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.