eMedicine Specialties > Cardiology > Congenital Heart Disease in the Adult
Ebstein Anomaly: Treatment & Medication
Updated: Nov 11, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
Treatment
Medical Care
Ebstein anomaly presents with a spectrum of congenital abnormalities of the tricuspid valve and the right ventricle. The age of presentation with symptoms is variable, and a wide range of treatment options is available. Treatment of Ebstein anomaly is complex and dictated mainly by the severity of the disease itself and the effect of accompanying congenital structural and electrical abnormalities. Treatment options include medical therapy, radiofrequency ablation, and surgical therapy.
- Antibiotic prophylaxis for infective endocarditis
- Medical therapy for heart failure - Angiotensin-converting enzyme (ACE) inhibitors, diuretics, and digoxin
- Arrhythmia treatment - Medical treatments such as anti-arrhythmic drugs or radiofrequency ablation of the accessory pathways
- Curative therapy of SVT with radiofrequency ablation is currently the treatment of choice.
- The success rate is lower than that in patients without significant structural heart disease.
- Factors associated with lower likelihood of success include the following:
- Accessory pathways located along the atrialized right ventricle
- Multiple accessory pathways
- Complex geometry of the pathways
- Abnormal morphology of the endocardial action potentials in this region
Surgical Care
Surgical care includes correction of the underlying tricuspid valve and right ventricular abnormalities, correction of any associated intracardiac defects, palliative procedures in early days of life as a bridge to more definitive surgical treatment later, and surgical treatment of associated arrhythmias.
- Indications for surgery are generally as follows:
- New York Heart Association (NYHA) class I-II heart failure with worsening symptoms or with a cardiothoracic ratio of 0.65 or greater5
- NYHA class III-IV heart failure
- History of paradoxical embolism
- Significant cyanosis with arterial O2 saturation of 80% or less and/or polycythemia with hemoglobin of 16 g/dL or more
- Arrhythmias refractory to medical and radiofrequency ablation
- Generally, the trend is to perform surgery earlier rather later in the course of heart failure.
- Various approaches are available to treat structural abnormalities.
- Tricuspid valve repair is preferred over valve replacement, and bioprosthetic valves are preferred over mechanical prosthetic valves.
- The atrialized portion of the right ventricle can be resected surgically, and the markedly dilated, thin-walled right atrium can be resected.
- Associated septal defects may be closed.
- Palliative procedures include creation of atrial septal defect, closure of tricuspid valve with plication of the right atrium, and maintenance of pulmonary blood flow through aortopulmonary shunt. Palliative procedures usually are reserved for severely ill infants with otherwise dire prognosis.
- Left ventricular dysfunction should not be considered a contraindication to tricuspid valve surgery. In these patients, although early mortality is greater with tricuspid valve surgery, the late results are favorable and left ventricular function seems to improve postoperatively.6,7
- Functional status improves after surgery.
- Surgical treatments of arrhythmias include the following:
- Ablation of the accessory pathways
- Maze procedure for atrial arrhythmias
- Cardiac transplantation is appropriate in selected patients.
Consultations
- Electrophysiologist
- Cardiac surgeon
Diet
A low-sodium diet is recommended for symptomatic relief from fluid overload.
Activity
Activity may be allowed as tolerated.
Medication
Ebstein anomaly requires drug treatment for cardiovascular consequences resulting from tricuspid atrialization of the right ventricle, valvular regurgitation, and septal defects. Patients may require antibiotic prophylaxis for bacterial endocarditis. Treatment of SVT is typically by radiofrequency ablation rather than drug therapy. CHF is treated with ACE inhibitors, diuretics, and digoxin.
Diuretics
These agents promote excretion of water and electrolytes by the kidneys. They are used in treatment of hypertension, heart failure, and hepatic, renal, or pulmonary disease when salt and water retention has resulted in edema or ascites.
Furosemide (Lasix)
Increases excretion of water by interfering with chloride-binding cotransport system, which in turn inhibits sodium and chloride reabsorption in ascending loop of Henle and distal renal tubule. Dose must be individualized. Depending on response, administer at increments of 20-40 mg no sooner than 6-8 h after previous dose until desired diuresis occurs. When treating infants, titrate with 1 mg/kg/dose increments until satisfactory effect achieved.
