The present study reports a case of biopsy-proven pulmonary veno-occlusive disease as a cause of severe pulmonary hypertension in a patient suffering from a chronic myeloproliferative disorder. The pulmonary disease evolved favourably under treatment with defibrotide, a pro-fibrinolytic medication used in hepatic veno-occlusive disease.KEYWORDS: Anagrelide, chronic myeloproliferative disorder, defibrotide, pulmonary hypertension, pulmonary veno-occlusive disease A 66-yr-old female was admitted to the intensive care unit (ICU) for severe dyspnoea at rest and minor exertion (New York Heart Association (NYHA) class IV). Her past medical history was marked by a myeloproliferative and myelodysplastic syndrome, which was treated with hydroxyurea for 4 yrs. There was no clinical or radiographical evidence of a preexisting pulmonary disease. Anagrelide, a selective inhibitor of megacaryocyte maturation, was introduced (4 mg?day -1 ) 6 weeks prior to admission because of refractory thrombocytaemia and hydroxyurea-induced neutropenia. After several weeks, the patient experienced a progressive worsening of dyspnoea resulting in orthopnoea. The patient also presented with a nonproductive cough, without fever.On admission to the ICU, oxygen saturation on air was 75% and increased to 99% upon oxygen supply. Bibasilar rales were present on lung auscultation. A chest radiograph showed bilateral pulmonary infiltrates suggestive of pulmonary oedema. Helical angio-computed tomography (CT), ventilation-perfusion scans and echoDoppler examination of the lower limb veins showed no pulmonary embolism or deep venous thrombosis. A bronchioloalveolar lavage demonstrated the absence of pathogenic organisms. Transthoracic echocardiography displayed a markedly dilated and hypocontractile right ventricle with paradoxical septal motion and an estimated pulmonary artery systolic pressure of .60 mmHg. There was no left sided myocardial (systolic and diastolic) dysfunction or valvular disease. High-resolution CT of the thorax showed dilatation of central pulmonary arteries, septal thickening, diffuse ground-glass opacities and bilateral pleural effusion ( fig. 1a). According to the clinical, echocardiographic and CT findings, pulmonary veno-occlusive disease (PVOD) was diagnosed. Right heart catheterisation confirmed pulmonary hypertension (pulmonary artery pressure: 69/31 mmHg; mean: 48 mmHg), and the pulmonary arterial occlusion pressure was 10 mmHg. The cardiac output was 3.9 L?min fig. 1b). Estimated systolic pulmonary artery pressure was 42 mmHg. Defibrotide was discontinued while enoxaparin (40 mg q.d.) and oral methylprednisolone (in tapered doses) were continued and the patient was discharged from hospital.The clinical symptoms relapsed 2 months later and the patient was readmitted to the ICU because of severe pulmonary oedema requiring noninvasive ventilation. Chest radiographs showed severe diffuse pulmonary infiltrates. Echocardiography and thoracic CT scans demonstrated the recurrence of right ventricular dysfunction, pulm...
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