The emergency department physician pages you to ask you to evaluate a 17-year-old girl who has abdominal pain and swelling. She has a complex cardiac history including situs inversus with isolated levocardia, transposition of the great arteries, pulmonary atresia, large ventricular septal defect, and right aortic arch with mirror image branching.
The Case
The emergency department (ED) physician pages you to ask you to evaluate a 17-year-old girl who has abdominal pain and swelling. She was asked to come to the ED after seeing her cardiologist, who was concerned that she had ascites on her exam. She has a complex cardiac history including situs inversus with isolated levocardia, transposition of the great arteries, pulmonary atresia, large ventricular septal defect, and right aortic arch with mirror image branching. She underwent a modified Fontan procedure approximately 10 years ago and has had an unremarkable cardiac course since that time. You are now worried about this new abdominal pain and swelling.
The abdominal pain has been bothering her for the last 2 months. The pain is in this patient's lower abdomen but also in her left upper quadrant. It occurs almost daily and is worse at night. The severity is 7 out of 10 on the numeric pain-intensity scale. The pain is worse with prolonged walking or lying briefly on her left side.
She reports a 15-pound weight loss since the beginning of her illness, some of which she has regained over the last month. The only medication she takes is a baby aspirin daily.
On review of systems, she reports intermittent headache and cough over the last 2 months. She has had menses up to 2 times per month since her symptoms started. Before that, her menses occurred every 4 weeks and lasted about 4 days.
She is currently sexually active. She has had 3 male partners and reports that she uses condoms. She has never been pregnant. There is no family history of malignancy, liver disease, or congenital heart disease. Also of note, she reports being exposed to a classmate at school with confirmed tuberculosis.
She was seen at an outside hospital several weeks ago for abdominal pain. A computed tomography (CT) scan was reassuring, with the exception of mild pelvic ascites. She was discharged from the ED with instructions to follow-up with her pediatrician.
She saw her cardiologist earlier today for a routine visit. Her exam was concerning for abdominal distension. She was sent for labs and imaging that showed elevated inflammatory markers and ascites.
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