Chronic Thromboembolic Pulmonary Hypertension With Cold Agglutinins Needing Pulmonary Thromboendarterectomy: The Use of Therapeutic Plasma Exchange.
6 affiliations
- 1Division of Pulmonary and Critical Care Medicine Loma Linda University Medical Center Loma Linda California USA.
- 2Division of Pulmonary, Critical Care, Sleep Medicine and Physiology UC San Diego Health La Jolla California USA.
- 3Division of Nephrology-Hypertension UC San Diego Health San Diego California USA.
- 4Division of Cardiovascular and Thoracic Surgery UC San Diego Health San Diego California USA.
- 5Department of Pathology UC San Diego Health San Diego California USA.
- 6Division of Hematology/Oncology UC San Diego Health La Jolla California USA.
Abstract
Cold agglutinins can be detected in patients with chronic thromboembolic pulmonary hypertension (CTEPH) during pulmonary thromboendarterectomy (PTE) pre-operative testing. Peri-operative management of these patients in anticipation of deep hypothermic circulatory arrest can be challenging and has not been standardized. We describe our relevant experience and suggest a potential protocolized pre-operative approach with this observational, retrospective cohort study. A chart review of all patients undergoing PTE surgery for CTEPH (1107 patients) at UC San Diego between 2019 and 2025 was performed. An Institutional Review Board approved quality improvement database on all patients undergoing PTE surgery was used. Twenty-one patients (1.9%) screened positive for cold agglutinins pre-PTE. A high co-prevalence of antiphospholipid syndrome and cold agglutinins was noted (17/21; 81%). Thermal amplitude testing (when possible) was positive for nine patients between 15°C and 18°C. Fifteen patients underwent therapeutic plasma exchange (median of two sessions) with albumin and plasma replacement fluid followed by PTE surgery with standard cooling protocol. There were no incidents of agglutination or hemolysis peri- or post-operatively. Three patients had post-operative airway hemorrhage, two of which developed heparin-induced thrombocytopenia (HIT). There was one mortality in the setting of HIT with recurrent thrombosis and bleeding. Median intensive care unit and hospital length of stays were 3 and 12.5 days, respectively. We suggest a protocolized approach for the identification and management of high-risk patients with cold agglutinins, which may include therapeutic plasma exchange pre-operatively. This can successfully mitigate the potential risk of hypothermic circulatory arrest causing agglutination during surgery.
Related
- Comparison of Clinical, Hemodynamic and Imaging Profiles Between Isolated Pulmonary Vasculitis and Chronic Thromboembolic Pulmonary Hypertension
- Reduction in Right Ventricular Stroke Work Index After Balloon Pulmonary Angioplasty is Associated With Long-Term Prognosis in Chronic Thromboembolic Pulmonary Hypertension.
- Obstructive Ventilatory Defects in Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension
- Evaluating preoperative imaging in patients with chronic thromboembolic pulmonary hypertension: Is computed tomography pulmonary angiography a reliable alternative to pulmonary angiography?
- Right Ventricular-Pulmonary Arterial Coupling by Serial TAPSE/sPAP: A Noninvasive Predictor for Clinical Outcomes in Chronic Thromboembolic Pulmonary Hypertension.
BibTeX
@article{naqvi2026chronic,
title = {{Chronic Thromboembolic Pulmonary Hypertension With Cold Agglutinins Needing Pulmonary Thromboendarterectomy: The Use of Therapeutic Plasma Exchange.}},
author = {Beenish Naqvi and Marie A Bautista and Amber P Sanchez and Jenny Z Yang and Timothy M Fernandes and David S Poch and Kim M Kerr and Nick H Kim and Michael M Madani and Victor G Pretorius and Elizabeth S Allen and Patricia M Kopko and Laura D Dilly and Charles Ginsberg and Soo J Park and Michael Choi and Dinna N Cruz and Demosthenes G Papamatheakis},
journal = {Pulmonary circulation},
year = {2026},
month = oct,
doi = {10.1002/pul2.70423},
url = {https://europepmc.org/article/MED/42924162},
note = {CC BY-NC},
}
RIS
TY - JOUR
TI - Chronic Thromboembolic Pulmonary Hypertension With Cold Agglutinins Needing Pulmonary Thromboendarterectomy: The Use of Therapeutic Plasma Exchange.
AU - Beenish Naqvi
AU - Marie A Bautista
AU - Amber P Sanchez
AU - Jenny Z Yang
AU - Timothy M Fernandes
AU - David S Poch
AU - Kim M Kerr
AU - Nick H Kim
AU - Michael M Madani
AU - Victor G Pretorius
AU - Elizabeth S Allen
AU - Patricia M Kopko
AU - Laura D Dilly
AU - Charles Ginsberg
AU - Soo J Park
AU - Michael Choi
AU - Dinna N Cruz
AU - Demosthenes G Papamatheakis
PY - 2026
DA - 2026/10/22
JO - Pulmonary circulation
DO - 10.1002/pul2.70423
UR - https://europepmc.org/article/MED/42924162
AB - Cold agglutinins can be detected in patients with chronic thromboembolic pulmonary hypertension (CTEPH) during pulmonary thromboendarterectomy (PTE) pre-operative testing. Peri-operative management of these patients in anticipation of deep hypothermic circulatory arrest can be challenging and has not been standardized. We describe our relevant experience and suggest a potential protocolized pre-operative approach with this observational, retrospective cohort study. A chart review of all patients undergoing PTE surgery for CTEPH (1107 patients) at UC San Diego between 2019 and 2025 was performed. An Institutional Review Board approved quality improvement database on all patients undergoing PTE surgery was used. Twenty-one patients (1.9%) screened positive for cold agglutinins pre-PTE. A high co-prevalence of antiphospholipid syndrome and cold agglutinins was noted (17/21; 81%). Thermal amplitude testing (when possible) was positive for nine patients between 15°C and 18°C. Fifteen patients underwent therapeutic plasma exchange (median of two sessions) with albumin and plasma replacement fluid followed by PTE surgery with standard cooling protocol. There were no incidents of agglutination or hemolysis peri- or post-operatively. Three patients had post-operative airway hemorrhage, two of which developed heparin-induced thrombocytopenia (HIT). There was one mortality in the setting of HIT with recurrent thrombosis and bleeding. Median intensive care unit and hospital length of stays were 3 and 12.5 days, respectively. We suggest a protocolized approach for the identification and management of high-risk patients with cold agglutinins, which may include therapeutic plasma exchange pre-operatively. This can successfully mitigate the potential risk of hypothermic circulatory arrest causing agglutination during surgery.
SN - 2045-8932
SN - 2045-8940
ER -