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Table of Contents
CASE REPORT
Year : 2021  |  Volume : 15  |  Issue : 4  |  Page : 351-353

En bloc combined heart–liver transplantation for Ebstein's anomaly and cardiac cirrhosis - A case report


1 Department of Cardiothoracic Surgery and Transplantation, Apollo Hospitals, Chennai, Tamil Nadu, India
2 Department of Hepato-Biliary and Liver Transplantation, Apollo Hospitals, Chennai, Tamil Nadu, India

Date of Submission29-Dec-2020
Date of Decision01-May-2021
Date of Acceptance03-May-2021
Date of Web Publication30-Dec-2021

Correspondence Address:
Dr. Madhan Kumar Kuppuswamy
52, Gangadeeswar Koil Street, NuTech-Sree Nivas Apartment, Flat: C9, Purasawakkam, Chennai - 600 084, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijot.ijot_159_20

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  Abstract 

In this report, we present our experience with combined en bloc combined heart and liver transplantation performed in 2015 on a 29-year-old male with Ebstein's anomaly with cardiac cirrhosis and decompensation. Entire procedure was done under cardiopulmonary bypass. Portocaval shunting was done during the procedure to reduce bowel congestion. He remains well on regular follow-up at 4 years. This is the first procedure to be reported from India.

Keywords: Cardiac cirrhosis, combined heart and liver transplantation, en bloc


How to cite this article:
Kuppuswamy MK, Ramamurthy A, Thirugnanasambandam S, Thangaraj PR. En bloc combined heart–liver transplantation for Ebstein's anomaly and cardiac cirrhosis - A case report. Indian J Transplant 2021;15:351-3

How to cite this URL:
Kuppuswamy MK, Ramamurthy A, Thirugnanasambandam S, Thangaraj PR. En bloc combined heart–liver transplantation for Ebstein's anomaly and cardiac cirrhosis - A case report. Indian J Transplant [serial online] 2021 [cited 2022 Aug 10];15:351-3. Available from: https://www.ijtonline.in/text.asp?2021/15/4/351/334427




  Introduction Top


A combined heart and liver transplantation (CHLT) is the only treatment option for end-stage combined heart and liver failure. One of the indications for CHLT is congenital heart disease with secondary cardiac cirrhosis. In South East Asia and India, the majority of organ transplants are performed from living donors. Deceased donation rates are gradually improving, especially in certain regions, enabling multiorgan transplants including the heart.


  Case Report Top


A 29-year-old male was referred with end-stage heart and liver failure with severe ascites [Figure 1]a. He was diagnosed to have Ebstein's anomaly, Grade 4 (Celermajer classification) with cirrhosis of the liver (Model for End-Stage Liver Disease Score: 11, Child-Turcotte-Pugh Score: 9/15, Child's B). He had refractory ascites requiring repeated paracentesis to relieve pressure symptoms. A comprehensive multidisciplinary evaluation for orthotopic heart and liver transplantation was performed, and he was found to be fit with no contraindication.
Figure 1: (a and b) Preoperative and postoperative images of the patient's chest and abdomen

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Upon availability of a suitable donor (27-year-old male), the recipient was taken up for CHLT. Injection methylprednisolone 500 mg intravenous (IV) was given at induction. The chest was opened by midline sternotomy incision and abdomen by bilateral subcostal incision [Figure 1]b. Cardiopulmonary bypass (CPB) was established using extracorporeal membrane oxygenation circuit to minimize trauma to the blood. Aorta and direct superior vena cava cannulation was performed. The right femoral vein was cannulated with tip of the cannula below the proposed line of anastomosis in the infrahepatic inferior vena cava (IVC). The patient was cooled to 32°C. On completion of recipient hepatectomy, a portacaval shunt was performed to decrease bowel congestion. Diaphragm was incised till the IVC opening. Heart and liver were explanted using standard techniques.

The donor heart and liver were retrieved en bloc with intact IVC between the heart and the liver [Figure 2] and [Figure 3]. Histidine-tryptophan-ketoglutarate solution was used for the heart and liver preservation.
Figure 2: (a) Hear t with Ebstein's anomaly with cirrhotic liver. (b) Transplanted heart and liver

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Figure 3: Harvested en bloc donor heart and liver with intact inferior vena cava

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Heart transplantation was completed, and the suprahepatic IVC was clamped. Aortic cross-clamp was released after giving injection methylprednisolone 500 mg IV. Reperfusion of the heart was started using leukocyte-depleted blood with saturation of 80% for 10 min and subsequently increased to 100%. Liver implantation was then performed after disconnecting the portacaval shunt, anastomosing the infrahepatic IVC and portal vein. The liver was reperfused while on CPB, which was gradually weaned off. The diaphragm was reconstructed using prolene suture. Chest and abdomen were closed. He was extubated on the 1st postoperative day (POD). He was started on triple immunosuppressive therapy (prednisolone, tacrolimus, and mycophenolate mofetil [MMF]). He recovered uneventfully and was discharged from the hospital on the 9th POD. He had endomyocardial biopsies on the 7th day, 3rd month, 6th month, 9th month, and 1 year which showed Grade 0 rejection on the 7th day and 1 year. The rest of the biopsies showed Grade 1R.

