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Ebstein Anomaly

Kamran Riaz, MD, Clinical Assistant Professor, Department of Internal Medicine, Section of Cardiology, Wright State University School of Medicine

Updated: Nov 11, 2009

Introduction

Background

Ebstein anomaly is a congenital malformation of the heart that is characterized by apical displacement of the septal and posterior tricuspid valve leaflets, leading to atrialization of the right ventricle with a variable degree of malformation and displacement of the anterior leaflet.

Wilhelm Ebstein first described a patient with cardiac defects typical of Ebstein anomaly in 1866. In 1927, Alfred Arnstein suggested the name Ebstein's anomaly for these defects. In 1937, Yates and Shapiro described the first case of the anomaly with associated radiographic and electrocardiographic data.

Pathophysiology

The embryological development of tricuspid valve leaflets and chordae involves undermining of the right ventricular free wall. This process continues to the level of the atrioventricular (AV) junction. In Ebstein anomaly, this process of undermining is incomplete and falls short of reaching the level of the AV junction. In addition, the apical portions of the valve tissue, which normally undergo resorption, fail to resorb completely. This results in distortion and displacement of the tricuspid valve leaflets, and a part of the right ventricle becomes atrialized. In one study involving 50 hearts with the anomaly, the entire right ventricle was found to be morphologically abnormal.1

Ebstein anomaly is commonly associated with other congenital, structural, or conduction system disease, including intracardiac shunts, valvular lesions, and accessory conduction pathways (eg, Wolff-Parkinson-White [WPW] syndrome).

The hemodynamic consequences of Ebstein anomaly result from displaced and malformed tricuspid leaflets and atrialization of the right ventricle. The leaflet anomaly leads to tricuspid regurgitation. The severity of regurgitation depends on the extent of leaflet displacement, ranging from mild regurgitation with minimally displaced tricuspid leaflets to severe regurgitation with extreme displacement.

The atrialized portion of the right ventricle, although anatomically part of the right atrium, contracts and relaxes with the right ventricle. This discordant contraction leads to stagnation of blood in the right atrium. During ventricular systole, the atrialized part of the right ventricle contracts with the rest of the right ventricle, which causes a backward flow of blood into the right atrium, accentuating the effects of tricuspid regurgitation.

Frequency

United States

True prevalence is unknown because mild forms frequently are undiagnosed. Currently, with wide application of echocardiography, more cases are being diagnosed. Ebstein anomaly probably accounts for 0.5% of cases of congenital heart diseases.

Mortality/Morbidity

The natural course of the disease varies according to the severity of tricuspid valve displacement.

  • Patients presenting in infancy generally have severe disease and unfavorable prognosis.2,3
  • Mean age of presentation is in the middle teenage years. According to older observational data, approximately 5% of these patients survive beyond age 50 years. The oldest recorded patient lived to age 85 years.

Race

Ebstein anomaly is more common in children of white females.

Sex

No specific sex predominance exists.

Age

Ebstein anomaly can present at various stages of life.

  • Fetal life: Ebstein anomaly is usually diagnosed incidentally by echocardiography.
  • Neonatal life and infancy: Ebstein anomaly presents with cyanosis and/or severe heart failure; typically, symptoms present in infancy improve as pulmonary vascular resistance decreases.
  • Adult life: Ebstein anomaly presents with fatigue, exertional dyspnea, cyanosis, tricuspid regurgitation and/or right heart failure, and palpitations; arrhythmias are common.

Clinical

History

Patients can have a variety of symptoms related to the anatomical abnormalities of Ebstein anomaly and their hemodynamic effects or associated structural and conduction system disease.

  • Cyanosis
    • Fairly common and frequently due to right-to-left shunt at the atrial level and/or severe heart failure
    • Transient in neonatal life with recurrence in adult life
    • May appear for the first time in adult life
    • Transient appearance/worsening of cyanosis in adult life due to paroxysmal arrhythmias
    • Once apparent, progressively worsens
  • Fatigue and dyspnea: These are due to poor cardiac output secondary to right ventricular failure and decreased left ventricular ejection fraction.
  • Palpitations and sudden cardiac death
    • Due to paroxysmal supraventricular tachycardia (SVT) in as many as one third of patients
    • Fatal ventricular arrhythmias, which may be due to the presence of accessory pathways
  • Symptoms of right heart failure: These include ankle edema and ascites.
  • Other less common presenting symptoms
    • Brain abscess due to right-to-left shunt
    • Bacterial endocarditis
    • Paradoxical embolism, stroke, and transient ischemic attacks

Physical

Physical findings, like the symptoms, span a spectrum from subtle to dramatic.

  • Cyanosis and clubbing - Varying degrees of cyanosis at various times in life and transient worsening with arrhythmias
  • Precordial asymmetry
    • Usually left parasternal prominence and occasionally right parasternal prominence
    • Absent left parasternal (ie, right ventricular) lift an important negative sign
  • Jugular venous pulse
    • May be normal owing to a large, thin-walled right atrium, which can absorb the volume and pressure transmitted from the right ventricle through an incompetent tricuspid valve
    • Large a and v waves late in the course of the disease, with development of right heart failure
  • Arterial pulses
    • Usually normal
    • Diminished volume late in the course of the disease due to severe right heart failure and decreased left ventricular stroke volume
  • Heart sounds
    • First heart sound is widely split with loud tricuspid component secondary to delayed closure of the elongated anterior tricuspid leaflet, which has an increased excursion. Mitral component may be soft or absent in the presence of prolonged PR interval.
    • Second heart sound usually is normal but may be widely split when the pulmonary component is delayed due to right bundle-branch block (RBBB).
  • Additional heart sounds and murmurs
    • Third and fourth heart sounds are commonly present, even in the absence of congestive heart failure (CHF). Summation of third and fourth heart sounds, especially with prolonged PR interval, can mimic an early diastolic murmur.
    • The holosystolic murmur of tricuspid regurgitation is heard maximally at the lower left parasternal area and sometimes at the apex owing to the displaced location of the tricuspid valve; murmur intensity and duration increase during inspiration.

