Fritche-Salazar JF, Herrera-Bello H, Kuri AJ, Gaxiola MMBA, Cossio AJ, Ávila-Vanzzini N

Language: English
References: 12
Page: 43-47
PDF size: 199.09 Kb.

ABSTRACT

Introduction: Ebstein’s anomaly (EA) is a right ventricular cardiomyopathy, current echocardiographic severity criteria are not helpful to predict disease progression or identify patients at higher risk of mortality. Objective: We analyzed clinical and echocardiographic data of adult patients with EA to identify predictors of mortality. Methods and results: Clinical and echocardiographic parameters were analyzed from historical data of 60 adult patients with EA, six of them have died. Variables were compared between groups. Among clinical characteristics only the presence of cyanosis was statistically higher in the mortality group (100 vs 26%, p ‹0.001). When analyzing the echocardiographic parameters, both the right and left ventricular function were worse in the mortality group (LVEF: 56.7±7 vs 49±%, p ‹0.015, RV FAC: 27.1±11 vs 15.3±7.8%, p ‹0.022). Nor the severity of the EA, nor the pulmonary systolic pressure were different between groups. Conclusion: EA is a cardiomyopathy and not a disease limited to the tricuspid valve. Both right and left systolic dysfunction are predictors of mortality.

REFERENCES

1. Dearani JA, Mora BN, Nelson TJ et al. Ebstein anomaly review: what’s now, what’s next? Expert Rev Cardiovasc Ther. 2015; 13: 1101-1109.

2. Monibi AA, Neches WH, Lennox CC et al. Left ventricular anomalies associated with Ebstein’s malformation of tricuspid valve. Circulation. 1978; 57: 303-306.

3. Brown ML, Dearani JA. Ebstein malformation of the tricuspid valve: current concepts in management and outcomes. Curr Treat Options Cardiovasc Med. 2009; 11: 396-402.

4. Attenhofer CH, Connolly HM, Edwards WD, et al. Ebstein’s anomaly - review of a multifaceted congenital cardiac condition. Swiss Med Wkly. 2005; 135: 269-281.

5. Celermajer DS, Bull C, Till JA et al. Ebstein’s anomaly: presentation and outcome from fetus to adult. J Am Coll Cardiol. 1994; 23: 170-176.

6. Attie F, Rosas M, Rijlaarsdam M et al. The adult patient with Ebstein anomaly. Outcome in 72 unoperated patients. Medicine. 2000; 79: 27-36.

7. Giuliani ER, Fuster V, Brandenburg RO et al. Ebstein’s anomaly: the clinical features and natural history of Ebstein’s anomaly of the tricuspid valve. Mayo Clin Proc. 1979; 54: 163-173.

8. Edwards WD. Embryology and pathologic features of Ebstein’s anomaly. Prog Pediatr Cardiol. 1993; 2: 5-15.

9. Warnes CA, Williams RG, Bashore TM et al. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults with Congenital Heart Disease). J Am Coll Cardiol. 2008; 52: e143-e163.

10. Sarubbi B, D’Alto M, Vergara P et al. Electrophysiological evaluation of asymptomatic ventricular pre-excitation in children and adolescents. Int J Cardiol. 2005; 98: 207-214.

11. Frescura C, Basso C, Thiene G et al. Anomalous origin of coronary arteries and risk of sudden death: a study based on an autopsy population of congenital heart disease. Hum Pathol. 1998; 29: 689-695.

12. Sarris GE, Giannopoulos NM, Tsoutsinos AJ et al. Results of surgery for Ebstein anomaly: a multicenter study from the European Congenital Heart Surgeons Association. J Thorac Cardiovasc Surg. 2006; 132: 50-57.