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Case Report Adolescent presentation of interrupted aortic arch with
Case Report Adolescent presentation of interrupted aortic arch with

... them, echocardiogram can provide a wealth of information which other imaging techniques can not provide such as color doppler flow and associated with intracardiac anomalies. Several surgical means have been used for repair of IAA. The end-to-end anastomosis is the most commonly performed repair in ...
Heart Valve Disease
Heart Valve Disease

... Dilating LV, compensated for years 6-10 years: complains Severe MR: mortality 6-7% yearly ...
Aortic Valve Pathology and Treatment
Aortic Valve Pathology and Treatment

... Symptoms begin: 60-80 yrs. of age ...
Pediatric emergency case conference
Pediatric emergency case conference

...  Adequate sedation before the procedure ...
CONGENITAL BICUSPID AORTIC VALVE – A CASE REPORT
CONGENITAL BICUSPID AORTIC VALVE – A CASE REPORT

... pulse and wide pulse pressure are the characteristic clinical findings. As the disease progresses, these symptoms start occurring more frequently, even without exerciset . The diagnosis can done by proper history, characteristic clinical findings and assisted with echocardiography or magnetic resona ...
Chronic Mitral Regurgitation
Chronic Mitral Regurgitation

... Management Principles • Severe MS - is usually symptomatic - Percutaneous mitral commissurotomy (PMC) is the treatment modality of choice in the vast majority - PMC in optimal anatomy has acturial survival rate of 95% after 7 years - PMC in skilled centers has a mortality of < 1% ...
Mechanism of Aortic Valve Opening: Beyond the Pressure Gradient
Mechanism of Aortic Valve Opening: Beyond the Pressure Gradient

... interval: 1.6 to 3.9]). A mild CAC score (>10 to 100 AU) was associated with a 12-fold increase in odds for progression (odds ratio: 12.0 [95% confidence interval: 6.2 to 23.1]). The relationship between baseline CAC and progression did not differ by diabetes status. We have shown, in a young populat ...
Corneal Interface Morphology After Descemet Stripping
Corneal Interface Morphology After Descemet Stripping

... CEIOL, scleral buckle for retinal detachment repair, and strabismus surgery Preoperative BCVA ranged from 20/40 to 20/70 All patients required rebubbling during the postoperative course for management of graft dislocation Of the three patients who developed pupillary block glaucoma, one required reb ...
Down syndrome Associated with Epstein`s Anomaly and Pulmonary
Down syndrome Associated with Epstein`s Anomaly and Pulmonary

... In children with Down syndrome (DS) there is higher chance for concomitant congenital heart defects and organic disorders. However Epstein’s anomaly is a rare association of DS. We report a 2.5 months DS boy that referred to our hospital with tachypnea. In echocardiography the septal leaflet displac ...
Heart sounds and murmurs
Heart sounds and murmurs

... Common murmurs and timing: 1. systolic murmur: harsh turbulent flow (from increase in turbulence) a. aortic stenosis: ejection murmur because the valves are too tight./don’t open completely b. pulmonary stenosis: ejection murmur + S2 splitting c. mitral/tricuspid regurgitation: not properly closed ...
Bicuspid Aortic Valve and Aortopathy: See the First, Then Look at
Bicuspid Aortic Valve and Aortopathy: See the First, Then Look at

... to other series, this study found that fusion of the right and left coronary cusps (anterior–posterior [AP] leaflet type) was the most common pattern, occurring in 56% of patients, with fusion of the right and noncoronary cusps (right–left [RL] leaflet type) seen in the remaining 44% of patients. Al ...
S 2
S 2

...  Apical impulse In newborn and infants 4. intercostal space/midclavicular line In older children and adults 5. intercostal space/midclavicular line ...
Full Text  - Iran Red Crescent Med J
Full Text - Iran Red Crescent Med J

... in the infarct size promoted by the early coronary reperfusion, and the shorter exposure of the immune system to the myocardial antigens, promoted the virtual disappearance of this entity (10, 11). This case highlights the importance of the clinical assessment of patients admitted with chest pain. A ...
Aorto-Left Atrial Fistula
Aorto-Left Atrial Fistula

... ably a complication of aortic valvular endocarditis, are rare and infrequently diagnosed premortem. We describe a patient who presented with this entity and review the reports of five other patients for whom a diagnosis was made premortem. A number of causative organisms have been identi¬ fied. The ...
Aorto-Left Atrial Fistula
Aorto-Left Atrial Fistula

