ON THE CUSP-ID OF VALVULAR DISEASE: CARCINOID'S UNEXPECTED ROLE

ABSTRACT

Background: Primary causes of tricuspid regurgitation (TR) account for 8-10% of cases, whereas secondary causes account for >90%. Given this disparity, there is paucity of data to help guide treatment. Case A 55-year-old man presented with DOE, fatigue, and diarrhea. He initially presented to urgent care to be tested for COVID-19, however, was found to have a pulsatile neck and was sent to the emergency department. Further history significant for lethargy, bilateral lower extremity swelling, and PND. On presentation, he was normotensive and tachycardic to 110 bpm. Pertinent physical exam findings included facial erythema, severe jugular venous distention with prominent C-V waves, a holosystolic murmur without radiation, and 2+ lower extremity pitting edema. Pertinent laboratory studies include NT-Pro-BNP of 802 pg/mL (reference range, 15 - 125 pg/mL). Infectious workup was positive for SARS-CoV-2. Transthoracic echocardiogram (TTE) demonstrated preserved ejection fraction of 55-60%, dilation of the right atrium and ventricle with normal function, and a small pericardial effusion. Evaluation of the tricuspid valve showed wide-open regurgitation with thickened and restricted leaflets. Decision-making Given concern for carcinoid valvular disease, oncologic workup was performed that revealed a serum serotonin of 1493 ng/mL (reference range, 21-321 ng/mL), 24-hour urine serotonin of >300 mg/24 hr (reference range, 0.0-14.9 mg/24 hr), and chromogranin A of 806.7 ng/mL (reference range, 0.0-101.8 ng/mL). PET/CT demonstrated Dotatate uptake within the liver and mesenteric lymph nodes. Percutaneous liver biopsy confirmed metastatic well-differentiated neuroendocrine tumor, grade 1. He was started on octreotide and furosemide for symptomatic management. Currently, he is pending endoscopic tricuspid valve replacement which will be guided by decreased tumor progression. Conclusion: Carcinoid heart disease, while rare, represents an important etiology of valvular dysfunction. Despite a well-recognized clinical entity, establishing a diagnosis and making an individualized treatment plan remains a significant challenge utilizing several subspecialties.