A 33-year-old man with no morbid history.
The patient had progressive fatigue for 4 years and weight loss.
Occasional appearance of "flux" with alcohol, abdominal colic and semi-formed stools.
In addition, progressive dyspnea on minimal exertion and lower limb edema were observed.
Physical features: Jaundice engorged at 45o, tricuspid insufficiency murmur III/VI.
Indurated palpable liver 4 cm below costal margin, lower limbs with bilateral oedema.
(a) ECG sinus rhythm and left anterior hemiblock.
(b) thoracic-abdominal and pelvic CT (TAP): hypervascular lesion measuring 3.6 x 2.8 cm in the tail of the pancreas associated with multiple secondary hepatic locations.
(d) Indoleacetic acid in 24 h urine → 1,687 umol (< 34).
Intraplatelet serotonin → 3,886 ng/109 platelets (400-800).
Percutaneous puncture of focal hepatic lesion under TAC, with biopsy compatible with tumor.
(e) PET-CT: compatible with a pancreatic tail tumor, peritoneal nodules and multiple liver implants, consistent with secondary involvement.
(f) transesophageal echocardiography: left ventricle (LV) with normal size and function.
Left atrial fibrillation.
Right ventricle (RV) relaxed with normal systolic function.
Right atrium.
Thickened and rigid tricuspid valve, with insufficiency and severe stenosis.
Pulmonary valve regurgitation and severe stenosis.
Normal functioning aortic and mitral valves.
No pulmonary hypertension.
1.
She started treatment with monthly Sandostatin LAR® and was referred to cardiosurgery, performing tricuspid replacement with prosthesis Saint Jude No 27 and pulmonary valve replacement with prosthesis Saint Jude HP No 19.
Valve biopsy: extensive fibromyxoid degeneration.
1.
The patient was treated with furosemide 20 mg/day, isoniazid 25 mg/day, statins LAR® monthly and acenocoumarol.
At 3 years of follow-up she remains with FC I.
Intraplatelet serotonin was 4.306 ng/109 platelets.
Imaging study showed pancreatic tumor stability and secondary locations.
Echocardiograms showed normal functioning prostheses, with persistent dilated RV with decreased systolic function.
