A 35-year-old man with hypothyroidism and primary hypothyroidism syndrome in the proximal ileum was diagnosed in 1999 (due to compromised general weight status, underweight, influx, pathological antecedents and shock).
(a) Intraplatelet serotonin: 4.658 ng/109 platelets (400-800).
b) 24 h urine indoleacetic acid → 2,064 umol (< 34).
He loses control, because he's going to live abroad.
From 2010 with progressive deterioration of general status and progressive edema to the anasarca.
Physical examination revealed no jugular engorgement.
Holosystolic murmur of tricuspid insufficiency IV/ VI.
Moderate ascites and oedema of the lower limbs
(a) ECG: right atrial reaction.
(b) surface echocardiography: LV of preserved size and function.
Atrial and RV dilatation with preserved systolic function.
tricuspid valve disease with stenosis and severe regurgitation.
Pulmonary valve disease with moderate stenosis and severe regurgitation.
No pulmonary hypertension.
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(c) TAP TAC: Hypervascular adenopathies at the root of the mesentery and focal hypervascular hepatic lesions.
(d) PET-CT: Somatostatin receptor overexpression in the proximal ileum with extensive hepatic involvement, adenopathies affecting the mesentery and retroperitoneal glands.
It requires multiple paracentesis for ascites refractory to diuretic treatment, with partial improvement.
She started treatment with monthly Sandostatin LAR®, with less erythema and commitment of the general state.
Right-sided HF, predominantly FC decreased from III to IV.
Given the persistence of symptoms despite optimal treatment, it was decided to perform tricuspid valve replacement with St Jude No 29 prosthesis + pulmonary valve replacement with St Jude No 21 HP prosthesis.
Valve biopsy: deforming fibromyxoid valvulopathy.
The patient was managed conservatively with methotrexate 25 mg/day, furosemide 20 mg/day, acenocoumarol and Sstatin LAR® monthly.
In the imaging follow-up, CT shows stability of the hepatic and mesenteric-retroperitoneal adenopathic lesions.
Echocardiogram: RV with slightly decreased systolic function.
Mechanical prosthesis in tricuspid and pulmonary normofunctioning position.
Currently, asymptomatic cardiovascular with isolated episodes of "flux".
Intraplatelet serotonin: 3,072 ng/109 platelets.
