Insuficiencia cardíaca por un síndrome carcinoide: un reto diagnóstico. Reporte de caso / Carcinoid heart disease: a diagnostic challenge. Case Report

Resumen Introducción. La insuficiencia cardíaca es una patología frecuente que no solo es causada por isquemia miocárdica, hipertensión o valvulopatías, sino también por un gran número de enfermedades; sin embargo, en la práctica, muy pocas veces se aborda su etiología, y, por lo tanto, su manejo usualmente es sintomático, situación que puede repercutir negativamente en la evolución natural de la enfermedad, ya que es sabido que un diagnóstico etiológico oportuno puede mejorar el pronóstico de cualquier enfermedad. Los principales signos clínicos del tumor carcinoide típico son diarrea, flushing facial y broncoespamo, sin embargo el compromiso cardíaco también ha sido descrito en pacientes con esta condición (menos del 20%). Presentación del caso. Paciente masculino de 77 años que acudió al servicio de urgencias con un cuadro clínico de insuficiencia cardíaca descompensada. Teniendo en cuenta la pobre respuesta del paciente ante el manejo propuesto y la evidencia de diarrea crónica, se realizaron varios estudios diagnósticos, lo que permitió diagnosticarlo con un tumor neuroendocrino. Conclusiones. La insuficiencia cardíaca es una patología frecuente en la población general, sin embargo la identificación temprana de causas poco comunes, tales como el síndrome carcinoide, permitirá implementar un manejo oportuno e identificar complicaciones asociadas como la cardiopatía carcinoide, lo que tendrá un impacto positivo en la calidad de vida y pronóstico del paciente.

Abstract Introduction: Heart failure is a frequent disease that is not only caused by myocardial ischemia, hypertension or valve disease, but also by many other diseases. However, its etiology is rarely addressed, and therefore its management is usually symptomatic, which may affect the course of the disease, taking into account that an early etiological diagnosis could lead to better outcomes. The main clinical signs of the typical carcinoid tumor are diarrhea, facial flushing and bronchospasm, although heart failure symptoms have also been described in patients with this condition (less than 20%). Case presentation: A 77-year-old man visited the emergency department due to decompensated heart failure. Considering his poor response to the proposed treatment and symptoms such as chronic diarrhea, several diagnostic studies were performed, which allowed diagnosing him with a neuroendocrine tumor. Conclusions: Heart failure is a frequent pathology in the general population. However, the early detection of rare causes, such as carcinoid syndrome, will allow implementing adequate studies, staging and treatment in order to prevent complications such as carcinoid heart disease, which will have a positive impact on the patient's quality of life and prognosis.

Quadruple valve replacement for carcinoid heart disease

Abstract Introduction: Carcinoid heart disease most frequently involves the tricuspid or, more rarely, the pulmonary valve and presents with right heart failure as 5-HT is metabolized by the lung. Left-sided valve involvement is quite rare. We describe our experience of 3 patients presenting with heart failure secondary to carcinoid heart disease affecting all four cardiac valves. There are only four previous isolated case reports in the literature. Methods: All three patients underwent quadruple valve replacement during a single operation. Right ventricular outflow tract reconstruction with a pericardial patch was performed in all patients. For 24 hours prior to surgery, all patients received intravenous octreotide, which continued in intensive care for at least 24 hours. Results: Mean cross-clamp and bypass times were 175 (range 164-197 minutes) and 210 (range 195-229 minutes) minutes, respectively. Mean intensive treatment unit (ITU) and inpatient stays were 2.3 (range 2-3 days) and 12 (range 9-16 days) days, respectively. One patient was reopened for bleeding 4 hours postoperatively from a ventricular pacing wire site. None required a permanent pacemaker postoperatively. There were no other complications in any patient. The quality of life was excellent at 6-16 months clinic follow-up as they were in NYHA 1. Postoperative echocardiography showed no paravalvular leaks and well-functioning prostheses in all cases. Conclusion: Surgery to replace all four valves is feasible with excellent medium-term survival and a very low rate of complications. Patients with carcinoid heart disease should always be considered for surgery irrespective of the extent of valvular involvement.

