PDF(Pubmed)
Abstract：
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.
摘要：
家族性淀粉样多发性神经病,由于转甲状腺素蛋白基因突变导致的常染色体显性疾病，经常影响心脏和肝脏，并且最好采用心脏-肝脏联合移植（CHLT）。尽管它仍然是一个不常见的程序，接受CHLT的患者数量正在增加.由于与双重病理生理学相关的复杂性，CHLT对麻醉管理提出了非凡的挑战。心脏和肝脏移植均在体外循环（CPB）下进行；或心脏移植在CPB上进行，然后进行静脉旁路移植术。最近的报道表明，可以使用下腔静脉保留技术在没有旁路的情况下进行肝移植。然而,旁路和腔静脉保留技术都有自己的并发症。这里,我们介绍了使用常规腔静脉交叉钳夹技术而没有静脉静脉搭桥的序贯心脏-肝脏移植的麻醉管理。一名48岁的男子抱怨胸闷，胸痛,呼吸急促被诊断为淀粉样心肌病。心脏超声检查显示心室壁增厚和左心室收缩功能不全（6个月内射血分数从46%下降到20%），这是难以治疗的药物。症状反复出现。因此,CHLT是计划好的。在全身麻醉和标准CPB下顺利进行心脏移植。多巴酚丁胺和肾上腺素输注后，他的心脏功能良好。随后，患者脱离CPB。计划使用piggyback程序和保留腔的技术进行肝移植。然而,在腔室夹紧时，意外失血。测试了腔室的夹紧，然后进行了交叉夹紧。去甲肾上腺素,肾上腺素,并给予多巴酚丁胺。肝静脉吻合后，释放夹钳并给予硝酸甘油.血流动力学稳定,住院37天后出院。该病例表明，在选定的患者中，可以使用腔内夹进行CHLT而无需静脉旁路。
