366 RIGHT HEART FAILURE IN CARCINOID HEART DISEASE: A MULTIDISCIPLINARY APPROACH
Authors: Foti Marco , Tarantini Roberto , Acone Lorenzo , Toscano Evelina , Lanzillo Guido , Mortara Andrea 
Published: December 2022

Abstract

Introduction
Carcinoid heart disease occurs in more than 50% of patients with neuroendocrine gastrointestinal tumors, and it is the initial presentation of carcinoid syndrome in up to 20% of patients. The disease is characterized by pathognomonic plaque-like deposits of fibrous tissue in the endocardium of valvular cusps, cardiac chambers, and occasionally, the intima of the pulmonary arteries or aorta. The tricuspid and the pulmonary valve are most often affected by carcinoid disease, with several combinations of valve dysfunction (usually pulmonic stenosis + tricuspid regurgitation). Valvular dysfunction can lead to peripheral edema, ascites and right-sided heart failure, extreme cases may present with low cardiac output syndrome. Valve surgery may relieve symptoms and it should be considered in patients with controlled neoplasia.

Case report
a 47-years-old man with a history of operated neuroendocrine gastrointestinal tumor (ileal resection+right hemicolectomy+mesenteric lymphadenectomy) was admitted to our department for right heart failure. His clinical examination was remarkable for ankle swelling, flushing, liver congestion, ascites; moreover the patient reported progressive compromise of functional capacity and weight loss due to anorexia. Laboratory findings were in the normal range except for BNP 184 pg/ml (UNL &lt;100 pg/dl). ECG: sinus rhythm 80 bpm, diffuse repolarization abnomarlities. Transthoracic echocardiography showed: severe right chambers remodeling with RV+RA dilatation associated with systo-diastolic septal bulge (pressure + volume overload), torrential tricuspid regurgitation (secondary to fibrotic retraction of valve leaflets and severe coaptation deficiency with apical tethering), moderate-to-severe pulmonic regurgitation associated with valve stenosis; on the left side: mitral and aortic valves were normal, there was mild reduction of left ventricle ejection fraction secondary to ventricular interdipendence.
The patient was discussed in Heart Team: right sided valves were both considered responsible for patient's symptoms, so he was scheduled for surgical intervention after achieving adeguate haemodinamic stability. Pre-surgery right catetherization showed normal pulmonary pressures. Coronary arteries were normal. Before, during and after surgery he was treated with octreotide to reduce the risk of Carcinoid Crisis in the perioperative period. The patient underwent surgical replacement of tricuspid and pulmonary valves with biological prosthesis Mosaic n°33 and Avalus n°23 respectively. Histological examination of the valves showed extensive deposits of fibrous tissue in the valvular endocardium. Post-surgical hospital stay was uneventful, the patient was discharged 14 days after surgery on normal sinus rhythm and in good clinical conditions. In order to prevent thromboembolic events anticoagulant therapy with Warfarin was introduced.

Conclusion
this is the report of a rare cardiac condition responsible for right heart failure symptoms. Heart Team discussion and multidisciplinary approach involving surgeons and oncologist are mandatory in order to establish the best treatment strategy and timing for interventions. However, since this is a rare condition, more evidence is needed to better understand long term clinical outcomes and treatment options (including future percutaneous perspectives).