Case Presentation: A 46-year-old male with a past medical history of hypertension and macular edema was referred to the Cardiology office by his primary care provider for uncontrolled hypertension and bilateral lower extremity edema. An echocardiogram was obtained to rule out structural heart disease which revealed a reduced left ventricular ejection fraction of 35-40% and moderate to severe tricuspid valve regurgitation suggestive of Epstein’s anomaly. He subsequently underwent a transesophageal echocardiogram which demonstrated tricuspid valve thickening and torrential tricuspid valve insufficiency consistent with carcinoid heart disease (CHD). A DOTA-TATE PET/CT was performed which revealed scattered somatostatin receptor positive hepatic metastases and prominent uptake in the terminal ileum. Chromogranin A level was over 1,900. A diagnosis of metastatic neuroendocrine tumor was confirmed via liver biopsy. Subsequent inquiries, following diagnosis, revealed a history of intermittent flushing, diarrhea, and mild weight loss for the past two years, previously unreported and therefore unrecognized as indicative of carcinoid syndrome. He subsequently underwent transarterial chemoembolization of the right hepatic lobe and is now undergoing monthly lanreotide treatment. He is also being considered for valve replacement surgery in the near future and will eventually require primary tumor resection.
Discussion: CHD is a rare and complex condition with a poor long-term prognosis that can be difficult to diagnose and manage. With only 12-22% of neuroendocrine tumors being metastatic at presentation, diagnosis can be even more challenging in the absence of reported carcinoid syndrome symptoms such as flushing, diarrhea, and bronchospasm (1). The delayed recognition of carcinoid syndrome in this case, identified post-imaging, challenges the conventional association of CHD solely with symptomatic carcinoid syndrome, highlighting the diagnostic challenges posed by atypical presentations. Notably, the discovery of metastatic cardiac involvement preceding symptomatology underscores the necessity for heightened clinical vigilance in evaluating cardiac abnormalities, irrespective of typical associated symptoms. Once diagnosis is confirmed, collaboration among oncology, cardiology, surgery, and interventional radiology is crucial in order to individualize treatment plans and optimize patient outcomes.
Conclusions: In conclusion, this case highlights the importance of considering CHD in patients with valvular disease in the absence of overt carcinoid syndrome, emphasizing the need for a comprehensive and multidisciplinary approach to evaluation and treatment of metastatic neuroendocrine tumors.