docEbstein malformation - Advanced echocardiography
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Ebstein malformation - Advanced echocardiography
Wilhelm Ebstein 1836 - 1912 Ebstein Malformation Heidi M. Connolly, MD Advanced Echocardiography Dunblane, Scotland No disclosures Ebstein Anomaly vs Normal Ebstein Anomaly • June 28, 1864 • 19-year-old laborer admitted to Allerheiligen Hospital in Breslau with shortness of breath and palpitations • Died on July 6th • Ebstein performed an autopsy next day • 1866 published characteristic features Ebstein: Arch Anat Physiol 1866 Ebstein Anomaly • 1 in 200,000 live births, <1% of CHD • Spectrum of abnormal TV leaflets and RV • Abnormalities of all leaflets Variable failure of delamination Inferior displacement and anterior/apical Rotation of septal leaflet • More than valve problem- cardiomyopathy 1 Thin, Translucent, Redundant, Sail-like Anterior Leaflet Normal Delamination (Separation) of TV from RV Myocardium RA RCA RA EC EC TV RV RV Courtesy of W.D. Edwards Failed TV Delamination in Ebstein Anomaly RA Failed Delamination RA • Adherence of leaflets to underlying RV myocardium Atrialized RV RV • Displacement of the annular hinge points RV Ebstein Anomaly Embryology and Genetics Ebstein Anomaly • Most cases are sporadic • Endocarditis • Rare familial cases • Rheumatic heart disease • MYH7 mutation + LVNC • TV prolapse • NKX 2.5 mutation • TV dysplasia Differential Diagnosis • Carcinoid • Trauma • RV infarction, RV myopathy 2 Ebstein - Clinical Evaluation • 10% diagnosed prenatally Spectrum of Presentation Severe Mild • 60% diagnosed age <1 year 38% cyanotic 66% of neonates and infants • Murmur most common in older children • JVP often normal • 10% diagnosed as adults The Patient With This CXR… … Had This Echo Typical Ebstein ECG Ebstein Malformation • Apical displacement septal TV leaflet • Atrialized RV, small RV • >50% ASD or PFO • RV always abnormal • LV often abnormal Prolonged PR, RBBB, tall “Himalayan” P waves 3 Ebstein Malformation Apical Displacement Index Normal <8 mm/m2 Ebstein Anomaly RA Displacement >8 mm/m2 LV RV 23 mm/1.5 m2 = 15.3 mm/m2 Spiral Displacement of TV Anterior/Apical Rotation of Functional Orifice Ebstein Anomaly Associated Lesions • ASD or PFO 50 - 90% • Ventricular septal defect • Pulmonary stenosis • Left heart anomalies 40% LV dysfunction/noncompaction Bicuspid AV MV prolapse LVOT obstruction • ≥1 accessory pathways 10 – 30% Modified from Schrieber and Anderson: J Thorac Cardiovasc Surg, 1999 50-Year-Old Male • Murmur for many years Examples • Recent in exercise capacity • Episode of syncope EPS (+) – ICD placed 2 yr ago • DM – on insulin, HT on Rx 4 50-Year-Old Man What would you recommend? 1) MRI 2) Right heart cath 3) TV repair, PFO closure 4) TV replacement 5) Something else Operative Procedure Preop Postop • TVR 35-mm bioprosthesis • PFO suture closure • Right reduction atrioplasty • ICD lead exterior to TVR sewing ring 28-Year-Old Female with Ebstein Another Example 5 28-Year-Old Female with Ebstein Ebstein Anomaly TV repair, RA and RV plication, PFO closed Preop 6 months postop GUCH Guidelines 2010 Danielson Repair - 1974 Gordon K. Danielson Joseph A. Dearani Ebstein Anomaly 1972 – 2006 Overall Survival 100 80 Survival (%) Ebstein Anomaly n=539 60 10 year = 84.7% 20 year = 71.2% 40 ≤18 years >18 years 20 n=246 n=293 0 539 0 332 5 200 121 54 10 15 20 Years Brown, Dearani, et al JTCS 2008 Brown, Dearani, et al JTCS 2008 6 Survival Free from Late Reoperation Patient age > 12 yr 100 Multivariate Predictors of Overall Mortality Survival free of late reoperation (%) TV repair HR P Mild mitral regurgitation 6.84 0.01 Mild PS 3.20 0.01 Moderate ↓ RV function 2.76 0.01 Accessory tract ablation 0.20 0.01 80 TV replacement 60 40 P=0.77 20 129 217 0 0 90 102 63 45 5 39 17 10 15 Years Brown, Dearani, et al JTCS 2008 Late Functional Status NYHA class I or II 83% NYHA class III or IV 16% Pre-Cone Era Anatomic Tricuspid Valve Repair ↑ early mortality ↑ late mortality RV dysfunction MV surgery and RVOTO LV dysfunction Early referral 7 Cone Repair Cone Repair for Ebstein Anomaly • Mobilization of the anterior and posterior TV leaflets from their anomalous attachments in RV • Clockwise rotation of free edge of this complex to be sutured to septal border of anterior leaflet • This creates a cone the vertex of which remains fixed at RV apex and base is sutured to true TV annulus level ©2011 MFMER | 3138928-43 Cone Technique Repair at True Anatomic Annulus via Surgical Leaflet Delamination Preop Postop Echo Features of Ebstein Anomaly Septal > post TV leaflet displacement Tethering and elongation anterior leaflet Tricuspid valve regurgitation Atrialized RV - right heart enlargement Ventricular dysfunction and dysplasia RV > LV Associated defects ASD or PFO, PS, VSD, MVP, BAV, LVOTO Repairability Ebstein Anomaly - Conclusion • Ebstein is a complex form of CHD • No two hearts with EA are same • Precise knowledge of different anatomic variables, associated malformations and management options are essential • With alternative management strategies, survival of patients with EA will continue to improve Questions or Comments? 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