Neuroendocrine Carcinoma Presenting as Carcinoid Heart Disease

Liza Zachariah, MD
Sherine Salib, MD
Alejandro Moreno, MBBS, MPH, JD
Volume 55 - Issue 11 - November 2015

A previously healthy 31-year-old woman presented with a 3-month history of worsening dyspnea and exercise intolerance. She also reported dry cough, loose stools, and a recent 8 lb weight loss.

Physical examination. The patient was tachycardic and tachypneic. Cardiovascular examination revealed jugular venous distention, left parasternal heave, a loud blowing holosystolic murmur in the left lower sternal border, and a right-sided S3 gallop. Moreover, she had marked hepatomegaly and lower extremity edema.

Laboratory tests. These findings revealed anemia (hemoglobin, 8 g/dL) and mildly elevated hepatic enzymes (alkaline phosphatase, 145 U/L; aspartate aminotransferase, 43 U/L; and alanine aminotransferase, 92 U/L).

The patient was admitted for right heart failure and diuresed. An echocardiogram revealed severe tricuspid regurgitation, right atrial dilation, and evidence of right ventricular pressure and volume overload (Figure 1). Because of the persistent hepatomegaly and elevated liver enzymes, abdominal imaging was obtained, revealing extensive metastatic disease of the liver (Figures 2A and 2B).

Diagnosis. Based on the constellation of findings, a preliminary diagnosis of carcinoid heart disease was made.
A CT-guided liver biopsy confirmed the diagnosis of metastatic neuroendocrine carcinoma. Esophogastroduodenoscopy and colonoscopy were negative for malignancy.

Outcome of the case. The patient was started on octreotide, which led to significant improvement of her diarrhea and cough. She is being evaluated for tricuspid valve replacement.

Discussion. Carcinoid heart disease may be the initial presentation of neuroendocrine carcinoma in up to 20% of
patients.1,2 Only tumors that invade the liver can cause pathologic changes of the heart, including endocardial plaques of fibrous tissue distorting the valves.2 The predilection for right-sided involvement is due to inactivation of vasoactive substances by the lungs.2 The 2 key investigations for the diagnosis of carcinoid heart disease are transthoracic echocardiography and 24-hour 5-hydroxyindoleacetic acid urine test.2

Treatment entails supportive therapy for heart failure and somatostatin analogues, which reduce the effect of vasoactive peptides.1,3,4 Valve replacement should be considered in suitable candidates, since these patients are more likely to die from heart failure than from metastatic disease.1,4

References:

  1. Patel C, Mathur M, Escarcega RO, Bove AA. Carcinoid heart disease: current understanding and future directions. Am Heart J. 2014;167(6):789-795.
  2. Martinez-Quintana E, Avila-Gonzalez Mdel M, Suarez-Castellano L, Rodriguez-Gonzalez F. Metastatic carcinoid tumor presenting as right sided heart failure. Int J Endocrinol Metab. 2013;11(2):120-125.
  3. Bhattacharyya S, Davar J, Dreyfus G, Caplin ME. Carcinoid heart disease. Circulation. 2007;116(24):2860-2865.
  4. Luk A, Ezzat S, Butany J. Pathology, pathophysiology, and treatment strategies of endocrine disorders and their cardiac complications. Sem Diagn Pathol. 2013;30(3):245-262.