Presentation
Cystic medial necrosis present in the aneurysmal wall probably arose as a consequence of poststenotic dilatation. Adequate noninvasive evaluation of the ascending aorta requires echocardiographic studies, as well as a chest X-ray film. [jhu.pure.elsevier.com]
Current ACC/AHA guidelines recommend familial echocardiographic screening which, to the present authors' knowledge, is not performed routinely and uniformly. The aim of this pilot project was to explore the practicalities of running such a program. [f1000.com]
Eight high impact variants were identified by at least two of the commonly used selection strategies; however, these were either common in the general population ( 10%) or present in the majority of the unaffected family members. [diagenode.com]
Published on Dec 14, 2012 Amelia Carro's predoctoral presentation at the 6th VHIR Scientific Session. [slideshare.net]
First, we sought to identify variants present in affected but not in unaffected individuals. [circgenetics.ahajournals.org]
Workup
Sometimes, a family doctor may hear a small heart murmur while listening to the heart, but since it is such a minor heart murmur and there are no symptoms or limitations, no workup or evaluation is necessary. [badaorta.com]
The size of the proximal aorta should be evaluated carefully during the workup. [en.wikipedia.org]
X-Ray
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Calcified Aortic Valve
The proband, at the age of 39 years, presented severe calcified aortic valve and normal diameter of ascending aorta (39 mm). One year later, the patient underwent elective aortic valve and ascending aorta repair (48 mm) replacement. [bmcmedgenet.biomedcentral.com]
Treatment
Apply today's best medical and surgical approaches with comprehensive, expert guidance on today's full range of clinical heart disease treatments. [books.google.de]
Children with mild aortic stenosis rarely require treatment. However, it is important to note that aortic stenosis may be progressive, and that children with mild disease may eventually require treatment later in life. [cincinnatichildrens.org]
Treatment plans have been developed to assist people who may be at risk of complications from a defective aortic valve. [cedars-sinai.edu]
The treatment of an aortic aneurysm depends upon the severity of the aneurysm or dissection and may include medical management (medication) or surgery. For some hospitals that specialize in treatment of aortic disease, please see the Resources page. [johnritterfoundation.org]
Prognosis
Text In Reply We thank Martín et al for their interest in our work, 1 which described the association between bicuspid aortic valve (BAV) morphology (presence of raphe vs non-raphe) and degree of valve dysfunction as well as presence of aortopathy and prognosis [jamanetwork.com]
Fortunately the prognosis for most children diagnosed with a bicuspid aortic valve is good. [kidsheartshouston.com]
Medicines may include: Drugs that lower the workload on the heart (beta-blockers, ACE inhibitors) Drugs that make the heart muscle pump harder (inotropic agents) Water pills (diuretics) Outlook (Prognosis) How well the baby does depends on the presence [huronregional.org]
[…] edit ] Bicuspid aortic valves may assume three different types of configuration: [14] "Real" bicuspid valves with two symmetric leaflets A tricuspid architecture with a fusion of two leaflets A tricuspid architecture with a fusion of three leaflets Prognosis [en.wikipedia.org]
even a normally functioning BAV can progress and damage gradually to reveal abnormal folding and creasing throughout the cardiac cycle, with more turbulent flow, as well as restricted motion (12) Aortopathy and coarctation have implications on the prognosis [escardio.org]
Etiology
Ehlers-Danlos syndrome (EDS type IV) Arterial tortuosity syndrome Aortic aneurysm Aortic root dilation Ascending aortic dilation Familial thoracic aortic aneurysm and dissection Bicuspid aortic valve with aortic dilation Arterial tortuosity of unknown etiology [texaschildrens.org]
While a limited number of genetic etiologies for BAV have been defined, family members of affected individuals display BAV along with other left-sided CHD. [link.springer.com]
TA is an inflammatory disease of unknown etiology predominantly affecting the aorta and its main branches [ 23 ]. [uscapknowledgehub.org]
However, the etiology of this common valve disease is largely unknown. Future studies based on new genetic screening strategies, the so-called next-generation sequencing, are needed in order to unravel the still largely unknown genetics of BAV. [bmcmedgenet.biomedcentral.com]
The case against its being of rheumatic etiology. Am J Med. 1970 ; 49 :151–159. Crossref Medline Google Scholar 6. Roberts WC. The congenitally bicuspid aortic valve. A study of 85 autopsy cases. Am J Cardiol. 1970 ; 26 :72–83. [circgenetics.ahajournals.org]
Epidemiology
View Article PubMed PubMed Central Google Scholar O’Brien KD: Epidemiology and genetics of calcific aortic valve disease. J Investig Med. 2007, 55: 284-291. 10.2310/6650.2007.00010. [bmcmedgenet.biomedcentral.com]
Complications stemming from structural heart issues are most often treated through surgical intervention, which could include aortic valve replacement, or balloon valvuloplasty. [16] Epidemiology [ edit ] Bicuspid aortic valves are the most common cardiac [en.wikipedia.org]
Pathophysiology
Cardiol Res Pract. 2012;2012:478259. doi: 10.1155/2012/478259. 26 - Pathophysiological implications of different bicuspid aortic valve configurations. Kari FA, Beyersdorf F, Siepe M. [escardio.org]
Ascending aortic dilatation associated with bicuspid aortic valve: pathophysiology, molecular biology, and clinical implications. Circulation. 2009;119:880-90. Medline 2. Siu SC, Silversides CK. Bicuspid aortic valve disease. [revespcardiol.org]
Pathophysiology [ edit ] Heart bicuspid aortic valve anatomy. [en.wikipedia.org]
Bicuspid aortic valve aortopathy:genetics, pathophysiology and medical therapy. Interact Cardiovasc Thorac Surg. 2013;17:554–559. Chan KL, Stinson WA, Veinot JP. [lawarencepress.com]
Prevention
Medicine may be needed to relieve symptoms or prevent complications. [huronregional.org]
Heimlich valve a small one-way valve used for chest drainage, emptying into a flexible collection device; the valve prevents return of gases or fluids into the pleural space. [medical-dictionary.thefreedictionary.com]
The estimated mean cost of echocardiogram screening to prevent 1 aortic dissection was 363 911. The estimated cost to prevent 1 death between ages 20 and 40 years was 712 237. With 3% discounting, the cost per life-year saved was 74 884. [pediatrics.aappublications.org]
Smidt Heart Institute offers preventive, rehabilitative, diagnostic and both nonsurgical and surgical treatments for heart disease, including angioplasty, bypass surgery and heart transplantation. [cedars-sinai.edu]