Aortic diseases

ESC CardioMed ◽  
2018 ◽  
pp. 2861-2863
Author(s):  
Bernard Iung
Keyword(s):  
Aortic Valve ◽  
Aortic Dissection ◽  
Marfan Syndrome ◽  
Bicuspid Aortic Valve ◽  
Young Women ◽  
Beta Blockers ◽  
Aortic Aneurysms ◽  
Maximum Diameter ◽  
Aortic Diseases

Aortic diseases encountered in young women are mainly associated with syndromic diseases, which are often heritable, or bicuspid aortic valve. The most frequent syndromic disease is Marfan syndrome. In Marfan syndrome, the risk of aortic dissection is low during pregnancy when the maximum diameter of the ascending aorta is less than 45 mm. Dissection may affect the thoracic ascending or descending aorta. The risk of aortic dissection is low in bicuspid aortic valve when the aortic diameter is less than 50 mm. Beta blockers are recommended throughout pregnancy in Marfan syndrome and are often used in other causes of aortic aneurysms. Close echocardiographic follow-up is needed during pregnancy and after delivery.

Aortic diseases

ESC CardioMed ◽  
2018 ◽  
pp. 2861-2863
Author(s):  
Bernard Iung
Keyword(s):  
Aortic Valve ◽  
Aortic Dissection ◽  
Marfan Syndrome ◽  
Bicuspid Aortic Valve ◽  
Young Women ◽  
Beta Blockers ◽  
Aortic Aneurysms ◽  
Maximum Diameter ◽  
Aortic Diseases

Aortic diseases encountered in young women are mainly associated with syndromic diseases, which are often heritable, or bicuspid aortic valve. The most frequent syndromic disease is Marfan syndrome. In Marfan syndrome, the risk of aortic dissection is low during pregnancy when the maximum diameter of the ascending aorta is less than 45 mm. Dissection may affect the thoracic ascending or descending aorta. The risk of aortic dissection is low in bicuspid aortic valve when the aortic diameter is less than 50 mm. Beta blockers are recommended throughout pregnancy in Marfan syndrome and are often used in other causes of aortic aneurysms. Close echocardiographic follow-up is needed during pregnancy and after delivery.

scholarly journals Cardiomyopathy in Genetic Aortic Diseases

2021 ◽  
Vol 9 ◽  
Author(s):  
Laura Muiño-Mosquera ◽  
Julie De Backer
Keyword(s):  
Marfan Syndrome ◽  
Myocardial Dysfunction ◽  
Genetic Defect ◽  
Positive Family History ◽  
Aortic Disease ◽  
Aortic Aneurysms ◽  
Aortic Diseases ◽  
Pathogenic Variants ◽  
Fibrillin 1

Genetic aortic diseases are a group of illnesses characterized by aortic aneurysms or dissection in the presence of an underlying genetic defect. They are part of the broader spectrum of heritable thoracic aortic disease, which also includes those cases of aortic aneurysm or dissection with a positive family history but in whom no genetic cause is identified. Aortic disease in these conditions is a major cause of mortality, justifying clinical and scientific emphasis on the aorta. Aortic valve disease and atrioventricular valve abnormalities are known as important additional manifestations that require careful follow-up and management. The archetype of genetic aortic disease is Marfan syndrome, caused by pathogenic variants in the Fibrillin-1 gene. Given the presence of fibrillin-1 microfibers in the myocardium, myocardial dysfunction and associated arrhythmia are conceivable and have been shown to contribute to morbidity and mortality in patients with Marfan syndrome. In this review, we will discuss data on myocardial disease from human studies as well as insights obtained from the study of mouse models of Marfan syndrome. We will elaborate on the various phenotypic presentations in childhood and in adults and on the topic of arrhythmia. We will also briefly discuss the limited data available on other genetic forms of aortic disease.

Abstract 349: Outcomes Among Women With Bicuspid Aortic Valve and/or Bicuspid Aortopathy During Pregnancy

2020 ◽  
Vol 13 (Suppl_1) ◽  
Author(s):  
Sarina Meikle ◽  
Jenny Wu ◽  
David Bach ◽  
Marion Hofmann Bowman ◽  
Mark Norris ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Aortic Dissection ◽  
Bicuspid Aortic Valve ◽  
Gestational Age ◽  
Beta Blockers ◽  
Post Partum ◽  
Inclusion Criteria ◽  
Vaginal Deliveries ◽  
Increased Risk ◽  
Aortic Dissections

