Patient 2
Patient 2 was a 34-year-old woman with mitral atresia and pulmonary atresia who underwent atriopulmonary Fontan palliation at age 4. At 17 years old, she underwent conversion to a lateral tunnel Fontan due to recurrent atrial arrhythmias that persisted despite radiofrequency ablation.
She first developed ascites at age 22. At paracentesis, the SAAG was 0.7, and fluid protein was 6.2 g/dL. She had a markedly elevated serum CRP of 169.6 mg/L. Evaluation by rheumatology was inconclusive, but given her significant illness, she was treated with prednisone and methotrexate. After 2 years without ascites, the prednisone was weaned off. Methotrexate was stopped another 3 years later, but her ascites recurred shortly afterward.
Evaluation for causes of ascites included (1) blood tests showing normal liver synthetic function; (2) abdominal CT showing a cirrhotic-appearing liver without discrete lesions; (3) esophagogastroduodenoscopy showing no varices; (4) liver biopsy showing hepatic fibrosis and regenerative hepatocellular changes; (5) peritoneal biopsy showing mesothelium-lined soft tissue without significant pathologic change; and (6) cardiac catheterization showing CI of 1.9 L/min/m2, Fontan pressure of 8 mmHg, PCWP of 4 mmHg, PVR of 1.3 iWU, and a HVPG of 3 mmHg. Within 1 year, she was requiring paracentesis every 2-3 weeks, each time with a low SAAG. Fluid triglycerides were 50 mg/dL, and Gram stain, cultures, and cytology were negative. Her CRP was 173.0 mg/L. Based on these findings and on the temporal association of ascites with discontinuation of methotrexate, it was felt that an anti-inflammatory agent would be appropriate. Given concerns about toxicity related to long-term use of these agents, we decided to try intraperitoneal steroids.
After removal of 5 L of fluid by paracentesis, 500 mg of triamcinolone hexacetonide was introduced into the peritoneal space. She experienced post-procedural hyperglycemia that persisted for one week; the procedure was otherwise well tolerated. After 3 weeks, she was readmitted for ascites and underwent a second paracentesis with removal of 4.3 L of fluid followed again by administration of 500 mg of triamcinolone hexacetonide into the peritoneal space. Again, she experienced post-procedural hyperglycemia that resolved after a few days.
She did not require paracentesis again until 20 weeks after the second administration of intraperitoneal triamcinolone. By 8-1/2 months, she began presenting frequently for paracentesis. After the sixth paracentesis in a 3-month period, she again received 500 mg of intraperitoneal triamcinolone hexacetonide after removal of 4.5 L of ascites. Although she did not experience hyperglycemia this time, adrenocorticotropic hormone stimulation testing 2 weeks later showed evidence of adrenal insufficiency. She was started on low-dose hydrocortisone. She required paracentesis two more times over the next 6 weeks but then did not need paracentesis again until 45 weeks later. During this time, her hydrocortisone was weaned without any adverse effects. Although her ascites became intractable, no more intraperitoneal triamcinolone was given due to the previous adverse effects; she underwent paracentesis 43 more times over the next 3 years, including 23 times in her final year. She ultimately developed Candida peritonitis and died within 2 weeks of diagnosis.