176 J. L. Cornibe and A. H. Katz: J Extra Corpor Technol 2025, 57, 174--177
Discussion
Patients with PV not only present with a wide variety of coagulopathies, but coupled with the use of CPB and its hemodynamic implications, a whole subset of challenges arise. As previous literature suggests, many cases involving extracorporeal circuits have experienced issues with high CPB line pressure, oxygenator failure / dysfunction, and or thrombosis within the disposable circuit components [ 5--7 ]. Since this patient was not of an emergent nature, planning, and research took place in preparation for this case. Suggestions from an offpump CABG case reported phlebotomizing the patient once anesthetized, as well as liberal administration of fluids during the perioperative period [ 10 ]. Our aim during the perioperative period was to stay safely anticoagulated and continue repetitive hemodilution techniques while keeping close account of his various hypercoagulable attributes. Our first step was removing a 400 mL unit of autologous blood from the patient once anesthetized to reduce RBC volume. Utilizing the HMS compared to other ACT devices, we were also able to observe the Hepcon level, which proved to be imperative in a case such as this, as the ACT itself does not appear accurate. Normally, we would run an adjusted Hepcon level at 3.0 mg / kg, but we prophylactically increased to a Hepcon of 4.0 mg / kg for this patient. This was particularly done since previously published cases with elevated CPB ACTs, showed no benefit in the prevention of thrombosis of CPB circuit components [ 5, 6 ]. Due to inconsistencies in ACT levels and their inability to produce adequate anticoagulation in previous cases [ 5, 6 ], the Hepcon level was heavily relied upon in comparison to the ACT. Heparin was liberally administered upon each Hepcon result so as not to get behind.
Standardly, before initiation of CPB, we would VAP the entire CPB circuit. It was decided to allow the hemodilution of the circuit prime in this scenario. Once CPB was initiated, we were able to separate off a liter of autologous blood, replacing it with PlasmalyteA via the quick prime line for gradual re-introduction later in the bypass run. Han et al. describe an ideal target range for HCT between 25 % and 30 % while on extracorporeal support [ 11 ]. However, there is also evidence an HCT below 22 % of CPB can cause complications postoperatively [ 12 ]. Our goal was a target HCT < 30 %, which was achieved by the addition of PlasmalyteA for ANH. Since the laboratory PLT results returned beyond the high limits with secondary confirmation as well as PLT aggregation seen in the pump suckers and the cell saver bowl, the divided-off liter of autologous blood from the beginning of the pump run was sent to the cell saver. The intent was to remove as many of the non-RBC components as possible to reduce the PLT aggregation that is starting to evolve. Extra wash volume and leukocyte reduction filters were utilized before the slow administration back to the CPB circuit with additional heparin. There was no change in pump arterial line pressure or need to increase flow that would allude to issues within the circuit / oxygenator, nor was any thrombosis seen within the enclosed part of the reservoir or CPB circuit. The cross-clamp was removed, and the patient was soon to be weaned from bypass at this point, which proved of great benefit. Cerebral saturation did not waver at any point in the perioperative period.
When the patient returned to the operating room on POD1 for re-exploration, this could have well been a bleed induced by surgical insult due to his Factor X deficiency, similarly reported in a cardiac tamponade case reported by Othman et al. [ 9 ].
In hindsight, we could have removed a second unit of autologous harvest from the patient instead of one when first anesthetized, as his HCT minimally decreased. We could have also more liberally hemodiluted with PlasmalyteA during the CPB run, aiming for an HCT in the 25 % range as opposed to 30 %. However, since there are scarce cases of PV reported with the use of CPB, we are hopeful that our uneventful CPB run and the way we navigated throughout may be of potential assistance for future cases of this rare blood disease.
Funding This research did not receive any specific funding.
Conflicts of interest The authors declare that there is no conflict of interest.
Data availability statement Data availability is not applicable.
Author contribution statement
JLC produced original manuscript and AHK contributed data and reviewed. Both JLC & AHK had input on revisions and final manuscript.
Ethics approval
This paper was presented before submission to hospital risk management for approval.
References
1. Hatalova A, Schwarz J, Gotic M, et al. Recommendations for the diagnosis and treatment of patients with polycythaemia vera. Eur J Haematol. 2018; 1( 5): 654--664.
2. Ruggeri M, Rodeghiero F, Tosetto A, et al. Postsurgery outcomes in patients with polycythemia vera and essential thrombocythemia: a retrospective study. Blood. 2008; 111( 2): 666--667.
3. Strauss E, Mazzeffi M, Williams B, et al. Perioperative management of rare coagulation factor deficiency states in cardiac surgery. Br J Anaesth. 2017; 119( 3): 354--368.
4. Kwaan HC, Wang J. Hyperviscosity in polycythemia vera and other red cell abnormalities. Semin Thromb Hemost. 2003; 29: 451--458.
5. Kaiser P, Zuckermann A, Horvat J, et al. Acute oxygenator occlusion in two cases of polycythemia vera: bailout strategies. J Card Surg. 2020; 35( 10): 2835--2837.
6. Lehot JJ, Was B, Dendeleu L, et al. Oxygenator thrombosis without heparin resistance in polycythemia vera. Ann Fr Anesth Reanim. 2004; 23( 2): 153--156.
7. Ursulet L, Pierrakos C, Cudia A, et al. High hemoglobin level as a limiting factor for extracorporeal membrane oxygenation. ASAIO J. 2019; 65: e97--e99.
8. Chatterjee T, Philip J, Nair V, et al. Inheritied factor X( Stuart- Prower Factor) deficiency and its management. Med J Armed Forces India. 2014; 71( Suppl 1): S184--S186.