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Aortic Stenosis with Bicuspid Valve


Presentation

  • Presented at: American College of Cardiology Scientific Session; March 17-19, 2017; Washington, D.C. Yoon SH, et al. J Am Coll Cardiol . 2017;doi:10.1016/j.jacc.2017.03.017 . Disclosure: Yoon reports no relevant financial disclosures.[healio.com]
  • Abstract We herein present a rare case of severe aortic valve stenosis with a bicuspid valve in a patient with systemic lupus erythematosus.[link.springer.com]
  • 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]
  • Physical examination is notable for a 3/6 crescendo-decrescendo systolic murmur present at the right upper sternal border with radiation to the carotid arteries. Random blood glucose is 205 mg/dL.[medbullets.com]
  • The characteristic murmur may occasionally be faint and so the patient may present as heart failure of unknown cause.[patient.info]
Coarctation of the Aorta
  • THIS IS CASE 4,555 OF 4,932 JUMP TO CASE 4,554 Coarctation of the Aorta with Dilated Left Subclavian Artery 4,553 Type A Dissection Nicely Seen with Gated Acquisition CURRENT CASE Bicuspid valve with Extensive Calcification and Aortic Stenosis 4,556 Pericardial[ctisus.com]
  • […] of the aorta: Narrowing of the aorta at some point along its length[nm.org]
  • […] of the aorta. [1] Pathophysiology of Aortic Stenosis The pathophysiology of aortic stenosis is described in the main chapter on the pathophysiology of aortic stenosis here.[wikidoc.org]
  • Other associated lesions include VSD, coarctation of the aorta, and the Shone complex (coarctation of the aorta, parachute mitral valve, supravalvular mitral ring, sub-AS).[ecrjournal.com]
  • This arrangement is much more common in patients with coarctation of the aorta, whose valves function adequately.[emedicine.medscape.com]
Heart Disease
  • Prevention of cardiovascular events and death with pravastatin in patients with coronary heart disease and a broad range of initial cholesterol levels. ‏[books.google.com]
  • An echocardiographic study of valvular heart disease associated with systemic lupus erythematosus. N Engl J Med 1996; 335: 1424–30. PubMed CrossRef Google Scholar 8. Sabet HY, Edwards WD, Tazelaar HD, Daly RC.[link.springer.com]
  • Cedars-Sinai is pioneering the use of minimally invasive techniques to treat heart disease, as well as noninvasive approaches to managing heart conditions.[cedars-sinai.edu]
  • […] defect (Medical Encyclopedia) [ Read More ] Heart Valve Diseases Also called: Valvular heart disease Your heart has four valves.[icdlist.com]
  • Valvular Heart Disease: A Companion to Braunwald's Heart Disease. 4th ed. Philadelphia, PA: Elsevier Saunders; 2014:chap 13. Carabello BA. Valvular heart disease. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 25th ed.[medlineplus.gov]
Heart Murmur
  • The main sign of heart valve disease is an unusual heartbeat sound called a heart murmur. Your doctor can hear a heart murmur with a stethoscope. But many people have heart murmurs without having a problem.[icdlist.com]
  • A patient with a bicuspid aortic valve usually comes to attention due to the presence of a heart murmur or an abnormal heart sound. There are many other different causes of heart murmurs, including normal causes.[pediatricheartspecialists.com]
  • An early indicator of BAV is a heart murmur, which is an abnormal sound caused by the turbulent flow of blood over a diseased heart valve.[nm.org]
  • Other signs of bicuspid aortic valve include: Enlarged heart Heart murmur Weak pulse in the wrists and ankles Aortic stenosis Aortic regurgitation To learn more about bicuspid aortic valve and its diagnosis and treatment, download: Bicuspid Aortic Valve[marfan.org]
Systolic Murmur
  • 3/6 or louder systolic murmur.[aafp.org]
  • Physical examination is notable for a 3/6 crescendo-decrescendo systolic murmur present at the right upper sternal border with radiation to the carotid arteries. Random blood glucose is 205 mg/dL.[medbullets.com]
  • Aortic stenosis may develop a soft, harsh ejection systolic murmur at the first aortic area with possible radiation into the carotids. With more stenosis, thrills can be palpable.[lecturio.com]
  • Both conditions are associated with an early, harsh systolic murmur that is transmitted to the carotids. A similar murmur may occur without stenosis if turbulence is due to aortic aneurysm causing dilation of the proximal aorta.[patient.info]
  • In symptomatic adults with systolic murmurs,echocardiography is essential to identify those likely to benefit from surgery.[rjmatthewsmd.com]
Exertional Syncope
  • ), syncope (on exertion or at rest), and heart failure; exertional dyspnea is the most common complaint. 2,6 Manifestation of these symptoms most commonly occurs after age 60 years. 6 Complications of aortic stenosis may include infective endocarditis[uspharmacist.com]
  • Exertional syncope in aortic stenosis: evidence to support inappropriate left ventricular baroreceptor response. Am Heart J. 1991;121(2 pt 1):603–606. 15. Schwartz LS, Goldfischer J, Sprague GJ, Schwartz SP.[aafp.org]
Pulsus Parvus et Tardus
  • parvus et tardus weak pulses with a delayed peak Imaging Radiography indication for all patients findings cardiomegaly calcification pulmonary congestion Echocardiography indications for all patients diagnostic test views transthoracic echocardiogram[medbullets.com]
  • Signs Examination of the pulse in significant AS reveals a slow-rising, flat character called pulsus parvus et tardus. [ 7 ] Blood pressure will show a narrow pulse pressure (difference between systolic and diastolic pressures).[patient.info]
Fracture
  • Intraoperative and pathologic examination showed bicuspid aortic valve with symmetric cusps, straight and fibrotic cusp edges and fractured calcific nodules of the aortic valve.[ncbi.nlm.nih.gov]
  • Inflating the balloon is meant to fracture some of the calcium deposits on the valve, thus allowing the valve to open more completely, and relieving part of the obstruction.[verywell.com]
Dizziness
  • […] become more severe and cause: chest pain brought on by physical activity (angina) – caused by your heart having to work harder shortness of breath – at first you may only notice this when you exercise, but later you may experience this even when resting dizziness[nhs.uk]
  • Should a child develop moderate or severe tightness (aortic stenosis) or leaking (aortic regurgitation), or severe dilation of the ascending aorta, he or she might show signs of shortness of breath, exercise intolerance, fatigue, chest pain, dizziness[childheartassociates.com]
  • Symptoms of a stenotic valve include chest pain, shortness of breath and dizziness or fainting caused by inadequate blood flow to the brain. If the bicuspid valve does not close completely, blood can flow backwards into the heart.[my.clevelandclinic.org]
  • Although some people with a bicuspid valve are asymptomatic, common symptoms include: Shortness of breath Constant tiredness or fatigue Coughing at night or when in bed Rapid or fluttering heart palpitations Dizziness Chest pain (tightness, angina) Fainting[healthblog.uofmhealth.org]
  • […] with a bicuspid valve are asymptomatic, the most common symptoms include: Shortness of breath, during exercise or when you are lying in bed Constant tiredness or fatigue Coughing at night or when in bed Rapid or fluttering heart palpitations Feeling dizzy[uofmhealthblogs.org]

