Portal de Periódicos da CAPES

An unusual case of cardiac failure following orthotopic liver transplantation

2018; Elsevier BV; Volume: 18; Issue: 4 Linguagem: Inglês

10.1111/ajt.14704

1600-6143

Neal Bhutiani, Dylan Adamson, Mary Eng, Christopher M. Jones,

Liver Disease and Transplantation

The patient is a 55-year-old gentleman who presented with hypoxemia and chest pain 18 years after standard whole-organ orthotopic liver transplantation for primary sclerosing cholangitis. His posttransplant course had been unremarkable until presentation. He had no rejection episodes and required no instrumentation of his liver or biliary tract. Immunosuppression was tacrolimus monotherapy. Laboratory studies at presentation revealed normal liver tests, an elevated creatinine, hyperkalemia, and a supratherapeutic tacrolimus level. The patient required intubation, and systemic anticoagulation was initiated due to concern for pulmonary embolus. Transthoracic echocardiogram demonstrated elevated right ventricular volume and pressure with severe pulmonary hypertension (mean pulmonary artery pressure of 48 mmHg) and a dilated inferior vena cava. While left ventricular size and systolic function were otherwise normal, the right ventricle was moderately enlarged and demonstrated global hypokinesis. Cardiac catheterization failed to identify significant coronary artery disease. However, computed tomography of the chest, abdomen, and pelvis with IV contrast demonstrated an abnormality in the liver allograft (Figure 1) and multiple pulmonary emboli. The liver allograft was subsequently evaluated using right upper quadrant abdominal ultrasound with color Doppler, which demonstrated a large, dilated vascular structure with multiple branching vessels in the right hepatic lobe (Figure 2). The patient underwent treatment with improvement of his cardiac pathophysiology and normalization of his liver tests.FIGURE 2Color Doppler abdominal ultrasound of the right upper quadrant demonstrates abnormal communication between the right hepatic artery (thin arrow) and the right aspect of the portal vein (thick arrow). Waveform demonstrates turbulent, high-velocity (>50 cm/s) flowView Large Image Figure ViewerDownload Hi-res image Download (PPT) 1A 50-year-old gentleman who underwent orthotopic liver transplantation 10 years ago presents to the emergency department with progressively worsening dyspnea, lower extremity edema, and chest pain. He undergoes serologic evaluation for metabolic, hematologic, and infectious perturbations and computed tomography of the chest and abdomen. Which of the following tests should also be included in the patient’s initial workup?aElectrocardiography and echocardiographybComputed tomography of the headcMagnetic resonance cholangiopancreatography (MRCP)dVentilation/perfusion (V/Q) scaneNo additional testing required 2Which of the following factors, if present in the patient in Question 1, would be a known risk factor for new-onset right heart failure?aElevated preoperative right-sided heart pressurebElevated pulmonary artery wedge pressurecLower preoperative ejection fractiondHigher pretransplant Model for End-Stage Liver Disease (MELD) scoreeHistory of alcoholic cirrhosis 3After further evaluation, the systolic heart failure in this case presentation was the result of a large arterio-portal fistula. What is the most likely cause of this patient’s fistula?aBlunt liver traumabHepatic tumorcPreexisting aneurysm rupturedAutoimmune vasculitisePercutaneous liver biopsy 4Symptomatic arterio-portal fistula is associated with which of the following?aAbnormal liver testsbPulmonary hypertensioncAbdominal paindRight ventricular dysfunctioneGastrointestinal bleeding 5When cardiac evaluation fails to identify other causes contributing to heart failure in a liver transplant recipient with an arterio-portal fistula, which is the preferred primary treatment?aOpen ligation of the hepatic artery involved in the arterio-portal fistulabRetransplantationcHepatic lobectomydEndovascular embolization of the arterio-portal fistulaeHepatic segmentectomy