Hedinger Syndrome with Intraoperative Carcinoid Crisis — Understanding the Pathophysiology for a Successful Management

Abstract Carcinoid tumors can be a cause for right heart valve disease, also known as Hedinger syndrome or carcinoid heart disease. Proper understanding of the pathophysiology is of the uttermost importance for adequate treatment of these patients, especially during heart surgery.

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.

Compromiso cardiovascular secundario a tumor carcinoide metastásico / Cardiovascular compromise secondary to metastatic carcinoid tumor

Resumen Los tumores carcinoides son neoplasias pertenecientes a la familia de tumores neuroendocrinos y presentan baja incidencia en la población en general; sin embargo, hasta el 50 % pueden debutar con compromiso cardiovascular. Se presenta el caso de un paciente, sin antecedentes cardiovasculares quien presentó signos y síntomas sugestivos de falla cardiaca derecha. Se le realizó ecocardiografía transtorácica que evidenció compromiso de ambas válvulas cardíacas derechas por tumor neuroendocrino metastásico, llevando al diagnóstico de sindrome carcinoide. Con el fin de tener presente dicha entidad en la práctica diaria de la cardiología clínica se revisan datos claves de abordaje diagnóstico y terapéutico.

Abstract Carcinoid tumors are neoplasms belonging to the neuroendocrine tumor family and have a low incidence in the general population; however, up to 50% may debut with cardiovascular involvement. We present the case of a patient, without a cardiovascular history, who presented signs and symptoms suggestive of right-sided heart failure. A transthoracic echocardiography was performed, which evidenced the involvement of both right heart valves by a metastatic neuroendocrine tumor, leading to the diagnosis of carcinoid syndrome. In order to take this entity into account in the daily practice of clinical cardiology, key data of diagnostic and therapeutic approach are reviewed.

Tumor carcinoide primario de ovario que se presenta como enfermedad cardiaca carcinoide / Primary carcinoid tumor of the ovary presenting with carcinoid heart disease / Primary carcinoid tumor of the ovary presenting with carcinoid heart disease

RESUMEN El tumor carcinoide primario de ovario es una entidad poco frecuente que se caracteriza por la producción de sustancias vasoactivas que pasan directamente al torrente circulatorio y producen el llamado síndrome carcinoide. Estas sustancias también son las causantes de la formación de placas de fibrosis en las válvulas cardiacas, principalmente las derechas. Presentamos un caso de tumor carcinoide primario de ovario con afectación de la válvula tricúspide, que fue diagnosticado a partir de la lesión cardiaca. En la literatura están descritos casos clínicos aislados y revisiones de casos que nos pueden ayudar en el diagnóstico y en la toma de decisiones terapéuticas. Debemos conocer la existencia de estos tumores; la clínica debe alertarnos sobre su presencia. Es primordial un manejo multidisciplinar junto con endocrinólogos, cardiólogos, oncólogos, anatomo-patólogos, anestesistas, ginecólogos y cirujanos cardiacos, para ofrecer un tratamiento completo.

ABSTRACT Primary ovarian carcinoid tumor is a rare neoplasm characterized by the production of vasoactive substances that enter the bloodstream and give rise to the so-called carcinoid syndrome. These substances may also cause fibrotic plaques in the heart valves, mainly those in the right cardiac chambers. We describe the case of a primary ovarian carcinoid tumor with tricuspid valve involvement in which the diagnosis was prompted by identification of cardiac involvement. Knowledge of the clinical manifestations of neuroendocrine tumors is important in diagnosis and patient management. Recognition of typical clinical features may help suggest the correct diagnosis. Multidisciplinary discussion allows the formulation of a comprehensive management strategy involving endocrinologists, cardiologists, oncologists, pathologists, anaesthesiologists, gynaecologists and heart surgeons.

Bioprosthetic valve thrombosis in carcinoid heart disease

Tricuspid regurgitation in carcinoid syndrome leads to significant morbidity and mortality that may warrant a tricuspid valve replacement. However, for patients with high serotonin levels and known hypercoagulable risks, the optimum timing for surgery and postoperative anticoagulation approaches remain unclear. High serotonin-triggered hypercoagulability makes prosthetic valves susceptible to thrombosis. Despite appropriate management with a somatostatin analog, some patients continue to have high markers of serotonin that causes platelet aggregation and rapid clot formation. In severely symptomatic patients who require valve surgery, it may not be feasible to postpone surgery until these metabolites are normalized, which may add a substantial risk for postoperative valve thrombosis to an otherwise uneventful procedure. In some, there is a significant need to predict and prevent bioprosthetic valve thrombosis in carcinoid heart disease and to identify best anticoagulation practices across a spectrum of its complex coagulation dynamics and clinical presentation.

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.

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.

Carcinoid Heart Disease: A Review.

Carcinoid tumors are rare malignancies able to spread and produce bioactive humoral products, mainly serotonin, which is responsible for the Carcinoid Syndrome (CS); its features are: flushing, diarrhea, bronchospasm and valvular heart disease. The Carcinoid Heart Disease (CHD) importantly worsens prognosis and it is found in up to 50% of patients with CS. After being produced by liver implants, serotonin finds its way straight into the right heart cavities, leading to valve tissue aggression and ventricular dysfunction. Early CHD diagnosis is still a challenge due to the asymptomatic initial stage, until right heart failure develops along with ascites, swelling and hepatomegaly. Echocardiography is still the main tool for diagnosis, especially due to its ability to appropriately evaluate ventricular and valve function, cardiac morphology and hemodynamics. Tricuspid regurgitation, pulmonic stenosis and dilated cardiomyopathy are the main impairments found in CHD. Magnetic resonance imaging has also developed well in this field, and it is now believed to be essential, due to accurate right cavities evaluation and fibrosis detection. For better prognosis, early diagnosis must be pursued, which has impact on clinical management and valve repair surgical decision.

Síndrome carcinoide cardiaco / The carcinoid heart disease

El síndrome carcinoide cardiaco es una patología muy infrecuente que ocurre en el 50 por ciento de los pacientes con síndrome carcinoide maligno, y es caracterizado por la fibrosis de las válvulas tricúspide y pulmonar, así como del endocardio ventricular derecho, lo cual puede provocar una insuficiencia cardiaca derecha, que repercute directamente en el pronóstico de estos pacientes. Se reporta el caso de un masculino de 29 años, portador de síndrome carcinoide con metástasis hepáticas, referido para valoración cardiológica por presentar disnea de pequeños esfuerzos, con posterior diagnóstico de síndrome carcinoide cardiaco. Se discuten las características clínicas, diagnóstico y tratamiento de esta patología tan infrecuente...

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.

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.

Carcinoid heart disease / 中国实用内科杂志

Carcinoid tumors are relatively rare neuroendocrine malignancies most commonly originating from enterochromaffin cells in the gastrointestinal tract.Carcinoid syndrome occurs when tumor cells metastasize to the liver,as the vasoactive substances produced are able to reach the systemic circulation via the hepatic vein.The development of carcinoid heart disease (CHD) is thought to be related to the vasoactive substances secreted by the metastatic tumor cells in the liver,reaching the right heart.This is associated with deposition of fibrous tissue on the endocardial surfaces of the heart.Several series have reported CHD in up to 70% of cases of carcinoid syndrome.Somatostatin analogue use is a therapeutic cornerstone,effective in symptomatic control of the endocrine syndrome.In patients with severe cardiac involvement and well-controlled systemic disease,valve replacement surgery is an effective treatment modality that can relieve intractable symptoms and contribute to improved outcome.