... ably a complication of aortic valvular endocarditis, are rare and infrequently diagnosed premortem. We describe a patient who presented with this entity and review the reports of five other patients for whom a diagnosis was made premortem. A number of causative organisms have been identi¬ fied. The ...
Advances in Perinatal Genetics
Advances in Perinatal Genetics

... Name that syndrome… syndrome…. ...
Aortic Valve Disease
Aortic Valve Disease

... Aortic Stenosis Aortic stenosis is a term that refers to narrowing of the aortic valve opening during systole (Figure 1). This can be caused by a congenital abnormality of the valve (for instance, one could be born with a valve that has only 2 cusps instead of the normal 3) and thus may be detected ...
IOSR Journal of Dental and Medical Sciences (IOSR-JDMS)
IOSR Journal of Dental and Medical Sciences (IOSR-JDMS)

... Diabetes mellitus is a group of metabolic diseases characterized by hyperglycemia resulting from defects in insulin secretion, insulin action, or both[10]. The chronic hyperglycemia of diabetes is associated with long-term damage, dysfunction, and failure of various organs, especially the eyes, kidn ...
Aortic Stenosis Explained - New - CardioRespiratory Pet Referrals
Aortic Stenosis Explained - New - CardioRespiratory Pet Referrals

... Blood is pumped from the left ventricle to a particularly large blood vessel called the ‘aorta’. (The aorta is the body’s largest artery.) The valve that separates the left ventricle from the aorta is called the ‘aortic valve’. The left ventricle narrows as it leads to the aorta and this area is cal ...
Aortic Stenosis Client Handout PESC
Aortic Stenosis Client Handout PESC

... Blood is pumped from the left ventricle to a particularly large blood vessel called the ‘aorta’. (The aorta is the body’s largest artery.) The valve that separates the left ventricle from the aorta is called the ‘aortic valve’. The left ventricle narrows as it leads to the aorta and this area is cal ...
Valvular Heart Disease
Valvular Heart Disease

... smaller valve areas. in severe stenosis, a significant gradient is present at rest. ...
Unusual Presentation of Sturge Weber`s Syndrome with Cataract : A
Unusual Presentation of Sturge Weber`s Syndrome with Cataract : A

... symptomatic and prophylactic therapy for headache, glaucoma treatment to reduce the IOP, and laser therapy for PWS. l ...
AV Patterns and Other Strabismus 01212010
AV Patterns and Other Strabismus 01212010

...  Thyroid eye disease often co-exists with myasthenia so eval for this also  If optic nerve involved, may need orbital decompression prior to strabismus surgery  Surgery: optional oral prednisone for 2 weeks (“to soften things up”); then surgery (ie inferior rectus recession: aim to undercorrect ...
Premature opening of aortic valve in severe - Heart
Premature opening of aortic valve in severe - Heart

... valve has been noted (Wright, Toscano-Barboza, and Brandenburg, 1956): this phenomenon has been confirmed by echocardiography (Pridie, Benham, and Oakley, 197I). More recently it has also become possible to examine the aortic valve using reflected ultrasound, and characteristic features can be recog ...
Anaesthetic management of a patient with severe aortic stenosis for
Anaesthetic management of a patient with severe aortic stenosis for

... and the baby was breast fed. Further course in the hospital was uneventful and the patient was discharged 5 days later and advised to follow up with the cardiologist. DISCUSSION ...
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Marfan syndrome



Marfan syndrome (also called Marfan's syndrome) is a genetic disorder of connective tissue. It has a variable clinical presentation, ranging from mild to severe systemic disease. The most serious manifestations involve defects of the heart valves and aorta, which may lead to early death if not properly managed. The syndrome also may affect the lungs, eyes, dural sac surrounding the spinal cord, the skeleton, and the hard palate. People with Marfan syndrome tend to be unusually tall, with long limbs and long, thin fingers and toes.The syndrome is caused by the misfolding of fibrillin-1, a glycoprotein which forms elastic fibers in connective tissue and contributes to cell signaling activity by binding to and sequestering transforming growth factor beta (TGF-β). The mutated fibrillin binds poorly to TGF-β, which results in an accumulation of excess TGF-β in the lungs, heart valves, and aorta. This in turn causes abnormal structure and function of vascular smooth muscle and reduced integrity of the extracellular matrix, which weaken the tissues and cause the features of Marfan syndrome.Named after Antoine Marfan, the French pediatrician who first described the condition in 1896, the disease is an autosomal dominant disorder. Management often includes the use of angiotensin II receptor antagonists (ARBs) and beta blockers.
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