Mitral Valve Prolapse,

Flailed Mitral Valve

Mitral Annular Calcification
Mitral Valve Prolapse
 What is a syndrome?
 Mitral valve prolapse is called by many names.
What makes it a syndrome?
 What population is affected predominately
with this condition?
 What does the words myxomatous
degeneration mean and how does it relate to
mitral valve prolapse and mitral regurgitation?
 What portions of the mitral valve apparatus
can be affected?
 3-5% of the population
 Young thin females within reproductive years
 Hereditary incidence and autosomal dominant
 “Since a diagnosis of MVP carries with it a
need for antibacterial prophylaxis, as well as
the emotional burden of ‘heart disease,’ it is
important not to overdiagnosis it.” (M. Allen)
Classical Vs. Non-classical
MVP
 Classical MVP
 Associated with many conditions.
 Characterized by increased redundancy or thickening
(myxomatous changes) of varying portions of the
mitral valve leaflets
 Occurs quite commonly in inheritable disorders of
connective tissue
 Marfan syndrome
 Osteogenesis imperfecta
 Ebstein’s anomaly
 Ischemic papillary muscle
 Ballet dancers and patients with anorexia nervosa
Classic MVP cont…
 High incidence of patients with
asthenic habitus
 Thoracic deformities (pectus
excavatum)
 Scoleosis
Non-classic MVP
 Primary condition unassociated
with other diseases
 Mitral valve leaflet coaptation point
on the ventricular side of the mitral
annulus and no or minimal mitral
regurgitation
Secondary MVP
 Associated with CAD due to
displacement of a ischemic
papillary muscle
Functional MVP
 Disproportion of the mitral valve leaflets and
chordae in relation to the internal left
ventricular dimension.
 A reduction or alteration in left ventricular
cavity size or shape may cause normal
mitral valve leaflets to move past the mitral
valve annulus during ventricular systole
Myxomatous Appearance
 Loss or dissolution of normal dense
collagen fibers, with replacement and
invasion of a less sturdy type of
connective tissue (spongiosa)
 Leaflets, chordae, annulus all may be
affected by myxomatous proliferation
 Leaflets are thickened and redundant,
chordae can be elongated.
 Both leaflets can be affected, but the
posterior leaflet is more commonly
involved.
What constitutes
prolapse?
 Abnormal superior systolic displacement of
the mitral valve leaflets; one or both of the
leaflets extend beyond the normal systolic
coaptation point allowing MR to occur.
 The MR jet is not usually central but
eccentric depending on the leaflet that is
affected. E.g., if the anterior leaflet
prolapses, the MR jet usually extends
posteriorly.
 During systole, individual scallops
or an entire leaflet may billow
excessively into the left atrium.
 For severe MR to be present, both
leaflets must be affected or one
leaflet may be flail.
Signs and Symptoms
 Large majority are asymptomatic
 Anxiety, perhaps precipitated by having
been diagnosed with the presence of
heart disease.
 Palpitations, chest discomfort, dyspnea,
fatigability, syncope, pre-syncope.
 Rare cases of sudden death due to
malignant ventricular dysrhythmias
Physical Examination
 MVP is one of the few cardiac conditions in
which the outward appearance may suggest
the diagnosis
 Familial variety
 Red haired, fair skinned
 Asthenic with long extremities
 Some degree of pectus excavatum and less
commonly pectus carinatium
 Scoliosis
 Kyphosis
 Straight back (narrow A/P chest diameter)
 Small breasted in women
Heart Sounds
 Systolic click
 Most important auscultatory finding at
least 0.14 sec after S1.
 The mitral valve begins to prolapse
when the reduction of left ventricular
volume during systole reaches a
critical point at which the valve leaflets
no longer coapt; at that instant, the
click occurs and the murmur
commences.
 The click normally is found in mid to late
systole after S1
 Any maneuver that decreases left
ventricular volume, such as a reduction of
impedance to LV outflow, a reduction in
venous return, or an augmentation of
contractility, results in an earlier
occurrence of the prolapse in systole.
 What maneuvers would enhance?
 What maneuvers would diminish?
 Why?
 The click and onset of the murmur
move closer to S1.
 The S1 sound will appear to
increase in intensity
Systolic Murmur
 MVP can be associated with our
without a mid to late systolic,
crescendo or crescendo-
decrescendo medium to high-
pitched murmur. This is due to
what condition?
Chest X-ray
 May show the thoracic
abnormalities
 Cardiomegaly due to left atrial
enlargement and left ventricular
enlargement suggesting significant
MR.
Echocardiography
Treatment
 Usually no treatment
 MVR/replacement may be
indicated for the significant MR
Flailed Mitral Valve
 Define the term Flail.
 Flailed mitral valve is a ruptured
chordae/papillary muscle that will
result in the abnormal coaptation
of the mitral valve
Etiology
 MVP
 Severe MR
 Papillary muscle dysfunction from
MI or endocarditis
Signs and Symptoms
 Acute sudden onset of CHF and acute
pulmonary edema due to sudden increase in
volume in the left atrium during systole.
 The LA does not have time to take defense
against the sudden onset of blood volume an
rise in pressure.
 The edges of the valve leaflet slips
completely and points towards the left atria
during systole
 Acute MR is poorly tolerated and frequently
results in profound clinical deterioration.
 A large volume of regurgitation into a normal,
non-compliant atrium results in high left atrial
pressures.
 The left ventricle does not tolerate an acute
volume load when compensatory mechanisms
of dilation and hypertrophy do not have time to
develop; left ventricular diastolic and left atrial
pressures increase markedly
 Patients experience
 Dyspnea
 Orthopena
 Paroxysmal nochturnal dyspnea (PND)
 Chest discomfort
 Pulmonary edema
 Shock
 Surgery is need immediately
 Carpentier or Duran ring
Carpentier or Duran Ring
Mitral Annular Calcification
 MAC
 Found in the valve
rings or fibroskeleton
 Degenerative process
>40 yrs old, esp.
women
 Most common cardiac
abnormality found at
autopsy
MAC
 Usually limited to the posterior medial portion of
the annulus at the base of the posterior leaflet
and there usually is no complication
 When the calcification is more extensive, it may
involve the valve leaflets and cause limitation of
motion leading to regurgitation, when it is
severe it may be an important cause for MR or
cause of a gradient during diastole
Etiology
 Accelerated by systemic
hypertension, AS, chronic renal
failure with secondary
hyperparathyroidism and diabeties
 Marfan and Hurler syndromes
Pathophysiology
 Annulus normally measure 10 cm in
circumference, it is soft and flexible during
diastole and contraction of the ventricle
cause the annulus to constrict.
 When there is MAC it may immobilize the
basal portion of the mitral leaflets,
preventing their normal excursion in
diastole and coaptation in systole and
aggravating the MR that results from loss of
normal sphincteric action of the mitral valve
Signs and Symptoms
 Arrhythmias (atrial fibrillation,
PACs)
 Twice as likely to have
thromboembolic events