Combined heart-liver transplantation
Alexander Chernyavskiy MD 1
Sergey Alsov MD 1
Kseniya Guliaeva1
Ivan Porshennikov MD, PhD 2
1Department of Aortic and Coronary Artery Surgery,
National Medical Research Center named after academician E.N. Meshalkin, Novosibirsk, Russian Federation.
2Department of Transplantation and Liver Surgery,
State Novosibirsk Regional Clinical Hospital, Novosibirsk, Russian
Federation.
Guliaeva Kseniya Konstantinovna Address: 15, Rechkunovskaya,
Novosibirsk, 630055,
Russian Federation, 8-913-468-89-12,
e-mail: guliaeva_k@meshalkin.ru
Funding statement: The work was not supported by any sources
Abstract: We report a rare case of liver alveolar
echinococcosis with an invasion of the hepaticocaval confluence,
inferior vena cava, pericardium, right atrium, atrial septum, and
superior vena cava, and its successful treatment by combined heart-liver
transplantation.
Keywords: alveolar echinococcosis, vascular invasion, combined
heart-liver transplantation.
Introduction: The first combined heart-liver transplantation
(CHLT) was performed in a 6-year-old girl with familial
hypercholesterolemia and secondary heart failure in 1984 (1). From 1988
to February 2020, 329 cases of CHLT have been recorded in the United
Network for Organ Sharing (2). Indications for CHLT include cardiac
cirrhosis, concurrent liver-heart diseases, and amyloidosis, but not
alveolar echinococcosis (AE) of the heart (3). AE is a helminthiasis
caused by the Echinococcus multilocularis larvae, which are
retained by the liver while passing into the portal bloodstream from the
bowel and form tumor-like neoplasms. Treating patients with liver AE is
difficult as it shows signs of a slowly growing malignant tumor with
infiltrative growth, possible invasion of adjacent organs, and ability
to metastasize (4). Endemic hotbeds are Western Siberia, Far East,
Yakutia, Central Asia, Central Europe, Alaska, and Northern Canada.
CASE REPORT: The patient was a 36-year old woman who
experienced severe, colicky pain in the right hypochondrium for about a
year. For several years, she had contact with wild animals, mainly
foxes, and was involved in processing of fur. She was diagnosed with AE
of the liver.
Further examination revealed that in addition to the liver, her heart
was affected. Computed tomography showed a parasitic lesion with
complete occlusion of the hepaticocaval confluence and inferior vena
cava (IVC), narrowing of the right atrium, sinus of the superior vena
cava (SVC), and coronary sinus (Fig. 1 A), and a solitary 3-cm AE in the
left lung. No brain AE metastases were detected. Echocardiography
revealed a new growth in the roof and septum of the right atrium with a
clear uneven contour reducing the atrial cavity by approximately
40%-50% and obstructing the mouth of the SVC (Fig. 1 B). As the AE
involved the liver, heart chambers, and IVC, isolated liver
transplantation was impossible; therefore, CHLT was performed.
The patient was in the waiting list for 76 days. At the time of
transplantation, she had ascites, severe shortness of breath, and
distended veins of the neck.
The procedure first involved a J-shaped laparotomy. The liver showed
typical features of Budd-Chiari syndrome. Parasitic lesion was localized
in liver segments 1, 2, 3, 4, 7, and 8 and grew in hepaticocaval
confluence, retro- and suprahepatic IVC, diaphragm, and pericardium. The
liver was completely mobilized, and anteroposterior phrenotomy was
achieved. The diaphragm and pericardium were partially resected in the
circumference of the IVC, and its supraphrenic segment was encircled
from the abdomen (Fig. 1 C).
The next step involved a median sternotomy. The heart was not enlarged.
Cardiopulmonary bypass was initiated with peripheral IVC cannulation
through the femoral vein, SVC and aorta were cannulated directly. The
completely mobilized heart was lowered through the diaphragm into the
abdominal cavity and removed en bloc with the liver and
retrohepatic segment of the IVC (Fig. 2 A, B).
Orthotopic heart transplantation was performed using the standard
bicaval technique. IVC cardiac anastomosis formed with the interposition
fresh venous allograft (infrarenal IVC from the same donor) between
heart and liver which was placed into the abdominal cavity. The heart
was reperfused without IVC inflow. Heart ischemia time was 3 hours 50
minutes. Liver was transplanted using the conventional technique.
Superior caval anastomosis was formed using interposition venous
allograft (Fig. 2 C). Liver cold and warm ischemia time were 4 hours 7
minutes and 41 minutes respectively.
We used the triple immunosuppression protocol based on tacrolimus in
combination with mycophenolic acid and steroids. The postoperative
period was complicated by bile leakage, which caused anastomotic
arterial bleeding and was successfully treated by Roux-en-Y
hepaticojejunostomy with hepatic artery reconstruction. The total
hospital stay was 4 months. Before
discharge, multiple small AE metastases were detected in both lungs and
adjuvant chemotherapy with albendazole was initiated.
On 1-year follow-up, the patient
was in a good condition and lung metastases remained stable.
DISCUSSION: Radical surgery associated with antiparasitic
chemotherapy is the only curative option for liver AE. But due to
asymptomatic process, many patients have advanced disease with vascular
invasion when standard liver resection is unlikely to be feasible.
However, in most of these cases the radical treatment may be provided
using total vascular exclusion with in vivo or ex vivohypothermic liver surgery (5). Liver transplantation is regarded as a
possible treatment option for nonresectable AE and can be performed even
if the suprahepatic IVC is involved but not the heart.
In conclusion, CHLT is a rare procedure that treats complex and often
fatal conditions, and, to date, does not include AE in the indications.
Acknowledgements: None
Conflict of Interest Statement: There are no conflicts of interest.
Author contribution statement: All authors are familiar and agree with
the content of the article
Informed patient consent obtained
Figure legends:
Figure 1. (A) Preoperative computed tomography, (B) Preoperative
echocardiography, (C) Parasitic lesion in the liver
Figure 2. (A) Liver of the recipient, (B) Heart removed with liveren bloc , (C) Transplanted heart and liver.
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