Making Living-donor Liver Transplantation a Viable Option for Patients With Portopulmonary Hypertension

Abstract

Liver transplantation (LT) in patients with significant portopulmonary hypertension (PoPH) is associated with an increased risk of several complications, including graft failure. Graft loss is one of the major reasons. Living donor LT (LDLT) is not routinely performed in the United States in this patient population. In addition, ethical considerations often preclude donation from healthy donors in the setting of a procedure associated with an elevated risk of recipient morbidity and mortality. However, LDLT allows LT to be performed electively, using a superior graft with an improved probability of a good outcome. The key to success in managing these patients is establishing a multidisciplinary team that follows an institutional protocol with clear evaluation and management criteria. These criteria include screening and early diagnosis as well as treatment of PoPH with the goal of optimizing pulmonary arterial hemodynamics and maintaining right ventricular function. Any protocol should include admitting the patient to the hospital a day before surgery for placement of a pulmonary artery catheter to measure and derive relevant hemodynamic variables. A multidisciplinary team should determine the fitness for a transplant a after a careful review of the most up-to-date clinical information. Finally, the team prescribes and executes a plan for optimization and safe perioperative management of the patient. In this report, we discuss our approach to the perioperative management of a patient with significant PoPH who safely underwent LDLT with an excellent postoperative outcome.

Graphical abstract

FIGURE 1.

Penn Transplant Institute Guideline: portopulmonary hypertension. CO, cardiac output; LT, liver transplantation; mPAP, mean pulmonary artery pressure; PoPH, portopulmonary hypertension; PAWP pulmonary capillary wedge pressure; PVR, pulmonary vascular resistance; RHC, right heart catheterization; RV, right ventricle; sPAP, systolic pulmonary artery pressure; TTE, transesophageal echocardiography. Modified based on DuBrock.

FIGURE 2.

A–C, Transthoracic echocardiography 12 mo before liver transplantation. A moderate-to-severely dilated right ventricle with moderately decreased function, moderate-to-severe tricuspid regurgitation with pulmonary artery systolic pressure measuring 87 mm Hg. Mid-systolic notching of the right ventricular outflow track velocity time integral is present.

FIGURE 3.

Change of mean pulmonary artery pressure and pulmonary resistance over 12 mo time period. Pretransplant mPAP and PVR as obtained by serial RHC. Time 0 denotes the month of LDLT. The upward arrow signifies initiation of oral pulmonary vasomodulator therapy (macitentan and sildenafil). LDLT, living-donor liver transplantation; mPAP, mean pulmonary artery pressure; PVR, pulmonary vascular resistance; RHC, right heart catheter.

FIGURE 4.

A and B, Intraoperative transesophageal echocardiography. RV dilation with preserved systolic function and mild TR on inhaled epoprostenol. Postreperfusion, RV function was hyperdynamic on epinephrine and vasopressin (not shown). RV, right ventricle; TR, tricuspid regurgitation.

FIGURE 5.

Intraoperative hemodynamics. Data illustrating MAP and mean PA pressure throughout prehepatic (procedure start to hepatectomy), anhepatic (hepatectomy to portal reperfusion), and neohepatic (postreperfusion) phases. The time of portocaval shunt creation was estimated to be 40 min post arterial reperfusion. MAP, mean arterial pressure; mean PA, mean pulmonary artery pressure.