Anesthesia management of combined sequential heart–liver transplantation using a caval clamp without venovenous bypass: A case report

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 undergoi...

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Main Authors: Ye-Ke Zhu, Yan-Feng Zhou, Tian-Xiang Zhang, Yong-Xing Yao
Format: Article
Language:English
Published: Elsevier 2022-09-01
Series:Heliyon
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S2405844022020187
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author Ye-Ke Zhu
Yan-Feng Zhou
Tian-Xiang Zhang
Yong-Xing Yao
author_facet Ye-Ke Zhu
Yan-Feng Zhou
Tian-Xiang Zhang
Yong-Xing Yao
author_sort Ye-Ke Zhu
collection DOAJ
description 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.
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spelling doaj.art-3babc98a299d4a6cb579678ca2f8f12d2022-12-22T04:32:32ZengElsevierHeliyon2405-84402022-09-0189e10730Anesthesia management of combined sequential heart–liver transplantation using a caval clamp without venovenous bypass: A case reportYe-Ke Zhu0Yan-Feng Zhou1Tian-Xiang Zhang2Yong-Xing Yao3Department of Anesthesia, First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, People's Republic of ChinaDepartment of Anesthesia, First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, People's Republic of ChinaDepartment of Anesthesia, First Affiliated Hospital Beilun Branch, Zhejiang University School of Medicine, Ningbo, People's Republic of ChinaDepartment of Anesthesia, First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, People's Republic of China; Corresponding author.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.http://www.sciencedirect.com/science/article/pii/S2405844022020187Transthyretin familial amyloid polyneuropathyCombined heart–liver transplantationCardiopulmonary bypassVenovenous bypass
spellingShingle Ye-Ke Zhu
Yan-Feng Zhou
Tian-Xiang Zhang
Yong-Xing Yao
Anesthesia management of combined sequential heart–liver transplantation using a caval clamp without venovenous bypass: A case report
Heliyon
Transthyretin familial amyloid polyneuropathy
Combined heart–liver transplantation
Cardiopulmonary bypass
Venovenous bypass
title Anesthesia management of combined sequential heart–liver transplantation using a caval clamp without venovenous bypass: A case report
title_full Anesthesia management of combined sequential heart–liver transplantation using a caval clamp without venovenous bypass: A case report
title_fullStr Anesthesia management of combined sequential heart–liver transplantation using a caval clamp without venovenous bypass: A case report
title_full_unstemmed Anesthesia management of combined sequential heart–liver transplantation using a caval clamp without venovenous bypass: A case report
title_short Anesthesia management of combined sequential heart–liver transplantation using a caval clamp without venovenous bypass: A case report
title_sort anesthesia management of combined sequential heart liver transplantation using a caval clamp without venovenous bypass a case report
topic Transthyretin familial amyloid polyneuropathy
Combined heart–liver transplantation
Cardiopulmonary bypass
Venovenous bypass
url http://www.sciencedirect.com/science/article/pii/S2405844022020187
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