Abstract (EN):
Background: The Osler-Weber-Rendu syndrome (OWRS), also known as hereditary hemorrhagic telangiectasia, is a rare vascular pathology, inherited as an autosomal dominant trait. Typical clinical manifestations includes multiorgan arteriovenous malformations (AVMs) and associated hemorrhagic complications (epistaxis, gastrointestinal bleeding, etc.).
Case report: We describe the case of a 63 year-old man, diabetic, hypertensive, dyslipidemic and former smoker, with OWRS and history of previous surgical resections of a pulmonary arteriovenous fistula and of a brain abscess complicated with sequelar epilepsy.
In February 2007, he was admitted to the Cardiology Department for acute anterior wall myocardial infarction. The emergent cardiac catheterization revealed occlusion of the middle segment of the left anterior descending artery (LAD) and the patient underwent lesion angioplasty with drug eluting stent implantation. The echocardiogram showed moderate left ventricular systolic dysfunction (LVSD).
He remained clinically stable until October 2010, when he presented with anemia, probably related to recurrent epistaxis episodes, leading to interruption of aspirin therapy. Four days after antithrombotic therapy interruption, the patient was admitted to the emergency room with persistent chest pain and he was diagnosed with acute anterior wall myocardial infarction. He was treated with loading doses of double anti-aggregation and underwent emergent catheterization which revealed intra-stent thrombotic occlusion of the LAD. Angioplasty of the intra-stent lesion was done by thrombus aspiration, with no complication. Analytically, there was a hypochromic microcytic anemia (Hb: 8.8 g/dL), requiring transfusion support. Echocardiographic evaluation showed severe LVSD. Neverthless, the patient had a clinical favorable evolution, under double antiplatelet therapy, maintaining stable anemia and no apparent blood loss.
Conclusion: Appropriateness of anti-thrombotic therapy, in groups at risk, as the OWRS patients, requires a careful and continuing evaluation to ensure that the risks of bleeding do not outweigh the benefits from antithrombotic therapy. This case report shows how this clinical approach may be so challenging.
Language:
English
Type (Professor's evaluation):
Scientific
Notes:
Rangel I, Sousa C, Oliveira S, Lebreiro A, Sousa A, Correia AS, Paiva M, Ribeiro V, Melão F, Araújo V, Maciel MJ. Osler-Weber-Rendu syndrome: the challenging approach to antithrombotic therapy. Eur J Heart Fail Suppl 2012; 11(Suppl 1): P523. ISSN 1567-4215. Heart Failure Congress 2012, Belgrade, 2012