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Hans Dietz is a 25 year old male patient with a family historyof Marfan Syndrome (MFS). He is generally well and enjoys attendingregular fitness sessions in his local gymnasium. During a recentgym session, he experienced dizziness followed by a faintingepisode. He presented at the emergency department and a newsystolic murmur was heard. Mr Dietz subsequently underwentscreening for cardiovascular abnormalities associated with hisfamily history of Marfan Syndrome.

Question 1

The fibrous structure of the heart is comprised predominantly ofconnective tissue proteins.

Describe the microscopic composition of the layers of the mitralvalve. Your answer must include a description of the connectivetissue element, the cell types found in each layer and how thelayers are arranged (i.e. their structure or architecture).(3 marks)           

Question 2

Upon further evaluation with an echocardiogram (cardiacultrasound), Mr Dietz was found to have an enlarged (dilated)proximal ascending aorta, mitral valve prolapse with myxomatouschanges and elongated chordae.

The patient was noted as having myxomatous changes in the mitralvalve leaflets and associated elongation of the chordae tendineae.Explain what is meant by the term myxomatous changes. Your answermust include a description of the cellular and structural changesthat occur within the valve. (2marks)

Question 3

Where on the body would be the ideal position for the clinicianto auscultate for a systolic murmur associated with the mitralvalve? (2 marks)

Question 4

Describe the secondary complications (or consequences) that willoccur as a result of the changes in the structure of the mitralvalve in a patient with Marfan syndrome. Your answer should link tothe clinical sign of the new systolic murmur noted in this patient.(3 marks)