eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology
Ebstein Anomaly: Treatment & Medication
Updated: Mar 9, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
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. In neonates who have a severe form of this disease, an adequate atrial communication is crucial. 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%.4 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.
Surgical Care
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.
In a study by Brown et al from the Mayo clinic the results of 539 patients who had 604 cardiac operations is described.5 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.
Diet
Special dietary restrictions are not usually required. An infant with severe tricuspid regurgitation may require a high caloric density formula.
Activity
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.
Medication
In patients with Ebstein anomaly who are asymptomatic, the only drug therapy normally required is prophylaxis for bacterial endocarditis. In patients with congestive heart failure, digoxin and diuretics may be required. Patients with cyanosis are at an increased risk for a paradoxical embolus and may require therapy with warfarin (Coumadin) or aspirin. Patients who have paroxysmal SVT may require a beta-blocking or calcium channel–blocking agent or, possibly, a Vaughn-Williams class I or III antiarrhythmic agent.
The choice of antiarrhythmic agent somewhat depends symptom severity, tachycardia mechanism, potential medical contraindications, patient and physician preferences, and alternate therapeutic choices, such as catheter ablation. Antibiotics for endocarditis prophylaxis are required before performing procedures that may cause bacteremia. For more information, see Antibiotic Prophylactic Regimens for Endocarditis.
Inotropic agents
These agents increase cardiac output.
Digoxin (Lanoxin)
Cardiac glycoside with direct inotropic effects in addition to indirect effects on the cardiovascular system. Acts directly on cardiac muscle, increasing myocardial systolic contractions. Its indirect actions result in increased carotid sinus nerve activity and enhanced sympathetic withdrawal for any given increase in mean arterial pressure.
Adult
Pediatric
Maintenance dose:
Preterm infant: 5-7.5 mcg/kg/d PO divided bid
Term infant: 6-10 mcg/kg/d PO divided bid
1 month to 2 years: 10-15 mcg/kg/d PO divided bid
2-5 years: 7.5-10 mcg/kg/d PO divided bid
5-10 years: 5-10 mcg/kg/d PO divided bid
>10 years: 2.5-5 mcg/kg/d PO qd
Medications that may increase digoxin levels include alprazolam, benzodiazepines, bepridil, captopril, cyclosporine, propafenone, propantheline, quinidine, diltiazem, aminoglycosides, PO amiodarone, anticholinergics, diphenoxylate, erythromycin, felodipine, flecainide, hydroxychloroquine, itraconazole, nifedipine, omeprazole, quinine, ibuprofen, indomethacin, esmolol, tetracycline, tolbutamide, and verapamil
Medications that may decrease serum digoxin levels include aminoglutethimide, antihistamines, cholestyramine, neomycin, penicillamine, aminoglycosides, PO colestipol, hydantoins, hypoglycemic agents, antineoplastic treatment combinations (including carmustine, bleomycin, methotrexate, cytarabine, doxorubicin, cyclophosphamide, vincristine, procarbazine), aluminum or magnesium antacids, rifampin, sucralfate, sulfasalazine, barbiturates, kaolin/pectin, and aminosalicylic acid
Documented hypersensitivity; severe hypokalemia; renal failure; WPW syndrome with antegrade conduction of accessory pathway; AV block; idiopathic hypertrophic subaortic stenosis or constrictive pericarditis
Pregnancy
Precautions
Check renal function and previously prescribed medications before starting digoxin therapy; hypokalemia, hypomagnesemia, hypercalcemia, and hypermagnesemia predispose patients to digoxin toxicity
Diuretics
These agents decrease pulmonary or systemic edema.
Furosemide (Lasix)
Increases excretion of water by interfering with chloride-binding cotransport system, which inhibits sodium and chloride reabsorption in ascending loop of Henle and distal renal tubule.
Adult
Pediatric
0.5-1 mg/kg/dose PO q8-24h
Metformin decreases furosemide concentrations; furosemide interferes with hypoglycemic effect of antidiabetic agents and antagonizes muscle relaxing effect of tubocurarine; auditory toxicity appears to be increased with coadministration of aminoglycosides and furosemide; hearing loss of varying degrees may occur; anticoagulant activity of warfarin may be enhanced when taken concurrently with this medication; increased plasma lithium levels and toxicity are possible when taken concurrently with this medication
Documented hypersensitivity; severe hypovolemia; severe electrolyte imbalance
Pregnancy
Precautions
Carefully monitor electrolytes and volume status; may increase risk of renal stones
Prostaglandins
Neonates with severe Ebstein anomaly and insufficient or ductal dependent pulmonary blood flow require prostaglandin E1 therapy.
