Challenges in the diagnosis and management of the carcinoid heart disease

Introduction: Carcinoid tumors are a type of rare, slow growing, malignant neuroendocrine neoplasm, most commonly developing in the intestinal wall, but may be seen in several places throughout the body. This type of neoplasm can synthesize and release hormones in the blood that cause symptoms and signs such as chronic diarrhea and flushing. The etiology of this type of tu-mor remains unclear. In a number of cases, carcinoid heart disease develops, because of an intrinsic tricus-pid and pulmonary valve disease leading to right heart failure. According to the literature, there is a direct re-lationship between liver metastasis and right heart val-vular disease.

Methods: We present the case of a 38 years old female patient, with carcinoid syndrome of unknown primary and highly active biologically and structurally liver me-tastasis. The patient was clinically stable at the follow up for several years, without relevant echocardiographic changes (mild tricuspid and pulmonary regurgitation). Results: She returnedin our clinic for her annual check, but this time, she described bilateral pitting edema and mild/moderate dyspnea, approximately 1 month before presentation. On admission the patient was hemodina-mically stable, BP=110/70 mmHg, SO2=97%, HR=93 bpm and afebrile. Lab workup: elevated level of tumor biomarkers compared with previous examination (se-rotonine, 5-HIAA). ECG at rest: sinus rhythm with ri-ght axis deviation and nonspecific T wave changes. The transthoracic echocardiography revealed severe tricus-pid regurgitation and moderate pulmonary stenosis, but with preserved left heart function. Liver metastasis on ultrasonography were evolutive concerning dimen-sions, number and blood supply. We considered that carcinoid syndrome was the cause of the severe valvu-lar disease, most likely because of highly active multiple liver metastasis. At the time, we decided a conservative treatment of the tricuspid pathology and right heart fa-ilure and direct the patient to oncology for chemoem-bolization and systemic carcinoid treatment, then to be surgically evaluated.

Conclusions: The rapid evolution of this type of heart disease in a young patient, without additional patholo-gies or risk factors was unexpected, considering that, this type of neuroendocrine tumors are slow growing an the transthoracic echocardiography performed for several years did not point out any relevant hemodyna-mic valvular pathology.

ISSN – online: 2734 – 6382
ISSN-L 1220-658X
ISSN – print: 1220-658X
The Romanian Journal of Cardiology is indexed by:
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CODE: 379
CME Credits: 10 (Romanian College of Physicians)