When should we intervene surgically in pediatric patient with MR?

Transcription

1 When should we intervene surgically in pediatric patient with MR? DR.SAUD A. BAHAIDARAH CONSULTANT, PEDIATRIC CARDIOLOGY ASSISTANT PROFESSOR OF PEDIATRICS HEAD OF CARDIOLOGY AND CARDIAC SURGERY UNIT KAUH

2 Mitral valve anatomy Saddle shape not a real planner circle two high points one at the site of aortic-mitral continuity and at the posterior aspect of the valve two low points are along the lines of the commissure between the aortic and mural leaflets Two leaflet : Anterior leaflet is semicircular ( aortic cusp) Posterior leaflet is rectangular (mural cusp) Each leaflet usually subdivided by minor commissures into three or more semicircular scallop Two Commissures chordae tendineae Papillary muscles : Antrolateral Posteromedial

3 Assesment of mitral regurgitation Vena contracta measurement Jet area in LA LA and LV dimentions Pulmonary Venous Doppler Pattern

4 Continuos Doppler Regurgitant Jet Profile Mitral E Velocity Dominance

5 Qualitative assessment method Regurgitation stroke volume Regurgitation fraction PISA (Proximal isovelocity surface area) EROA ( Effective regurgitation Orifice Area)

6 Assessment of mitral valve apparatus morphology and function, including: the integrity of chordal and leaflet support structures; length of the chordae; chordal attachments and papillary muscle location and number careful definition of the annular plane; identification of MVP; localization of the prolapsing leaflet segment identification of MV cleft attachment location identification of associated conotruncal and atrioventricular canal lesions feasibility of biventricular repair by visualizing the pathway from LV to outflow as it relates to the ICMV or SMV attachments Full interrogation of all MR jets: localize the regurgitation orifice; assess the degree of MR using multiple methods to overcome limitations of each: color flow mapping (spatial distribution width of the jet at the vena contracta continuous-wave Doppler with analysis of the spectral pattern; pulsed-wave spectral Doppler of pulmonary veins; consider quantification of the regurgitant volume. Anatomic and hemodynamic assessment of the MV flow orifice: annulus size dimensions in two orthogonal planes assessment of anatomic flow orifice by two dimensional (2D) imaging recognize physiology that leads to reduced orifice flow atrial septal defect (ASD) and/or reduced cardiac output; Doppler interrogation. Left atrial size Left ventricular size Estimation of right ventricular pressure Ventricular function. Echocardiography in Pediatric and Congenital Heart Disease From Fetus to Adult, Second Edition by Wyman W. Lai MD, Luc L. Mertens MD, Meryl S. Cohen MD, Tal Geva MD

7 Objectives of Preoperative TEE Examination Define morphology and function of the mitral valve and its support apparatus Obtain measurements of the mitral valve annulus, preferably from several different planes Determine whether the primary pathology is stenosis or regurgitation (or both) If stenosis is present, define the nature of the obstruction,and any additional level(s) of obstruction If regurgitation is present, assess the mechanism of regurgitation Evaluate the hemodynamic severity of stenosis/regurgitation and secondary effects on cardiac structures Estimate right ventricular/pulmonary artery pressures Characterize associated lesions as indicated Assess left atrial and ventricular size, and ventricular function Transesophageal Echocardiography for Congenital Heart Disease Pierre C. Wong Wanda C. Miller-Hance

8 3D Echocariography role Qualitative assessment Detailed anatomy and mechanism of pathology

9 Causes of Mitral Regurgitation in Pediatrics Mitral Valve Prolapse : Systolic extension of a leaflet segment beyond the annular plane. Classic MVP : is defined as systolic leaflet displacement of >2 mm and diastolic leaflet thickness exceeding 5 mm Non classic MVP : when the systolic displacement is >2 mm with leaflet thickness <5mm. Borderline displacement <2mm is considered a normal variant and is not associated with morbidity or progression Prevalence and clinical outcome of mitral-valve prolapse.freed LA, Levy D, Levine RA, et al N Engl JMed 1999;341(1):1 7-. Threedimensional echocardiographic reconstruction of the mitral valve, with implications for the diagnosis of mitral valve prolapse.levine RA, Handschumacher MD, Sanfilippo AJ, et al Circulation 1989;80(3):

