Zhu Ye-Ke, Zhou Yan-Feng, Zhang Tian-Xiang, Yao Yong-Xing
Department of Anesthesia, First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, People's Republic of China.
Department of Anesthesia, First Affiliated Hospital Beilun Branch, Zhejiang University School of Medicine, Ningbo, People's Republic of China.
Heliyon. 2022 Sep 22;8(9):e10730. doi: 10.1016/j.heliyon.2022.e10730. eCollection 2022 Sep.
Familial amyloid polyneuropathy, an autosomal-dominant disease due to mutations in the transthyretin gene, often affects the heart and liver, and is treated best with a combined heart-liver transplantation (CHLT). Although it remains an uncommonly performed procedure, the number of patients undergoing CHLT is increasing. Because of the complexity associated with dual pathophysiology, CHLT poses an extraordinary challenge for anesthesia management. Either both heart and liver transplantation are performed on cardiopulmonary bypass (CPB); or heart transplantation is performed on CPB, followed by liver transplantation with venovenous bypass. Recent reports suggested that liver transplantation can be performed without bypass using the inferior vena cava-sparing technique. However, both bypass and caval sparing technique have their own complications. Here, we present the anesthesia management in a case of sequential heart-liver transplantation using a routine caval cross-clamp technique without venovenous bypass. A 48-year-old man complaining of chest tightness, chest pain, and shortness of breath was diagnosed with amyloid cardiomyopathy. Cardiac ultrasonography revealed thickening of ventricular walls and left ventricular systolic insufficiency (ejection fraction decreased from 46% to ∼20% in 6 months), which was refractory to medical therapy. Symptoms occurred repeatedly. Therefore, CHLT was planned. Heart transplantation was performed smoothly under general anesthesia and standard CPB. His heart functioned well with dobutamine and epinephrine infusion. Subsequently, the patient was weaned from CPB. Liver transplantation was planned using the piggyback procedure with the caval sparing technique. However, upon caval clamping, unexpected blood loss occurred. Clamping of the caval was tested followed by cross-clamping. Norepinephrine, epinephrine, and dobutamine were administered. After the hepatic vein was anastomosed, the clamp was released and nitroglycerin was administered. Hemodynamics was stable, and the patient was discharged after 37 days of hospitalization. The case indicates that CHLT could be performed using caval clamp without venovenous bypass in selected patients.
家族性淀粉样多神经病是一种由于转甲状腺素蛋白基因突变导致的常染色体显性疾病，常累及心脏和肝脏，最佳治疗方法是进行心脏-肝脏联合移植（CHLT）。尽管这仍是一种不常施行的手术，但接受CHLT的患者数量正在增加。由于与双重病理生理相关的复杂性，CHLT对麻醉管理构成了巨大挑战。心脏和肝脏移植要么在体外循环（CPB）下进行；要么心脏移植在CPB下进行，随后肝脏移植采用静脉-静脉旁路。最近的报告表明，使用保留下腔静脉技术可以在无旁路的情况下进行肝脏移植。然而，旁路和保留腔静脉技术都有其自身的并发症。在此，我们介绍一例采用常规腔静脉交叉钳夹技术且无静脉-静脉旁路的序贯心脏-肝脏移植病例的麻醉管理。一名48岁男性，主诉胸闷、胸痛和呼吸急促，被诊断为淀粉样心肌病。心脏超声显示心室壁增厚和左心室收缩功能不全（射血分数在6个月内从46%降至约20%），药物治疗无效。症状反复出现。因此，计划进行CHLT。在全身麻醉和标准CPB下顺利进行了心脏移植。通过输注多巴酚丁胺和肾上腺素，他的心脏功能良好。随后，患者脱离CPB。计划采用保留腔静脉技术的背驮式手术进行肝脏移植。然而，在腔静脉钳夹时，发生了意外失血。先测试腔静脉钳夹，然后进行交叉钳夹。给予去甲肾上腺素、肾上腺素和多巴酚丁胺。肝静脉吻合后，松开钳夹并给予硝酸甘油。血流动力学稳定，患者住院37天后出院。该病例表明，在选定的患者中，可以使用腔静脉钳夹且无静脉-静脉旁路进行CHLT。
