Pediatric Ebstein Anomaly Treatment & Management
- Author: Raymond T Fedderly, MD; Chief Editor: Stuart Berger, MD more...
Asymptomatic patients with Ebstein anomaly who have mild tricuspid regurgitation need only outpatient clinic evaluation, which may include periodic ECG, chest radiography, and oxygen saturation measurement. All patients with this diagnosis require lifetime prophylaxis for bacterial endocarditis.
Neonates with severe Ebstein anomaly initially require admission to a neonatal intensive care unit (NICU) for stabilization. If pulmonary blood flow is insufficient or ductal dependent, they also require prostaglandin E1 therapy.
In addition, it is crucial that neonates with a severe form of this disease have an adequate atrial communication. If the patient is born with only a patent foramen ovale (PFO) or a restrictive atrial septal defect (ASD), a balloon atrial septostomy or urgent surgical intervention may be required. Atrial septostomy can be accomplished at the bedside with echocardiographic guidance or in the cardiac catheterization laboratory, under echocardiographic and/or fluoroscopic guidance.
Electrophysiological studies are performed both as a diagnostic tool to determine the cause of an arrhythmia and as a curative procedure using radiofrequency catheter ablation. Catheter ablation for paroxysmal supraventricular tachycardia (SVT) is highly successful in children, with a low complication and recurrence rate; however, the subset of patients with Ebstein anomaly and SVT has been shown to be more challenging to cure, likely because of the derangement in tricuspid valve alignment with the tricuspid annulus and the increased likelihood of multiple accessory pathways.
The reported acute success rate in the Pediatric Radiofrequency Ablation Registry and other sources ranges from 75-90%, and the recurrence rate is reported to be as high as 32%. As expected, success rates, complications, and recurrence rates vary with complex pediatric radiofrequency catheter ablation procedures, depending on operator and institutional experience. Radiofrequency ablation appears to be most successful in patients with a mild degree of tricuspid regurgitation.
The surgical care of these patients depends on the severity of the leaflet displacement and on the degree of associated right ventricular outflow tract obstruction.
In neonates with the most severe form of Ebstein anomaly, the functional right ventricle is hypoplastic, and the patient is usually best treated by closing the tricuspid valve and, in effect, creating a tricuspid atresia physiology (Starnes procedure). In addition, these infants require a systemic artery–to–pulmonary artery shunt. When the patient is aged approximately 6 months, a bidirectional Glenn procedure (superior vena cava–to–pulmonary artery anastomosis) and shunt takedown is performed. Fontan completion (inferior vena cava–to–pulmonary artery anastomosis) is usually performed when the patient is aged 2-4 years.
In infants with mild-to-moderate tricuspid regurgitation and severe right ventricular outflow tract obstruction, a systemic artery–to–pulmonary artery shunt is performed in addition to an unrestrictive atrial communication being created. In patients with moderate-to-severe tricuspid regurgitation, the abnormal valve can be replaced with a mechanical or prosthetic valve, a surgical reconstruction, or a combination of the two.[6, 7, 8]
In a study by Brown et al from the Mayo clinic the results of 539 patients who had 604 cardiac operations is described. The mean age of this group was 24 years. The first repair consisted of tricuspid valve repair in 182 patients and tricuspid valve replacement in 337 patients. Late survival was 84.7% at 10 years and 71.2% at 20 years. Preoperative characteristics associated with mortality included increased hematocrit levels, associated mitral valve repair, prior cardiac operation, and moderate-to-severe reduction in right ventricular systolic function.
One study evaluated the outcomes of 32 patients who underwent surgery for Ebstein anomaly. After 16-year follow-up, the results found early and late survival rates of 60% ± 12% versus 85% ± 10 (P = .06), respectively, for patients without pulmonary atresia and early and late survival rates of 60% ± 12% (9/15) versus 94.1% (16/17) (P < .05), respectively, for those with pulmonary atresia. The results conclude that biventricular and tricuspid valve repair of Ebstein anomaly is feasible in neonates and young infants with good results, especially those without pulmonary atresia.
Special dietary restrictions are not usually required. An infant with severe tricuspid regurgitation may require a high caloric density formula.
The activity restrictions of these patients depend on the severity of the leaflet displacement. If the displacement is mild and patients do not have an associated paroxysmal SVT, they should be allowed to determine their own level of activity. For patients with cyanosis, sports participation is usually somewhat restricted. An exercise stress test and other noninvasive assessments might be helpful in making this determination.
Celermajer DS, Bull C, Till JA, et al. Ebstein's anomaly: presentation and outcome from fetus to adult. J Am Coll Cardiol. 1994 Jan. 23(1):170-6. [Medline].
Freud LR, Escobar-Diaz MC, Kalish BT, et al. Outcomes and Predictors of Perinatal Mortality in Fetuses With Ebstein Anomaly or Tricuspid Valve Dysplasia in the Current Era: A Multicenter Study. Circulation. 2015 Aug 11. 132 (6):481-9. [Medline].
Connolly HM, Warnes CA. Ebstein's anomaly: outcome of pregnancy. J Am Coll Cardiol. 1994 Apr. 23(5):1194-8. [Medline].
Kipps AK, Graham DA, Lewis E, Marx GR, Banka P, Rhodes J. Natural history of exercise function in patients with Ebstein anomaly: A serial study. Am Heart J. 2012 Mar. 163(3):486-91. [Medline].
Reich JD, Auld D, Hulse E, Sullivan K, Campbell R. The Pediatric Radiofrequency Ablation Registry's experience with Ebstein's anomaly. Pediatric Electrophysiology Society. J Cardiovasc Electrophysiol. 1998 Dec. 9(12):1370-7. [Medline].
