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Cystic Medial Necrosis of the Aorta

Erdheim Cystic Medial Necrosis of Aorta


Presentation

  • Presents comprehensive coverage of valvular heart disease to provide you with a complete reference and one-stop shop for this specialty in cardiac medicine.[books.google.com]
  • The presence of a diastolic murmur at initial presentation was associated with a poor prognosis (p 0.03).(ABSTRACT TRUNCATED AT 250 WORDS).[ncbi.nlm.nih.gov]
  • Benefit from matchless Netter illustrations that offer precision, clarity, detail and realism as they provide a visual approach to the clinical presentation and care of the patient.[books.google.com]
  • The presence of a diastolic murmur at initial presentation was associated with a poor prognosis (p 0.03).[onlinejacc.org]
  • Most of these patients present with aortic regurgitation and on investigation aneurysm is detected. Owing to its rarity and disguised presentation it is important to be aware of this entity.[heartindia.net]
Collapse
  • For the vast majority, there is an underlying anatomic disorder of the cardiovascular system responsible for the ultimate hemodynamic collapse known as sudden cardiac death.[books.google.com]
  • Medionecrosis, or laminar medial necrosis, is defined as coagulative necrosis of medial smooth muscle cells, resulting in loss of nuclei and collapse of the elastic lamellae; [3] this finding is not specific for any TAA etiology.[emedicine.medscape.com]
Pallor
  • Aortic dissections usually cause severe, sudden chest or back pain, and may also result in unusually pale skin (pallor), a very faint pulse, numbness or tingling (paresthesias) in one or more limbs, or paralysis.Familial TAAD may not be associated with[icdlist.com]
Hunting
Regurgitation
  • Cardiac catheterization in 76 patients demonstrated aortic root dilation in 78%, aortic regurgitation in 72%, aortic dissection in 32% and coronary artery disease in 32%.[ncbi.nlm.nih.gov]
  • (D) Coronal cine gradient echo image in diastole demonstrating flow void in the left ventricle corresponding to severe aortic regurgitation (arrow).[heart.bmj.com]
  • Features new chapters-Genetic, molecular and cellular mechanisms of valvular disease; Bicuspid aortic valve disease; and Ischemic mitral regurgitation-for the latest in cutting-edge research and clinical data.[books.google.com]
  • One had aortic regurgitation but there was no reason to believe that the aorta in the other two patients had been subjected to unusual haemodynamic stresses.[ncbi.nlm.nih.gov]
Chest Pain
  • The proband (II-1) was thought to be in good health until she developed chest pain at age 56 and died of a dissecting aneurysm of the ascending aorta that ruptured into the pericardial space.[nature.com]
  • […] cystic medial degeneration (which can also be a separate AD hereditary condition: cystic medial necrosis) nb also mitral and aortic valve prolapse etc and Ehlers Danlos - defective collagen [COL3A1 etc, Tenascin-X, PLOD1, ADAMTS2] Presents Sharp tearing chest[alancam.com]
  • Case report A previously healthy 42-year-old man presented to our hospital with the complaints of severe chest pain.[heartindia.net]
  • […] surgically* DeBakey Type III (most common) Stanford Type B Descending aorta only Hypertension Atherosclerosis Usually medically *Goal is to prevent backward involvement of the aortic valve or rupture into pericardium · Clinical o Sharp, tearing, intractable chest[learningradiology.com]
  • The American College of Cardiology recently recognized Leesburg Regional Medical Center and The Villages Regional Hospital for their demonstrated expertise and commitment in treating patients with chest pain by awarding the Chest Pain Center Accreditation[centralfloridahealth.org]
Vascular Disease
  • Disease 1496 Cerebrovascular Disease 1511 Disease of Peripheral Vessels 1531 Renal Artery Disease 1549 Venous Thromboembolism 1569 Index 1585 Genetic Factors in Atherosclerosis 1589 Gene Therapy 1602 حقوق النشر مقاطع مشهورة الصفحة 108 - Heart Association[books.google.com]
  • See also Vascular disease. Aortic dissection. References[librepathology.org]
  • The vascular disease in these patients is particularly aggressive, with a mean age of death of 26 years.[nap.edu]
  • disease, yet as many as 20% of these individuals have at least 1 first-degree family member with a known aneurysm in the arterial tree. [15] Radiologic screening in family members of patients presenting with aneurysms or with complications thereof have[emedicine.medscape.com]
Diastolic Murmur
  • The presence of a diastolic murmur at initial presentation was associated with a poor prognosis (p 0.03).(ABSTRACT TRUNCATED AT 250 WORDS).[ncbi.nlm.nih.gov]
  • The presence of a diastolic murmur at initial presentation was associated with a poor prognosis (p 0.03).[onlinejacc.org]
  • Erdheim-Gsell cystic medial necrosis as a cause of giant aneurysm of the ascending aorta Statistics from Altmetric.com Images in cardiology A previously healthy 42 year old woman was referred after the discovery of a 3/6 diastolic murmur maximal at the[heart.bmj.com]
  • On examination, diastolic murmur was discovered, which was maximum at the aortic region with vigorous pulsation of the suprasternal notch. There was no significant past or family history of similar complain.[heartindia.net]
Cardiomegaly
  • Sixty-eight percent of the patients had a diastolic murmur and chest roentgenogram revealed a dilated aortic arch in 58% and cardiomegaly in 63%.[ncbi.nlm.nih.gov]
Left Ventricular Dysfunction
  • Effect of captopril on mortality and morbidity in patients with left ventricular dysfunction after myocardial infarction: results of the survival and ventricular enlargement trial. ‏ الصفحة 248 - Group.[books.google.com]
Excitement
  • This classic Netter reference has been updated to mirror the many exciting advances in cardiovascular medicine and imaging – offering unparalleled insights into anatomy, physiology, and clinical conditions.[books.google.com]

