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Ebstein's anomaly

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Ebstein’s anomaly is a rare congenital heart disorder occurring in ≈1 per 200 000 live births and accounting for <1% of all cases of congenital heart disease. This anomaly was described by Wilhelm …

Ebstein’s anomaly is a rare congenital heart disorder occurring in ≈1 per 200 000 live births and accounting for <1% of all cases of congenital heart disease. This anomaly was described by Wilhelm Ebstein in 1866 in a report titled, “Concerning a very rare case of insufficiency of the tricuspid valve caused by a congenital malformation.” The patient was a 19-year-old cyanotic man with dyspnea, palpitations, jugular venous distension, and cardiomegaly. At autopsy, Ebstein described an enlarged and fenestrated anterior leaflet of the tricuspid valve. The posterior and septal leaflets were hypoplastic, thickened, and adherent to the right ventricle. There was also a thinned and dilated atrialized portion of the right ventricle, an enlarged right atrium, and a patent foramen ovale. By 1950, only 3 cases of this anomaly had been published.

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  • 1. Ebstein's anomaly RAMACHANDRA
  • 2. Preface • Ebstein’s anomaly is a rare congenital heart disorder occurring in ≈1 per 200 000 live births and accounting for <1% of all cases of congenital heart disease. This anomaly was described by Wilhelm Ebstein in 1866 in a report titled, “Concerning a very rare case of insufficiency of the tricuspid valve caused by a congenital malformation.” The patient was a 19-year-old cyanotic man with dyspnea, palpitations, jugular venous distension, and cardiomegaly. At autopsy, Ebstein described an enlarged and fenestrated anterior leaflet of the tricuspid valve. The posterior and septal leaflets were hypoplastic, thickened, and adherent to the right ventricle. There was also a thinned and dilated atrialized portion of the right ventricle, an enlarged right atrium, and a patent foramen ovale. By 1950, only 3 cases of this anomaly had been published.
  • 3. Pathology in 3 leaflets of TV  THE ANTERIOR LEAFLET IS THE LARGEST LEAFLET AND IS USUALLY ATTACHED TO THE TRICUSPID VALVE ANNULUS.  THE POSTERIOR AND SEPTAL LEAFLETS ARE VESTIGIAL OR ABSENT AS THERE IS FAILURE OF DELAMINATION (SEPARATION OF THE LAYER OF VALVE TISSUE) FROM THE MYOCARDIUM. WHEN PRESENT, THE FREE EDGES ARE GENERALLY DISPLACED POSTERIORLY AND DOWNWARD FROM THE ATRIOVENTRICULAR JUNCTION AND PROGRESSIVELY TOWARD THE RIGHT VENTRICULAR OUTFLOW TRACT AND APEX  FUNNEL-SHAPED  INCOMPETENT  STENOTIC. ("SAIL-LIKE")
  • 4. Change in RV • Tripartite Atrialized RV Small RV Out flow tract
  • 5. EMBRYOLOGY • FAILURE OF DELAMINATION OF EACH OF THE THREE LEAFLETS
  • 6. NATURAL HISTORY  Actuarial survival for all live born patients was 67 percent at one year and 59 % at 10 years   The major causes of death were heart failure, perioperative, and sudden death The main predictors of death were echocardiographic grade of severity (relative risk 2.7 for each increase in grade; the grade was based upon the location of displaced tricuspid valve, right ventricular size, and clinical presentation), foetal presentation (relative risk 6.9), and RVOT obstruction (relative risk 2.1).  The outcome was better in patients with less severe disease who presented in later childhood or adulthood  50 % female  Majority reach adulthood despite tricuspid regurgitation and abnormal right ventricular function
  • 7. Classification Attenhofer Jost CH et al: Based on mild/moderate/severe apical displacement,TR and RV size Carpentier classification(on surgery table finding):A,B,C,D Danielson GK(Based on Carpentier classification):I,II,III &IV
  • 8. Danielson GK I The ATL is larger and mobile but the posterior and STL are apically displaced, dysplastic, or absent. The ARV size varies from relatively small to large. II The anterior, posterior, and often septal leaflets are present, but are relatively small and displaced in a spiral fashion toward the apex. The atrialized ventricular chamber is moderately large. III The ATL is restricted motion with shortened, fused, and tethered chordae. Direct insertion of papillary muscles into the anterior leaflet is frequently present. The posterior and septal leaflets are displaced, dysplastic, and usually not reconstructable. The ARV is large. IV The ARV is severely deformed and displaced into the RV outflow tract. There may be few or no chordae, and direct insertions of the PM into the leading edge of the valve are common. PTL is typically dysplastic or absent, and the septal leaflet is represented by a ridge of fibrous material descending apically from the membranous septum. TV displaced into the RVOT and may cause
