Multiple anesthesia experiences for two Loeys-Dietz syndrome (LDS) patients in the same family

Honda Takahiro, Yamashita Atsushi, Sekita Akihiko, Sugimura Kousuke, Ando Hisae, Toda Masaya, Okamoto Hirotsugu

doi:10.11478/jscva.2022-2-012

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Other Title

同一家系内の二人のLoeys-Dietz症候群（LDS）患者に対する複数回の麻酔経験
ドウイッカケイナイノフタリノ Loeys-Dietz ショウコウグン(LDS)カンジャニタイスルフクスウカイノマスイケイケン

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Abstract

<p>　We experienced multiple anesthesia in two patients with Loeys-Dietz syndrome within the same family. Case 1: 50-year-old female. She underwent emergency surgeries for two aortic dissections. In both surgeries, it was difficult to intubate and secure the arterial line, and there was significant blood loss for the patient. After discharge, she died of aortic rupture at the distal end of the replacement. Case 2: 30-year-old female, eldest daughter of the patient in Case 1. She underwent a standby basal to thoracoabdominal aortic replacement for an enlarged aorta, which was performed in triplicate. Based on our previous experience with Case 1, intubation and securing of the arterial line went smoothly, and the patient is currently doing well. This patient has an analog of Marfan syndrome, which can result in aortic dissection or rupture at a younger age. The patient is also prone to vascular tortuosity, premature skull fusion, congenital heart disease, joint contractures, and characteristic facial features and requires careful perioperative management accordingly.</p>

Journal

Cardiovascular Anesthesia

Cardiovascular Anesthesia 26 (1), 81-85, 2022-09-01

Japanese Society of Cardiovascular Anesthesiologists

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