10 Other congenital mitral valve anomalies : Mitral valve cleft Straddling of mitral valve Double orifice mitral valve Para shout valve

11 Acquired causes of mitral valve regurgitation Rheumatic heart disease Infective endocarditis Myocardiatis Dilated cardiomyopathy Ischemic cardiomyopathy

12 Carpentier classification for mitral regurgitation Carpentier A, Branchini B, Cour JC, et al. Congenital malformations of the mitral valve in children. Pathology and surgical treatment. J Thorac Cardiovasc Surg. 1976;72:

13 Aim of Mitral Valve surgery Improving the outcome of the individual patient with regard to longterm mortality and morbidity. Preserving left ventricular function. Achieving mitral valve repair. Management of valvular mitral regurgitation:the importance of risk stratification Raphael Rosenhek (MD), Gerald Maurer (MD) Journal of Cardiology (2010) 56,

14 Why we prefer to delay MV surgery? Patient size Risks of mitral valve replacement including appropriate valve size and anticoagulation The early the surgery the high is the reoperation risk Late Left Ventricular Function After Surgery for Children With Chronic Symptomatic Mitral Regurgitation Usha S. Krishnan, Welton M. Gersony, Erika Berman-Rosenzweig, Howard D. Apfel Circulation. 1997;96:

15 Why the pediatric population is different from adult? Variable age, body size including Hight and weight Changing parameter with age Limitations of quantitative mitral valve assessment Structural abnormalities of mitral valve Associated cardiac lesions

16 6 year ( jan 2005-Dec 2010) 778 MV procedure in 673 Diagnoses : AV canal 67% Congenital MS isolated or associated 17% Congenital MR 3.7 % VSD/TOF/DORV 2.7% RHD 2.5 % Marfan/Ehker Danlos 1.5% Others 33.5 %

17 364 MV repair (AV canal excluded ): MR 53% MR & MS 25 % MS 12 % Other indication 10 % 54 pt had MV replacement : MS & MR 48% MS 37 % MR 13 % Other indication 2 % Results Mortality : Early 2.8 % Overall 4.1 % 5 year survival (excluding AV canal) : MV repair 95% MV replacement 81% Reoperation : 11% AV canal, MS patients Over all reoperation free at 5 years 89 %

18 in two affiliated hospital in Shanghai china 107 patient with MR 89 associated with cardiac lesions 6 due to infective endocarditis 12 isolated MR Age : 2months 6 years median 19 month Less than 1 year 42 % Weight : 4 21 Kg MR : Median 12.3 kg 47.7 % less than 10 kg Moderate 38/107 Severe 69/107 Based on the maximum length and width of jet in relation of LA

19 Follow up : 3-80 months with mean 46 mo ( about 4 y) in only 85% cohort Post OP mortality 0.9 % (VSD,PHTN) 1 died after 10 months with CHF 5 moderate MR responding for treat 1 severe MR needed replacement after 1 y In VSD + MR moderate to severe: Abnormal MV repair Normal structure repair to smooth post OP period

20 Conclusion Pediatric patients with moderate or severe MR need early surgical treatment. At present, MV annuloplasty is the objective in children with significant MR, and the concomitant cardiac anomalies should be treated at the same time. Individualized treatment based on the specific pathology is the key to successful surgical therapy for pediatric

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22 56 patient : 52 MR 4 Ms 82% associated with cardiac lesions Mitral valve repair Number of procedure Mitral valve replacement Age (mean) 3m-15y (3.6 y), 5 pt < 1y 2m-16y (5.7) Weight (mean) (12.7 kg) (18.5 kg) Hospital deaths 2 (5.6%) mediastinitis,infective end Survival at 10 years 87.2% 90.3 Freedom from reoperation 72.7% 67.3 conclude that the current operative risk of mitral valve operation is low, irrespective of severe deformation of the mitral valve apparatus and associated complex cardiac anomalies. 0

