Endocrine Abstracts

Introduction: Carcinoid heart disease can occur in up to 20 % of patients with carcinoid syndrome. It is a regarded as a rare complication and associated with high morbidity and mortality as a sequela of vasoactive peptides resulting in plaque formation on the valve leaflets classically tricuspid and pulmonary valves and causing right heart failure. Definitive treatment is surgical valve replacement, but percutaneous valve management is an attractive alternative for those deemed unsuitable for surgery.

Case: We describe a 56 year old lady, presented with diarrhea and flushing in 2015 diagnosed with NET of mid gut with multiple metastasis. Investigations revealed elevated Chromogranin A level 283 mg/ml and Urinary 5 HIAA 1321 mg/24 hours (5 hydroxy indole acetic acid). Her octreotide scan with SPECT revealed right iliac fossa lesion with multiple liver metastasis, metastasis in abdominal lymph node, left femur, right pleura, left breast and bilateral orbits. She was treated with 4 cycles of Lutathera in 2016 and 2 cycles in 2020. She has developed carcinoid crisis in 2019. She was treated with Lanreotide autogel 120 mg deep sc every 2 weeks with rescue subcutaneous octreotide top-ups and denosumab for bone metastasis. Orbital metastasis treated with steroid injections in 2021. She has developed shortness of breath and bilateral ankle edema in 2021 and diagnosed with severe carcinoid heart disease. Her 2D-ECHO and CT cardiac angiogram revealed thickened right heart valves with pulmonary more significantly involved. She underwent percutaneous intervention of pulmonary valve in 2022 and currently her right heart failure is improved.

Conclusions: Hedinger syndrome is a challenging entity in cardiac surgery due to right sided valvular lesions and risk of open surgery. Percutaneous valve intervention or transcatheter valve repair of tricuspid and pulmonary valve has become a suitable entity over invasive surgical intervention in non-surgical candidates in recent years.