1. The surgical approach to Ebstein's Disease at IBCvT Tîrgu-Mureş
Marisiensis Congress Tîrgu Mureș 2014
The surgical approach to Ebstein's Disease at IBCvT Tîrgu-Mureş
Author: Denisa Pop1
Second authors: Dominic Pascal Keller1
Andrei Feier1
Coordinators: Prof. Horațiu Suciu, MD, PhD 2
Valentin Stroe, MD, PhD Student2
1.Universitatea de Medicină și Farmacie Tîrgu Mureș
Facultatea de Medicină Generală
2.Institutul de Boli Cardiovasculare și Transplant Tîrgu Mureș

2. Ebstein’s disease is a congenital heart malformation
Downward displacement of the tricuspid valve
Atrialized right ventricle

3. - based on the echocardiographic aspect
Celermajer’s grading
- based on the echocardiographic aspect
RA + atrialized RV _
functional RV + left heart
The classification proposed by Carpentier
- based on ventricular morphology, leaflet movement, and RVOT obstruction
GRADE
RATIO 1
<0,5 2
0,5- 0,99 3
1-1,49 4
>1,5
Ratio=
TYPE
DESCRIPTION A
adequate functional RV B
large atrialized RV
the anterior leaflet moves freely C
the anterior leaflet is severely restricted
may obstruct the RVOT D
almost complete atrialization of the RV
small infundibular component

4. Clinical aspects Cyanosis Cardiac failure Respiratory failure
More important for survival is the timing of symptoms!!
Clinical aspects
Degree of displacement
Obstruction of the RVOT by the anterior leaflet
Associated cardiac anomalies
Cyanosis
Cardiac failure
Respiratory failure
Tricuspid valve
regurgitation
Cardiomegaly
Displacement of the valve
Atrialized ventricle
Conduction disturbances
Structural abnormalities of the AV conduction system

5. Diagnosis Echocardiography
Attenhofer Jost C H et al. Circulation. 2007;115:

6. chest x-ray
MRI
cardiac catheterization
angiography

7. Surgical indications[4]
Asymptomatic patients are followed until symptoms arise!!
Surgical indications[4]
Class III or IV NYHA
Cyanosis
↑ cardiothoracic index (>0.80)
Severe tricuspid regurgitation
Severe rhythm disturbances
Under medical treatment

8. Institutul de Boli Cardiovasculare şi Transplant Tîrgu Mureș
Material and method
Institutul de Boli Cardiovasculare şi Transplant Tîrgu Mureș
8 patients
7 female(87%)
1 male (13%)
2005
All cases had similar clinical presentation:
Cyanosis
Systolic murmur
Enlarged right atrium
Cardiomegaly
2013

9. Age Associates cardiac anomalies: Atrial septal defect
16 yrs
15 yrs
Age
8 yrs
1.33 yrs
2.16 yrs
2.08 yrs
0.05 yrs
0.06 yrs
Patient 1
Patient 2
Patient 3
Patient 4
Patient 5
Patient 6
Patient 7
Patient 8
The mean age at the time of surgery was 5.58 years.
Associates cardiac anomalies:
Atrial septal defect
Patent ductus arteriosus
Pulmonary artery hypoplasia
Pulmonary valve stenosis
Pulmonary atresia

10. Surgical management and treatment
Surgical principles
Valve replacement/ Valvuloplasty
Right-to-left shunting
(when exists a risk of RV dysfunction and ↑ pulmonary vascular resistance)
Repair of associated defects
Ablation of arrhytmias

11. The access to the abnormal valve

12. The valvuloplasty

13. The closure

14. Results Systemic-pulmonary anastomosis Tricuspid valvuloplasty
By-pass time
115 min
113 min
114 min
Aortic clampage time
68.3 min
67.5 min
43 min
Intensive care unit stay
11.5 days
3.5 days
17.3 days
Systemic-pulmonary anastomosis
Tricuspid valvuloplasty
Tricuspid valve replacement

15. Mortality 1 patient included in our study died.
16 years old patient after tricuspid valve replacement
2nd day → right ventricle dysfunction with low cardiac output → reintervention
4th day → death

16. Conclusion Early diagnosis of Ebstein’s disease For best results:
→ surgical correction in the first years of life
→ precise analysis of
anatomy
hemodynamic consequences
management options
associated malformations
→ individualized surgical treatment
(NO standard treatment)

17. Discussions Study limitations IBCVT Tîrgu-Mureș small number of cases
the lack of long term follow-up
IBCVT Tîrgu-Mureș
only hospital in Romania where the surgical correction of Ebstein’s disease is performed

18. References
Attenhofer JCH, Connoly HM, Edwards WD, Hayes D, Warnes CA, Danielson GK. Ebstein’s anomaly—review of a multifaceted congenital cardiac condition. Swiss Med Wkly 2005
Christine H. Attenhofer Jost; Heidi M. Connolly; Joseph A. Dearani; William D. Edwards; Gordon K. Danielson, Congenital Heart Disease for the Adult Cardiologist. Ebstein’s Anomaly, Circulation. 2007; 115:
Gerold L. Schiebler, Paul Adams Jr., Ray C. Anderson, Kurt Amplatz and Richard G. Lester. Clinical Study of Twenty-Three Cases of Ebstein's Anomaly of the Tricuspid Valve, Circulation, American Heart Association; 1959; 19:
Constantine Mavroudis MD, Carl L. Backer MD, Pediatric Cardiac Surgery, Fourth Edition; 2013; 29:
Sylvian Chauvaud, Alain Carpentier. Ebstein’s anomaly: the Broussais approach. Oxford Journals, Multimedia Manual Cardio-Thoracic Surgery,Volume 2008, Issue 0626
J. Stark, M. de Leval, V. T. Tsang, Surgery for Congenital Hearts Defects, Third Edition; 2006; 43:

19. Thank you for your attention!