes,hypertension , angina and dysnea on exertion was present Preoperative anesthesia work up was done

4. AN UNUSUAL CALL- ALERT After sendling all the baseline blood samples, we got a call from the lab that blood samples showed error MCV, MCH and hematocrit ratio was deranged PBF showed large red cell mass Moreover, sample when incubated at 37 degree in the lab,CBC , MCV and MCH values came out to be normal

5. SUSPICION OF COLD AGGLUTININ ANTIBODY POSITIVE

6. OTHER TESTS DONE AS PER PROTOCOL LDH ( contribute to the diagnosis of hemolysis ) COOMBS test ( C3b antibody positive and titres were noted ) Hb-10.6 g/dl, plt-2.2 lac , overall CBC, KFT, LFT - normal

7. IHD WITH LMTVCAD with severe LV dysfunction with Moderate MR PREOPERATIVE TESTING DEMONSTRATED- ELEVATED CA TITRES(1:1025) AT THERMAL AMPLITUDE OF 32 DEGREE CELSIUS LDH- 735 IU/L BILIRUBIN AND COAGULATION PROFILE WAS NORMAL LAB TESTS FOR IM , SYPHILLIS, CYTOMEGALOVIRUS, MYCOPLASMA- NEGATIVE RETIC COUNT- 2%

8. CONCERNS 1.left main coronary artery disease 2.Poor LV function 3.Moderate Mitral Regurgitation 4.Recently diagnosed cold agglutinin disease (WHAT TO DO AND HOW TO DO) PLAN OF ACTION

9. CHALLENGES TO CARDIAC ANESTHETIST

10. BALANCED ANESTHESIA TECHNIQUE Baseline ACT= 132 sec Smooth induction of anesthesia was done with titrated doses of midazolam , fentanyl , etomidate and rocuronium Midline sternotomy was performed Heparin @ 400 iu /kg given CPB initiated after aortic cannulation and cavoatrial cannulation , ACT-842 sec

11. CPB CHALLENGES PLASALYTE CRYSTALLOID SOLUTION, SODIUM BICARBONATE AND MANNITOL PRE WARMED PRIMING SOLUTIONS ARE USED PRIMING VOLUME COLD CARDIOPLEGIA AVOIDED WARM BLOOD ANTEROGRADE CARDIOPLEGIA(34 DEGREE) GIVEN MORE FREQUENT DOSING OF CARDIOPLEGIA CARDIOPLEGIA CORE TEMPERATURE MAINTAINED AT 34 DEGREE CELSIUS OPERATING ROOM TEMPERATURE AND IV FLUIDS TEMPERATURE WAS MAINTAINED TEMPERATURE

12. Temperature was maintained at 34 degree celsius ACT throughout pump maintained more than 480 sec No visible agglutinates seen on pump No hematuria seen Ht mainatined between 25-30% MAP maintained >60mmhg Patient was easily weaned from the cpb machine with minimal ionotropes Heparin was neutralized (ACT-140)

13. Patient received 2 units of PRBC(warmed) in postoperative period Postoperative retic count was 2.2% On day o only patient was started with tab ecosprin (after the satisfactory drain output) POSTOPERATIVE COURSE REMAINS STABLE AND WAS DISCHARGED ON 7 th POST OPERATIVE DAY

14. KNOWING COLD AGGLUTININ DISEASE Rare hematologic disease Can present acutely in ER or with more chronic symptoms Challenging at presentation, if clinician is unfamiliar with this rare condition Clinicians need to be aware of pathogenesis, signs, symptoms, and potential risks associated with CAD, along with treatment options

15. Prevalence of Patients with CAD Affects ~one person per million every year Affects middle-aged and elderly people(40–80 years of age)- mean age- 60 years More common in women than men Represents 15% of all AIHA Paul L. Swiecicki . et al Cold agglutinin disease Division of Hematology, Mayo Clinic, Rochester, MN,2022

16. Clinical Features of Cold Agglutinin Disease Hemolysis/Hemolytic anemia Fatigue Dyspnea Complement-driven symptoms 1-4 Hemoglobinuria Jaundice Acrocyanosis Raynaud‘s phenomena Livedo reticularis Gangrene Cold-induced agglutination (IgM)-driven symptoms 5-7

17. COLD AGGLUTININ DISEASE

18. Pathogenesis of Cold Agglutinin Disease Rare form or subtype of AIHA caused by cold-reacting IgM autoantibodies Characterized by both: IgM -mediated agglutination of erythrocytes Hemolysis mediated by activation of the classical complement pathway

19. Firstly, there is paucity in literature Secondly, METHODS AND DIAGNOSTIC TESTS FOR SUCH DISEASE ARE NOT AVAILABLE AT EVERY C ENTRE. While during surgery on detection of ongoing catastrophe giving the clue to retrospectively evaluation of the patient: then the come to know that such patients are cold agglutinins positive

20. TAKE HOME MESSAGE SUCCESS OF CARDIAC SURGERY DEPENDS UPON

21. ALWAYS A MULTI DISCIPLINARY APPROACH LAB MEDICINE ARE OUR BACKBONE FOR TELLING US NOT ONLY THE GENERAL STATUS OF THE PATIENT BUT SOMETIMES RARE DISEASES CAN BE DIAGNOSED WITH JUST A SMALL CLUE AS IS THE CASE WITH OUR PATIENT  IRRESPECTIVE OF CLINICAL FEATURES, EVERY PATIENT SCHEDULED FOR CARDIAC SURGERY SHOULD UNDERGO PREOPERATIVE SCREENING OF COLD AGGLUTININ. PROPER VIGILANCE AND TAILORED APPROACH ARE KEY TO SUCCESS OF THESES PATIENTS