Chapter 78: Combined Heart–Liver Transplantation

BACKGROUND

• This is a rare and lifesaving surgical procedure.

• The first combined heart–liver transplantation (CHLT) case in the United States was performed in 1984 in a 6-year-old female with familial hypercholesterolemia and heart failure secondary to coronary artery disease.¹

• The Organ Procurement and Transplantation Network (OPTN) reported 231 CHLT procedures in the United States between January 1, 1988, and October 31, 2017.

• As noted in Fig. 78-1, the number of CHLT has been increasing over the past decades

INDICATIONS

• Familial amyloidosis polyneuropathy (FAP):^3,4

  • A progressive and disabling condition due to the increased production of transthyretin in the liver that leads to the abnormal accumulation in the peripheral nervous system and end organs such as the heart

  • Most common indication of CHLT to prevent the accumulation in the cardiac graft (26.8% from a United Network for Organ Sharing [UNOS] database of CHLT between 1987 and 2010)⁵

• Heart failure with associated hepatic cirrhosis^5,6

• Other

  • Late-stage congenital heart disease status–post previous repair

    • For example, polysplenia and dextrocardia with situs ambiguus^7,8

  • Familial hypercholesterolemia

• Hereditary hemochromatosis^9,10

MANAGEMENT

• There are no current guidelines regarding pretransplant workup in patients requiring CHLT^11,12

• Waitlist is reduced in CHLT compared to orthotopic heart transplant (OHT) and orthotopic liver transplant (OLT)¹³

• Mortality on the waiting list for CHLT is higher compared to single-organ transplant candidates

• The heart is usually implanted first (as it is less tolerant of ischemia) followed by the liver

• Techniques reported:

  • OHT and OLT on full cardiopulmonary bypass (CPB)

  • OHT on CPB and OLT on venovenous bypass (VVB)

  • OHT on CPB and OLT en bloc technique¹⁴

  • Sequential transplantation^4,15

• The risks and benefits of using CPB during the entire procedure should be addressed in a patient-dependent manner. (See Table 78-1.)

OUTCOMES

• CHLT is a safe procedure with survival rates similar to single-organ recipients⁶ (Table 78-2)

• Rates of acute rejection were found to be lower in patients receiving CHLT than in single-organ recipients⁵

  • Acute liver rejection in CHLT= 5.2% versus liver transplant alone = 12.2% (p = 0.060)

  • Acute cardiac rejection in CHLT= 8.9% versus cardiac transplant alone = 23.9% (p = 0.060)

• Immunosuppression ...