77. Case Report: Carcinoid Heart Disease with Severe Tricuspid Regurgitation – Boston University Medical Center

CardioNerds (Amit Goyal & Daniel Ambinder) join Boston University cardiology fellows (Yuliya Mints, Anshul Srivastava, and Michel Ibrahim) for some hotdogs at Fenway Park in Boston, MA. They discuss an educational case of carcinoid heart disease with severe tricuspid regurgitation. Program director, Dr. Omar Siddiqi provides the E-CPR and APD Dr. Katy Bockstall provides a message for applicants. Episode notes were developed by Johns Hopkins internal medicine resident Bibin Varghese with mentorship from University of Maryland cardiology fellow Karan Desai. Jump to: Patient summary - Case media - Case teaching - References Episode graphic by Dr. Carine Hamo The CardioNerds Cardiology Case Reports series shines light on the hidden curriculum of medical storytelling. We learn together while discussing fascinating cases in this fun, engaging, and educational format. Each episode ends with an “Expert CardioNerd Perspectives & Review” (E-CPR) for a nuanced teaching from a content expert. We truly believe that hearing about a patient is the singular theme that unifies everyone at every level, from the student to the professor emeritus. We are teaming up with the ACC FIT Section to use the #CNCR episodes to showcase CV education across the country in the era of virtual recruitment. As part of the recruitment series, each episode features fellows from a given program discussing and teaching about an interesting case as well as sharing what makes their hearts flutter about their fellowship training. The case discussion is followed by both an E-CPR segment and a message from the program director. CardioNerds Case Reports PageCardioNerds Episode PageCardioNerds AcademySubscribe to our newsletter- The HeartbeatSupport our educational mission by becoming a Patron!Cardiology Programs Twitter Group created by Dr. Nosheen Reza Patient Summary A woman in her mid 60s with history of neuroendocrine tumor (NET) presented to the cardio-oncology clinic with chronic progressive SOB and fatigue. She was diagnosed with NET after presenting with a small bowel obstruction (SBO) several years prior. At the time, she was found to have liver and pulmonary metastasis with MR enterography showing thickening of the terminal ileum. Ileocecetomy and biopsy of the liver lesions confirmed metastatic NET. Despite treatment with octreotide and everolimus, follow up CT showed progression of liver lesions and she was eventually started on telotristat and enrolled in a clinical trial. On presentation, she was not tachycardiac, hypotensive or requiring oxygen supplementation (KD: Correct?). On exam, she demonstrated elevated JVP with a positive hepato-jugular reflex and a 3/6 holosytolic murmur loudest at the LLSB that increased with inspiration. Lab work revealed urinary 5-HIAA was 212 (nl 300 mmol/24 h conferred a 2- to 3-fold increased risk for developing or progression of carcinoid heart disease TTE is the imaging modality of choice for patients with signs and/or symptoms of carcinoid heart disease, in patients with elevated NT-proBNP , and any patient undergoing surgical liver or abdominal intervention. The findings of carcinoid heart disease are on a spectrum, but there are some characteristic findings as outlined above. TEE can be an additional test to fully characterize valvular involvement and/or for surgical planning. Furthermore, cardiac CT and CMR may be valuable as adjuncts 4. How do you manage carcinoid heart disease? The only definitive and effective therapy for carcinoid heart disease is valve intervention. Diuretics and aldosterone antagonists can be helpful to relieve symptoms, but typically only have temporary effectiveness. Telotristat ethyl, an oral tryptophan hydroxylase inhibitor used in combination with a somatostatin analog for management of diarrhea associated with carcinoid syndrome, has been used to try to prevent the development and progression of carcinoid heart disease. Surgical valve intervention should be considered in patients with severe valvular disease and/or signs of right heart failure, with at least 12 months of anticipated post-operative survival fromt heir NET disease. Symptomatic management primarily involves loops diuretics and aldosterone antagonists for relieving symptoms associated with RHF. Digoxin, vasodilators, and ACEi have no proven efficacy in this population. Bioprosthetic valves may be preferred over mechanical valves due to the inherent increased risk of bleeding in patients with advanced liver disease and hepatic dysfunction from carcinoid disease. However, bioprosthetic valves may be more prone to premature dysfunction and degeneration due to the underlying carcinoid process and thrombosis formation. A careful multi-disciplinary team and approach is needed to individualize valve choice for each patient. Transcatheter valve replacement has been undertaken for pulmonic valve involvement, but transcatheter tricuspid valve replacement is not common. 5. What is the overall prognosis of patients with carcinoid heart disease with and without surgical management? Carcinoid heart disease with NYHA III or IV symptoms have a poor prognosis and median survival is only 11 months. In carcinoid patients with cardiac symptoms and controlled systemic disease,