Showing posts with label Marfan syndrome. Show all posts
Showing posts with label Marfan syndrome. Show all posts

Tuesday, July 2, 2013

Aortic Valve

Aortic valve
What an aortic valve is:

The heart valve present in between the left ventricle and aorta is called aortic valve which is a tricuspid valve. In some people about 1% of the population has inborn bicuspid aortic valve. The valve is named so because it is present in between the left ventricle and aorta as well as it controls the flux of blood from left ventricle towards aorta.

Function:

When there is a ventricular systole the pressure in the left ventricle increases and this leads to the greater pressure in the left ventricle as compared to the aorta and ultimately this all results in the opening of the aortic valve. As the aortic valve the blood flux moves from left ventricle into the aorta. At the completion of the ventricular systole the ventricular pressure drops suddenly and at the same time the pressure in the aorta rises and this leads to the closure of the aortic valve. This opening and closing of the aortic valve produces the heart sound S2.

Disorders relating aortic valve:

There are two types of disorders responsible for the improper working of the aortic valve and those are:

• Aortic stenosis
• Aortic regurgitation

Aortic stenosis stands for the incomplete opening of the aortic valve hence blood doesn’t completely move into the aorta while aortic regurgitation is the disorder in which the aortic valve doesn’t close properly and in return the blood moves towards the wrong direction i.e. back towards the left ventricle is the disorder in which the aortic valve doesn’t close properly and in return the blood moves towards the wrong direction i.e. back towards the left ventricle.

Causes of aortic valve disorders:

Rheumatic fever is the common cause of both aortic stenosis and aortic regurgitation while other causes of aortic stenosis are degenerative calcification and inborn bicuspid aortic valve. Enlargement of the aorta, infective endocarditis, myxomatous, Marfan’s syndrome and breakdown of the aortic valve are responsible for the aortic valve regurgitation.

Bicuspid aortic valve:

It is an inborn disorder of aortic valve and it is present in only 1% of the population. In this congenital disorder there are two leaflets of the aortic valve instead of the three leaflets. This disorder is left undiagnosed until the symptoms of aortic stenosis occur in later life. The occurrence of aortic stenosis in bicuspid aortic valve people is faster then the people with normal tricuspid aortic valve. Turner’s syndrome is also a cause of associated bicuspid aortic valve.

Aortic valve replacement:

The replacement of a diseased aortic valve with a new healthy valve is called aortic valve replacement. There are different diseases which can leads to the aortic valve replacement. Two conditions can lead to aortic valve replacement which is either the leakage of the aortic valve or the partial closure of the aortic valve. There are further two types of aortic valve replacement which depends upon the type of valve used to replace the diseased aortic valve. There are two types of aortic valves which are:

• Biological aortic valve:
This type of aortic valve is extracted from a living being and is placed in the diseased patient.

• Mechanical valve:
This type of valve is also called artificial aortic valve as this valve is made up of cloth, metal or artificial tissues.

Wednesday, September 5, 2012

Marfan syndrome life expectancy

When we talk about Marfan syndrome should really talk about Marfan syndrome life expectancy because it is a disease that undiagnosed, timely can lead to the death of the patients.
Marfan syndrome is a genetic disorder (autosomal dominant) characterized by connective tissue damage. This syndrome includes multiple malformations of organs (bones, eyes, skin, intestines, heart and lungs).

Marfan syndrome symptoms

Most of the visible signs or symptoms occur in the skeletal system. The patients  suffering from Marfan syndrome are taller than patients without the disease and arachnodactyly ( very thin and long fingers). Another anomaly in the skeletal system is pectus excavatus (deformity of the anterior wall of the chest which penetrates inside sternum) and scoliosis (abnormality of the spine that consists of spine deformity in the form of the letter S). Other signs include abnormal joint flexibility and sometimes pain in joint.
The most important signs and symptoms  associated with Marfan syndrome are the cardiovascular symptoms. This are mitral or aortic valve prolapse (the most common heart valve abnormality) and aneurysm of the aorta.  Many patients with Marfan syndrom have some type of vision problems of which the most important are myopia (a visual anomaly in which distant objects appear blurred), glaucoma and cataracts. This can severely influence Marfan syndrome life expectancy.

Marfan syndrome life expectancy

Marfan syndrome life expectancy may be different from patient to patient. There is no treatment for the Marfan syndrome, but life expectancy may be influenced by monitoring and treatment of complications that can occur. Marfan syndrome life expectancy increased lately because of the advancement in medical techniques and appearance of new treatments.
Best way to expend Marfan syndrome life expectancy is tracking the aneurysm of the aorta and mitral valve prolapse. This is done by a cardiologist annual control for monitoring the health of the heart valves and the aorta. This is done by performing an ECG or another radiological method for the visualization of the anomaly (such as CT or MRI image). The goal of treatment in Marfan syndrome is to slow the progression of the aortic dilatation and to prevent the dissection of the aorta. Another goal of the treatment is to eliminate arrythmias and diminuate the heart rate.
The skeletal manifestations of this syndrome are also important for Marfan syndrome life expectancy. They can be serious and can affect patients life. To solve these problems are used different kinds of pain medication or muscle relaxants.
Because some of the Marfan symptoms of the spine may be asymptomatic, but serious if are not treated, they can be solved by surgery and thus increase the Marfan syndrome life expectancy.
Lung damage involves spontaneous pneumothorax and Marfan syndrome life expectancy depends on the volume of air in the pleural space.  It may resolve by itself or can be made by chest drainage.
Conclusion

Today patients with this syndrome needs to know is not the end of life and Marfan syndrome life expectancy can be good if patients are addressed in time to the doctor.

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