Abstract Introduction Carcinoid neoplasms (CaN) are neuroendocrine tumors that develop over years with few symptoms until they are large or have metastasized. The incidence of CaN is approximately 1-2 in 100,000 annually with cardiac involvement in most cases. Here, we present a case of a middle-aged male who presented with severe isolated tricuspid regurgitation and concomitant right ventricular/atrial dilatation, which was later found to be secondary to a newly diagnosed gastrointestinal neuroendocrine tumor. Case Presentation A 47-year-old previously healthy male presented with palpations, shortness of breath, leg swelling, and diarrhea for a duration of two weeks. Physical examination was significant for tachycardia, a non-tender 3cm left submandibular lymph node, 2+ bilateral pretibial pitting edema. Chest x-ray was unremarkable, and computed tomography (CT) pulmonary angiography revealed a markedly dilated right atrium in addition to multiple calcified mediastinal/left hilar lymph nodes. These findings prompted an echocardiogram which showed severe right ventricular/atrial dilatation, severe tricuspid regurgitation, and moderate pulmonary hypertension. Further investigation via right upper quadrant ultrasound/CT abdomen-pelvis revealed numerous hepatic hyperechoic central cystic lesions and a hypervascular mass in the proximal to mid ileum. Laboratory and uptake testing revealed a markedly elevated Chromogranin A/24 hour 5-Hydroxyindoleacetic Acid and Octreoscan identified numerous Somatostatin expressing lesions, all of which are consistent with a neuroendocrine tumor with cardiac involvement. Throughout the entire hospital course, the patient remained hemodynamically stable and at a New York Heart Association class II. The patient was started on octreotide therapy and diuresed to comfort with appropriate outpatient follow up after discharge. Discussion Carcinoid neoplasms are tumors of neuroendocrine origin most commonly arising from the gastrointestinal and bronchopulmonary tract. Typically, these neuroendocrine tumors secrete vasoactive substances that are metabolized in the lungs which spares the left side of the heart but reach the right side of the heart due to poor hepatic metabolism resulting in carcinoid heart disease (CaHD). CaHD is an important initial manifestation of CaN; with cardiac involvement associated with a poor prognosis. This case illustrates the importance of considering carcinoid syndrome in the differential diagnosis of right heart disease, especially in the absence of nonspecific symptomology and overt malignancy. Early recognition and prompt workup are essential, as delayed diagnosis can result in treatment delays and increased morbidity and mortality. This abstract is funded by: None
Albeg et al. (Fri,) studied this question.
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