Long-Term Risk Factors for Dilatation of the Proximal Aorta in a Large Cohort of Children With Bicuspid Aortic Valve

Blais, Samuel; Meloche-Dumas, Léamarie; Fournier, Anne; Dallaire, Frédéric; Dahdah, Nagib

doi:10.1161/circimaging.119.009675

Cited by 23 publications

(23 citation statements)

References 47 publications

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“…However, fetuses with aortic valve stenosis were excluded from our cohort as were fetuses with other lesions such as coarctation of the aorta with a known association with bicuspid aortic valve. However, in pediatric patients with BAV early signs of dilated ascending aorta in the absence of aortic stenosis or regurgitation and irrespective of the pattern of aortic cusp fusion has been reported 1–3 . Abnormal flow dynamics in the ascending aorta have been implicated in dilation of the ascending aorta in the presence of a bicuspid aortic valve, even in the absence of aortic valve stenosis 3,13 .…”

Section: Resultsmentioning

confidence: 99%

“…Dilatation of the ascending aorta is well described in children and adults most commonly in association with a bicuspid aortic valve (BAV) or connective tissue disorders such as Marfan syndrome and Loeys‐Dietz syndrome. With respect to BAV, which is present in 1%–2% of the population, recent data has confirmed that dilation of the ascending aorta may be present in infancy 1–3 . This is clinically important because severe dilatation of the ascending aorta is associated with an increased risk of aortic dissection 4 .…”

Section: Introductionmentioning

confidence: 99%

“…This is clinically important because severe dilatation of the ascending aorta is associated with an increased risk of aortic dissection 4 . Aortic valve morphology and coexistence of aortic stenosis and/or regurgitation are important predictors of progressive aortic dilatation 1–3 . However, even in the absence of BAV dysfunction, aortic dilation may occur, reflecting abnormal aortic wall properties with aortic elasticity 5 …”

Section: Introductionmentioning

confidence: 99%

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Dilated ascending aorta in the fetus

et al. 2021

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Introduction Prenatal recognition of dilated aortic root is extremely rare and there are significant challenges in counselling these patients. The primary aim of this case series is to describe the prevalence, associations and outcome of dilated ascending aorta diagnosed during fetal life. Methods This is a retrospective cohort study from two tertiary fetal cardiology centres. Dilated ascending aorta was defined as gestation‐specific standard deviation > 1.96 at some point during gestation. Results Sixteen infants were live born and underwent postnatal echocardiography. Prenatally suspected bicuspid aortic valve (BAV) (n = 6) was confirmed in 5 cases (83%) postnatally. Thirteen children have been followed up for a period of minimum one year. No connective tissue disease was found. Conclusions Prenatal dilated ascending aorta is a rare finding (0.06%). It is associated with BAV in 37% of cases and extracardiac abnormalities in 15.7%. Nuchal translucency measurement was >3.5 in 13% of cases. Connective tissue disease was not diagnosed postnatally. This is the largest prenatal cohort with dilated ascending aorta and postnatal outcomes to date. We showed a postnatal persistence of ascending aortic dilatation in 43% of babies. In the absence of extra‐cardiac abnormalities, medium term outcome appears good but postnatal surveillance of aortic dilation is required.

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Section: Resultsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Dilated ascending aorta in the fetus

et al. 2021

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“…AS is a very common valve disease, affecting between 2% and 4% of adults over the age of 65, and is associated with high mortality once it becomes symptomatic [ 1 ]. While tricuspid aortic valve degeneration is the most prevalent cause of AS in older individuals, bicuspid aortic valve degeneration is the most common cause of AS in younger people, with AS being one of the key determinants of ascending aortic dilatation in those patients [ 6 , 7 ]. HT has a high prevalence among patients with AS, leading to worse LV remodeling and faster degeneration and calcification of the valve [ 3 ].…”

Section: Epidemiologymentioning

confidence: 99%

Arterial Hypertension in Aortic Valve Stenosis: A Critical Update

Basile¹,

Fucile²,

Lembo³

et al. 2021

JCM

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Aortic stenosis (AS) is a very common valve disease and is associated with high mortality once it becomes symptomatic. Arterial hypertension (HT) has a high prevalence among patients with AS leading to worse left ventricle remodeling and faster degeneration of the valve. HT also interferes with the assessment of the severity of AS, leading to an underestimation of the real degree of stenosis. Treatment of HT in AS has not historically been pursued due to the fear of excess reduction in afterload without a possibility of increasing stroke volume due to the fixed aortic valve, but most recent evidence shows that several drugs are safe and effective in reducing BP in patients with HT and AS. RAAS inhibitors and beta-blockers provide benefit in selected populations based on their profile of pharmacokinetics and pharmacodynamics. Different drugs, on the other hand, have proved to be unsafe, such as calcium channel blockers, or simply not easy enough to handle to be recommended in clinical practice, such as PDE5i, MRA or sodium nitroprusside. The present review highlights all available studies on HT and AS to guide antihypertensive treatment.

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“…Bicuspid aortic valve (BAV) is associated with aortic root dilation and dissection in adults, but the age and conditions when dilation begins are unknown Patients with bicuspid aortic valve (BAV) have a higher risk of developing aortic valve dys-function and progressive proximal aorta dilatation, which can lead to aortic dissection. To this day, the identification of children at risk of developing severe aortic dilatation during their pediatric follow-up is still challenging because most studies were restricted to adult subjects (Blais et al, 2020).…”

Section: Introductionmentioning

confidence: 99%

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

Feituri

Megasbi

Maadani

et al. 2021

MJSc

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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.

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Long-Term Risk Factors for Dilatation of the Proximal Aorta in a Large Cohort of Children With Bicuspid Aortic Valve

Cited by 23 publications

References 47 publications

Dilated ascending aorta in the fetus

Dilated ascending aorta in the fetus

Arterial Hypertension in Aortic Valve Stenosis: A Critical Update

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

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