scholarly journals The bicuspid aortic valve and related disorders

2010 ◽  
Vol 128 (5) ◽  
pp. 296-301 ◽  
Author(s):  
Shi-Min Yuan ◽  
Hua Jing
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Thrombus Formation ◽  
Beta Blockers ◽  
Ductus Arteriosus ◽  
Treatment Strategies ◽  
Hereditary Diseases ◽  
Laboratory Findings ◽  
Male Predominance ◽  
Congenital Cardiac Malformation

Bicuspid aortic valve (BAV) is the most common congenital cardiac malformation, affecting 1-2% of the population, with strong male predominance. Individuals may have a normally functioning BAV, and may be unaware of its presence and the potential risk of complications. However, they may easily develop aortic valve disorders: either stenotic or regurgitant, or both. Today, BAV is recognized as a syndrome incorporating aortic valve disorders and aortic wall abnormalities, including aortic dilation, dissection or rupture. Congenital or hereditary diseases such as ventricular septal defect, patent ductus arteriosus, coarctation of the aorta, Turner's syndrome, Marfan's syndrome etc., may frequently be associated with BAV. Infective endocarditis and occasionally thrombus formation may develop during the lives of BAV patients. Elevated cholesterol or C-reactive protein may be seen in laboratory findings of these patients. Beta-blockers and statins are the possibilities for medical treatment, and aortic valve repair/replacement and ascending aorta replacement are indicated for patients with a severely diseased aortic valve and aorta. Rigorous follow-up throughout life is mandatory after BAV has been diagnosed. The aim of the present article was to describe the implications of BAV and its associated disorders, and to discuss diagnostic and treatment strategies.

scholarly journals Cardiac disease and pregnancy: hyper vigilance and extreme caution for optimal outcome

2020 ◽  
Vol 9 (2) ◽  
pp. 825
Author(s):  
Naresh Dhawan ◽  
Rohin Kumar ◽  
Reema Kumar Bhatt
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Cardiac Disease ◽  
Congenital Heart ◽  
Single Ventricle ◽  
Heart Diseases ◽  
Fetal Outcome ◽  
Coordinated Care ◽  
Extreme Caution ◽  
Congenital Cardiac Malformation

Cardiac disease in pregnancy is a leading cause of maternal death in more so high-income countries. The armamentarium for winning this difficult battle involves shared decision-making with communication across the clinical team and the patient. There is limited clinical evidence concerning effective approaches to managing such complex care and moreover involvement of different specialists makes coordinated care challenging. Bicuspid aortic valve (BAV) is the most common congenital cardiac malformation, occurring in 1-2% of the population whereas a single ventricle is a rare congenital heart disease that accounts for less than 1% of all congenital heart diseases. We had two cases of pregnancy with bicuspid aortic valve in one case and the other with single ventricle. The involvement of multidisciplinary team involving cardiologist, cardiothoracic anaesthetist and fetal maternal medicine specialist resulted in good maternal and fetal outcome in both the cases.

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.

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 Current and Future Aspects of Multimodal Imaging, Diagnostic, and Treatment Strategies in Bicuspid Aortic Valve and Associated Aortopathies

2020 ◽  
Vol 9 (3) ◽  
pp. 662
Author(s):  
Shazia Afzal ◽  
Kerstin Piayda ◽  
Oliver Maier ◽  
Shouheng Goh ◽  
Katharina Hellhammer ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Aortic Valve Replacement ◽  
Valve Replacement ◽  
Bicuspid Aortic Valve ◽  
Treatment Options ◽  
Treatment Strategies ◽  
Fusion Imaging ◽  
Surgical Aortic Valve Replacement ◽  
Transcatheter Aortic Valve ◽  
Depth Analysis

Bicuspid aortic valve (BAV) is the most frequent congenital cardiac abnormality leading to premature aortic valve apparatus dysfunction and is often associated with aortopathy. Therefore, current guidelines recommend a surgical aortic valve replacement (SAVR), even if many patients are deemed inoperable owing to their comorbidities and require alternatives such as transcatheter aortic valve replacement (TAVR). However, BAV variations remain challenging for procedural success. Therefore, the latest development in different imaging modalities (echocardiography, multislice-computertomographie, cardiovascular magnetic resonance) allows in-depth analysis for preprocedural risk stratification, follow up, and patient selection. Furthermore, we shed light on the latest developments in pre- and periprocedural fusion imaging as well as on current and future treatment options.

scholarly journals Bicuspid Aortic Valve in 2 Model Species and Review of the Literature

2020 ◽  
Vol 57 (2) ◽  
pp. 321-331 ◽  
Author(s):  
Borja Fernández ◽  
María Teresa Soto-Navarrete ◽  
Alejandro López-García ◽  
Miguel Ángel López-Unzu ◽  
Ana Carmen Durán ◽  
...  
Keyword(s):  
Animal Models ◽  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Wide Spectrum ◽  
Genetically Modified ◽  
Type A ◽  
Mouse Strains ◽  
Genetic Modifiers ◽  
Interspecific Differences ◽  
Congenital Cardiac Malformation

