Patient 1
Patient 1 was a 30-year-old woman with double-outlet right ventricle and mitral atresia who underwent atriopulmonary Fontan palliation at 4 years old. At age 28, she underwent Fontan conversion for recurrent arrhythmia. Afterward, she developed ascites requiring frequent paracentesis.
Her evaluation included (1) blood tests showing normal liver synthetic function; (2) liver biopsy showing “established cirrhosis,” and (3) cardiac catheterization showing cardiac index (CI) of 4.5 L/min/m2, Fontan pressure of 14 mmHg, pulmonary capillary wedge pressure (PCWP) of 9 mmHg, pulmonary vascular resistance (PVR) of 1.3 Wood units (iWU), and a hepatic vein pressure gradient (HVPG) of 4 mmHg. At the first paracentesis, the fluid was non-chylous; SAAG was 0.8, and fluid protein was 5.3 g/dL. Fluid Gram stain, cultures, and cytology were negative for infection or malignancy. Serum C-reactive protein (CRP) was 15.8 mg/L.
Given her poor prognosis, she agreed to a trial of intraperitoneal steroids when she started requiring paracentesis more than once a month despite escalating doses of diuretic. After therapeutic paracentesis during which 5.2 L of fluid was removed, 500 mg of triamcinolone hexacetonide was introduced into the peritoneal space. The procedure was well tolerated. She did not require paracentesis again until 45 later; 6 L of fluid was removed, after which another 500 mg of triamcinolone hexacetonide was administered intraperitoneally. Again, there were no immediate complications. One week later, she experienced sudden death at home. Her family declined autopsy.