Source: International Congress 2016 – Pulmonary hypertension and pulmonary embolism: from the bench to the bedside
Disease area: Interstitial lung diseases, Pulmonary vascular diseases

Abstract

Introduction: PAD is an extremely rare condition. Over the past two centuries, around 70 cases of pulmonaryartery dissection have been described, of which only nine were diagnosed during lifetime.Case report: A 39 year old man presented in the pulmonary clinic with notable exertional dyspnea, cough and occasional fever, which lasted for a month before admission. Blood gas analysis showed severe hypoxemia (PaO2 4.3 kPa). The patient was diagnosed 7 years ago with diffuse interstitial lung disease-desquamative pneumonia using transbronchial biopsy. The patient received corticosteroid and immunosuppressive treatment. Echocardiography displayed increased mean pulmonary artery pressure (PAP) >70 mmHg, TAPSE 17 mm. The HRCT imaging showed signs of chronic pulmonary embolism of the left pulmonary artery with infarction formation, which was confirmed with VP scan. MRI showed marked enlargement of the right heart (ventricle 64.2 mm, atrium 57 mm), pericardial effusion and dissection of the left branch of the pulmonary artery with flap formation. The patient was assessed eligible for anticoagulant treatment, which led to improvement of the dyspnea. Surgical treatment was discussed, but assumed not appropriate due to severe hypoxemia. He was discharged with recommendation for home oxygen treatment and oral anticoagulant therapy. Patient follow up for 5 years showed stable condition (PAP ~100 mmHg) and no further progression of PAD on control MRI.The co-existence of interstitial lung disease with CTEPH and PAD is very rare and physicians should be aware of this possibility. There should be multidisciplinary approach to these patients.


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Citations should be made in the following way:
Velin Stratev (Varna, Bulgaria), Velin Stratev, Valentina Dimitrova, Tanya Dobreva, Diana Petkova. Pulmonary artery dissection (PAD) in a patient with diffuse interstitial lung disease and chronic thromboembolic pulmonary hypertension (CTEPH). Eur Respir J 2016; 48: Suppl. 60, 2491

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