Congenital Mitral Valve Disease - PowerPoint PPT Presentation

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Congenital Mitral Valve Disease

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Types of Congenital Mitral Stenosis Supravalvular * Fibrous ring * Shone s syndrome Valvular * Fusion of commissure & papillary muscle * Double ... – PowerPoint PPT presentation

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Title: Congenital Mitral Valve Disease


1
Congenital Mitral Valve Disease
  • Seoul National University Hospital
  • Department of Thoracic Cardiovascular Surgery

2
Congenital Abnormalities of Mitral Valve
  • Incidence
  • 0.2-0.4 of all CHD


  • MS 0.6 in autopsy
  • MR 0.5 in autopsy
  • Associated lesions ASD, VSD, PS, COA,
    LVOTO
  • Mitral valve malformations may
    constitute an integral part of
  • complex lesions such as hypoplastic
    left heart syndrome,
  • Shones complex, atrioventricular
    canal, double inlet ventricle.
  • Pathophysiology
  • MS (75-80 have associated lesions)
  • MR
  • MSR

3
Pathophysiology of Congenital Mitral Valve Disease
  • Malformation of the mitral valve apparatus
    results in mitral stenosis, insufficiency, or
    both.
  • Significant flow obstruction or regurgitation of
    the mitral valve results in elevated pulmonary
    venous pressure and pulmonary congestion.
  • Left untreated, pulmonary vascular occlusive
    changes and pulmonary hypertension lead to right
    ventricular hypertrophy and right heart failure.

4
Clinical Features of Congenital Mitral Valve
Disease
  • Symptoms
  • Fatigue, DOE, recurrent pulmonary infection,
  • tachypnea, growth retardation, central
    cyanosis.
  • ? Severely affected infants have CHF with
    gross
  • pulmonary edema
  • Signs
  • Systolic diastolic murmur, redistribution
    of
  • pulmonary vascularity, cardiac enlargement,
  • LA enlargement, increased MPA, LV
    enlargement in MR

5
Patterns of Congenital MV Diseases
  • Mitral Stenosis
  • 1) Associated lesions
  • 25 isolated
  • 30 VSD
  • 40 LVOT obstruction( 2 of COA has
    congenital MS)
  • others TOF, VSDPS, PS
  • 2) Symptom signs
  • usually severe if untreated, death
    during 1st 45 years of life
  • Mitral Insufficiency
  • Often only moderate in severity in early
    life, and 1/2 patients
  • come to operation until older than about 5
    years of age.

6
Types of Congenital Mitral Stenosis
  • Supravalvular
  • Fibrous ring
  • Shones syndrome
  • Valvular
  • Fusion of commissure papillary muscle
  • Double orifice
  • Excessive valve tissue
  • Annular hypoplasia( usually associated
    with HLHS)
  • Subvalvular
  • Single papillary muscle (Parachute valve)
  • Abnormally large or numerous papillary
    muscle (Hammock valve)
  • Absent papillary muscle
  • Abnormalities associated with LVOTO
  • Abnormal attachment of the subvalvular
    apparatus to the septum.
  • Systolic anterior movement( usually with
    hypertrophic cardiomyopathy)

7
Supramitral Ring
8
Congenital Mitral Stenosis
Functional Classification (Carpentier)
  • 1. Normal papillary muscle
  • 1) Commissural papillary fusion
  • 2) Excessive leaflet tissue
  • 3) Valvar ring
  • 4) Annular hypoplasia
  • 2. Abnormal papillary muscle
  • 1) Parachute mitral valve
  • 2) Hammock mitral valve

9
Parachute-like Asymmetric Mitral Valve
  • Unequal distribution of chords
  • One normal and one elongated papillary muscle
  • Elongated papillary muscle is displaced toward
  • the MV annulus.
  • The anterolateral papillary muscle is usually
  • abnormal.
  • Clinical implications are mitral stenosis and
  • other malformations are present.