Carcinoid syndrome: update on the pathophysiology and treatment

Approximately 30-40% of patients with well-differentiated neuroendocrine tumors present with carcinoid syndrome, which is a paraneoplastic syndrome associated with the secretion of several humoral factors. Carcinoid syndrome significantly and negatively affects patients' quality of life; increases costs compared with the costs of nonfunctioning neuroendocrine tumors; and results in changes in patients' lifestyle, such as diet, work, physical activity and social life. For several decades, patients with neuroendocrine tumors and carcinoid syndrome have been treated with somatostatin analogues as the first-line treatment. While these agents provide significant relief from carcinoid syndrome symptoms, there is inevitable clinical progression, and new therapeutic interventions are needed. More than 40 substances have been identified as being potentially related to carcinoid syndrome; however, their individual contributions in triggering different carcinoid symptoms or complications, such as carcinoid heart disease, remain unclear. These substances include serotonin (5-HT), which appears to be the primary marker associated with the syndrome, as well as histamine, kallikrein, prostaglandins, and tachykinins. Given the complexity involving the origin, diagnosis and management of patients with carcinoid syndrome, we have undertaken a comprehensive review to update information about the pathophysiology, diagnostic tools and treatment sequence of this syndrome, which currently comprises a multidisciplinary approach.

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.

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 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.

Acupuntura na assistência de enfermagem ao paciente portador de arritmias cardíacas: pesquisa bibliográfica / Acupuncture in nursing care for patients with cardiac arrhythmias: bibliographic search

Introdução: Entre as patologias cardíacas, parte dos eventos está relacionada com as arritmias. Inúmeros tratamentos são encontrados; medicamentosos e cirúrgicos; que nem sempre apresentam resultados satisfatórios, com uso de drogas que provocam reações adversas significativas e, a busca pela adequação da dosagem causa grande desconforto. As cirurgias são onerosas e nem sempre acessíveis aos sofredores de arritmias. Atualmente, busca-se por tratamentos alternativos, entre os quais, a Acupuntura, técnica da Medicina Tradicional Chinesa (MTC) sobressai às outras pela grande divulgação midiática. São conhecidos dos Acupunturistas seus benefícios no que tange as doenças cardíacas e, em especial, as arritmias. Objetivo: Buscar subsídios para o uso da Acupuntura como técnica efetiva usada pelo Enfermeiro na assistência do paciente cardiopata. Metodologia: Pesquisa bibliográfica realizada com consulta de livros, uma vez que a busca por trabalhos na Biblioteca Virtual de Saúde ­ BVS, utilizando as bases de dados LILACS, IBECS E MEDLINE, com as palavras-chave acupuntura e enfermagem, cardiologia e acupuntura e enfermagem e cardiologia não apresentou resultados que sustentem a possibilidade da ocorrência e prática dos pressupostos afirmados no corpo do projeto. Resultados: A Acupuntura é técnica da MTC, nesta as patologias são explicadas por concepções energéticas de saúde e fisiologia. Na MTC, o coração regula o sangue e os vasos sanguíneos, e armazena a consciência. É patente que as disfunções cardíacas acarretam prejuízo à totalidade do organismo, em órgão salvos e ao estado de consciência, daí a preocupação com as síndromes energéticas, pois são a origem dos distúrbios cardiocirculatórios. Por esse olhar, os padrões de desarmonia do coração podem ser divididos em dois grupos: Deficiência de Yang e deficiência de Yin e suas síndromes. Foram selecionados 35 livros, entre estes, 19 específicos de Acupuntura e Medicina Tradicional Chinesa e encontrados 15 pontos de Acupuntura com ação direta sobre as arritmias cardíacas, são eles: B14, B15, B19, CS5, CS6, C5, C7, C8, VC6, VC7, VC14, VC15, VC17, R3, R10. Todavia, não foi possível a quantificação da eficácia no uso desses pontos por carência de trabalhos relacionados. Conclusão: A Acupuntura, como técnica que busca a harmonização das energias do corpo, muito pode oferecer aos portadores de arritmias, visto que suas causas e manifestações se mostram complexas e oriundas de um organismo em desequilíbrio. Os conhecimentos que o enfermeiro detém qualificam-no para a prática segura dessa técnica terapêutica de cuidado.

