Case Presentation: The patient is an 83-year-old female with hypertension, hyperlipidemia and carcinoid tumor with metastasis to the liver. Two months after her oncologic diagnosis, she presented to the hospital for severe dyspnea and palpitations. Her dyspnea had been progressively worsening for six months; the palpitations were associated with exertion and resolved with rest. Her exam was notable for jugular venous distention, a 3/6 holosystolic murmur at the left lower sternal border and 2+ pitting edema of the lower extremities. Chest x-ray only showed a small right pleural effusion. Electrocardiogram (ECG) demonstrated normal sinus rhythm. Transthoracic echocardiogram (TTE) without a bubble study showed a preserved ejection fraction, severe tricuspid regurgitation from poor valve coaptation, and a massively dilated right atrium and right ventricle. A transesophageal echocardiogram (TEE) was obtained for better visualization of the valves and confirmed these findings; in addition, it demonstrated moderate aortic regurgitation and the presence of a patent foramen ovale (PFO). Her presentation was consistent with right-sided heart failure secondary to advanced carcinoid heart disease. The patient was started on diuretics for symptom relief and cardiothoracic surgery was consulted for valve replacement, which was eventually performed. Intraoperatively, the aortic valve also appeared to have evidence of infiltration from the carcinoid tumor. She thus underwent aortic and tricuspid valve replacement and PFO closure. Microscopic and histologic examination of the involved valves showed plaques of fibrous tissue. Upon outpatient follow-up, the patient reported doing well with symptom improvement.
Discussion: Carcinoid tumors most often occur in the gastrointestinal tract and secrete vasoactive substances such as serotonin, histamine and tryptophan. Carcinoid heart disease can be seen in up to 50% of patients. Elevated levels of serotonin reach the right side of the heart and induce fibrosis of the right-sided valves and endocardium. Examination of the affected valves shows the pathognomonic plaque-like deposits of fibrous tissue made up on smooth muscle cells, myofibroblasts, and endothelial cells, as was seen in this patient. Left-sided carcinoid disease is rare, since it is postulated that the valves are spared due to inactivation of these vasoactive products by the lungs. A PFO, however, permits their entry into the left heart chambers. In similarity with literature review findings, providers should consider a PFO as a likely cause of bilateral carcinoid heart disease and heart failure when such patients present in the clinical setting. This should prompt investigation with a bubble study or TEE as these patients have a higher likelihood of disease progression.
Conclusions: This report describes an interesting case of advanced carcinoid heart disease with involvement of the tricuspid valve and aortic valve in the presence of a PFO. PFO assessment should be performed in patients with carcinoid disease as these patients are at high risk of heart disease progression. A multidisciplinary treatment approach often leads to recovery of cardiac function and improved patient outcomes.