Mitral Valve Prolapse

Mitral valve prolapse (MVP) is a common dang usually asymptomatic billowing of

mitral leaflets into the left atrium during ventricular systole, sometimes accompanied by
Mitral regurgitaion.

Risk Factors

MVP may occur with greater frequency in individuals with:

1. Ehlers-Danlos Syndrome

Also known as "Cutis hyperelastica". Ehlers-Danlos Syndrome is a group of inherited

connective tissue disorders, caused by a defect in the synthesis of collagen (a protein in
connective tissue - usually Type I and III). The collagen in connective tissue helps tissues
to resist deformation (decreases its elasticity). In the skin, muscles, ligaments, blood
vessels and visceral organs, collagen plays a very significant role and with increased
elasticity, secondary to abnormal collagen, pathology results. Depending on the individual
mutation, the severity of the syndrome can vary from mild to life-threatening. There is no
cure, and treatment is supportive, including close monitoring of the digestive, excretory
and particularly the cardiovascular systems.

2. Marfan syndrome
Marfan syndrome is a genetic disorder of the connective tissue. It is sometimes inherited
as a dominant trait. It is carried by a gene called FBN1, which encodes a connective
 protein called fibrillin-1. People have a pair of FBN1 genes. Because it is dominant,
people who have inherited one affected FBN1 gene from either parent will have Marfan's.
This syndrome can run from mild to severe. People with Marfan's are typically tall, with
long limbs and long thin fingers.
The most serious complications are the defects of the heart valves and aorta. It may also
affect the lungs, eyes, the dural sac surrounding the spinal cord, skeleton and the hard
palate.
In addition to being a connective protein that forms the structural support for tissues
outside the cell, the normal fibrillin-1 protein binds to another protein, transforming
growth factor beta (TGF-β). TGF-β has deleterious effects on vascular smooth muscle
development and the integrity of the extracellular matrix. Researchers now believe that
secondary to mutated fibrillin there is excessive TGF-β at the lungs, heart valves, and
aorta, and this weakens the tissues and causes the features of Marfan syndrome.
3. Polycystic kidney disease

PKD is characterized by the presence of multiple cysts (hence, "polycystic") in both

kidneys. The cysts are numerous and are fluid-filled resulting in massive enlargement of
the kidneys. The disease can also damage the liver, pancreas, and in some rare cases, the
heart and brain. The two major forms of polycystic kidney disease are distinguished by
their patterns of inheritance.
Mitral valve prolapse in patients with prior rheumatic
fever
NJ Lembo, LJ Dell'Italia, MH Crawford, JF Miller, KL Richards and RA O'Rourke
Department of Medicine, University of Texas Health Science Center, San Antonio.

It is known that rheumatic heart disease frequently results in isolated mitral regurgitation
without concomitant mitral stenosis, especially in countries with a high prevalence of
rheumatic fever. However, more recent surgical pathologic data also have demonstrated a
high incidence of mitral valve prolapse in cases of rheumatic heart disease, which suggests
that rheumatic fever may be a cause of mitral valve prolapse. To determine whether this
association of mitral valve prolapse and rheumatic heart disease is present in a stable clinic
population, we studied 30 patients who had an apical systolic murmur and a well-
documented history of rheumatic fever with dynamic auscultation, two- dimensional
echocardiography, and pulsed Doppler examinations. Twenty of the 30 patients (67%) had
findings on physical examination consistent with isolated mitral regurgitation and 25 patients
(84%) had mitral regurgitation by Doppler examination. Echocardiography demonstrated
mitral valve prolapse in 24 patients (80%), whereas only one of the total study group had
echocardiographic findings consistent with mitral stenosis. We conclude that (1) the presence
of an isolated systolic murmur in patients with a history of rheumatic fever frequently
represents pure mitral regurgitation secondary to mitral valve prolapse and (2)
postinflammatory changes in valvular tissue resulting from rheumatic fever may be the
etiology of mitral valve prolapse in these patients.
 RHEUMATIC MITRAL VALVE PROLAPSE
The present study is a trial to find out the incidence of mitral valve prolapse in
patients with well documented history of rheumatic fever with or without rheumatic valvular
heart disease.
139 patients were included in our study " They were classified into 4 groups:-
Group I : Patients with well documented history of rheumatic fever.
Group II : Patients with rheumatic mitral regurgitation
Group III : Patients with rheumatic mitral stenosis.
Group-IVA : Patients with rheumatic combined mitral valve disease.
Group IVB : Patients with rheumatic multivalvular lesion.
Patients were refered on the basis of presence of one or more auscultatory signs of
M.V.P., well documented history of rheumatic fever and/or rheumatic valvular heart disease.
Control group of 25 healthy subjects was also included in the study.
M.V.P. on M. mode echocardiogram was considered when at least 3mm mid-late
systolic or 5 mm holosystolic posterior motion of one or both mitral leaflets relative to the
line joining the point of valve closure in systole and the point of valve opening in diastole.
On two dimensional echocardiogram, the parasternal long axis and apical 4 chamber
views were used for analysis of the pattern of mitral valve motion. Definite diagnosis of
M.V.P required leaflet bowing or protrusion to be of severe degree enough to bring the
coaptation point above or at least at the level of the mitral annulus. When failure of leaflet
coaptation occur (with mitral regurge) the distance between the maximally prolapsed segment
and the mitral annular plane should be 5 mm in either parasternal long-axis or apical 4-
chamber view for certain diagnosis.
Doppler echocardiogram was performed using the pulsed and continous mode. The
presence of associated mitral regurge was defined as a clear cut late or holosystolic
regurgitant jet with a peak velocity 150 m. and the severity of the regurge was
semiquantitated according to the depth of the regurgitant jet within the atrial cavity.
The most important symptoms among our patients were chest pain, palpitations,
fatigue, dyspnea, and syncope. The chest pain. palpitation showed no relation to the severity
of prolapse, however fatique, dyspnea and syncope were common with grade III M.V.P.
The auscultatory findings in our study groups were mid-late systolic or holosystolic
murmur with or without mid-late systolic click, and /or auscultatory signs of associated
valvular disease.
 The auscultatory findings were more common in groups (I,III). due to lower age
range when compared with other groups.Patients with M.V.P. in group I had higher incidence
of non ejection click, which was explained to be due to the higher incidence of A.L.P.
Patients of group I were found to have higher incidence of A.L.P., less severe degree
of M.V.P. (grade II), while patients of group II had a higher incidence of both L.P.
The incidence of M.V.P. was ( 62,86%) in group I, and (73.91%) in group II showing
a highly significant increase when compared with control group, however the incidence of
M.V.P. in other groups show no significant difference when compared with control group.
The incidence of M.V.P. was found to be more prevalent in patients with grade I
regurge, but has no relation to severity of mitral stenosis.
Echocardiographic L.V. systolic function was preserved. in patients with M.V.P. in
the group with well documented hisstory oF rheumatic fever (group I.) inspite of hemo-
dynamic alteration induced, by changes in L.V.E- S. D, and L.V.E.D.D. when compared
with control group.
We concluded that:-
1. The post inflammatory changes resulting from rheumatic activity may be the etiology
of M.V.P. in patients with prior rheumatic fever.
2. M.V.P. should be considered as one of the important causes of isolated mitral
regurge in rheumatic patients.
3. Patients with rheumatic isolated M.V.P. must be followed for progression to mitral
regurge and should have chemo-prophylaxis against rheumatic fever to prevent
further valvular damage.