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cessation of cardiotomy suction upon delivery of protamine. Anaesthesia should also take great care to avoid injecting air with intravenous medications and drug syringe stopcocks 7.
Patients with HHT are at risk of both haemorrhagic and embolic complications from cardiac surgery. Though the condition is rare, it is worth considering strategies to mitigate risk for this vulnerable group of patients when surgery is unavoidable. With some preoperative investigations, intraoperative management strategies and postoperative blood conservation, a series of small variations to practice can aggregate into a safer cardiac surgery for those affected by hereditary haemorrhagic telangiectasia.
References
1. McDonald, J, Pyeritz, RE, ‘Hereditary Hemorrhagic Telangiectasia’ in GeneReviews. June 2000, viewed on 12th March 2021, https://www.ncbi.nlm.nih.gov/ books/NBK1351/
2. Karkouti, K, Wijeysundera, DN, Beattie, WS, ‘Risk Associated With Preoperative Anemia in Cardiac Surgery, A Multicenter Cohort Study’, Circulation. Vol. 117, 2008, pp.478–484
3. Papagiannis, J, Apostolopoulou, S, Sarris, G, Rammos, S. ‘Diagnosis and management of pulmonary arteriovenous malformations’. Images in Paediatric Cardiology vol. 4, 2002; pp. 33–49.
4. Garcia-Tsao, G, Korzenik, JR, Young, L, Henderson, KJ, Jain, D, Byrd, B, Pollak, JS, White, RI Jr., ‘Liver disease in patients with hereditary hemorrhagic telangiectasia’. New England Journal of Medicine, vol. 343, 2000, pp.931–936
5. Thunberg, CA, Gaitan, BD, Grewal, A, Ramakrishna, H, Stansbury, LG, Grigore, AM, ‘Pulmonary hypertension in patients undergoing cardiac surgery: pathophysiology, perioperative management and outcomes’, Journal of Cardiothoracic and Vascular Anaesthesia, vol. 27, 2013, pp. 551–572
6. Robinson, D, Rogers, B, Kapoor, R, Swan, J, Speas, G and Gutmann, R, ‘Anesthetic considerations for a patient with hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu Syndrome) undergoing a five-box thoracoscopic maze procedure for atrial fibrillation. Journal of Investigative Medicine High Impact Case Reports, vol. 2, 2014
7. Weingarten, TN, Hanson, JW, Anusionwu, KO, Moncrief, ML, Opdahl, TJ, Schneider, DD, and Sprung, J (2013). ‘Management of patients with hereditary hemorrhagic telangiectasia undergoing general anesthesia: a cohort from a single academic center's experience’, Journal of Anesthesia, vol. 27, 2013, pp. 705–711
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