Title: Congenital Mitral Valve Disease
1Congenital Mitral Valve Disease
- Seoul National University Hospital
- Department of Thoracic Cardiovascular Surgery
2Congenital 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
3Pathophysiology 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.
4Clinical 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
5Patterns 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.
6Types 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)
7Supramitral 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
9Parachute-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.
10Congenital 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
12LV 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)
14Indications 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
15Operative 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
16Mitral Valve Repair for Multiple Leaflets
- Remodeling the annulus
- with Ring(1) after annular
- plication with resected
- posterior median leaflet(2)
17Anatomy 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
18Aims 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 )
19Complications 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
20Postoperative 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.