Transplant Medicine · Supplementary · from Transplant Medicine

Case 2: Liver Transplant for Decompensated Cirrhosis

Patient Presentation

Demographics: 51-year-old male former restaurant owner

Chief Complaint: "My belly keeps filling with fluid and I turned yellow again"

History of Present Illness: This 51-year-old male with a history of hepatitis C virus (HCV)-related cirrhosis presents with progressive jaundice, worsening ascites, and confusion over the past 2 weeks. He was diagnosed with chronic hepatitis C (genotype 1a) 12 years ago, likely acquired through IV drug use in his twenties. He achieved sustained virologic response (SVR) after a 12-week course of sofosbuvir/velpatasvir 4 years ago, but his cirrhosis had already progressed to a decompensated state by that time.

Over the past year, he has had three hospitalizations for hepatic encephalopathy and four large-volume paracenteses (8-12 liters each). His most recent MELD-Na score has risen from 22 to 28 over the past 3 months. He developed his first episode of spontaneous bacterial peritonitis (SBP) 2 months ago, treated with IV ceftriaxone, and has been on secondary prophylaxis with ciprofloxacin. He has been evaluated and listed for deceased donor liver transplantation. He presents today with worsening symptoms concerning for recurrent encephalopathy and possible recurrent SBP.

His wife reports increasing confusion over the past 5 days. He has been sleeping during the day and awake at night, has difficulty recognizing family members, and had an episode of fecal incontinence yesterday. She also noticed progressive jaundice and increasing abdominal girth despite compliance with diuretics and sodium restriction.

Past Medical History:

• HCV-related cirrhosis (Child-Pugh C12, MELD-Na 28), SVR achieved 4 years ago
• Recurrent hepatic encephalopathy (West Haven Grade II-III)
• Refractory ascites
• Prior SBP (2 months ago)
• Esophageal varices (Grade II, banded x2)
• Portal hypertensive gastropathy
• Hepatorenal syndrome type 2 (baseline creatinine 1.8 mg/dL)
• History of IV drug use (heroin, age 18-25), in recovery for 26 years
• Remote history of polysubstance use, no relapse
• Hepatocellular carcinoma screening: No HCC detected

Medications:

• Lactulose 30 mL four times daily (titrated to 3-4 bowel movements/day)
• Rifaximin 550 mg twice daily
• Spironolactone 200 mg daily
• Furosemide 80 mg daily
• Ciprofloxacin 500 mg daily (SBP prophylaxis)
• Nadolol 20 mg daily
• Zinc sulfate 220 mg daily
• Midodrine 10 mg three times daily
• Albumin 25 g IV twice weekly

Social History:

• Former restaurant owner, now unable to work (on disability)
• Remote history of IV heroin use (ages 18-25), 26 years of sustained recovery
• Attends NA meetings monthly
• No alcohol use for 28 years
• Non-smoker for 20 years (10 pack-years prior)
• Married with two adult children (ages 22 and 24)
• Strong family support system
• Social work and psychiatry clearance for transplant listing completed
• Health care proxy designated to wife

Family History:

• Father died of alcoholic cirrhosis at age 58
• Mother alive with type 2 diabetes and hypertension
• No family history of liver cancer

Physical Examination

• Vital Signs: BP 98/56 mmHg, HR 92 bpm, RR 18/min, Temp 38.2°C (100.8°F), SpO2 94% on room air, BMI 26.8 (with ascites), Weight 84 kg
• General: Cachectic male with massive ascites and jaundice. Somnolent but arousable. Oriented to person only
• HEENT: Deeply icteric sclerae and skin. Fetor hepaticus on breath. Temporal muscle wasting. Dry mucous membranes
• Neck: No JVD. No lymphadenopathy
• Cardiac: Tachycardic, regular rhythm. Grade II/VI systolic flow murmur. Hyperdynamic circulation
• Lungs: Decreased breath sounds at bilateral bases with dullness to percussion (bilateral pleural effusions, right > left). No crackles
• Abdomen: Massively distended with tense ascites. Fluid wave positive. Caput medusae. Umbilical hernia with 4 cm defect, reducible. Liver and spleen not palpable due to ascites. Mild diffuse tenderness without rebound
• Extremities: 3+ bilateral pitting edema to the thighs. Marked muscle wasting in extremities. Terry's nails. Palmar erythema. Dupuytren's contractures bilaterally
• Skin: Jaundice. Multiple spider angiomata (>10) on face, chest, and upper arms. Excoriations from pruritus. Multiple ecchymoses
• Neurologic: West Haven Grade III hepatic encephalopathy. Somnolent but arousable. Disoriented to time and place. Asterixis present bilaterally. Hyperreflexia in lower extremities. No focal motor deficits

Workup and Results

Laboratory Studies:

Imaging/Additional Studies:

