Patient 3
Patient 3 was a woman with double-inlet left ventricle and pulmonary atresia who underwent atriopulmonary Fontan palliation at age 23. At 47, she underwent conversion to an extracardiac conduit Fontan due to recurrent atrial arrhythmias. She remained stable until presenting at age 56 with new-onset ascites.
Evaluation for causes of ascites included (1) blood tests showing normal liver synthetic function; (2) liver biopsy showing bridging fibrosis and focal nodule formation consistent with cirrhosis, and (3) cardiac catheterization showing CI of 2.7 L/min/m2, Fontan pressure of 16 mmHg, PCWP of 12 mmHg, PVR of 1.7 iWU, and HVPG of 1 mmHg. At her initial paracentesis, the fluid was non-chylous. SAAG was 0.7, and fluid protein was 5.1 g/dL. Fluid Gram stain, cultures, and cytology were negative for infection or malignancy. Serum CRP was 16.8 mg/L.
Nine months after paracentesis, she was involved in a motor vehicle accident that resulted in hemoperitoneum. She was hospitalized for 5 days during which she underwent paracentesis on two occasions and was transfused a total of 3 units of red blood cells and 4 units of fresh frozen plasma. Although her hemoperitoneum resolved, she developed recurrent ascites. She agreed to a trial of intraperitoneal triamcinolone. After 3.4 L of serous fluid was removed by paracentesis, 150 mg of triamcinolone hexacetonide was introduced into the peritoneal space. The procedure was well tolerated. She underwent paracentesis again 3-1/2 months later but then never again accumulated significant ascites. She died 15 months after this last paracentesis from complications following surgery for epicardial pacemaker lead placement.