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.