[Liver involvement in hereditary haemorrhagic telangiectasia (Rendu-Osler-Weber disease). Report of one case and review of the literature]. / Atteinte hépatique au cours de la maladie de Rendu-Osler. A propos d'un cas et revue de la littérature.

INTRODUCTION: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular dysplasia; mutations in at least three genes (ENG, ACVRL1 et MADH4), which are components of transforming growth factor (TGF)-beta, may lead to the clinical picture of HHT. HHT is a multisystemic angiodysplasia, resulting in multiple vascular malformations, involving notably the liver. EXEGESIS: We report the case of a patient with anicteric cholestasis, revealing HHT. Abdominal CT-scan revealed hepatic artery dilation and multiple arteriovenous fistula. At 2-year follow-up, the patient exhibited liver involvement-associated high output cardiac insufficiency. CONCLUSION: Liver involvement is frequent in HHT, occurring in 8-31% of patients; it may lead to life-threatening complications, such as high output cardiac failure, portal hypertension or severe cholangitis. Abdominal Doppler ultrasonography is a non-invasive accurate method, suitable for first-line imaging of the liver in patients with HHT; it should be done in all patients, in order to detect HHT-related hepatic vascular malformations.

[Balloon aortic valvuloplasty in the adult. Immediate results and in-hospital complications in the latest series of 141 consecutive patients at the University Hospital of Rouen (2002-2005)]. / Valvuloplastie aortique par ballonnet chez l'adulte. Résultats immédiats et complications intrahospitalières dans la dernière série de 141 patients consécutifs au CHU de Rouen (2002-2005).

UNLABELLED: With the aging population, elderly patients with degenerative, severely calcified, and symptomatic aortic stenosis are becoming a frequent problem in medical decision making, particularly because many are declined for surgical valve replacement. For these patients, balloon aortic dilatation could be a palliative treatment. PATIENTS AND METHODS: we analyzed retrospectively our techniques and complications during hospitalization for percutaneous aortic balloon dilatation in 141 consecutive patients (45% women, mean age 83 +/- 10 y) from January 2002 to April 2005. The Parsonnet's score was 41 +/- 8 and the Euroscore was 10 +/- 2. We excluded the patients enrolled for percutaneous aortic valve implantation. This technique was performed under local anesthesia, from retrograde or antegrade approach. Rapid pacing stimulation (220/bpm) with a pacing-lead placed in the right ventricle was performed during balloon inflation. RESULTS: the aortic valve area was increased from 0.59 +/- 0.19 to 1.02 +/- 0.34 cm2 (p < 0.001) and the transvalvular mean gradient decreased from 49.3 +/- 21.2 to 22.2 +/- 11.8 mmHg (p < 0.001). Six patients (4%) died (3 during aortic valve dilatation) and 9 patients (6%) had non fatal complications: 2 transitory strokes, 8 non surgical vascular complications and 5 episodes of atrio-ventricular complete block (these complication were non exclusive). The patients were discharged at 5, 6 +/- 3 days. CONCLUSION: In the hands of skilled operators, aortic percutaneous balloon dilatation has become a simple technique, associated with a low risk of complications despite a very ill patient population. This technique remains a good option for a number of patients before the onset of percutaneous aortic valve implantation.