Alprostadil (Prostaglandin E1, Prostin VR Pediatric injection)
Temporary maintenance of patency of ductus arteriosus in neonates with ductal-dependent congenital heart disease until surgery can be performed. The underlying conditions may present as cyanotic or acyanotic heart disease. Provides vasodilation by direct effects on vasculature and ductus arteriosus smooth muscle.
Adult
Pediatric
Neonates and infants: 0.05-0.1 mcg/kg/min IV; reduce to lowest effective dose; if unresponsive, dose may be slowly increased; maintenance range is typically 0.01-0.4 mcg/kg/min IV
Caution with concomitant drugs that may lower blood pressure, decrease serum glucose, cause respiratory depression, or have proarrhythmic effects
Documented hypersensitivity; hyaline membrane disease, respiratory distress syndrome
Pregnancy
Precautions
May cause apnea (10-12%), which most often occurs in neonates <2 kg at birth and usually appears during the first hour of the infusion, monitor respiratory status throughout treatment and have ventilatory assistance immediately available; caution with bleeding tendencies; monitor for hypotension, bradycardia, and arrhythmias; prolonged infusions associated with hypocalcemia or hypoglycemia
More on Ebstein Anomaly |
| Overview: Ebstein Anomaly |
| Differential Diagnoses & Workup: Ebstein Anomaly |
 Treatment & Medication: Ebstein Anomaly |
| Follow-up: Ebstein Anomaly |
| Multimedia: Ebstein Anomaly |
| References |
| « Previous Page | Next Page » |
References
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. Aug 1994;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. Sep 1994;46(2):113-9. [Medline].
Celermajer DS, Bull C, Till JA, et al. Ebstein's anomaly: presentation and outcome from fetus to adult. J Am Coll Cardiol. Jan 1994;23(1):170-6. [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. Dec 1998;9(12):1370-7. [Medline].
Brown ML, Dearani JA, Danielson GK, et al. The outcomes of operations for 539 patients with Ebstein anomaly. J Thorac Cardiovasc Surg. May 2008;135(5):1120-36, 1136.e1-7. [Medline].
Paranon S, Acar P. Ebstein's anomaly of the tricuspid valve: from fetus to adult: congenital heart disease. Heart. Feb 2008;94(2):237-43. [Medline].
Connolly HM, Warnes CA. Ebstein's anomaly: outcome of pregnancy. J Am Coll Cardiol. Apr 1994;23(5):1194-8. [Medline].
Ahmed S, Nanda NC, Nekkanti R, Pacifico AD. Transesophageal three-dimensional echocardiographic demonstration of Ebstein's anomaly. Echocardiography. Apr 2003;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. Jun 1997;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. Feb 2006;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. Apr 2006;81(4):1443-9. [Medline].
Endo M, Ohmi M, Sato K, et al. Tricuspid valve closure for neonatal Ebstein's anomaly. Ann Thorac Surg. Feb 1998;65(2):540-2. [Medline].
Garson Jr A, Bricker JT, Fisher DJ. Ebstein's anomaly of the tricuspid valve. In: 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. Nov 1998;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. Jun 1997;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. Jun 1998;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. Jan 1999;117(1):148-55. [Medline].
Snider AR, Serwer GA, Ritter SB. Abnormalities of ventricular flow. In: 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. Jul 28 2006;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. Mar 1998;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. Mar 15 1998;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. Feb 2006;131(2):469-71. [Medline].
Further Reading
Keywords
Ebstein anomaly, Ebstein's anomaly, Ebstein anomaly of tricuspid valve, Ebstein's anomaly of tricuspid valve, Ebstein disease, Ebstein's disease, treatment, medication, diagnosis, congenital heart defect, heart, tricuspid valve, atrial septal defect, ASD, patent foramen ovale, PFO, pulmonary stenosis, pulmonary atresia, congestive heart failure, supraventricular tachycardia, SVT, Wolff-Parkinson-White syndrome, WPW syndrome, arrhythmia, cyanosis, heart murmur, tricuspid valve regurgitation, right ventricular outflow tract obstruction