Adult
20-80 mg/d PO/IV/IM; titrate up to 600 mg/d for severe edematous states
Pediatric
1-2 mg/kg/dose PO; not to exceed 6 mg/kg/dose; not to administer more frequently than q6h
1 mg/kg IV/IM slowly under close supervision; not to exceed 6 mg/kg
Metformin decreases concentrations; interferes with hypoglycemic effect of antidiabetic agents and antagonizes muscle-relaxing effect of tubocurarine; aminoglycosides increase auditory toxicity—hearing loss of varying degrees may occur; may enhance anticoagulant activity of warfarin; may increase plasma lithium levels and toxicity
Documented hypersensitivity; hepatic coma; anuria; severe electrolyte depletion
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Perform frequent serum electrolyte, CO2, glucose, creatinine, uric acid, calcium, and BUN determinations during first few months of therapy and periodically thereafter
Cardiac glycosides
These agents possess positive inotropic activity, which is mediated by inhibition of sodium-potassium adenosine triphosphatase (Na+/K+ ATPase). Also, cardiac glycosides reduce conductivity in the heart, particularly through the atrioventricular node, and therefore have a negative chronotropic effect. The cardiac glycosides have very similar pharmacological effects but differ considerably in their speed of onset and duration of action. They are used to slow the heart rate in supraventricular arrhythmias, especially atrial fibrillation, and also are administered in chronic heart failure.
Digoxin (Lanoxin, Lanoxicaps)
Cardiac glycoside with direct inotropic effects in addition to indirect effects on 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
0.125-0.375 mg/d PO
Pediatric
Digitalization in infants and children not generally recommended; suggested doses are as follows:
Total digitalizing dose (TDD)
Preterm neonates: 20-30 mcg/kg/d PO in divided doses
Full-term neonates: 25-35 mcg/kg/d PO divided in divided doses
1 month-2 years: 35-60 mcg/kg/d PO in divided doses
2-5 years: 30-40 mcg/kg/d PO in divided doses
5-10 years: 20-35 mcg/kg/d PO in divided doses
>10 years: 10-15 mcg/kg/d PO in divided doses
TDD divided into 3 doses: 0.5 TDD initial dose, then 0.25 TDD q6h for 2 doses
Maintenance dose: 25-35% of TDD PO
Medications that may increase digoxin levels include alprazolam, benzodiazepines, bepridil, captopril, cyclosporine, propafenone, propantheline, quinidine, diltiazem, aminoglycosides, oral 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, oral 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; beriberi heart disease; idiopathic hypertrophic subaortic stenosis; constrictive pericarditis; ventricular fibrillation; carotid sinus syndrome
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Hypokalemia may reduce positive inotropic effect; IV calcium may produce arrhythmias; hypercalcemia predisposes patient to toxicity, and hypocalcemia can render digoxin ineffective until serum calcium levels are normal; institute magnesium replacement therapy in patients with hypomagnesemia to prevent toxicity; incomplete AV block may progress to complete block when treated with digoxin; exercise caution in hypothyroidism, hypoxia, and acute myocarditis; anorexia, nausea, abdominal pain, or diarrhea may be early signs of toxicity; monitor ECG after each digitalizing dose (ie, loading dose)
Angiotensin-converting enzyme inhibitors
ACE inhibitors are beneficial in all stages of chronic heart failure. Dyspnea and exercise tolerance are improved. Unlike diuretics, studies demonstrate improvement of survival and reduced progression of mild or moderate heart failure to more severe stages. They have benefit in asymptomatic left ventricular dysfunction.
Enalapril (Vasotec)
Competitive inhibitor of ACE. Reduces angiotensin II levels, decreasing aldosterone secretion.
Adult
2.5-5 mg/d PO initial; 10-40 mg/d PO qd or divided bid; alternatively, 1.25 mg/dose IV over 5 min q6h
Pediatric
0.1 mg/kg/d PO divided qd/bid initial; may increase prn over 2 wk; not to exceed 0.5 mg/kg/d
NSAIDs may reduce hypotensive effects; may increase digoxin, lithium, and allopurinol levels; rifampin decreases levels; probenecid may increase levels; diuretics may enhance hypotensive effects
Documented hypersensitivity; angioedema secondary to ACE inhibitors
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Skull hypoplasia and renal failure in neonate; caution in renal impairment, valvular stenosis, or severe CHF
More on Ebstein Anomaly |
| Overview: Ebstein Anomaly |
| Differential Diagnoses & Workup: Ebstein Anomaly |
 Treatment & Medication: Ebstein Anomaly |
| Follow-up: Ebstein Anomaly |
| References |
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Further Reading
Keywords
Ebstein's anomaly, congenital heart disease, tricuspid regurgitation lithium ingestion during pregnancy, maternal benzodiazepine use, right heart failure, supraventricular tachycardia, accessory conduction pathways, bacterial endocarditis
