scholarly journals Marfan syndrome in children and adolescents: an adjusted nomogram for screening aortic root dilatation

Heart ◽  
1998 ◽  
Vol 79 (1) ◽  
pp. 69-72 ◽  
Author(s):  
L. Rozendaal ◽  
M. Groenink ◽  
M. S J Naeff ◽  
R. C M Hennekam ◽  
A. A M Hart ◽  
...  
Keyword(s):  
Children And Adolescents ◽  
Marfan Syndrome ◽  
Aortic Root ◽  
Aortic Root Dilatation

scholarly journals Aortic Root Dilatation in Children and Adolescents At Al-Hawary General Hospital, & National Benghazi Cardiac Center -Libya

2021 ◽  
Vol 36 (4) ◽  
pp. 300-307
Author(s):  
Rasmia H. Feituri ◽  
Hanan El Megasbi ◽  
Mariam M. El maadani ◽  
Amal Khazm
Keyword(s):  
Aortic Valve ◽  
Children And Adolescents ◽  
Marfan Syndrome ◽  
Aortic Root ◽  
Ehlers Danlos Syndrome ◽  
Aortic Root Dilatation ◽  
Adrenergic Blockers ◽  
Danlos Syndrome ◽  
Cardiac Center

Isolated dilatation of the aortic root and/or ascending aorta is a rare but well-known cardiovascular manifestation, can be caused by a variety of congenital or acquired conditions; that lead to the weakening of the aortic wall. The study aimed to detect the cause and the rate of the aortic root dilatation in children and adolescents, and to assess the effect of the Beta-adrenergic blockers in preventing further dilatation in the aortic root. A case series study was perform with five years of follow-up at Al-Hawary General Hospital, National Benghazi Cardiac Center. A total of 91 patients were seen with ascending aortic dilatation and/or root dilatation during the period from 6/2016 - 6/2021 included in the study diagnosed by clinical examination, chest x-ray, and echocardiogram. The diagnosis in 34/91(37%) was Tetralogy of fallout (TOF) and truncus arteriosus, 57/91 (63%) was dilated aortic root, 25/57 (44%) bicuspid aortic valve (BAV), 22/57 (38.5%) Marfan syndrome, 4/57(7%) Noonan syndrome, 2/57(3.5%) Turner syndrome, 3/57(5%) Ehlers-Danlos syndrome, 1/57(2%) idiopathic. Follow-up results of three months – five years: 57/91 patients with aortic root dilatation were followed up, none of the Marfan syndrome and Ehlers-Danlos syndrome patients who received beta-blockers had shown progression in the dilatation of the aortic root, and all patients who had bicuspid aortic valve did not show any progression in the dilatation without using medication. Conclusions: Dilated aortic root is a common finding in Marfan syndrome, bicuspid aortic root, and Ehlers-Danlos syndrome, and its progress could be decreased by using beta-adrenergic blockers in rapidly progressing dilation.

scholarly journals Losartan for Preventing Aortic Root Dilatation in Patients with Marfan Syndrome: A Meta-Analysis of Randomized Trials

2019 ◽  
Vol 8 (2) ◽  
pp. 365-372 ◽  
Author(s):  
Ayman Elbadawi ◽  
Mohamed A. Omer ◽  
Islam Y. Elgendy ◽  
Ahmed Abuzaid ◽  
Ahmed H. Mohamed ◽  
...  
Keyword(s):  
Marfan Syndrome ◽  
Aortic Root ◽  
Randomized Trials ◽  
Meta Analysis ◽  
Aortic Root Dilatation

445Effects of losartan and beta-blockers on aortic root dilatation in patients with Marfan Syndrome - results of the extended COMPARE trial

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
M M Van Andel ◽  
J Jalalzadeh ◽  
R Indrakusuma ◽  
R Balm ◽  
J Timmermans ◽  
...  
Keyword(s):  
Root Growth ◽  
Marfan Syndrome ◽  
Aortic Root ◽  
Favourable Effect ◽  
Study Cohort ◽  
Aortic Root Dilatation ◽  
Dilatation Rate ◽  
Β Blocker ◽  
Β Blockers

