Platypnea-orthodeoxia syndrome and Budd–Chiari syndrome: An unreported association

Demelo-Rodríguez, Pablo; del Toro-Cervera, Jorge

doi:10.1016/j.medcle.2014.03.002

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Said obstruction may occur in the hepatic veins or suprahepatic inferior vena cava. When the obstruction is caused by a thrombosis, it is called primary BCS. If it is caused by a tumour, it is called secondary BCS.<a class="elsevierStyleCrossRef" href="#bib0035">1</a>We presented the case of a 35-year-old female patient who, 2 years before, had been diagnosed with primary BCS during pregnancy (week 11), evidenced by ascites and dyspnoea. The hepatic Doppler scanning showed thrombosis of the right and left suprahepatic veins and reduced flow in the middle suprahepatic vein. Anticoagulation therapy was started with low-molecular-weight heparin, and the patient gave birth on week 38 through vaginal delivery using forceps. In the subsequent outpatient study, the patient was diagnosed with polycythemia vera (JAK2+, typical bone marrow biopsy and normal karyotype). Therefore, anticoagulation therapy was indefinitely maintained with acenocumarol.During the BCS progress control, a year after the diagnosis, there was still no flow in the right and left suprahepatic veins, and the middle suprahepatic vein presented a large venous vessel compatible with collateral circulation. The portal vein was normal and presented hepatopetal circulation (at a speed of 23cm/s). The liver and kidney functions were normal, and the endoscopic test ruled out the presence of varicose veins.The patient attended our practice because she had moderate-mild effort dyspnoea, with predominance during orthostatism (and, in particular, in some positions, such as when leaning forward) and improving in the decubitus position (platypnea). It had no other related symptoms.The physical examination revealed hepatomegaly of 2 finger breadths, without any other pathological findings. The baseline saturation in oxygen was normal. As to the complementary tests, the electrocardiogram, the cardiac stress test, the spirometry and the diffusion test were normal. A transthoracic echocardiogram revealed interauricular septal aneurysm with the presence of a permeable oval foramen (POF) with a right to left short circuit and high bubble load. The rest of the results were normal. Due to diagnostic suspicion, the cardiac stress test was repeated and the patient was specifically asked to lean forward during the test. In this way, she showed a sudden decrease in the oxygen saturation at maximum effort (up to a 78%) and a sudden decrease in the respiratory reserve from 70 (6min in the Bruce protocol) to 48% at maximum effort.The oval foramen was closed by means of a percutaneous catheter procedure using an Amplatzer cribriform device of 25mm, with good results and no complications.First described in 1949,<a class="elsevierStyleCrossRef" href="#bib0040">2</a> the POS results from a short circuit of non-oxygenated blood from the right auricle to the left auricle through an opening in the interauricular septum. The POS is characterised by the presence of two components: on the one hand, an interauricular short circuit or shunt (as an oval foramen or auricular communication) or an intrapulmonary short circuit (as the hepatopulmonary syndrome or pulmonary arteriovenous malformations).<a class="elsevierStyleCrossRef" href="#bib0045">3</a> On the other hand, the syndrome requires a functional component that favours the right-left short circuit when the patient moves from the decubitus position to orthostatism. This could be a defect in the auricular septum or right auricle, which increases blood flow from the inferior vena cava through the auricular defect. In this regard, various related diseases have been described, such as pneumonectomy, pulmonary hypertension or pleural effusion.<a class="elsevierStyleCrossRefs" href="#bib0050">4,5</a>Though the POF is present in up to one third of the general population,<a class="elsevierStyleCrossRef" href="#bib0060">6</a> it almost never has clinical manifestations. The most frequent presentation of the POS is a patient with an asymptomatic POF who, after developing a disease which favours increased pressure in the right cavities, has a clinical manifestation of dyspnoea and unsaturation with orthostatism. In our patient, the decrease in the flow of suprahepatic veins and the development of collaterals meet the requirements of a position-related redirection of the venous flow through the inferior vena cava to the auricular septum. The presence of this disease and an oval foramen would be the two necessary requirements for the diagnosis of BCS-related POS. There are currently no scientific publications on POS cases related to this condition.To date, the subsequent progress of the patient has been favourable, and dyspnoea related to position changes and mild efforts has not been observed. Moderate-effort mild dyspnoea, which is probably related to the baseline haematological disease, has persisted." "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "Please cite this article as: Demelo-Rodríguez P, del Toro-Cervera J. Síndrome de platipnea-ortodeoxia y síndrome de Budd-Chiari: una asociación inédita. Med Clin (Barc). 2015;144:94–95." ] ] "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" => "Budd–Chiari syndrome: a review by an expert panel" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:6 [ 0 => "H.L. Janssen" 1 => "J.C. Garcia-Pagan" 2 => "E. Elias" 3 => "G. Mentha" 4 => "A. Hadengue" 5 => "D.C. 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