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Clinical cases

Paradoxical arterial hypoxemia in a left-to-right shunt congenital heart disease

Efrén Martínez-Quintana, Fayna Rodríguez-González
1. Cardiology Service, Insular-Materno Infantil University Hospital, Las Palmas de Gran Canaria, Spain 2. “Dr. Negrín” University Hospital, Las Palmas de Gran Canaria, Spain


The hepatopulmonary syndrome is a rare complication of different types of chronic hepatic diseases with associated portal venous hypertension, resulting in pulmonary vascular dilatation, predominantly in the lower lung fields, and leading to ventilationperfusion mismatch, arterial hypoxemia and a poor prognosis. We present the case of 42-year-old male patient with an anomalous drainage of the right superior pulmonary vein into the azygos vein and a portal vein cavernomatosis with associated portal venous hypertension who presented severe oxygen desaturation, during exercise, in the context of a hepatopulmonary syndrome.

Keywords: hepatopulmonary syndrome, hypoxia, portal hypertension, congenital heart disease

Delayed diagnosis in a case of granulomatosis with polyangiitis (Wegener’s) with initial predominance of joint involvement

Anca Macri, Ruxandra Ulmeanu, Florin Mihălţan, Gabriela Popa, Radu Stoica
Institutul de Pneumoftiziologie „Marius Nasta” București 1. Secţia Pneumologie III 2. Serviciul de Bronhologie 3. Secţia ATI
The authors present the case of a 53-year-old female, initially admitted in a rheumatology department for fever and diffuse arthritis – being diagnosed with sero-positive rheumathoid arthritis. Although the chest X-ray and CT scan of thorax showed several abnormal features (medium lobe atelectasis, pseudo-cyst in the posterior segment of the right upper lobe with satellite milliary nodules, mediastinal lymph node enlargement), the investigations performed in our pneumology department couldn’t establish the etiology of radiological abnormalities. With non-steroidal antiinflamatory treatment, the patient got worse, being readmitted in our hospital after 3 months for high fever, diffuse arthralgia with functional impairment, small hemoptysis, loss of hearing and left ear ache and on chest X-ray with bilateral macronodules, some of these with cavitation. The investigations showed a slight alveolar hemorrhagic syndrome, positive cANCA antibodies, negative antiCCP antibodies – the diagnosis of Wegener’s granulomatosis with lung and ENT involvement being established. Puls-therapy with Solumedrol and i.v. Cyclophosphamide was thereafter initiated with a favorable evolution. This case is special because of the initial misdiagnosis due to the atypical pulmonary manifestations and the non-specific paraclinical findings, in the context of diffuse arthritis with positive rheumatoid factor.
Keywords: granulomatosis with polyangiitis, Wegener, rheumatoid factor, arthritis, alveolar hemorrhagic syndrome