MVP may occur with greater frequency in individuals with Ehlers-Danlos<unk> , Marfan<unk>  or polycystic kidney disease. Other risk factors include Graves disease and chest wall deformities such as pectus excavatum. For unknown reasons, MVP patients tend to have a low body mass index (BMI) and are typically leaner than individuals without MVP. Also women tend to have joint hypermobility. Rheumatic fever is common worldwide and responsible for many cases of damaged heart valves. Chronic rheumatic heart disease is characterized by repeated inflammation with fibrinous resolution. The cardinal anatomic changes of the valve include leaflet thickening, commissural fusion, and shortening and thickening of the tendinous cords. The recurrence of rheumatic fever is relatively common in the absence of maintenance of low dose antibiotics, especially during the first three to five years after the first episode. Heart complications may be long-term and severe, particularly if valves are involved. Rheumatic fever, since the advent of routine penicillin administration for Strep throat, has become less common in developed countries. In the older generation and in much of the less-developed world, valvular disease (including mitral valve prolapse, reinfection in the form of valvular endocarditis, and valve rupture) from undertreated rheumatic fever continues to be a problem. In an Indian hospital between 2004 and 2005, 4 of 24 endocarditis patients failed to demonstrate classic vegetations. All had rheumatic heart disease (RHD) and presented with prolonged fever. All had severe eccentric mitral regurgitation (MR). (One had severe aortic regurgitation (AR) also.) One had flail posterior mitral leaflet (PML). Micrograph demonstrating thickening of the spongiosa layer (blue) in myxomatous degeneration of the aortic valve. Movat's stain. The mitral valve, so named because of its resemblance to a bishop's mitre, is the heart valve that prevents the backflow of blood from the left ventricle into the left atrium of the heart. It is composed of two leaflets, one anterior and one posterior, that close when the left ventricle contracts. Each leaflet is composed of three layers of tissue: the atrialis, fibrosa, and spongiosa. Patients with classic mitral valve prolapse have excess connective tissue that thickens the spongiosa and separates collagen bundles in the fibrosa. This is due to an excess of dermatan sulfate, a glycosaminoglycan. This weakens the leaflets and adjacent tissue, resulting in increased leaflet area and elongation of the chordae tendineae. Elongation of the chordae tendineae often causes rupture, commonly to the chordae attached to the posterior leaflet. Advanced lesions—also commonly involving the posterior leaflet—lead to leaflet folding, inversion, and displacement toward the left atrium. In diagnostics Common risk factors in diagnostics for severe, arrhythmic mitral valve prolapse include: * Electrocardiography - Inferior T wave inversions in II, III, aVF leads, ST-segment depression, QT dispersion, QT prolongation, complex ventricular ectopy (PVCs), PVC-triggered ventricular fibrillation * Echocardiography - ≥ moderate mitral regurgitation, leaflet thickening, bi-leaflet MVP with redundancy, spiked systolic high-velocity signal (pickelhaube spike) when ≥ 16 cm/s in mitral annular tissue Doppler imaging (TDI), mitral annular disjunction (MAD) * Cardiac MRI - myocardial/papillary scars (fibrosis), myocardial stretch, systolic curling
complications of Marfan syndrome
medical diagnosis of Marfan syndrome
complications of Ehlers–Danlos syndromes
complications of Noonan syndrome
complications of Long QT syndrome
complications of Treacher Collins syndrome
complications of Hypertrophic cardiomyopathy
picture of Marfan syndrome