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24 Cases , 36 years 93 patients all MV repaired Median age 4.5 year Devided in to 2 groups : Moratlity : Group 1 : MR patient Group 2 : MS patient 72% associated with cardiac lesions Early 7.5 % Late 8 % with mean follow up of 10.3 years Reoperation in 12 patient 11 replacement and 1 repair Conclusion : surgical repair of MV dysplasia in infants and children is an effective and reliable treatment with a decreasing early and late mortality and reoperation rate, even in the most complex anatomic variants. MV reconstruction needs to be carefully planned and tailored to each patient, with the aim of achieving a physiological repair rather than an anatomic one

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26 data Inclusion criteria : Childern <18 years MV repair or replacement Exclusion criteria : New York Heart Association Class III or IV symptoms Acute MR Dominant mitral stenosis Cardiomyopathy Concurrent hemodynamically significant lesion that could contribute to LV enlargement (aortic insufficiency, ventricular septal defect, or patent ductus arteriosus) Postoperative follow-up of less 6 months

27 Data collected and Followed : LV dimentions : LVEDd, LVEDs with z scors EF SF Age : MV regurgitation assessment 6 m 17.4 y median 8.6 y 39 child with MV procedure : 25 pre OP asymptomatic with F/U more than 6 months 14 : 10 with symptoms and 4 without follow up data were excluded MV repaired in 17 MV replaced in 8

28 Reason for referral for surgery : 10 with significant or increasing LV enlargement 4 atrial arrhythmias 3 high likelihood of repair 2 pulmonary hypertension 2 increased fatigue without exercise intolerance 1 decreased LV systolic function 3 could not be determined from the medical record

29 Result : Residual MR less than moderate in 22/25 (88%) 9/25 (36%) had Late LV dysfunction ( EF< 55%, SF < 28% ) Type of surgery repair vs replacement has no risk LVESZ > 5 and SF <33 as risk, sensitivity of 89 % (95 % CI %), specificity of 88 %(95 % CI %), negative predictive value of 93 %, and positive predictive value of 80 % Conclusion : In children with asymptomatic chronic MR, the risk of late LV dysfunction increases with increased preoperative LV size (LVESZ) and decreased LV systolic function. To reduce the risk of late postoperative LV dysfunction in asymptomatic children with chronic MR, surgery should be considered before LVESZ > 5 and SF < 33 %. In asymptomatic patients with SF > 33 %, observation with serial echocardiographic measurements may be appropriate.

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31 patient ( excluded AV canal, straddling MV, acquired disease, Marfan,degenerative ) : 7 patient MV replacement 138 patient MV repair Age 2m 12 years Symptoms at the time of operation : 47 patients(32.2%) were in New York Heart Association class II 73 patient (50.7%) in class III 25 patient (17.1%) in class IV

32 In hospital mortality : Repair 5 % Replacement no mortality 10 years survival : Repair 88% Replacement 51 % Conclusion : Despite the complexity of the malformations, congenital MR can be effectively treated by reconstructive surgery.

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35 Mitral regurgitation in adult Chronic Primary MR : Abnormal mitral valve Causes most common : MVP Chronic secondary MR : Normal structure valve Causes : Ischemic related to CAD Barlaw s valve : maxymatous degeneration of valve component severe, prolong MR leads to myocardial damage Non ischemic myocardial disease Fixing the valve is not a cure Disease needs to be fixed

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41 Conclusion Carful assessment for mitral valve structure and regurgitation is needed for decisions The most common indication for MV procedures : Symptoms refractory to control LV dilatation and/or dysfunction Arrhythmia Pulmonary artery hypertension Associated cardiac lesions Mitral valve repair is preferred than replacement Both MV repair and replacement have low mortality, reoperation risk and good survival rate Improvement of LV function on late follow up after MV procedure unlike adult population

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