A 59-year-old Caucasian male was referred to our liver center for refractory right-sided pleural effusion and abdominal ascites. His history is significant for Scleroderma/CREST syndrome and chronic kidney disease (CKD). He had 12 paracenteses in 2015 and starting from February 2016 was undergoing thoracentesis 3 times weekly (about 8-9 liters/week) and a single weekly paracentesis up to 5 liters. Medications included Spironolactone 50 mg and Furosemide 20 mg, which were limited dosages due to CKD. In February 2016, prior to our visit, he underwent a liver transplant evaluation: Na 138 mg/dL, Cr 4.4 mg/dL, eGFR 31 mL/min, INR 1.0, Hb 13 g/dL, Platelets 342 Th/mm3, TB 0.5 g/dL, AlkPhos 278 IU/L, ALT/AST 46/40 IU/L, albumin 2.7 mg/dL, negative viral serology, ANA 1 : 320, SMA and AMA negative, C282Y/H63D, and MELD-Na score 14. Abdominal ultrasound revealed a heterogeneous liver and ascites. A thoracentesis demonstrated a SAAG 1.9 and total protein 3.3 gm/dL suggesting posthepatic portal hypertension. A right heart catheterization was notable for RA 5 mmHg, PA 31/15 mm/Hg, mean 22 mmHg, normal Echo LV function, and grade 1 diastolic dysfunction. At this juncture, the etiology of his presumed cirrhosis had not been determined and a liver biopsy with portal pressures was to be scheduled but he wanted a second opinion and presented to us in March.
The physical exam revealed a pleasant frail appearing gentleman with stable vital signs: B/P 87/58, HR 80, and BMI 24.5 kg/m2; labs demonstrated TB 0.5 md/dL, AlkPhos 462 IU/L, ALT/AST 62/65 IU/L, total protein 5.7 IU/L, albumin 3.2 mg/dL, and Cr 2.4 mg/dL (spironolactone had been discontinued one month earlier). A large right-sided-pleural effusion with moderate abdominal ascites was noted on examination. He underwent a transjugular intrahepatic portal systemic shunt study (TIPS) and liver biopsy after 100 gm of IV albumin (given for renal dysfunction) with the following results: RA 13 mmHg, FHVP 16 mmHg, WHVP 17 mmHg, and HVPG 1 mmHg (Table 1). The TIPS was aborted due to the normal sinusoidal portal pressure gradient and elevated right-sided pressures. A right heart catheterization 5 hours later revealed RA 13 mm/Hg, PA 57/30 mm/Hg, mean 39 mm/Hg, PWP 20, and C.O 5.5 L/min. Presumptive diagnosis was mild/moderate mixed arterial and venous pulmonary hypertension. The liver biopsy revealed mild sinusoidal dilatation, no inflammation or fibrosis, trace iron deposition and was considered unremarkable, other than mild outflow obstruction (Figure 1). The patient was subsequently referred for a pulmonary work-up as well as a dysphagia evaluation. Pulmonary function tests demonstrated a low DLco 9.33 L, vital capacity 2.75 L, and a repeat heart catheterization after a thoracentesis and paracentesis without albumin: RA 3 mm/Hg, PA 30/10 mm/Hg, mean 18 mm/Hg, and PWP 12 mm/Hg (Table 1). An EGD demonstrated trace esophageal varices (Figure 2). A second interpretation of the liver biopsy noted mild venous outflow obstruction and possibly Nodular Regenerative Hyperplasia (NRH, Figures 3 and 4). Taken together, in the setting of normal right heart pressures and a possible diagnosis of NRH, the patient could have portal hypertension due to a presinusoidal obstruction. He underwent a repeat shunt study this time with direct and indirect portal pressures: RA 2 mm/Hg, FHVP 4 mm/Hg, WHVP 5 mm/Hg, PVP 15 mm/Hg, and portal pressure gradient (PPG = PVP minus FHVP) 11 mm/Hg (Table 1). He successfully underwent a TIPS shunt with a post-TIPS PPG of 7 mm/Hg. Prior to TIPS, the patient underwent 50 thoracenteses and 20 paracenteses over that past year and after TIPS he had 1 thoracentesis/paracentesis 10 days after and none over the past 18 months. Other than mild hepatic encephalopathy, controlled on medication, no other adverse effects have been reported.