Abstract Introduction Despite several randomized trials, beneficial effects of Losartan and β-blockers in adults with Marfan syndrome (MFS) are not entirely clear. The COMPARE trial previously showed a small but significant beneficial effect of Losartan on top of β-blocker use on aortic root dilatation rate. Yet, this effect was not reproduced by other trials, although in general a favourable effect of Losartan could be demonstrated. All trials in adults with MFS showed lower than expected aortic root dilatation rates, suggesting mildly affected study populations. Therefore we extended the follow-up period of the COMPARE trial up to 10 years and aimed to assess the clinical outcomes of the study cohort, as well as the effect of medication regimes on aortic root dilatation rates in the subgroup of patients with a native aortic root at initial randomization. Methods Patients previously enrolled in the COMPARE trial were retrospectively analyzed. Cardiovascular events (aortic dissections, ruptures and operations) and all-cause mortality were assessed. Individual aortic root dilatation rates were estimated in patients with a native aortic root at time of randomization on the basis of linear regression analysis of multiple transthoracic echocardiogram (TTE) results. Correlations between aortic root dilatation rates and cumulative Losartan or β-blocker treatment days were assessed with Spearman's rho (ρ). Results During a median follow-up of 8.0 years, two dissections and three deaths occurred in the 151 patients with a native aortic root at time of randomization. The 122 patients that were eligible for aortic root dilatation analysis, underwent a median of 6 TTEs. The median aortic root dilatation rate in these patients was 0.28 (interquartile range 0.09 - 0.59) mm/y. These patients were further classified as either patients with a stable aortic root (n=102) or with aortic root growth (n=20), based on the threshold of 0.9 mm per year. Patients with aortic root growth had significantly more aortic root replacements during follow-up (17/20 vs 18/102, P=0.001). Furthermore, aortic root dilatation rate was negatively correlated with the number of Losartan treatment days (ρ=−0.272, P=0.003), β-blocker treatment days (ρ=−0.217, P=0.017) and with the duration of follow-up (ρ=−0.437, P<0.001). Conclusions Our results support previous findings that Losartan and β-blockers appear to be equally effective on aortic root dilatation rate in Marfan Syndrome patients. The low event rate in the long term follow-up of this subgroup of the COMPARE trial represent a relatively mildly affected study population and an aggressive prophylactic surgical regime.

Zespół Marfana u 7-letniej dziewczynki – opis przypadku

2018 ◽  
Vol 22 (2) ◽  
Author(s):  
Małgorzata Ludzia ◽  
Radosław Pietrzak ◽  
Bożena Werner
Keyword(s):  
Marfan Syndrome ◽  
Aortic Root ◽  
Body Height ◽  
Premature Death ◽  
Joint Hypermobility ◽  
Left Ventricular ◽  
Ventricular Enlargement ◽  
Angiotensin Ii Receptor ◽  
Aortic Root Dilatation ◽  
Left Ventricular Enlargement

Marfan syndrome is a systemic, autosomal dominant connective tissue disorder with variable expressivity. An early diagnosis is challenging but important, because Marfan syndrome is associated with premature death in untreated patients. The authors present a case of a 7-year-old girl with Marfan syndrome. The child’s father was diagnosed with Marfan syndrome confirmed by genetic tests. The first symptoms of Marfan syndrome in the presented patient occurred at the age of 2 years, when she presented with mitral and tricuspid valve prolapse, scoliosis, joint hypermobility and body height above 97 percentile. In regular check-ups, aortic root dilatation and the enlargement of the left ventricle were first described one year later. It was decided to introduce beta blocker therapy. Due to the further progression of left ventricular enlargement the girl was given additionally angiotensin II receptor antagonist. In echocardiography follow up no increasing of the aortic root dilatation and the left ventricular enlargement is observed.

scholarly journals Incidence of Aortic Root Dilatation in Pectus Excavatum and Its Association With Marfan Syndrome

2008 ◽  
Vol 162 (9) ◽  
pp. 882 ◽  
Author(s):  
Diane Rhee ◽  
David Solowiejczyk ◽  
Karen Altmann ◽  
Ashwin Prakash ◽  
Welton M. Gersony ◽  
...  
Keyword(s):  
Marfan Syndrome ◽  
Aortic Root ◽  
Pectus Excavatum ◽  
Aortic Root Dilatation

Abstract 12138: Angiotensin Blockade Reduces Aortic Dilatation in Patients With Marfan Syndrome: A Meta-Analysis

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Partha Sardar ◽  
Amartya Kundu ◽  
Omosalewa Adenikinju ◽  
Gerald Pekler ◽  
Saurav Chatterjee ◽  
...  
Keyword(s):  
Marfan Syndrome ◽  
Aortic Root ◽  
Meta Analysis ◽  
Conclusive Evidence ◽  
Sensitivity Analyses ◽  
Current Evidence ◽  
Cochrane Central Register ◽  
Aortic Dilatation ◽  
Observation Study ◽  
Aortic Root Dilatation

Introduction: Recently few studies evaluated the effects of ACE/ARB in patients with Marfan syndrome. However, as majority of the studies were small and mainly pilot studies, conclusive evidence of benefit with ACE/ARB in this scenario is still pending. The objective of the present meta-analysis was to evaluate the effect of ACE/ARB on the progression of aortic root dilatation in patients with Marfan syndrome. Hypothesis: Treatment with ACE/ARBs is effective for the prevention of aortic-root enlargement in patients with Marfan syndrome. Methods: We searched PubMed, EMBASE, and Cochrane Central Register of Clinical Trials from the inception to April 30, 2013. The main outcome of the present analysis was the change in aortic root diameter with ACE/ABR therapy compared to control. The random effects model of DerSimonian and Laird was used. Results: Four studies were included in the final analysis; three studies were randomized trial and one was observation study. ACE/ARB significantly reduced enlargement of aortic root diameters compared to control; mean difference of -0.97 (95% confidence interval, -1.42 to -0.52; P<0.0001). Consistent benefit of ACE/ARB was observed in all sub-group and sensitivity analyses; against beta blocker, against placebo, in adult patients, with only ARB, analysis with the results from only RCTs. Conclusions: This is the first meta-analysis indicating a beneficial effect of ACE/ARB treatment on aortic root dilatation in patients with Marfan Syndrome. Current evidence supports the use of ARB, specially losartan for this indication.

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