NET-Induced Carcinoid Heart Disease Affecting Both Tricuspid and Aortic Valves Due to Patent Foramen Ovale and Right/Left Shunt: A Multi-imaging Challenge to Nuclear Medicine.

ABSTRACT: Carcinoid heart disease (Hedinger syndrome) is a long-term consequence in hormone-active neuroendocrine tumors with hepatic metastases and carcinoid syndrome. Because of serotonin, excess multiple cardiac and pulmonary symptoms evolve, which are further complicated by a patent foramen ovale due to right-left shunting. We present a 53-year-old man with an ileum-neuroendocrine tumor including gross liver metastases and long-term stable disease who subsequently developed Hedinger syndrome. Initially experiencing progressive dyspnea, he eventually experienced severe hypoxemia due to patent foramen ovale. 99mTc-MAA lung perfusion scintigraphy quantitatively identified the right-left shunting, whereas 68Ga-FAPI-46 PET/CT characterized the typical fibrous heart valve thickening due to serotonin-induced fibroblast proliferative properties.

Estenosis Tricuspídea Severa secundaria a enfermedad carcinoide cardiaca: presentación de caso clínico y revisión de la literatura / Severe Tricuspid Stenosis Secondary to Cardiac Carcinoid Disease: Case Report and Literature Review

RESUMEN: La estenosis tricuspídea (ET) es una valvulopatía infrecuente cuyas principales etiologías son la enfermedad reumática y la endocarditis infecciosa. En raras ocasiones puede deberse a un fenómeno carcinoide subyacente, en lo que se conoce como la enfermedad carcinoide cardiaca (ECC). Esta condición lleva a la fibrosis del endocardio del ventriculo derecho, principalmente de sus válvulas, lo cual puede provocar falla cardiaca derecha, complicando el pronóstico. En este artículo se presenta un caso de una ET severa por una posible ECC, en conjunto con las imagenes ecocardiográficas obtenidas durante el abordaje diagnóstico (imágenes bidimensionales, imagen multiplanar y ecocardiografía en 3D). Se discuten las implicaciones clínicas, los retos diagnósticos, las opciones terapeuticas y el pronóstico de esta rara entidad.

ABSTRACT Severe Tricuspid Stenosis Secondary to Cardiac Carcinoid Disease: Case Report and Literature Review Tricuspid stenosis is an unfrequent valvulopathy that can be caused by multiple etiologies, including rheumatic disease and infectious endocarditis. In rare occasions, it occurs in the context of a carcinoid syndrome, in what is known as carcinoid heart disease. This condition causes fibrosis of the valves and the endocardium of the right ventricule, which can progress into right ventricular failure, worsening the patient's prognosis. In this article, we present a case of a severe tricuspid stenosis in which this ethiology is suspected. We show the echocardiographic images obtained for the diagnosis (two-dimensional imaging, multimodal imaging and 3D echocardiography), and we discuss the clinical and diagnostic implications, therapeutic options and prognosis of this rare condition.

Carcinoid syndrome: update on the pathophysiology and treatment

Approximately 30-40% of patients with well-differentiated neuroendocrine tumors present with carcinoid syndrome, which is a paraneoplastic syndrome associated with the secretion of several humoral factors. Carcinoid syndrome significantly and negatively affects patients' quality of life; increases costs compared with the costs of nonfunctioning neuroendocrine tumors; and results in changes in patients' lifestyle, such as diet, work, physical activity and social life. For several decades, patients with neuroendocrine tumors and carcinoid syndrome have been treated with somatostatin analogues as the first-line treatment. While these agents provide significant relief from carcinoid syndrome symptoms, there is inevitable clinical progression, and new therapeutic interventions are needed. More than 40 substances have been identified as being potentially related to carcinoid syndrome; however, their individual contributions in triggering different carcinoid symptoms or complications, such as carcinoid heart disease, remain unclear. These substances include serotonin (5-HT), which appears to be the primary marker associated with the syndrome, as well as histamine, kallikrein, prostaglandins, and tachykinins. Given the complexity involving the origin, diagnosis and management of patients with carcinoid syndrome, we have undertaken a comprehensive review to update information about the pathophysiology, diagnostic tools and treatment sequence of this syndrome, which currently comprises a multidisciplinary approach.

Quadruple valve replacement for carcinoid heart disease

Abstract Introduction: Carcinoid heart disease most frequently involves the tricuspid or, more rarely, the pulmonary valve and presents with right heart failure as 5-HT is metabolized by the lung. Left-sided valve involvement is quite rare. We describe our experience of 3 patients presenting with heart failure secondary to carcinoid heart disease affecting all four cardiac valves. There are only four previous isolated case reports in the literature. Methods: All three patients underwent quadruple valve replacement during a single operation. Right ventricular outflow tract reconstruction with a pericardial patch was performed in all patients. For 24 hours prior to surgery, all patients received intravenous octreotide, which continued in intensive care for at least 24 hours. Results: Mean cross-clamp and bypass times were 175 (range 164-197 minutes) and 210 (range 195-229 minutes) minutes, respectively. Mean intensive treatment unit (ITU) and inpatient stays were 2.3 (range 2-3 days) and 12 (range 9-16 days) days, respectively. One patient was reopened for bleeding 4 hours postoperatively from a ventricular pacing wire site. None required a permanent pacemaker postoperatively. There were no other complications in any patient. The quality of life was excellent at 6-16 months clinic follow-up as they were in NYHA 1. Postoperative echocardiography showed no paravalvular leaks and well-functioning prostheses in all cases. Conclusion: Surgery to replace all four valves is feasible with excellent medium-term survival and a very low rate of complications. Patients with carcinoid heart disease should always be considered for surgery irrespective of the extent of valvular involvement.