A Case of Carcinoid Syndrome Due to Malignant Metastatic Carcinoid Tumor with Carcinoid Heart Disease Involving Four Cardiac Valves

Abstract

Patient: Female, 62Final Diagnosis: Carcinoid heart diseaseSymptoms: Abdominal discomfort • diarrhea • fatigue • weight lossMedication: —Clinical Procedure: —Specialty: CardiologyObjective:Rare diseaseBackground:Carcinoid tumor, benign, low-grade malignant, and high-grade malignant, can be associated with the release of vasoactive substances that cause symptoms including cutaneous flushing, diarrhea, and bronchospasm. In 50–60% of patients with carcinoid syndrome, the vasoactive substances cause fibrosis of the pulmonary and tricuspid heart valves which lead to regurgitation and right-sided heart failure. The right side of the heart is usually affected because monoamine oxidases in the lungs usually inactivate the vasoactive substances.Case Report:A 62-year-old woman presented with a four-month history of diarrhea and abdominal discomfort. The patient had bilateral pelvic masses and elevated serum CA125 and an elevated urinary 5-hydroxyindoleacetic acid (5-HIAA). Total abdominal hysterectomy (TAH) and bilateral salpingo-oophorectomy (BSO) identified a primary appendiceal carcinoid tumor with bilateral ovarian metastases. Post-operatively, a computed tomography (CT) scan showed cardiomegaly. Transthoracic echocardiography showed morphologic changes that affected all four heart valves, consistent with carcinoid heart disease but without a patent foramen ovale (PFO). The patient was evaluated for valve replacement surgery, but surgery was not performed due to the degree of heart failure.Conclusions:This report describes a rare case of carcinoid heart disease with involvement of all four cardiac valves, but without the presence of a PFO, possibly due to a large amount of vasoactive substances in the patient’s circulation, as supported by the patient’s elevated urinary 5-HIAA. This rare presentation highlights the importance of early diagnosis and treatment with tumor resection and, if possible, Cardiac valve replacement to prevent irreversible heart failure.

Development of severe intrapulmonary shunting in a patient with carcinoid heart disease after closure of a persistent foramen ovale: a case report

“…Noteworthy, a massive increase in the release of vasoactive substances also can lead to an involvement of left-sided valves even without the presence of a PFO. 21 In our case, 5-HIAA levels remained stable at that time. Nevertheless, it cannot be ruled out that the blood levels of other bioactive substances which are not measured in clinical routine may already have risen at the time of PFO closure.…”

Tumor carcinoide primario de ovario que se presenta como enfermedad cardiaca carcinoide

“…La clínica del síndrome carcinoide suele presentarse en 20 a 30% (5) de las pacientes y lo hace de forma precoz, cuando existen metástasis hepáticas o en los casos de tumores de ovario primarios, ya que liberan sustancias vasoactivas (histamina, bradiquininas, prostaglandinas, taquicininas y serotonina) (5)(6)(7) de forma directa al sistema circulatorio. En nuestro caso, los síntomas que presentaba la paciente eran diarrea y rubor cutáneo, pero ambos síntomas no la alertaron para consultar con anterioridad a la crisis de disnea y broncoespasmo.…”

“…Desde el punto de vista histológico, se dividen en insular (el más común), trabecular, estromal y mucinoso, aunque a veces pueden ser mixtos (2,4) . Estos tumores se caracterizan por la liberación de sustancias vasoactivas como la serotonina, taquiquininas y prostaglandinas, que normalmente son inactivadas por el hígado (5)(6)(7) . El aumento de estas sustancias ocasiona el síndrome carcinoide, que se caracteriza por rubor cutáneo, diarrea, broncoespasmo e hipotensión (1,2,5) .…”

“…El síndrome carcinoide ocurre cuando existen metástasis hepáticas o cuando las sustancias vasoactivas son liberadas directamente al torrente circulatorio, sin pasar el filtro hepático, como ocurre en los tumores carcinoides primarios de ovario. En este caso, estas sustancias, pasan directamente de la vena ovárica a la vena cava en el lado derecho o a la vena renal en el lado izquierdo (1,4,5) . Esto puede afectar las válvulas cardiacas, principalmente las del lado derecho (válvula tricúspide y pulmonar) (2,4,5) .…”

“…En este caso, estas sustancias, pasan directamente de la vena ovárica a la vena cava en el lado derecho o a la vena renal en el lado izquierdo (1,4,5) . Esto puede afectar las válvulas cardiacas, principalmente las del lado derecho (válvula tricúspide y pulmonar) (2,4,5) .…”