LVOTO and Pulmonary Valve stenosis课件

1、Left Ventricle Outflow Tract Obstruction (LVOFTO) and Pulmonary Valve stenosis.Tsinghua Surgical Program 2014.Dr P. PohlnerThe Prince Charles HospitalBrisbane Australia LVOFTO: types to consider. Asymmetric Septal Hypertrophy (ASH) one form of Hypertrophic Cardio-Myopathy (HCM) Sub-aortic stenosis.

2、Aortic valve stenosis (annular/leaflet) Supravalvar aortic stenosis (Williams syndrome)Asymmetric Septal Hypertrophy HCM incidence 1:500 ASH is ECHO. Contrast MRI locates scarring.Surgical management If peak gradient 50mmHg at rest or exercise. Trans-aortic septal myomectomy. (may require extended m

3、yomectomy for mid cavity obstruction). Mitral valve: assess papillary muscle attachments. Risk to survival 1%. Improves survival and functional capacity with minimal recurrence risk ( 40mmHg, LVH, (risk 1%)Discrete Subaortic membraneNote tethered base of anterior MV leafletGross Asymmetric Septal Hy

4、pertrphySubaortic Septal muscle 4cm thick.Fibro-muscular Septal HypertrophyFibromuscular septal hypertrophy Note involvement of base of aortic valve leafletAortic valve stenosis Incidence: - 1.3% of population; ( 3 - 8% of CHD) 3M : 1Female Presentation:- Foetal ECHO (?balloon) - Neonatal: ? fibroel

5、astosis, coarctation, VSD, PDA- Childhood: fixed valvar aortic stenosis (10-20%)- Adult: - Congenital - Acquired: Senile Ca+n, rheumatic, rheumatoid,Tubular subaortic stenosisRequires extensive muscular resection for relief.Normal Aortic Valve StructureRight Coronary arteryLeft Coronary ArteryNodule

6、 of Aranti (coaptation facets)Thickened aortic wall at commissuresLeaflet composition;- Lamina Ventricularis inflow surface (radial elastic fibres)- Corpus Spongiosum (fibroblasts, mucopolysaccharides, mesenchyme) - Lamina Fibrosa: outflow surface (circumferential collagen fibres.)Types of Congenita

7、l Aortic Valve Stenosis Congenital:1. 44%2. 11%3. 7%4. 0.6%5. 37%LCARCANeonatal aortic stenosis Frequently diagnosed by foetal U/Sound. Aortic arch development often abnormal. May be associated with L Ventricle fibro-elastosis. If severe - requires PGE1 use soon after delivery. If annulus 40mmHg wit

8、h LVH, AVA 50 yrs. Surgery - if mean gradient 40mmHg - AVA 0.5 cm2/m2. Risk to survival: R). Obstructive lesions may involve ascending aorta, arch and branches, descending aorta and visceral branches. (MAASyndrome) and MPA. Diagnosis: Clinically: - facies, sociability, ES murmur- ECHO, MRI/CT Angio

9、for systemic &pulmonary artery involvement Management: Surgical when gradient 40mmHg systolic, LVH, evidence of ischaemiaWilliams syndromeWilliams syndromeSupra valvar aortic stenosisSupra valvar Aortic stenosis Surgical points:- Sino-tubular region requires circumferential enlargement, retainin

10、g commissure integrity.- leaflet - commissural junction often requires thinning.- Coronary ostia may need enlargement- Proliferative medial tissue involving ascending aorta should be excised.- Enhance non-coronary sinus to ascending aorta with patch beyond involved aortic wall.Pulmonary valve Stenos

11、is/Atresia Pulmonary Atresia Intact Ventricular Septum (PAIVS)- Present with cyanosis, tachycardia- require PGE1 soon after birth + ? Septostomy- need RV angio to define sinusoids if present.- ECHO for RV and Tricuspid valve sizeSurgical management:- If sinusoids small shunt only- no sinusoids &

12、 Tricuspid valve 50% . TAP & shunt.Definitive management of PAIVS Biventricular repair: when RV and TV 50% normal size. 11/2 ventricular repair if RV & TV 25-50%. Fontan: if RV 25%. Results: - Hospital Mortality trileaflet valve, leaflets thickened. Pulmonary Valve Stenosis Diagnosis: CXR MP

13、A dilatation, oligaemic lungs, cyanosis. Management options:- balloon dilatation. (Size 1.2 annulus) single or double balloon technique. - Surgical valvotomy and relief of RVOFTO. Need to discuss case by case with cardiologists.Critical pulmonary valve stenosisCritical pulmonary valve stenosisValvot

14、omy Results Balloon Valvuloplasty: Infants/Neonate: - Mortality 3%, morbidity 10%.- 5-10% need shunt.- 10% redo 12 Mo- 15-20% still obstructed; need operation- mod-severe Pulmonary regurgitation = 74%. (PVR 3% at 10 years). Children / Adults: - Mortality 1%.- RVSP 35mmHg 90%- Freedom From Intervention (FFI)10yrs 83%.- Surgical intervention if complications (perforation, Tricuspid Valve damage), small annulus or dysplastic valve.- Valvotomy resultsSurgical valvotomy For Balloon failure / complications, shunt required (? PGE1), small annulus, dysplastic valve (Noo