  • 9. Associated cardiac defects • PFO/ASD • VSD • RVOT OBSTRUCTION • PDA • COA • ARRHYTHMIAS(20%) • DIASTOLIC AND SYSTOLIC DYSFUNCTION, MITRAL VALVE PROLAPSE, BICUSPID AORTIC VALVE, LEFT VENTRICULAR NONCOMPACTION AND LTGA
  • 10. CLINICAL PRESENTATION — an abnormal routine prenatal scan (86 percent) Foetuses Neonates Infants — cyanosis (74 percent) — heart failure (43 percent) Children — an incidental murmur (63 percent) Adolescents percent) and adults — arrhythmia (42
  • 11. Physical examination • CYANOSIS • PAN DIGITAL CLUBBING • MULTIPLE HEART SOUNDS • PULSATILE RVOT • RAISED JVP
  • 12. Differential diagnosis • UHL’S ANOMALY • TRICUSPID VALVE DYSPLASIA • TRICUSPID VALVE PROLAPSE • TRICUSPID VALVE ENDOCARDITIS • CARCINOID HEART DISEASE • TRAUMATIC INJURY OF THE TRICUSPID VALVE • ARRHYTHMOGENIC RIGHT VENTRICULAR CARDIOMYOPATHY
  • 13. Electrocardiogram  RAE  RBBB  PREEXCITATION WITH A LBBB WITH PREDOMINANT S WAVES IN THE RIGHT PRECORDIUM DUE TO RIGHT-SIDED ACCESSORY PATHWAY  LOW-VOLTAGE  SVT QRS OVER THE RIGHT-SIDED CHEST LEADS T WITH OR WITHOUT PREEXCITATION:AT/AF/AFIB  FIRST DEGREE ATRIOVENTRICULAR BLOCK
  • 14. Chest radiograph MILD SEVERE NORMAL  "WALL TO WALL" HEART  DIMINISHED PULMONARY VASCULARITY  RAE  INFUNDIBULAR DILATATION
  • 15. Echocardiography APICAL DISPLACEMENT OF THE SEPTAL TRICUSPID VALVE LEAFLET (BY ≥8 MM/M² OF BAS PARADOXICAL VENTRICULAR SEPTAL MOTION LOW VELOCITY TR ASD/ PFO OFTEN WITH BD SHUNTING. PFO/ASD/PDA/RVOT OBSTRUCTION
  • 16. Great Ormond Street Score (GOSE) COMMONLY A RATIO AREA  FOR NEONATE OF RA+ARV/TRV+LA+LV THE GREATER THE RATIO, THE WORSE THE PROGNOSIS
  • 17. Management MEDICAL INTERVENTION SURGERY MILDER FORM RFA:single accessory pathway in 52%, multiple accessory 29% , arrhythmias unrelated to an accessory pathway in 18%. Ablation of a right free wall or right septal pathway was initially successful in 79 and 89 percent, respectively; the recurrence rate was 32 and 29 %.Among those with arrhythmias unassociated with an accessory pathway, ablation was initially successful in 75 percent and 27% had recurrence        ASD CLOSURE  PDA CLOSURE Severe cyanosis GOSE score 3 or 4 CT ratio >80 percent Severe tricuspid regurgitation Arrhythmia Worsening of SOB/HF
  • 18. Tricuspid repair techniques Danielson repair Carpentier technique Cone reconstruction  Monocusp repair ATL  Right ventricular plication (vertical)  posterior annuloplasty is performed routinely  detaching ATL from annulus  Delamination of the anterior and inferior leaflets  clockwise rotation to the coronary sinus to create a monocusp valve  The atrialized portion of the RV is plIcated (vertical )  Final hinge is true RV annulus  Contemporary and the most anatomic  delaminating the anterior, posterior, and septal leaflets from the underlying myocardium. The mobilized anterior and posterior leaflets are rotated in a clockwise manner to meet the mobilized septal leaflet. This results in 360 degrees of leaflet tissue, ie, a cone of leaflet tissue, that is then re-anchored at the level of the true annulus.ARV is vertically plicated.
  • 19. TV repair/TVR follow up Ten year survival was 93 %  Freedom from bio prosthesis replacement at 10 and 15 years of 98 and 81 % respectively 92  % were NYHA class I or II 94 % were not receiving anticoagulation Hospital mortality was 3 %
  • 20. PREGNANCY  RV VOLUME OVERLOAD/RAISED RVEDP/CYANOSIS REAPPEAR/RIGHT VENTRICULAR FILLING PRESSURE/INTRACARDIAC SHUNT INCREASES/ PARADOXICAL EMBOLIZATION/HYPOXEMIA INCREASES THE FETAL RISK  ARRHYTHMIA RISK IS ALSO INCREASED  HEART FAILURE  PREMATURITY, FETAL WASTAGE, AND CONGENITAL HEART DISEASE REGARDLESS OF THE PRESENCE OR ABSENCE OF MATERNAL CYANOSIS  MATERNAL EBSTEIN’ ANOMALY :FOETAL RISK IS 4 % HAVING EBSTEIN’ ANOMALY  PATERNAL EBSTEIN’ ANOMALY :FOETAL RISK IS 1-2 % EBSTEIN’ ANOMALY 
  • 21. Job fitness • mild Ebstein's anomaly defined as no cyanosis, normal right ventricular size, and with no evidence of atrial or ventricular tachyarrhythmias :all jobs • Moderate tricuspid regurgitation can participate in low-intensity competitive sports (class IA) , if there is no evidence of arrhythmia on ambulatory ECG/ Holter monitoring other than isolated premature contractions. • Severe form (severe tricuspid regurgitation, right ventricular enlargement, and/or evidence of arrhythmias) are precluded from all jobs
  • 22. Plant tree ,Cardiology is not enough