Valvular disorders in carcinoid heart disease

Abstract Carcinoid heart disease is a rare but important cause of intrinsic right heart valve disorders leading to right heart failure. Occasionally, left-sided heart valves may also be involved. The characteristic cardiac pathological findings of carcinoid heart disease are endocardial thickening as a result of fibrous deposits on the endocardium. Echocardiographic examination and right heart catheterization are very useful for the diagnosis of the lesion. If more cardiac valves are affected, multiple valve replacement should be considered. The management of the pulmonary valve lesion depends on the extent of the diseased valve, either by valvulotomy, valvectomy, or valve replacement. Percutaneous valve implantations in the pulmonary and in the inferior vena cava positions have been advocated for high-risk patients.

Síndrome carcinoide y compromiso valvular cardiaco, a propósito de 2 casos: Report of two cases / Tricuspid and pulmonary valve involvement in carcinoid syndrome

Carcinoid is a rare neuroendocrine tumor typically arising in the gastrointestinal tract that can cause heart valve involvement. We report two patients with carcinoid syndrome and tricuspid/pulmonary valve lesions. A 33-year-old male presenting with fatigue and weight loss: A tumor in the tail of the pancreas was found on an abdominal CAT scan. The percutaneous biopsy was informed as a carcinoid tumor. A trans-esophageal echocardiogram showed a tricuspid and pulmonary valve involvement, which was replaced surgically. The biopsy informed an extensive fibrous and myxoid degeneration of the valves. A 35-year-old male with a carcinoid syndrome and cardiac failure: An echocardiogram showed a severe tricuspid stenosis and severe pulmonary regurgitation. The patient was subjected to a double surgical valve replacement. The pathology report of the excised valve showed a deforming fibrous and myxoid valvulopathy.

Síndrome carcinoide y compromiso valvular cardíaco / Carcinoid syndrome anf heart valve involvemen

El carcinoide es un tumor neuroendocrino raro que se origina típicamente en el tracto gastrointestinal y que puede resultar en un amplio espectro de síntomas mediado por sustancias vasoactivas. La enfermedad carcinoide con compromiso cardíaco es caracterizada por el depósito de tejido fibroso en forma de placas, afectando más frecuentemente las válvulas cardíacas derechas y el endocardio. El compromiso valvular izquierdo ocurre en menos del 10 por ciento de los casos y es casi siempre asociado a un cortocircuito de derecha a izquierda o a un carcinoide bronquial primario. Los pacientes con falla cardíaca derecha sintomáticos tienen limitadas opciones terapéuticas diferentes a la cirugía cardíaca. El reemplazo valvular cardíaco es el único tratamiento efectivo para la enfermedad valvular carcinoide y debiese ser considerada en pacientes sintomáticos, en los cuales la enfermedad metastásica y los síntomas del síndrome carcinoide se encuentran bien controlados. Por estos motivos, se recomienda un enfoque multidisciplinario en un centro de experiencia para este tipo de pacientes. El presente artículo es una revisión actualizada de la literatura al respecto, tratando de clarificar las principales dudas con respecto al manejo de estos pacientes.

Carcinoid is a rare neuroendocrine tumor typically originating in the gastrointestinal tract and which may result in a broad spectrum of symptoms mediated by vasoactive substances. Carcinoid heart disease is characterized by plaque-like deposits of fibrous tissue, most frequently affecting the right heart valves and endocardium. Left-sided valve disease occurs in less than 10 percent of patients with cardiac involvement and is almost always associated with an atrial right-to-left shunt or a primary bronchial carcinoid. Patients with symptomatic right heart failure have limited therapeutic options other than cardiac surgery. Cardiac valve replacement is the only effective treatment for carcinoid heart disease and should be considered for symptomatic patients whose metastatic carcinoid disease and symptoms of carcinoid syndrome are well controlled. A multidisciplinary approach at an experienced center is recommended for the care of these patients.

Carcinoid heart disease and the utility of 3D trans-thoracic and trans-esophageal echocardiography: two clinical cases

Carcinoid tumors are rare neuroendocrine malignancies. We present two cases of metastatic carcinoid tumors, complicated by carcinoid syndrome and by cardiac valve involvement. Carcinoid syndrome is characterized by secretory diarrhea, episodic flushing, and bronchospasm. Cardiac involvement occurs in up to 50% of patients with metastatic carcinoid tumors which commonly causes abnormalities of the right sided valves. Echocardiography is the best available and non invasive technique for diagnosis. Characteristic features of carcinoid heart disease are thickened, shortened, retracted, and fixed or partially fixed valve leaflets. Three-dimensional [3D] echocardiography provided an en face view of pulmonary and tricuspid valve, not obtainable by two-dimensional echocardiography, and improved delineation of the relationship between these structures and cardiac chambers

Síndrome de carcinoide con manifestación inicial a nivel valvular cardíaco: a raíz de un caso clínico / Carcinoid syndrome presenting as valvular diseas: report of one case

Carcinoid syndrome is observed in one third of carcinoid tumors and usually appears when there are liver metastases. One of the main complications of this syndrome is the appearance of tricuspid or pulmonary valvular disease. We report a 56 years old male presenting with malaise and a weight loss of 10 kg. On physical examination, a heart murmur suspicious of a double tricuspid lesion was found. The echocardiogram was suggestive of a carcinoid valvular disease. The abdominal CAT scan showed a small bowel tumor. Urinary 5-hydroxy-indol- acetic acid values were highly elevated. The patient was subjected to excision of the distal ileum, liver metastasectomy and hemicolectomy. The pathological study of the surgical piece confirmed the diagnosis of carcinoid tumor. Two years after surgery, the patient is in stable conditions.

Síndrome carcinoide: reporte de un caso con afección cardiaco y tumor primario de ovario / Carcinoid syndrome: report of a case with cardiac involvement and primary tumor of the ovary

Se presenta el caso de una paciente con tumor carcinoide primario de ovario que desarrolló enfermedad cardiaca carcinoide grave sin metástasis hepáticas. Se hace una revisión de la literatura, enfatizando el hecho de que la enfermedad cardiaca carcinoide secundaria a tumor ovárico primario es muy rara. Se discuten las indicaciones quirúrgicas relativas a este caso.

O ecocardiograma na síndrome do coraçäo carcinóide: relato de caso / The echocardiogram in carcinoid heart syndrome: a case report

Mulher de 42 anos, portadora de prótese mecânica em posiçäo aórtica, começou a apresentar sinais de insuficiência cardíaca direita, associados a diarréia, sensaçäo de "sufoco" e eritema facial. O estudo ecocardiográfico revelou sinais típicos de comprometimento cardíaco por síndrome do coraçäo carcinóide (SCC): sinais de sobrecarga de volume das câmaras direitas e espessamento da valva tricúspide que permanecia em posiçäo semi-aberto durante todo o ciclo cardíaco. A valva pulmonar também apresentou aspecto semelhante. O estudo com "Doppler Pulsátil" registrou acentuada insuficiência tricúspide e discreta incompetência pulmonar. Com base nos achados ecocardiográficos, foi sugerida dosagem de ácido 5-hidroxiindolacético na urina de 24 e os níveis elevados confirmam o diagnóstico daquela síndrome. Conclui-se que a ecocardiografia permite avaliar com detalhes o comprometimento cardíaco na SCC, apresentando imagens típicas, quase patognomônicas da afecçäo