Letter to the Editor
Carcinoid cardiopathy as the first manifestation of carcinoid syndrome
Cardiopatía carcinoide como presentación inicial de síndrome carcinoide
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The thorax radiography showed a significant overall cardiomegaly with bilateral pleural effusion, and abdominal ultrasound showed a heterogeneous mass of 13.3<span class="elsevierStyleHsp" style=""></span>cm<span class="elsevierStyleHsp" style=""></span>×<span class="elsevierStyleHsp" style=""></span>9.7<span class="elsevierStyleHsp" style=""></span>cm, described as probable liver metastasis. The echocardiogram was compatible with carcinoid heart disease with extensive pulmonary and tricuspid valve involvement. The presence of carcinoid syndrome was confirmed with increased 5-hydroxyindoleacetic acid (5-HIIA) in urine by 324<span class="elsevierStyleHsp" style=""></span>mg/g of creatinine (0–10) and chromogranin A by 112<span class="elsevierStyleHsp" style=""></span>ng/ml (0–6). On the seventh day after hospital admission she had extensive thrombosis of the right internal jugular vein, requiring anticoagulant therapy. Scintigraphy and SPECT were performed with somatostatin analogs (SSA), showing uptake indicative of disease activity in the liver parenchyma, without finding the primary lesion. For symptomatic carcinoid syndrome control, she was treated with lanreotide, 120<span class="elsevierStyleHsp" style=""></span>mg every 28 days, with marked improvement in digestive symptoms. However, the occurrence of refractory right heart failure was the limiting factor for the treatment of the disease.</p><p id="par0010" class="elsevierStylePara elsevierViewall">Approximately 30% of patients with carcinoid tumor have carcinoid syndrome, clinically manifested by vasomotor changes due to hypersecretion of vasoactive hormones (serotonin, histamine, bradykinin, 5-hydroxytryptophan, prostaglandins), causing episodes of facial flushing, intestinal hypermotility, secretory diarrhea, bronchospasm and hypotension. Diagnosis is based on clinical suspicion supported by primary tumor and/or metastasis associated with an increased urinary excretion of the serotonin metabolite: 5-HIIA. Carcinoid heart disease affects about half of the patients with carcinoid syndrome. It may be the first sign in up to 20%,<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a> and accounts for the main cause of death in cases involved.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a> It is characterized by a pathognomonic plaque-like deposit of fibrous tissue in the endocardium, and occasionally, in the intima layer of the pulmonary or aorta artery. Right valve disease is more frequent since the lung appears to inactivate vasoactive hormones.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a> Serotonin is crucial in the pathogenesis of the disease. Patients with heart disease have higher levels of serotonin in blood, platelet serotonin and 5-HIAA in urine, and tend to have typical carcinoid as primary tumor. Metastatic liver has been linked directly with the risk of carcinoid syndrome, acting permissively without inactivating the plasma secretion of vasoactive amines.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a> Clinically, carcinoid heart disease occurs with progressive symptoms and resembles a constrictive pericarditis with signs of right heart failure. If untreated, the median survival ranges from 12 to 38 months from onset of systemic symptomatology.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a> Treatment primarily involves symptomatic control with SSA. Prognosis is worse in patients with decompensated heart failure with NYHA functional class III/IV (median survival<span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>11 months).<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">5</span></a> Diuretics improve symptoms resulting from edema, but soon they reduce left ejection fraction, worsening asthenia. Treating the primary tumor does not improve the existing valvular involvement. Treatment with SSA has no significant impact on the remission of the valve lesions either. Valve replacement is the only procedure with a positive impact on the prognosis of the disease.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">6</span></a> It is indicated for symptomatic patients with acceptable control of the carcinoid syndrome symptoms and metastatic disease. Additionally, carcinoid syndrome is associated with high thrombotic risk, with a 4% risk of valve thrombosis, annually. The patient described showed extensive thrombosis of the right internal jugular vein in relation to blood stasis secondary to right HF and occurrence of procoagulant amines in the context of carcinoid syndrome. The patient described in this case was treated with SSA, and the secretory diarrhea was controlled. Valve replacement was not possible due to the advanced valvular dysfunction, extensive metastatic liver involvement and the high thrombotic risk.</p><p id="par0015" class="elsevierStylePara elsevierViewall">We describe an exceptional patient because of the carcinoid syndrome of unknown primary tumor whose initial symptomatology was congestive HF, predominantly right, with jugular vein thrombosis associated. The approach to carcinoid heart disease in these patients is very complex, and the effectiveness of medical treatments very limited when the disease is advanced; Further studies are required on the impact of SSA in patients with no extensive valvular involvement.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Cuesta Hernández M, Ruiz Gracia T, Estrada Lastra A, Díaz Pérez JÁ. Cardiopatía carcinoide como presentación inicial de síndrome carcinoide. Med Clin (Barc). 2015;145:324–325.</p>" ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:6 [ 0 => array:3 [ "identificador" => "bib0035" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Carcinoid heart disease. 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