10
Congenital Mitral Regurgitation
  • Annulus
  • 1) Dilation
  • secondary due to ventricular dilation (
    rare in primary causes)
  • (endocardial fibroelastosis,
    infarction, ischemia, ASD)
  • Leaflets
  • 1) Cleft
  • 2) Leaflet defect
  • hole due to localized agenesis
    (posterior)
  • 3) Mitral valve prolapse
  • rupture or elongated chordae tendinea
  • 4) Congenital perforation or displacement
  • Ebsteins anomaly of MV
  • 5) Duplication of orifice
  • Subvalvular apparatus
  • 1) Bridge of fibrous tissue ( anomalous
    arcade) with shortened or absent chordae
  • 2) Shortened chordae tendineae
  • 3) Abnormal papillary muscle

11
Congenital Mitral Regurgitation
Functional classification
(Carpentier)
  • 1. Normal motion of the leaflet (type I)
  • 1) Deformation dilatation
  • 2) Clefts
  • 3) Partial agenesis
  • 2. Prolapse of leaflet (type II)
  • 1) Absence of tendinous cords
  • 2) Elongation of tendinous cords
  • 3) Elongation of papillary m.
  • 3. Restricted motion of leaflet (type III)
  • 1) Normal papillary m.
  • commissural fusion / shortness of
    cords
  • 2) Abnormal papillary m.
  • parachute / Hammock / hypoplasia of
    papillary m. /
    Ebstein
    malformation / double orifice

12
LV Dysfunction in Mitral Regurgitation
  • 1. Indicators
  • 1) NYHA status
  • 2) Ejection fraction
  • 3) LV end-diastolic end-systolic
    dimension
  • 4) Rate of rise of LV pressure
  • 5) Left atrial size
  • 6) Pulmonary artery pressure
  • 2. Misleading
  • It is possible to have LV dysfunction in
    the presence
  • of normal LV dimension minimal symptoms
    due to
  • afterload reduction of the regurgitant
    mitral valve.

13
Pulmonary Hypertension in MVD
  • 1. Increased LAP transmitted on a retrograde
  • basis into the arterial circulation
  • 2. Vascular remodeling of pulmonary vasculature
  • in response to chronic obstruction to
    pulmonary
  • venous drainage(fixed component)
  • 3. Pulmonary arterial vasoconstriction
  • (reactive component)

14
Indications for Mitral Valve Operation
  • Severe symptoms signs of important pulmonary
    venous hypertension are indications of mitral
    valve anomalies
  • Infancy
  • 1) Mild to moderate symptoms with certainty
    without valve
  • replacement supravalvular ring,
    commissure fusion
  • 2) Other circumstances
  • reserved only for infants with
    intractable heart failure
  • Childhood
  • 1) Considerations for operation are similar
  • CHF
  • pulmonary hypertension secondary to
    MVD
  • 2) MVR should be withheld whenever possible

15
Operative Procedures for Mitral Valve
  • 1. Mitral regurgitation
  • 1) Eccentric annuloplasty
  • 2) Ring annuloplasty
  • 3) Leaflet plication chordal shortening
  • 4) Suture of cleft
  • 5) Valve replacement
  • 2. Mitral obstruction
  • 1) Resection of supravalvular ring
  • 2) Open commissurotomy splitting
  • 3) Correction of parachute deformity
    splitting fenestration
  • 4) Valve replacement

16
Mitral Valve Repair for Multiple Leaflets
  • Remodeling the annulus
  • with Ring(1) after annular
  • plication with resected
  • posterior median leaflet(2)

17
Anatomy of Mitral Annulus
  • C-shaped portion touches the underlying LV
  • wall, the remaining 25-30 of annulus is
  • intracavitary.
  • Muscular representation is the basis for
  • geometric relationship, being more elliptic
  • in systole and circular in diastole.
  • To be a support for the leaflet attachment
  • To insulate electrically the atrium from
    ventricle

18
Aims for Mitral Valve Annuloplasty
  • Reduce annular dilation mitral valve area
  • Increase the leaflet coaptation
  • Reinforce the annulus sutures when part of
  • the valve has been resected
  • Prevent future dilation of annulus
  • ( Stabilization of the posterior annulus )

19
Complications of Mitral Annuloplasty Ring
  • 1. Postoperative valvular incompetence
  • most common mild in 15 by Echo
  • 2. LVOT obstruction 3
  • 3. Suture line dehiscence 2
  • 4. Dehiscence of prosthetic ring, fracture,
    hemolysis
  • 1
  • 5. Aortic regurgitation infrequent

20
Postoperative Course Results
  • Mortality
  • Usually higher than adult ( LCO,
    pulmonary hypertension)
  • Complication
  • Low cardiac output
    Neurologic complication
  • Bleeding and infection
    Arrhythmia
  • Pulmonary insufficiency
  • Risk factors
  • Young age
  • Preoperative functional status
  • Associated cardiac anomaly
  • Long-term result
  • Most survivors improve functionally.
  • Better for mitral valve repair than
    replacement
  • Residual stenosis or regurgitation is
    present in most can progress.
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