A 30 year old female presented with complaints of progressive breathlessness since 6 months. There was history of intermittent chest pain and palpitation. On examination her HR was 88/min and BP was 140/80 mm of Hg and a late systolic murmur with midsystolic click at cardiac apex. Pathological examination of the heart is most likely to show which of the following in this patient?
A. Fatty replacement of the ventricle with thinning of the RV free wall
B. Hooding of the mitral valve leaflets with myxomatous degeneration C. Ischemia of the papillary muscles
D. Elongated and ruptured chordae tendineae
Ans. B. Hoodingof the mitral valve leaflets with myxomatous
degeneration.
Myxomatous
degeneration of the mitral valve with hooding and prolapse of the posterior
mitral leaflet into the left atrium is characteristic of mitral valve prolapse.
Prolapsing
,billowing. mitral valve:-
This
is also known as Barlow's syndrome or floppy mitral valve. It is due to
excessively large mitral valve leaflets, an enlarged mitral annulus, abnormally
long chordae or disordered papillary muscle contraction. Histology may
demonstrate myxomatousdegeneration of the mitral valve leaflets. It is more
commonly seen in young women than inmen or older women and it has a familial
incidence. Its cause is unknown but it
is associated with Marfan'ssyndrome, thyrotoxicosis, rheumatic or ischaemic
heart disease. It also occurs in association with atrial septal defect and as
part of hypertrophic cardiomyopathy. Mild mitral valve prolapse is so common
that it should be regarded as a normal variant.
Pathophysiology
- During ventricular systole, a mitral valve leaflet most commonly the
posterior leaflet. prolapses into the
left atrium. This may result in abnormal ventricular contraction, papillary
muscle strain and some mitral regurgitation. Usually.the syndrome is not
haemodynamically serious. Thromboembolism occurs.
Symptoms: Atypical chest pain is the most common
symptom. Usually the pain is left submammary and stabbing in quality. -
Sometimes it is substernal, aching and severe. Rarely it is similar to typical
angina pectoris. Palpitations may be experienced because of the abnormal
ventricular contraction or because of the atrial and ventricular arrhythmias
that are commonly associated with mitral valve prolapse. Sudden cardiac death due to fatal ventricular
arrhythmias is a very rare but recognized complication.
Signs The most common sign is a mid-systolic click, which is produced by
the sudden prolapse of the valve and the tensing of the chordae tendineae that
occurs during systole. This may be followed by a late systolic murmur owing to
some regurgitation. With more regurgitation, the murmur becomes pansystolic
mitral regurgitation.