Losartan Versus Atenolol for Prevention of Aortic Dilation in Patients With Marfan Syndrome

Teixidó-Turà, Gisela; Forteza, Alberto; Palomares, José F. Rodríguez; Mirelis, J. González; Gutiérrez, Laura; Sánchez, Violeta; Ibáñez, Borja; Garcı́a-Dorado, David; Evangelista, Artur

doi:10.1016/j.jacc.2018.07.052

Cited by 87 publications

(51 citation statements)

References 14 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…MFS patients were classified according to the past aortic dilation rate as following: four stable (if the growing rate was less than 0.6 mm year −1 ) and four dilating (if it was greater than or equal to 0.6 mm year −1 ). This threshold was established based on the reported average growth rate of MFS patients [31].…”

Section: Methodsmentioning

confidence: 99%

Fluid–structure interaction simulations outperform computational fluid dynamics in the description of thoracic aorta haemodynamics and in the differentiation of progressive dilation in Marfan syndrome patients

et al. 2020

View full text Add to dashboard Cite

show abstract

Section: Methodsmentioning

confidence: 99%

Fluid–structure interaction simulations outperform computational fluid dynamics in the description of thoracic aorta haemodynamics and in the differentiation of progressive dilation in Marfan syndrome patients

et al. 2020

View full text Add to dashboard Cite

show abstract

“…Clinical trials with losartan have shown variable results. Studies in the Netherlands 50 and the USA 51 showed a significant reduction in growth of aortic diameter in Marfan patients that used losartan, compared to patients that used beta blockers, whereas this was not confirmed by studies in France 52 and Spain 53 . A meta analysis of clinical trials with losartan, comprising 1398 subjects, revealed no significant effect of losartan 54 .…”

Section: Medicationmentioning

confidence: 95%

Marfan Syndrome, A Review

Pals

2018

J. Biomed. Transl. Res.

View full text Add to dashboard Cite

Marfan syndrome is named after the French pediatrician Antoine Bernard-Jean Marfan who described in 1896 a girl with arachnodactyly and long limbs1. The patient also had congenital contractures of the elbows and would not fulfill the current criteria for Marfan syndrome. She probably was suffering from a condition that we now call contractural arachnodactyly, caused by mutations in the FBN2 gene.The clinical features of Marfan syndrome affect many systems of the body. The most obvious are the skeletal features, long limbs, tall stature, long thin fingers (arachnodactyly or spider fingers). The skeletal features can be scored objectively as: arm span more than 1.05 x body length; wrist sign (thumb and index finger can encircle the wrist of the other hand with at least one digit overlap) and thumb sign (when making a fist around the thumb, one digit of the thumb sticks out). The main neurological symptom is dural ectasias. The most severe symptoms are cardiovascular: mitralis valve prolapse, aortic dilatation and thoracic aortic aneurysms and dissections, which may lead to sudden death5. However, I noticed in discussions with patients that they often consider the ocular symptoms, severe myopia and lens luxation, the worst for themselves, because the latter may lead to blindness.

show abstract

“…Based on the implication of TGF-β signaling observed in MFS, type-1 angiotensin receptor blockers (ARBs) which antagonize the pathway, have also been used in adults and children to reduce rate of aneurysm growth (Chiu et al, 2013;Franken et al, 2015;Groenink et al, 2013). The differential benefit of β-blockade versus ARBs, however, remains less well defined, although a recent trial with the potent ARB irbesartan in MFS was effective in controlling aortic root growth (Forteza et al, 2016;Lacro et al, 2013;Teixido-Tura et al, 2018).…”

Section: Managementmentioning

confidence: 99%

The genetics of aortopathies: Hereditary thoracic aortic aneurysms and dissections

Chou

Lindsay

2020

American J of Med Genetics Pt C

View full text Add to dashboard Cite

Aortopathies encompass a variety of inherited and acquired pathologies that increase risk of life‐threatening dissection or rupture. Identifying individuals with hereditary thoracic aortic aneurysm and dissection (HTAAD) for longitudinal monitoring, medical therapy, or elective and preventative repair is paramount to reduce risk of cardiovascular‐related mortality and complications from dissection and rupture. Over the past couple of decades, pathogenic variants in numerous genes have been identified in relation to HTAAD. The genetic diagnosis can help stratify patient risk and provide guidance on medical treatment, timing of prophylactic surgical repair, as well as longitudinal surveillance and imaging. Implicated genes and their associated proteins have been found to act on a diverse variety of pathways, cells and structural components linked to transforming growth factor beta (TGF‐β) signaling pathways, disruption of the vascular smooth muscle cell contractile apparatus, and primary disruption of extracellular matrix homeostasis. This review describes relevant genetic variants that may help identify and guide the management of hereditary thoracic aortic aneurysms and dissections.

show abstract

Losartan Versus Atenolol for Prevention of Aortic Dilation in Patients With Marfan Syndrome

Cited by 87 publications

References 14 publications

Fluid–structure interaction simulations outperform computational fluid dynamics in the description of thoracic aorta haemodynamics and in the differentiation of progressive dilation in Marfan syndrome patients

Fluid–structure interaction simulations outperform computational fluid dynamics in the description of thoracic aorta haemodynamics and in the differentiation of progressive dilation in Marfan syndrome patients

Marfan Syndrome, A Review

The genetics of aortopathies: Hereditary thoracic aortic aneurysms and dissections

Contact Info

Product

Resources

About