title: [Aortic root dilatation rate in pediatric patients with Marfan syndrome treated with losartan].
abstract: BACKGROUND
  Medical therapy with angiotensin II receptor blockers/angiotensin-converting enzyme inhibitors and/or beta-blockers was reported to reduce aortic root dilatation rates in pediatric patients with Marfan syndrome. No data are available in the literature on losartan effects after 3 years of therapy. The aim of our study was to establish whether losartan reduces aortic root dilatation rates in pediatric patients with Marfan syndrome in the mid and long term.
  
  
  METHODS
  This is a retrospective analysis of 38 pediatric patients with Marfan syndrome followed at the Marfan Clinic of S. Orsola-Malpighi Hospital of the University of Bologna (Italy). Aortic diameters were measured at sinuses of Valsalva and proximal ascending aorta with transthoracic echocardiography.
  
  
  RESULTS
  After a mean follow-up of 4.5 ± 2.5 years (range 2-9 years), aortic root z score at sinuses of Valsalva and proximal ascending aorta remained stable. The average annual rate of change in aortic root z score was -0.1 ± 0.4 and 0 ± 0.3 at sinuses of Valsalva and proximal ascending aorta, respectively. The mean dose of losartan was 0.7 ± 0.3 mg/kg/day. Three patients were non-responders, probably because of late beginning or low dose of therapy. Eight patients underwent cardiac surgery (aortic root surgery in 5 and mitral valve repair in 3), all of them started losartan later in life.
  
  
  CONCLUSIONS
  Despite the retrospective design of the study and the small sample size, a beneficial effect of losartan therapy was observed in pediatric patients with Marfan syndrome in the mid and long term. Late beginning or low doses of losartan can turn off the effects of therapy.
authors.affiliation: Cardiologia e Cardiochirurgia Pediatrica e dell'Età Evolutiva, Centro Marfan, Policlinico S. Orsola-Malpighi, Università degli Studi, Bologna.
authors.forename: Elisabetta
authors.initials: E
authors.lastname: Mariucci
authors.affiliation: Cardiologia e Cardiochirurgia Pediatrica e dell'Età Evolutiva, Centro Marfan, Policlinico S. Orsola-Malpighi, Università degli Studi, Bologna.
authors.forename: Marta
authors.initials: M
authors.lastname: Guidarini
authors.affiliation: Cardiologia e Cardiochirurgia Pediatrica e dell'Età Evolutiva, Centro Marfan, Policlinico S. Orsola-Malpighi, Università degli Studi, Bologna.
authors.forename: Andrea
authors.initials: A
authors.lastname: Donti
authors.affiliation: Cardiologia e Cardiochirurgia Pediatrica e dell'Età Evolutiva, Centro Marfan, Policlinico S. Orsola-Malpighi, Università degli Studi, Bologna.
authors.forename: Luigi
authors.initials: L
authors.lastname: Lovato
authors.affiliation: Cardiologia e Cardiochirurgia Pediatrica e dell'Età Evolutiva, Centro Marfan, Policlinico S. Orsola-Malpighi, Università degli Studi, Bologna.
authors.forename: Anita
authors.initials: A
authors.lastname: Wischmeijer
authors.affiliation: Cardiologia e Cardiochirurgia Pediatrica e dell'Età Evolutiva, Centro Marfan, Policlinico S. Orsola-Malpighi, Università degli Studi, Bologna.
authors.forename: Emanuela
authors.initials: E
authors.lastname: Angeli
authors.affiliation: Cardiologia e Cardiochirurgia Pediatrica e dell'Età Evolutiva, Centro Marfan, Policlinico S. Orsola-Malpighi, Università degli Studi, Bologna.
authors.forename: Gaetano D
authors.initials: GD
authors.lastname: Gargiulo
authors.affiliation: Cardiologia e Cardiochirurgia Pediatrica e dell'Età Evolutiva, Centro Marfan, Policlinico S. Orsola-Malpighi, Università degli Studi, Bologna.
authors.forename: Fernando M
authors.initials: FM
authors.lastname: Picchio
authors.affiliation: Cardiologia e Cardiochirurgia Pediatrica e dell'Età Evolutiva, Centro Marfan, Policlinico S. Orsola-Malpighi, Università degli Studi, Bologna.
authors.forename: Marco
authors.initials: M
authors.lastname: Bonvicini
mesh_terms: D000293:Adolescent; D047228:Angiotensin II Type 1 Receptor Blockers / Q000627:therapeutic use*; D001021:Aortic Valve; D000082882:Bicuspid Aortic Valve Disease; D002648:Child; D002675:Child, Preschool; D004108:Dilatation, Pathologic / Q000517:prevention & control; D005260:Female; D006330:Heart Defects, Congenital / Q000209:etiology* / Q000517:prevention & control*; D006349:Heart Valve Diseases / Q000209:etiology* / Q000517:prevention & control*; D006801:Humans; D019808:Losartan / Q000627:therapeutic use*; D008297:Male; D008382:Marfan Syndrome / Q000150:complications*; D012189:Retrospective Studies
chemical_list: D047228:Angiotensin II Type 1 Receptor Blockers; D019808:Losartan