He was readmitted to the hospital 2 months after transplantation with abdominal pain and vomiting. Evaluation revealed intestinal obstruction. Adhesions between small bowel loops and anterior abdominal wall obstructing the jejunum were released laparoscopically. He had an uneventful recovery and discharged on the 2nd POD. Since then, he recovered well. He was being followed up every 6 monthly with blood tests, echocardiogram, and ultrasound of the abdomen. He went on to get married in 2017 and was blessed with a child in 2018.

Comment

Historically, concurrent liver failure was considered to be a contraindication to heart transplant, as was heart failure to liver transplant.

The first CHLT was described by Starzl et al. in 1984.[1] CHLT is a relatively rare procedure performed at a few centers across the world even as there is a slow increase in frequency over time. Approximately 250 cases have been registered in United Network for Organ Sharing database between 1998 and 2018.[2]

There are three main indications for CHLT:

  1. Primary heart disease resulting in secondary cardiac cirrhosis caused by chronic hepatic venous outflow obstruction
  2. Hereditary amyloidosis leading to cardiomyopathy where liver transplant is concurrently performed to remove the source of abnormal protein
  3. Primary indication for a liver transplant in a patient with concurrent heart disease.


Familial amyloid polyneuropathy forms the most common indication for CHLT (29%) followed by congenital heart disease (17.5%).[3]

There are no standard protocols or guidelines for evaluation of patients undergoing CHLT. Patients are typically evaluated independently for each organ. All patients must fulfill listing criteria for both heart and liver transplants.

A team-based approach is adopted for CHLT with separate cardiac and liver teams involved during transplantation of the respective organs to minimize cold ischemia time, especially for the heart. Conventionally, heart transplants are performed on CPB, followed by discontinuation of bypass and reversal of anticoagulation for the liver transplant.[4]

Three techniques of implantation have been described, differentiated primarily by the use of bypass during reimplantation of the liver.[5] In en bloc technique, the heart and liver are connected by the IVC. The liver is kept cold while the heart is implanted first. The heart and liver are reperfused simultaneously.[6] The advantages of the en bloc technique performed on complete CPB include:

  1. Minimization of liver cold ischemia time for the liver, as only single outflow (infrahepatic cava) and portal vein anastomosis is performed before reperfusion
  2. Minimization of impact of hemodynamic and metabolic disturbances of liver reperfusion, namely hyperkalemia, metabolic acidosis, and fluid overload on the cardiac allograft
  3. Provision of optimal oxygenation to allow for cardiac and liver allografts to recover from ischemia-reperfusion injury.


These advantages outweigh the increased risk of coagulopathy and inflammatory response associated with longer CPB times.

Liver allografts are observed to be more tolerant to human leukocyte antigen (HLA) mismatch and immune injury. The liver allograft also confers some degree of immune protection in combined organ transplants.[7] Proposed mechanisms include phagocytosis of antibodies by Kupffer cells, shedding of HLA antigens, and dilution of antibody concentration by a large vascular bed.[5]

Patients undergoing CHLT usually do not receive induction immunosuppression. A steroid bolus is given prior to reperfusion followed by a taper. Maintenance therapy includes calcineurin inhibitors and MMF. Lower immunosuppression dose requirements and fewer episodes of cellular rejection of cardiac allografts after CHLT when compared with isolated heart transplants have been observed and reported.[8]

Graft survival after CHLT is similar to that of isolated liver or heart transplantation exceeding 80% at 1 year and 70% at 10 years.[3],[8]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Starzl TE, Bilheimer DW, Bahnson HT, Shaw BW Jr., Hardesty RL, Griffith BP, et al. Heart-liver transplantation in a patient with familial hypercholesterolaemia. Lancet 1984;1:1382-3.  Back to cited text no. 1
    
2.
Zhao K, Mclean RC, Hoteit MA, Olthoff KM. Combined heart and liver transplant: Indication, patient selection, and allocation policy. Clin Liver Dis (Hoboken) 2019;13:170-5.  Back to cited text no. 2
    
3.
Barshes NR, Udell IW, Joyce DL, Southard RE, O'Mahony CA, Goss JA. A pooled analysis of posttransplant survival following combined heart-liver transplantation. Transplantation 2007;83:95-8.  Back to cited text no. 3
    
4.
Beal EW, Mumtaz K, Hayes D Jr., Whitson BA, Black SM. Combined heart-liver transplantation: Indications, oucomes and current experience. Transplant Rev (Orlando) 2016;30:261-8.  Back to cited text no. 4
    
5.
Barbara DW, Rehfeldt KH, Heimbach JK, Rosen CB, Daly RC, Findlay JY. The peri-operative management of patients undergoing combined heart-liver transplantation. Transplantation 2015;99:139-44.  Back to cited text no. 5
    
6.
Bernier PL, Grenon M, Ergina P, Schricker T, Chaudhury P, Metrakos P, et al. Combined simultaneous heart and liver transplantation with complete cardiopulmonary bypass support. Ann Thorac Surg 2007;83:1544-5.  Back to cited text no. 6
    
7.
Atluri P, Gaffey A, Howard J, Phillips E, Goldstone AB, Hornsby N, et al. Combined heart and liver transplantation can be safely performed with excellent short- and long-term results. Ann Thorac Surg 2014;98:858-62.  Back to cited text no. 7
    
8.
Cannon RM, Hughes MG, Jones CM, Eng M, Marvin MR. A review of the United States experience with combined heart-liver transplantation. Transpl Int 2012;25:1223-8.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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