Sindrome carcinoide: achados ecocardiográficos na evolução clínica de um caso de síndrome carcinoide / Carcinoid syndrome: echocardiography findings in a clinical evolution of a carcinoid syndrome case

O comprometimento cardíaco na síndrome carcinoide ocorre em 50% dos pacientes, sendo o ecocardiograma um importante meio diagnóstico da doença. Os achados de exame físico podem passar despercebidos, como o sopro tricúspide e pulmonar, em virtude dabaixa pressão no território pulmonar. Apesar de alguns casos responderem bem aos análogos da somastotina, como o octeotride, a doença cardíaca evolui, inexoravelmente, para insuficiência cardíaca, sendo a indicação cirúrgica uma opção para determinados casos.

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.

Ovarian Tumor-Associated Carcinoid Heart Disease Presenting as Severe Tricuspid Regurgitation

Carcinoid heart disease is characterized by heart valve dysfunction as well as carcinoid symptomatology. We report a case of carcinoid heart disease associated with a primary ovarian tumor. A 60-year-old woman presented for dyspnea evaluation with a history of facial flushing, telangiectatic skin changes, and pitting edema of both lower extremities. Chest radiography showed cardiomegaly, and echocardiography revealed an isolated, severe tricuspid regurgitation without left-sided valvular dysfunction. The tricuspid leaflets were severely retracted and shortened, resulting in poor coaptation. Furthermore, mild pulmonary valve stenosis and moderate regurgitation were found along with this deformation. The 24-hour urine analysis revealed an increased level of 5-hydroxyindoleacetic acid, and an ovarian tumor was apparent on computed tomography images. The mass was surgically removed, and the patient was diagnosed as having a primary ovarian carcinoid tumor. She was treated with chemotherapy and regularly followed-up with supportive treatments, deferring surgical correction.

Carcinoid Heart Disease: A Rare Cause of Right Ventricular Dysfunction Evaluation by Transthoracic 2D, Doppler and 3-D Echocardiography

Carcinoid heart disease is a rare cause of heart failure with or without right valvular heart impairments. In this study, we showed a case of carcinoid tumour with hepatic metastases inducing carcinoid heart disease. Neuroendocrine heart involvement happens for severe tricuspid valve insufficiency and plaques on right ventricular (RV) walls produced by a release of serotonin (5-HT). A patient affected by primitive ileal tumour with 5-HT-secernent hepatic metastases inducing tricuspid insufficiency is showed. Transthoracic 2-D echocardiography showed tricuspid valve regurgitation and both right atrium, RV-walls plaques and RV dilation. Continue-wave Doppler showed a characteristic "dagger shaped" spectrum of tricuspid systolic flow. RV function was evaluated with 3-D transthoracic echocardiography. In particular, RV volumes, RV ejection fraction and stroke volume were defined by this technique. 2, 3-D echocardiography and Doppler method are useful techniques to show heart valves' derangements and RV function to non-invasively detect RV impairments in carcinoid heart disease.

Isolated Tricuspid Regurgitation Caused by Annular Dilatation

Isolated tricuspid regurgitation (TR) is rare. Generally, TR is caused by pulmonary hypertension secondary to mitral or aortic valve disease, commonly referred to as "functional" regurgitation. The causes of isolated TR in adults include trauma, endocarditis, carcinoid heart disease, and congenital malformation of the tricuspid valve apparatus. In addition, isolated TR should be distinguished from Ebstein anomaly. In the present case, the patient had no definite causes of TR, and neither mitral nor aortic valve disease. The tricuspid valve of this patient showed no abnormalities other than a severely dilated tricuspid annulus. Isolated TR caused by annular dilatation was diagnosed and then ring annuloplasty was perfomed. The subsequent clinical course was satisfactory.

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.