Sata S, Murin P, Hraska V. Cone reconstruction of Ebstein's anomaly in a neonate. Ann Thorac Surg. 2012 Oct. 94(4):e99-100; discussion e100. [Medline].
Vogel M, Marx GR, Tworetzky W, Cecchin F, Graham D, Mayer JE, et al. Ebstein's malformation of the tricuspid valve: short-term outcomes of the "cone procedure" versus conventional surgery. Congenit Heart Dis. 2012 Jan-Feb. 7(1):50-8. [Medline].
Anderson HN, Dearani JA, Said SM, et al. Cone reconstruction in children with Ebstein anomaly: the Mayo Clinic experience. Congenit Heart Dis. 2014 May-Jun. 9 (3):266-71. [Medline].
Brown ML, Dearani JA, Danielson GK, et al. The outcomes of operations for 539 patients with Ebstein anomaly. J Thorac Cardiovasc Surg. 2008 May. 135(5):1120-36, 1136.e1-7. [Medline].
Boston US, Goldberg SP, Ward KE, et al. Complete repair of Ebstein anomaly in neonates and young infants: A 16-year follow-up. J Thorac Cardiovasc Surg. 2011 May. 141(5):1163-9. [Medline].
Paranon S, Acar P. Ebstein's anomaly of the tricuspid valve: from fetus to adult: congenital heart disease. Heart. 2008 Feb. 94(2):237-43. [Medline].
Correa-Villasenor A, Ferencz C, Neill CA, Wilson PD, Boughman JA. Ebstein's malformation of the tricuspid valve: genetic and environmental factors. The Baltimore-Washington Infant Study Group. Teratology. 1994 Aug. 50(2):137-47. [Medline].
Jaiswal PK, Balakrishnan KG, Saha A, et al. Clinical profile and natural history of Ebstein's anomaly of tricuspid valve. Int J Cardiol. 1994 Sep. 46(2):113-9. [Medline].
Ahmed S, Nanda NC, Nekkanti R, Pacifico AD. Transesophageal three-dimensional echocardiographic demonstration of Ebstein's anomaly. Echocardiography. 2003 Apr. 20(3):305-7. [Medline].
Augustin N, Schmidt-Habelmann P, Wottke M, Meisner H, Sebening F. Results after surgical repair of Ebstein's anomaly. Ann Thorac Surg. 1997 Jun. 63(6):1650-6. [Medline].
Boston US, Dearani JA, O'Leary PW, Driscoll DJ, Danielson GK. Tricuspid valve repair for Ebstein's anomaly in young children: a 30-year experience. Ann Thorac Surg. 2006 Feb. 81(2):690-5; discussion 695-6. [Medline].
Chauvaud SM, Hernigou AC, Mousseaux ER, Sidi D, Hebert JL. Ventricular volumes in Ebstein's anomaly: x-ray multislice computed tomography before and after repair. Ann Thorac Surg. 2006 Apr. 81(4):1443-9. [Medline].
Endo M, Ohmi M, Sato K, et al. Tricuspid valve closure for neonatal Ebstein's anomaly. Ann Thorac Surg. 1998 Feb. 65(2):540-2. [Medline].
Garson Jr A, Bricker JT, Fisher DJ. Ebstein's anomaly of the tricuspid valve. The Science and Practice of Pediatric Cardiology. Williams & Wilkins; 1998. 1303-15.
Kiziltan HT, Theodoro DA, Warnes CA, et al. Late results of bioprosthetic tricuspid valve replacement in Ebstein's anomaly. Ann Thorac Surg. 1998 Nov. 66(5):1539-45. [Medline].
MacLellan-Tobert SG, Driscoll DJ, Mottram CD, et al. Exercise tolerance in patients with Ebstein's anomaly. J Am Coll Cardiol. 1997 Jun. 29(7):1615-22. [Medline].
Pavlova M, Fouron JC, Drblik SP, et al. Factors affecting the prognosis of Ebstein's anomaly during fetal life. Am Heart J. 1998 Jun. 135(6 Pt 1):1081-5. [Medline].
Schreiber C, Cook A, Ho SY, Augustin N, Anderson RH. Morphologic spectrum of Ebstein's malformation: revisitation relative to surgical repair. J Thorac Cardiovasc Surg. 1999 Jan. 117(1):148-55. [Medline].
Snider AR, Serwer GA, Ritter SB. Abnormalities of ventricular flow. Echocardiography in Pediatric Heart Disease. 2nd ed. Mosby-Year Book, Incorporated; 1997. 389-94.
Trojnarska O, Szyszka A, Gwizdala A, et al. Adults with Ebstein's anomaly--Cardiopulmonary exercise testing and BNP levels exercise capacity and BNP in adults with Ebstein's anomaly. Int J Cardiol. 2006 Jul 28. 111(1):92-7. [Medline].
van Son JA, Falk V, Black MD, Haas GS, Mohr FW. Conversion of complex neonatal Ebstein's anomaly into functional tricuspid or pulmonary atresia. Eur J Cardiothorac Surg. 1998 Mar. 13(3):280-4; discussion 284-5. [Medline].
Yetman AT, Freedom RM, McCrindle BW. Outcome in cyanotic neonates with Ebstein's anomaly. Am J Cardiol. 1998 Mar 15. 81(6):749-54. [Medline].
Yun TJ, Lee SH, Ko JK. Neonatal stenotic Ebstein's anomaly: a novel technique of right ventricular exclusion. J Thorac Cardiovasc Surg. 2006 Feb. 131(2):469-71. [Medline].