The importance of genotype-phenotype correlation in the clinical management of Marfan syndrome

VM Becerra-Muñoz, JJ Gómez-Doblas… - Orphanet journal of rare …, 2018 - Springer
Background Marfan syndrome (MFS) is a disorder of autosomal dominant inheritance, in
which aortic root dilation is the main cause of morbidity and mortality. Fibrillin-1 (FBN-1) …

Personalised external aortic root support for elective treatment of aortic root dilation in 200 patients

L Van Hoof, F Rega, T Golesworthy, P Verbrugghe… - Heart, 2021 - heart.bmj.com
Background and objectives In personalised external aortic root support (PEARS), a custom-
made, macroporous mesh is used to stabilise a dilated aortic root and prevent dissection …

Elective David I procedure has excellent long-term results: 20-year single-center experience

ML Shrestha, E Beckmann, F Abd Alhadi… - The Annals of thoracic …, 2018 - Elsevier
Background We present our results after elective, isolated David I procedures over the past
20 years. Methods Between 1993 and 2015, 197 patients (mean age 46±17 years, 73 …

Valve-sparing aortic root replacement (David I procedure) in Marfan disease: single-centre 20-year experience in more than 100 patients

A Martens, E Beckmann, T Kaufeld… - European Journal of …, 2019 - academic.oup.com
OBJECTIVES Valve-sparing aortic root replacement (VSARR) is recommended for patients
with aortic root dilatation and preserved aortic valve cusp morphology. The durability of …

Three-dimensional flows of incompressible Navier–Stokes fluids in tubes containing a sinus, with varying slip conditions at the wall

R Chabiniok, J Hron, A Jarolímová, J Málek… - International Journal of …, 2022 - Elsevier
The objective of this study is to understand the formation of vortices and other flow
characteristics associated with the three-dimensional motions of an incompressible Navier …

Marfan syndrome: A therapeutic challenge for long-term care

AH Wagner, M Zaradzki, R Arif, A Remes… - Biochemical …, 2019 - Elsevier
Marfan syndrome (MFS) is an autosomal dominant genetic disorder caused by mutations in
the fibrillin-1 gene. Acute aortic dissection is the leading cause of death in patients suffering …

[HTML][HTML] Aortic annulus does not dilate over time after aortic root remodeling with or without annuloplasty

T Kunihara, S Arimura, F Sata, C Giebels… - The Journal of Thoracic …, 2018 - Elsevier
Objective The lack of annular stabilization is the drawback of aortic root remodeling, and
recently the addition of annuloplasty has been proposed. Limited data, however, exist on …

Bicuspid aortic insufficiency with aortic root aneurysm: root reimplantation versus Bentall root replacement

P Vallabhajosyula, WY Szeto, A Habertheuer… - The Annals of Thoracic …, 2016 - Elsevier
Background In patients with a bicuspid aortic valve presenting with aortic insufficiency (AI)
and root aneurysm, we assessed whether outcomes with primary cusp repair with root …

[HTML][HTML] Reimplantation valve-sparing aortic root replacement is the most durable approach to facilitate aortic valve repair

T David - JTCVS techniques, 2021 - Elsevier
Reimplantation of the aortic valve has been performed for 3 decades, and experience shows
that patient selection and meticulous operative technique are crucial to provide excellent …

[HTML][HTML] Composite valve graft implantation for the treatment of aortic valve and root disease: results in 1045 patients

L Di Marco, D Pacini, A Pantaleo, A Leone… - The Journal of Thoracic …, 2016 - Elsevier
Objectives Aortic root replacement using a composite graft is the treatment of choice for a
large variety of aortic root conditions with a diseased aortic valve. The objective of the …