Bicuspid aortic valve (BAV) is the most common human congenital cardiac malformation. Although the etiology is unknown for most patients, formation of the 2 main BAV anatomic types (A and B) has been shown to rely on distinct morphogenetic mechanisms. Animal models of BAV include 2 spontaneous hamster strains and 27 genetically modified mouse strains. To assess the value of these models for extrapolation to humans, we examined the aortic valve anatomy of 4340 hamsters and 1823 mice from 8 and 7 unmodified strains, respectively. In addition, we reviewed the literature describing BAV in nonhuman mammals. The incidences of BAV types A and B were 2.3% and 0.03% in control hamsters and 0% and 0.3% in control mice, respectively. Hamsters from the spontaneous model had BAV type A only, whereas mice from 2 of 27 genetically modified strains had BAV type A, 23 of 27 had BAV type B, and 2 of 27 had both BAV types. In both species, BAV incidence was dependent on genetic background. Unlike mice, hamsters had a wide spectrum of aortic valve morphologies. We showed interspecific differences in the occurrence of BAV between humans, hamsters, and mice that should be considered when studying aortic valve disease using animal models. Our results suggest that genetic modifiers play a significant role in both the morphology and incidence of BAV. We propose that mutations causing anomalies in specific cardiac morphogenetic processes or cell lineages may lead to BAV types A, B, or both, depending on additional genetic, environmental, and epigenetic factors.

Familial patent ductus arteriosus and bicuspid aortic valve with hand anomalies: A novel heart-hand syndrome

1999 ◽  
Vol 87 (2) ◽  
pp. 175-179 ◽  
Author(s):  
Bruce D. Gelb ◽  
Jian Zhang ◽  
Robert J. Sommer ◽  
Jared M. Wasserman ◽  
Milton J. Reitman ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Patent Ductus Arteriosus ◽  
Bicuspid Aortic Valve ◽  
Ductus Arteriosus ◽  
Hand Anomalies ◽  
Patent Ductus

scholarly journals Approach to Asymptomatic Case of Bicuspid Aortic Valve with Coarctation and Massive Aortic Arteriopathy: a Ticking Time Bomb

2021 ◽  
Vol 04 (10) ◽  
pp. 01-04
Author(s):  
K.V Charan Reddy
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Medical Literature ◽  
Treatment Strategies ◽  
Good Alternative ◽  
Aortic Dilatation ◽  
Endovascular Stenting ◽  
Type A Aortic Dissection ◽  
Combined Lesions ◽  
Asymptomatic Case

The combination of aortic coarctation (CoA) and bicuspid aortic valve (BAV) is associated with high risk of ascending aortic dilatation and type A aortic dissection. Most of the patients having combined lesions remain asymptomatic until adulthood and are incidentally diagnosed. Several questions regarding their treatment strategies remain unanswered. Both single-and two-stage surgical procedures have been described in medical literature, to treat CoA and BAV. Lately, combined endovascular therapy (Balloon angioplasty or Stent implantation) and surgery, has become a good alternative in selected cases. Here, we describe such a asymptomatic case of BAV with massive aneurysmal root dilatation and CoA, which was managed with endovascular stenting and surgery.

scholarly journals Bicuspid Aortic Valve: Genetic and Clinical Insights

Aorta ◽  
2021 ◽  
Vol 09 (04) ◽  
pp. 139-146
Author(s):  
Idit Tessler ◽  
Juliette Albuisson ◽  
Guillaume Goudot ◽  
Shai Carmi ◽  
Shoshana Shpitzen ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Genetic Model ◽  
Wide Spectrum ◽  
Current Data ◽  
Incomplete Penetrance ◽  
Cardiac Events ◽  
Clinical Value ◽  
Cascade Screening ◽  
Male Predominance

AbstractBicuspid aortic valve (BAV) is the most common valvular congenital heart disease, with a prevalence of 0.5 to 2% in the general population. Patients with BAV are at risk for developing cardiovascular complications, some of which are life-threatening. BAV has a wide spectrum of clinical presentations, ranging from silent malformation to severe and even fatal cardiac events. Despite the significant burden on both the patients and the health systems, data are limited regarding pathophysiology, risk factors, and genetics. Family studies indicate that BAV is highly heritable, with autosomal dominant inheritance, incomplete penetrance, variable expressivity, and male predominance. Owing to its complex genetic model, including high genetic heterogenicity, only a few genes were identified in association with BAV, while the majority of BAV genetics remains obscure. Here, we review the different forms of BAV and the current data regarding its genetics. Given the clear heritably of BAV with the potential high impact on clinical outcome, the clinical value and cost effectiveness of cascade screening are discussed.

Sign in / Sign up

Export Citation Format

Share Document