Workup

  • Workup demonstrated marked dilatation of the ascending and descending aorta. This low-magnification view of the ascending aorta stained with Movat pentachrome stain shows necrotizing aortitis involving mainly the media.[emedicine.medscape.com]

Treatment

  • Abstract Percutaneous transluminal angioplasty has been shown to be both feasible and efficacious for the treatment of aortic coarctation.[ncbi.nlm.nih.gov]
  • 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]
  • In the third edition, Catherine Otto is joined by Robert Bonow and a team of expert contributors to bring you the latest developments in imaging and treatment.[books.google.com]
  • Clinical considerations and surgical treatment of annulo-aortic ectasia. J Thorac Cardiovasc Surg 1961; 42: 363–70. PubMed Google Scholar 4. Savunen T, Aho HJ. Annulo-aortic ectasia. Light and microscopic changes in aortic media.[link.springer.com]
  • TREATMENT The treatment for aortic aneurysms may involve surgical or endovascular repair of the weakened arterial wall. Endovascular aneurysm repair (EVAR) now accounts for 56 percent of repairs in patients with AAA.[nap.edu]

Prognosis

  • The presence of a diastolic murmur at initial presentation was associated with a poor prognosis (p 0.03).(ABSTRACT TRUNCATED AT 250 WORDS).[ncbi.nlm.nih.gov]
  • Abstract The characteristics and prognosis of patients with cystic medial necrosis of the aorta were reviewed. Subjects were 46 patients who underwent aortic and/or aortic valve surgery between August 1965 and October 1994.[ncbi.nlm.nih.gov]
  • The presence of a diastolic murmur at initial presentation was associated with a poor prognosis (p 0.03).[onlinejacc.org]
  • Toshihiko Ueda Hideyuki Shimizu Ryo Aeba Hankei Shin Toshiyuki Katogi Ryohei Yozu Shiaki Kawada Abstract The characteristics and prognosis of patients with cystic medial necrosis of the aorta were reviewed.[link.springer.com]
  • Type A Dissecting aneurysm Involves the descending aorta - more distal thus less dangerous and has a better prognosis.[quizlet.com]

Etiology

  • By providing an atlas style review of ischemic and non-ischemic etiologies of sudden cardiac death, this book opens a window to see the underlying pathology first hand.[books.google.com]
  • However, a few reasons suggested are that there is difference in composition between the ascending and descending aorta, which is responsible for difference in the etiology of aneurysms that develop in these areas.[heartindia.net]
  • The aim of the present article is to provide a comprehensive overview of the CMN lesion in terms of pathologic features, clinical implications and etiologies based on molecular research results.[ncbi.nlm.nih.gov]
  • Common etiologies for dissection are HTN, cystic medial necrosis as with Marfan's syndrome, jet lesions from valve defects, medial necrosis from thrombosis of the vasa vasorum, and other connective tissue disorders affecting the media of the aorta.[med.illinois.edu]
  • The relationship between the extent of cystic medial necrosis and the etiology of TAA has been extensive studied.[emedicine.medscape.com]

Epidemiology

  • Genetics Genetic variants include ACTA2 as well as: Associated Conditions There is an association between cystic medial necrosis and the following disorders: Marfan syndrome Ehlers-Danlos syndrome Annuloaortic ectasia Acromegaly [4] Epidemiology and Demographics[wikidoc.org]
  • […] aorta Type IIIa: involves descending thoracic aorta from left subclavian artery to celiac artery Type IIIb: involves descending thoracic aorta from left subclavian and tracts distal to celiac artery Synonym(s): dissecting aneurysm; dissecting hematoma Epidemiology[unboundmedicine.com]
  • Epidemiologic studies have pointed to an autosomal dominant inheritance, but no specific genetic aberration has been identified to date. [27] Marfan syndrome has been extensive studied clinically and genetically.[emedicine.medscape.com]
  • EPIDEMIOLOGY, NATURAL HISTORY, AND TYPES OF AORTIC ANEURYSMS Abdominal aortic aneurysms (AAAs), involving the infradiaphragmatic abdominal area, are the most common type of aneurysms.[nap.edu]
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]
  • […] unknown due to undiagnosed/misdiagnosed cases resulting in sudden death without subsequent autopsy Prevalence United States: 6,000 to 10,000 cases annually Present in 3 out of every 1,000 patients presenting to ED with chest and/or back pain Etiology and Pathophysiology[unboundmedicine.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]
  • Marfan’s Ehlers-Danlos Usually surgically* DeBakey Type III (most common) Stanford Type B Descending aorta only Hypertension Atherosclerosis Usually medically *Goal is to prevent backward involvement of the aortic valve or rupture into pericardium · Clinical[learningradiology.com]
  • […] arteritis, giant cell arteritis, Behçet arteritis Other: polycystic kidney disease, pregnancy Recreational drug use: tobacco use, cocaine, methamphetamine Trauma (catheters, motor vehicle accidents, vascular surgery, intra-aortic balloon pump) General Prevention[unboundmedicine.com]
  • It is currently accepted that the aneurysms are not due to flow-related disturbances secondary to valvar insufficiency or stenosis; for this reason, wrapping of the aorta to prevent progressive dilatation after valve repair is often advocated. [14] Patients[emedicine.medscape.com]
  • Failure to prevent progressive dilation of ascending aorta by aortic valve replacement in patients with bicuspid aortic valve: comparison with tricuspid aortic valve. Circulation 2003;108:II291-4. Russo CF, Mazzetti S, Garatti A, et al.[annalscts.com]

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