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

Ebstein’s disease is a congenital heart malformation http://medical-dictionary.thefreedictionary.com/_/viewer.aspx?path=dorland&name=anomaly_Ebstein.jpg Downward displacement of the tricuspid valve Atrialized right ventricle

- 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

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

Diagnosis Echocardiography Attenhofer Jost C H et al. Circulation. 2007;115:277-285

chest x-ray MRI cardiac catheterization angiography

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

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

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

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

The access to the abnormal valve

The valvuloplasty

The closure

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

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

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)

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

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: 277-285 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; 19:165-187 Constantine Mavroudis MD, Carl L. Backer MD, Pediatric Cardiac Surgery, Fourth Edition; 2013; 29: 584-600 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: 591-598

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