• CT abdomen with contrast (triple phase): Cirrhotic liver with nodular contour, caudate lobe hypertrophy. Massive ascites. Splenomegaly (19 cm). Patent portal vein with hepatopetal flow. No hepatocellular carcinoma (no arterially enhancing lesions). Bilateral pleural effusions
• Doppler ultrasound: Patent portal vein (flow velocity 12 cm/s), patent hepatic artery, patent hepatic veins. No portal vein thrombosis
• Chest X-ray: Moderate bilateral pleural effusions (right > left). No pneumonia
• Echocardiogram: LVEF 65%, no pulmonary hypertension (RVSP 30 mmHg), no significant valvular disease, no diastolic dysfunction — cardiac clearance for transplant
• Right heart catheterization: Mean PAP 18 mmHg, PCWP 10 mmHg, cardiac output 8.2 L/min (hyperdynamic). No portopulmonary hypertension

Clinical Image

Diagram illustrating the MELD score components, comparison of cirrhotic and normal liver, and orthotopic liver transplant surgical anatomy with vascular and biliary anastomoses. Source: Educational illustration.

Diagnosis

Decompensated HCV-Related Cirrhosis (Child-Pugh C12, MELD-Na 34) with Recurrent Spontaneous Bacterial Peritonitis, Grade III Hepatic Encephalopathy, Refractory Ascites, and Hepatorenal Syndrome Type 2 — Awaiting Liver Transplantation

Key Diagnostic Criteria:

• Biopsy-proven HCV cirrhosis with SVR but persistent decompensation
• MELD-Na 34 with rising trend (from 22 to 34 over 3 months)
• Ascitic fluid PMN count >250 cells/μL confirming SBP
• West Haven Grade III encephalopathy with asterixis
• Hepatorenal syndrome evidenced by rising creatinine in the setting of cirrhosis
• Completed transplant evaluation with cardiac and psychosocial clearance

Treatment Plan

1. Acute management:

• SBP treatment: IV ceftriaxone 2 g daily (change based on culture sensitivities) plus IV albumin 1.5 g/kg on day 1 and 1 g/kg on day 3 (to prevent hepatorenal syndrome progression)
• Hepatic encephalopathy: Increase lactulose to achieve 4-5 bowel movements/day, continue rifaximin, correct precipitating factors (SBP), check and replace zinc
• Hepatorenal syndrome: IV albumin infusion, midodrine 15 mg TID and octreotide 200 mcg TID (consider terlipressin if available), hold diuretics
• ICU admission for close monitoring given Grade III encephalopathy

1. Transplant status upgrade: Contact transplant coordinator to update MELD-Na score (now 34) for priority allocation. Apply for MELD exception points if criteria met. Contact UNOS regarding Status 1A consideration if further deterioration
2. Bridge to transplant: Optimize nutritional status (nocturnal oral nutritional supplement, high-protein diet unless Grade III-IV encephalopathy, enteral feeding if unable to eat). Continue SBP prophylaxis after treatment completion. Monitor for hepatocellular carcinoma (AFP and ultrasound every 6 months)
3. Liver transplant surgery (when organ available): Standard orthotopic liver transplantation with piggyback technique (recipient hepatectomy preserving IVC, donor hepatic veins anastomosed to recipient hepatic vein confluence). Portal vein, hepatic artery, and bile duct anastomoses. Duct-to-duct biliary anastomosis preferred
4. Post-transplant immunosuppression: Induction with methylprednisolone, maintenance with tacrolimus (target trough 8-12 ng/mL initially), mycophenolate mofetil, and prednisone taper. Adjust based on renal function (consider delayed tacrolimus introduction if hepatorenal syndrome persists post-transplant)
5. Post-transplant monitoring: HCV recurrence is not expected given SVR, but monitor HCV RNA at 3, 6, and 12 months. Screen for de novo or recurrent malignancy. Monitor metabolic complications (new-onset diabetes after transplant, hyperlipidemia, hypertension, obesity)

Key Learning Points

• The MELD-Na score (incorporating bilirubin, INR, creatinine, and sodium) is the primary allocation tool for deceased donor liver transplantation in the United States; higher scores reflect greater disease severity and shorter predicted survival without transplant
• Spontaneous bacterial peritonitis is diagnosed by ascitic fluid PMN count greater than or equal to 250 cells/μL; IV albumin administration with antibiotics reduces the incidence of hepatorenal syndrome and mortality
• Achieving HCV SVR before transplant eliminates the risk of post-transplant HCV recurrence, which historically was universal and accelerated by immunosuppression, leading to graft loss in 20-30% within 5 years
• Hepatorenal syndrome type 2 is characterized by slowly progressive renal impairment and is associated with refractory ascites; it can progress to type 1 (rapidly progressive) with precipitating events like SBP; liver transplantation is the definitive treatment
• Right heart catheterization is mandatory in the transplant evaluation to exclude portopulmonary hypertension (mean PAP >25 mmHg with PVR >240 dynes) and hepatopulmonary syndrome, which are contraindications or require specific management before transplantation

All cases for this lecture as Markdown