Abstract Introduction Carcinoid syndrome- a rare manifestation of neuroendocrine tumor in itself- causes uncontrolled release of serotonin and vasoactive mediators, resulting in multisystem disease. We discuss a case of refractory carcinoid syndrome despite all aggressive measures, resulting in torrential valvular disease and catastrophic outcomes. Case Presentation A 21-year-old male with valvular heart disease, severe tricuspid regurgitation (TR), had a chronic waxing and waning rash over his face, arms, trunk, and extremities. Right heart catheterization confirmed mild pulmonary hypertension with normal pulmonary capillary wedge pressure and cardiac output. A liver biopsy of incidental hepatic lesions and mesenteric lymphadenopathy confirmed a Grade 1 well-differentiated neuroendocrine tumor, consistent with carcinoid syndrome. Subcutaneous octreotide was initiated. He consequently had multiple admissions for recurrent massive pleural effusions, right heart failure, and ascites. He required repeated thoracentesis showing transudative effusions, which were consistent with hepatic hydrothorax. Bilateral VATS with pleurodeses were performed; pleural biopsies were negative for malignancy. The patient had multiple carcinoid crises during his clinical course, either post-procedural or during episodes of acute heart failure. During these crises, he required vasopressor support, in conjunction with steroids, antihistamines, and high-dose octreotide, with difficulty in titrating due to labile blood pressures. The patient also experienced sudden, unexplained episodes of acute hypercapnic respiratory failure and encephalopathy requiring intubation and mechanical ventilation. His mechanical ventilation was extremely challenging due to his torrential TR causing auto-triggering of the ventilator and restrictive pulmonary physiology from his bilateral pleurodesis. The severity of these episodes and ongoing carcinoid crises precluded him from surgical repair of TR. He was deemed to be a poor surgical candidate and was evaluated for EVOQUE and TricValve percutaneous tricuspid replacements. He was determined to be ineligible for both, due to extensive carcinoid valvulopathy. He palliatively underwent off-label transfemoral valve-in-IVC procedure using a 29 mm Edwards Sapien valve with Z-stent support. Unfortunately, he suffered another carcinoid crisis with refractory shock and hypercapnic respiratory failure, following which the family decided to transition to comfort care, ultimately resulting in his death. Discussion The complex interplay of metastatic carcinoid disease with severe right-sided valvular disease, refractory carcinoid crises, and post-procedural complications, including unexplained episodes of hypercapnia, made management of this case arduous. Despite multimodal treatment including advanced valve therapies, high doses of octreotide, and extensive ICU interventions, uncontrolled carcinoid activity precluded definitive surgical repair, culminating in a fatal outcome. This abstract is funded by: None
Khanal et al. (Fri,) studied this question.