Workup

  • The size of the proximal aorta should be evaluated carefully during the workup.[en.wikipedia.org]
  • Diagnosis of aortic stenosis is suspected on physical examination (abnormal heart sounds, abnormalities in the pulse) and confirmed by echocardiography. 2,9 (For a detailed description of the history, physical examination, differential diagnosis, and workup[uspharmacist.com]
Calcified Aortic Valve
  • Unfortunately, in older people with heavily calcified aortic valves, the results with valvulotomy have not been good.[verywell.com]
  • aortic valves, although it can occur in the geriatric patient population. 6 Hospital-acquired Staphylococcus aureus bacteremia can occur at any age and is often seen as a consequence of aortic valve replacement. 6 Prognosis Sudden death, while occurring[uspharmacist.com]
Pericardial Effusion
Left Ventricular Hypertrophy
  • If there are signs of left ventricular hypertrophy (thickening of the ventricle walls) or arrhythmia in conjunction with moderate stenosis, then the patient should be confined to moderate levels of isotonic exercise and should avoid isometric exercise[pted.org]
  • Left ventricular hypertrophy is present in bicuspid aortic stenosis. [5] Pathological Findings Images shown below are courtesy of Professor Peter Anderson DVM PhD [6] and published with permission.[wikidoc.org]
  • Not helpful in screening of BAV Image : “Left ventricular hypertrophy in short axis view.” by Patrick J. Lynch, medical illustrator.[lecturio.com]
  • . 4 Left ventricular hypertrophy secondary to aortic stenosis, as well as age-related changes, both promote diastolic dysfunction, rendering the geriatric patient with aortic stenosis more dependent on contraction of the atrium for filling the ventricle[uspharmacist.com]
  • ventricular hypertrophy (LVH) LVH and stiff, noncompliant walls result in oxygen demand and clinically manifests as angina over time, aortic stenosis results in blood flow to the vertebral, basilar, and carotid arteries, resulting clinically in syncope[medbullets.com]

