Hypothermic circulatory arrest for aortic dissection with cryoglobulinemia

Abstract

Cryoglobulinemia is a cold-reactive autoimmune disease. A 64-year-old man with active cryoglobulinemia presented Stanford type A acute aortic dissection. He had been treated with immunosuppressive drugs and plasma exchange (PE) at our hospital; subsequently, qualitative analysis of cryoglobulin (CG) was negative. He underwent emergency ascending aorta replacement using cardiopulmonary bypass (CPB) under deep hypothermia circulatory arrest with selective cerebral perfusion. The total CPB time, aortic cross-clamp time, and selective cerebral perfusion time were 255, 153, 56 minutes, respectively, and the minimal nasopharyngeal temperature was 17.3°C. Our patient had no significant perioperative complications. Hence, if PE is performed appropriately and CG is negative, patients with cryoglobulinemia who exhibit severe preoperative symptoms can safely undergo surgery with deep hypothermia.

Perioperative management of cryoglobulinemia in patients requiring cardiac surgery

“…That study was limited by a majority of patients having cold agglutinin disease, with only 3 patients having the more serious cryoglobulinemia. 3 The more recent series by Yamazaki and colleagues 4 , 5 provides a complete review of case reports, including only those with a diagnosis of cryoglobulinemia (n = 14) across several operations such as coronary, valve, and aortic surgery with 3 patients requiring hypothermic circulatory arrest. The series highlights a significant increase in the use of plasma exchange (57%; n = 8 out of 14, pre/intra/post) as well as warm/tepid blood cardioplegia (93%; n = 13 out of 14) with only 1 reported mortality (7.1% perioperative mortality).…”

“…The series highlights a significant increase in the use of plasma exchange (57%; n = 8 out of 14, pre/intra/post) as well as warm/tepid blood cardioplegia (93%; n = 13 out of 14) with only 1 reported mortality (7.1% perioperative mortality). 4 , 5 …”

“…Hypothermia and cold cardioplegic arrest are routine components of cardiac surgery that can precipitate activation of cryoglobulins, resulting in disastrous microvascular damage, cellular lysis, and vascular or extracorporeal circuit thrombosis. Several case reports have identified various approaches to safely perform cardiac surgery 3 , 4 ; however, the following case and discussion provide a summary and review of strategies for management of patients with cryoglobulinemia who require cardiac surgery. The patient provided written consent for publication of study data and this was approved by the West Virginia University Health Sciences Institutional Review Board (protocol #2006047334; June 23, 2022).…”

Cryoglobulinemia: the “cold” problem in cardiac surgery, a single-center experience and a literature review

“…As a second option, Yamazaki and colleagues, instead, performed a successful deep hypothermia in patients with Stanford type A aortic dissection which required cardiac arrest and deep hypothermia for neuroprotection [30]. In this case, a 64-year-old man diagnosed with symptomatic idiopathic cryoglobulinemia underwent immunosuppressive therapy plus plasma exchange and double filtration plasmapheresis; with the abovementioned therapies the qualitative analysis of cryoglobulins was found negative, hence, when the patient experienced chest and back pain and was diagnosed with aortic dissection, the medical team opted for deep hypothermia (minimal nasopharyngeal temperature was 17.3 °C) with selective cerebral perfusion.…”

“…Preoperative management -Plasmapheresis and/or plasma exchange [30,[32][33][34][35] -Immunosuppressive therapy [30,32,34] -Steroids [30,[32][33][34][35] Intraoperative management -Warm/cold blood cardioplegia and systemic warming [31][32][33]35] -Warm/cold blood cardioplegia and mild hypothermia (temperature above cryoprecipitation threshold) [28,34] -Hypothermia after negative cryo test [30]…”