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.