Treatment

  • الصفحة 24 - Third Report of the National Cholesterol Education Program (NCEP) Expert Panel on Detection, Evaluation, and Treatment of High Blood Cholesterol in Adults (Adult Treatment Panel III). ‏ الصفحة 134 - Sacks FM, Pfeffer MA, Moye LA et al.[books.google.com]
  • WASHINGTON — Treatment of bicuspid aortic stenosis with transcatheter aortic valve replacement was comparable to transcatheter aortic valve replacement in tricuspid aortic stenosis, but the procedure was more successful with newer-generation systems,[healio.com]
  • Although surgical correction is a more definitive treatment modality, BAV has a long-term durability and is the treatment of choice in patients presenting early.[louisville.edu]
  • Conversely, even if no symptoms are present, it may be advisable to proceed with treatment or repairs based on the test results. Possible treatments may include valve repair or valve replacement.[heart.org]
  • 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]

Prognosis

  • Natural History and Prognosis Valvular AS is frequently a progressive disease, the severity increasing over time.[rjmatthewsmd.com]
  • MSCT may be useful in quantifying the valve area and coronary calcification, which aids in assessing prognosis.[patient.info]
  • There is a favorable long-term outcome following aortic valve surgery and relatively low operative risk. 6 This should be considered in light of the prognosis of congestive heart failure in the very old as worse than the prognosis of most cancers ( 1[uspharmacist.com]
  • Fortunately the prognosis for most children diagnosed with a bicuspid aortic valve is good.[kidsheartshouston.com]

Etiology

  • […] definition narrowing of the aortic valve Epidemiology demographics bicuspid aortic valve calcification presents at a younger age tricuspid aortic valve calcification 65 years of age risk factors hypertension coronary artery disease rheumatic heart disease Etiology[medbullets.com]
  • Aortic stenosis refers to abnormal orifice narrowing of the aortic valve causing obstruction of blood flow from the left ventricle to the ascending aorta during systole. 2,3 In the elderly, etiologies for aortic stenosis include calcification of a normal[uspharmacist.com]

Epidemiology

  • Introduction Clinical definition narrowing of the aortic valve Epidemiology demographics bicuspid aortic valve calcification presents at a younger age tricuspid aortic valve calcification 65 years of age risk factors hypertension coronary artery disease[medbullets.com]
  • Epidemiology of Bicuspid Aortic Valve Disease Bicuspid aortic valve disease is one of the prominent causes of death worldwide and considered one of the most common causes of inherited heart diseases.[lecturio.com]
  • Epidemiology [ 1 ] AS has become the most frequent type of valvular heart disease in Europe and North America.[patient.info]
  • 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]
  • Morphological, Epidemiological, and Hereditary Aspects of Congenital Aortic Stenosis Congenital obstruction of the LVOT may involve the subvalvular, valvular, or supravalvular part of the outflow tract complex.[ecrjournal.com]
Sex distribution
Age distribution

Pathophysiology

  • 1243 Percutaneous Coronary Intervention 1258 Percutaneous Cardiac Procedures 1273 Coronary Artery Bypass Surgery 1290 Approaches to the Patient with Prior Bypass Surgery 1306 Percutaneous Valve Dilatations 1316 Global Pandemic of Heart Failure 1330 Pathophysiology[books.google.com]
  • […] of the aorta. [1] Pathophysiology of Aortic Stenosis The pathophysiology of aortic stenosis is described in the main chapter on the pathophysiology of aortic stenosis here.[wikidoc.org]
  • 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]
  • Pharmacotherapy: A Pathophysiologic Approach. 7th ed. New York, NY: McGraw-Hill Inc.; 2008:315-330. 15. Novaro GM, Tiong IY, Pearce GL, et al.[uspharmacist.com]
  • ,The Pathology,Pathophysiology,Recognition,and Treatment of Congenital Heart Disease,Hurst's Diseases of The Heart,10th edition,Vol.2,Pp.1837-1905.[rjmatthewsmd.com]

Prevention

  • Prevention of cardiovascular events and death with pravastatin in patients with coronary heart disease and a broad range of initial cholesterol levels. ‏[books.google.com]
  • Both are safe and effective ways of managing bicuspid aortic stenosis and both prevent the adverse effects described above.[louisville.edu]
  • 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]
  • Medicine may be needed to relieve symptoms or prevent complications.[medlineplus.gov]
  • Patients with severe or moderate to severe impairment of valve function require intervention to prevent long term damage to the heart.[pediatricheartspecialists.com]

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