Background: Bicuspid aortic valve (BAV) is associated with an increased risk of aortic dissection, but little is known about pregnancy-associated outcomes. Methods: ICD codes and a text recognition program (EMERSE) were used to identify women with BAV and pregnancy (2000-2018) at a single center. Charts were manually reviewed to confirm the diagnoses, echocardiographic data, co-morbidities, and outcomes. Inclusion criteria were congenital BAV pathology and pregnancy. Primary endpoints included expansion of aortic aneurysm and aortic dissection during pregnancy and up to 6 weeks post-partum. Results: We identified 622 women with diagnoses of BAV and pregnancy. After detailed review, 72 women (87 pregnancies) met inclusion criteria. Mean gestational age at delivery was 36.9 weeks. Ten (11.5%) patients had hypertensive disorders of pregnancy, 7 (8.0%) had gestational diabetes, and 25 (28.7%) were treated with beta-blockers. Two (2.3%) pregnancies had intra-uterine growth restriction. Vaginal deliveries occurred in 56.5% and cesarean section in 36.3%. No Type A or B aortic dissections occurred. During pregnancy, there were no significant changes in the aortic dimensions. Prior to pregnancy, 6 women had aortic measurements > 40mm (mean pre-pregnancy 40.8 ± 3.2 mm; mean post-pregnancy 42.5 ± 3.9 mm). In this higher-risk group, mean gestational age was 38.7 weeks, 4 (66.7%) had vaginal delivery, 1 (16.7%) had preeclampsia, 4 (66.7%) were treated with beta-blockers, and all breastfed. There were no delivery complications, no aortic dissections, and no maternal or fetal deaths. Conclusions: Women with BAV appear to be at relatively low risk for acute aortic dissection during pregnancy.

scholarly journals Aortic dissection in Marfan syndrome: is bicuspid aortic valve (BAV) a risk factor?

2017 ◽  
Vol 9 (1) ◽  
pp. 74
Author(s):  
O. Milleron ◽  
D. Baghdadi ◽  
M. Langeois ◽  
M. Spentchian ◽  
F. Arnoult ◽  
...  
Keyword(s):  
Risk Factor ◽  
Aortic Valve ◽  
Aortic Dissection ◽  
Marfan Syndrome ◽  
Bicuspid Aortic Valve

ALTERATION OF ELASTIC PROPERTIES IN CONGENITAL/INHERITED AORTIC DISEASES: MRI STUDY IN MARFAN SYNDROME AND BICUSPID AORTIC VALVE

2006 ◽  
Vol 06 (01) ◽  
pp. 47-53
Author(s):  
LUIGI LOVATO ◽  
PAOLO SBARZAGLIA ◽  
SILVIA MAGI ◽  
VINCENZO RUSSO ◽  
ROMANO ZANNOLI ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Marfan Syndrome ◽  
Elastic Properties ◽  
Bicuspid Aortic Valve ◽  
Aortic Distensibility ◽  
Control Group ◽  
Aortic Dilation ◽  
Aortic Diseases ◽  
Magnetic Resonance Imaging Mri ◽  
Mri Study

Marfan Syndrome and Bicuspid Aortic Valve (BAV) are characterized by the congenital/inherited alteration of the fibrillin proteins of aortic wall extracellular matrix, with higher incidence of aortic dissection/rupture even without aortic dilation. Therefore, early identification and treatment of aortic involvement could improve prognosis. The purpose of this study was to evaluate aortic elastic properties of BAV and Marfan Syndrome patients to identify an index of early aortic involvement. Methods: A Magnetic Resonance Imaging (MRI) study was performed on 38 BAV patients, 20 Marfan patients and 14 healthy volunteers as a control group. A high-resolution gradient-echo sequence was applied in the axial plane at the level of pulmonary artery bifurcation to evaluate aortic distensibility. Results: Aortic distensibility was significantly different among the three groups (ANOVA, p = 0.0001) and was sensibly reduced in BAV (0.0056 ± 0.0048 versus 0.025 ± 0.006 control group) and Marfan (0.0085 ± 0.006 versus 0.025 ± 0.006 control group). Intraobserver and interobserver reproducibility for diastolic measurements were 1.2% and 0.4% respectively and for systolic measurements, they were 1.1% and 0.3% respectively. Conclusion: In BAV or Marfan Syndrome patients, MRI evaluation of abnormal distensibility may provide a reliable index of early aortic involvement, even before aortic dilation occurs.

Maternal and fetal outcomes in pregnancies complicated by Marfan syndrome

Heart ◽  
2019 ◽  
Vol 105 (22) ◽  
pp. 1725-1731 ◽  
Author(s):  
Matthew Cauldwell ◽  
Philip J Steer ◽  
Stephanie L Curtis ◽  
Aarthi Mohan ◽  
Samuel Dockree ◽  
...  
Keyword(s):  
Aortic Dissection ◽  
Marfan Syndrome ◽  
Beta Blockers ◽  
Type B ◽  
Maternal Deaths ◽  
Type B Dissection ◽  
Maternal And Neonatal Outcomes ◽  
Preconception Counselling ◽  
Aortic Size

