Case Presentation: A 49 year old female with history of polysubstance abuse, recent diagnosis of diastolic heart failure, and COPD who initially presented to an outside hospital with progressive dyspnea on exertion and volume overload. Her hypoxic symptoms were thought to be from COPD,pulmonary hypertension and volume overload from predominantly right-sided heart failure. She was admitted for acute hypoxic respiratory failure and gradually improved with diuresis, nebulizers and steroids. Initial echocardiography showed preserved LV function, moderately dilated and dysfunctional RV, severe tricuspid and pulmonic regurgitation,moderate degenerative aortic insufficiency, and presumed pulmonary hypertension. Recognizing the peculiarity and rarity of severe PR, we re-performed the echocardiogram focusing on the right-sided structures. In addition to a PFO, thickened and restricted tricuspid valve leaflets were noted raising suspicion for carcinoid syndrome. Returning to the patient to investigate our new theory further, we noted a history of significant diarrhea that she had blamed on her recent cholecystectomy and episodic flushing. We also discovered a large protruding RLQ mass with related abdominal discomfort and history of vaginal spotting. CT and MRI revealed a lobulated 7.6 cm heterogeneously enhancing solid lesion of the right adnexa. In consultation with gynecology oncology, tumor markers including 5HIAA were obtained. 5HIAA was severely elevated at 90mg/24hr, CA125 was 225U/ml. Empiric octreotide was administered, and she subsequently underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy revealing an insular carcinoid tumor. She was discharged on octreotide with plans for octreotide-receptor scintigraphy to assess for metastasis upon her follow up with oncology for consideration of chemotherapy in the future. She will also follow up with CT surgery for consideration of tricuspid and pulmonic valvular replacements.
Discussion: Carcinoid heart disease is a rare and progressive manifestation of a unique disease process that affects the right-sided heart valves.Typically, the disease based in the colon metastasizes to the liver following the portal circulation before affecting the heart valves. In our very unique case, the ovarian location of the tumor may have limited the metastatic probability as the ovarian vein empties straight into the IVC.
Carcinoid heart disease is rare and can easily be missed in a patient with risk factors for right heart failure such as COPD. Early diagnosis of this rare condition is important to prevent irreversible valvular damage, improve the prognosis, or—as in this case—potentially curb an otherwise progressive disease.
Conclusions: Carcinoid heart disease is a unique manifestation of carcinoid syndrome mediated by the release of vasoactive substances. A timely diagnosis and early treatment is necessary to reduce mortality. We present an interesting case of carcinoid heart disease initially masquerading as progressive pulmonary hypertension and right heart failure from COPD and heart failure with preserved ejection fraction.