ObjectivesInformation to guide counselling and management for pregnancy in women with Marfan syndrome (MFS) is limited. We therefore conducted a UK multicentre study.MethodsRetrospective observational study of women with MFS delivering between January 1998 and March 2018 in 12 UK centres reporting data on maternal and neonatal outcomes.ResultsIn total, there were 258 pregnancies in 151 women with MFS (19 women had prior aortic root replacements), including 226 pregnancies ≥24 weeks (two sets of twins), 20 miscarriages and 12 pregnancy terminations. Excluding miscarriages and terminations, there were 221 live births in 139 women. Only 50% of women received preconception counselling. There were no deaths, but five women experienced aortic dissection (1.9%; one type A and four type B—one had a type B dissection at 12 weeks and subsequent termination of pregnancy). Five women required cardiac surgery postpartum. No predictors for aortic dissection could be identified. The babies of the 131 (65.8%) women taking beta-blockers were on average 316 g lighter (p<0.001). Caesarean section rates were high (50%), particularly in women with dilated aortic roots. In 55 women, echocardiographic aortic imaging was available prepregnancy and postpregnancy; there was a small but significant average increase in AoR size of 0.84 mm (Median follow-up 2.3 months)ConclusionThere were no maternal deaths, and the aortic dissection rate was 1.9% (mainly type B). There with no identifiable factors associated with aortic dissection in our cohort. Preconception counselling rates were low and need improvement. Aortic size measurements increased marginally following pregnancy.

Genetic diseases of the aorta: aortic diseases related to Marfan syndrome and other genetic abnormalities

ESC CardioMed ◽  
2018 ◽  
pp. 2611-2613
Author(s):  
Bernard Iung
Keyword(s):  
Aortic Valve ◽  
Aortic Dissection ◽  
Bicuspid Aortic Valve ◽  
Aortic Surgery ◽  
Genetic Diseases ◽  
Rapid Progression ◽  
Aortic Diseases ◽  
Genetic Abnormalities ◽  
History Of ◽  
Associated Risk Factors

Bicuspid aortic valve affects 1–2% of babies at birth and is frequently associated with thoracic aortic aneurysm but the risk of aortic dissection is low. Indications for aortic surgery are based on studies on natural history. Aortic surgery is recommended when the maximum aortic diameter is greater than 55 mm in patients with a bicuspid aortic valve. Intervention is indicated at an earlier stage when there are associated risk factors, in particular familial history of aortic dissection or rapid progression, or if there is an indication for aortic valve replacement. The choice between partial or total replacement of the aorta should be based on anatomy, but also take into account the patient’s age and operative risk.

Genetic diseases of the aorta: aortic diseases related to Marfan syndrome and other genetic abnormalities

ESC CardioMed ◽  
2018 ◽  
pp. 2611-2613
Author(s):  
Bernard Iung
Keyword(s):  
Aortic Valve ◽  
Aortic Dissection ◽  
Bicuspid Aortic Valve ◽  
Aortic Surgery ◽  
Genetic Diseases ◽  
Rapid Progression ◽  
Aortic Diseases ◽  
Genetic Abnormalities ◽  
History Of ◽  
Associated Risk Factors

Bicuspid aortic valve affects 1–2% of babies at birth and is frequently associated with thoracic aortic aneurysm but the risk of aortic dissection is low. Indications for aortic surgery are based on studies on natural history. Aortic surgery is recommended when the maximum aortic diameter is greater than 55 mm in patients with a bicuspid aortic valve. Intervention is indicated at an earlier stage when there are associated risk factors, in particular familial history of aortic dissection or rapid progression, or if there is an indication for aortic valve replacement. The choice between partial or total replacement of the aorta should be based on anatomy, but also take into account the patient’s age and operative risk.

scholarly journals P1762 Natural history and outcome of adults with bicuspid aortic valve: a middle-aged french cohort of 541 patients

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
Y Bohbot ◽  
C Cheng ◽  
F Fay ◽  
F Elmkies ◽  
A L Gugeinheim ◽  
...  
Keyword(s):  
Survival Rate ◽  
Aortic Valve ◽  
Natural History ◽  
Aortic Dissection ◽  
Bicuspid Aortic Valve ◽  
Large Population ◽  
Relative Survival ◽  
Surgical Indication ◽  
Middle Aged

Abstract Funding Acknowledgements none Background No data are available regarding the natural history and outcomes of middle-aged European patients with bicuspid aortic valve(BAV), as the only 2 large studies were conducted on the other side of the Atlantic. Purpose To determine the frequency of cardiovascular events and to assess the survival relative to that of the general population(GP) in a large population of adults diagnosed with BAV according to the presence or the absence of a surgical indication at baseline. Methods Between 2005 and 2017, 350 patients without surgical indication (mean age 53, 71% men) and 191 patients with surgical indication (mean age 59, 71% men) at baseline were included. Median follow-up was 80 months. Results In the medical group, 34 deaths(9.7%) were recorded(10 cardiovascular). The 10-years survival rate was 88 ± 2% with a relative survival compared to the age-and-sex matched population of 98.8%. Nine patients(2.6%) were diagnosed with infective endocarditis (IE) and no aortic dissection occurred. Aortic valve and/or ascending aorta surgery was performed in 102 patients(29%) during follow-up. In the surgical group, 12 deaths(6.3%) were recorded(5 cardiovascular). The 10-years survival rate was 90 ± 3% with a relative survival compared to the matched population of 99.2%. Five patients(2.6%) were diagnosed with IE and no aortic dissection occurred. Conclusion Middle-aged adults with BAV have a high likelihood of surgery but a survival comparable to the GP. The rate of IE is low and no aortic dissection occurred in our study. Abstract P1762 Figure. a) survival curves B) surgery incidence

Sign in / Sign up

Export Citation Format

Share Document