Causes

  • Ebstein anomaly is a congenital disease of often uncertain cause.
  • Environmental factors4 implicated in etiology include the following:
    • Maternal ingestion of lithium in first trimester of pregnancy
    • Maternal benzodiazepine use
    • Maternal exposure to varnishing substances
    • Maternal history of previous fetal loss
  • Risk is higher in whites than in other races.

Differential Diagnoses

Cyanosis
Tricuspid Atresia
Tricuspid Regurgitation

Other Problems to Be Considered

Rule out Ebstein anomaly in patients with the following:

  • Accessory pathway-mediated WPW syndrome and SVT
  • Cyanotic congenital heart diseases
  • Atrial septal defect (ASD)
  • Cases of severe right heart failure
  • Isolated severe tricuspid regurgitation
  • L-transposition of the great vessels
Ebstein anomaly should not be confused with Uhl anomaly, a congenital heart disease characterized by a hypoplastic, underdeveloped inflow segment of the right ventricle with tricuspid leaflets arising appropriately from the annulus.

Workup

Imaging Studies

  • Chest radiographs can reveal the following:
    • Normal findings
    • Cardiomegaly
    • Small aortic root and main pulmonary artery shadow
    • Decreased pulmonary vasculature
    • Large right atrium
  • Echocardiogram is the criterion standard for diagnosis.
    • M-mode
      • Paradoxical septal motion
      • Dilated right ventricle
      • Delayed closure of tricuspid valve leaflets more than 65 milliseconds after mitral valve closure
    • Two-dimensional
      • Apical displacement of the septal leaflet of tricuspid leaflet of greater than 8 mm/m2 - Most specific sign
      • Abnormalities in morphology and septal attachment of the septal and anterior tricuspid leaflets
      • Eccentric leaflet coaptation
      • Dilated right atrium
      • Dilated right ventricle with decreased contractile performance
      • Various left heart structural abnormalities (described recently in up to 39% of patients with Ebstein abnormality)
    • Doppler studies
      • Varying degrees of tricuspid regurgitation
      • Excludes associated shunts
    • Assessment of severity and surgical options by echocardiography
      • Functional right ventricular area less than 35% of total right ventricular area or an atrialized to functional right ventricular ratio greater than 0.5 associated with unfavorable prognosis
      • Functional right ventricular size
      • Degree of septal leaflet displacement
      • Amount of leaflet tethering
      • Magnitude of leaflet deformity and dysplasia
      • Aneurysmal dilatation of right ventricular outflow tract (right ventricular outflow tract-to-aortic root ratio of >2:1 on parasternal short axis view)
      • Moderate-to-severe tricuspid regurgitation
  • Cine MRI is currently not used routinely; it may become a useful noninvasive modality in the future.

Other Tests

Twelve-lead ECG

  • Rhythm
    • Usually normal sinus findings
    • Intermittently SVT, paroxysmal SVT, atrial flutter, atrial fibrillation, ventricular tachycardia
  • Abnormal P waves consistent with right atrial enlargement
  • PR interval
    • Most commonly prolonged
    • May be normal or short in patients with WPW syndrome
  • QRS complex
    • RBBB
    • Low voltage in many patients

Procedures

  • Cardiac catheterization
    • Rarely performed today
    • Confirms echocardiographic findings
    • Can reveal right ventricular electrical activity on the intracardiac ECG with simultaneous right atrial pressure and waveform when the catheter is withdrawn from the right ventricle, back across the tricuspid valve into the right atrium
  • Electrophysiologic studies
    • Of patients with Ebstein anomaly, 25-30% have accessory pathways, and 5-25% have evidence of preexcitation on the surface ECG.
    • Electrophysiologic (EP) studies in patients with tachyarrhythmias can delineate accessory conduction pathways and guide ablative therapy.
    • Right-sided pathways are more common.
    • Fifty percent of the patients have multiple pathways.

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.

Dosing

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

Interactions

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

Contraindications

Documented hypersensitivity; hepatic coma; anuria; severe electrolyte depletion

Precautions

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.

Dosing

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

Interactions

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

Contraindications

Documented hypersensitivity; beriberi heart disease; idiopathic hypertrophic subaortic stenosis; constrictive pericarditis; ventricular fibrillation; carotid sinus syndrome

Precautions

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.

Dosing

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

Interactions

NSAIDs may reduce hypotensive effects; may increase digoxin, lithium, and allopurinol levels; rifampin decreases levels; probenecid may increase levels; diuretics may enhance hypotensive effects

Contraindications

Documented hypersensitivity; angioedema secondary to ACE inhibitors

Precautions

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

Follow-up

Further Outpatient Care

Outpatient follow-up is directed toward the following:

  • Assessment of general symptoms (eg, fatigue, lassitude)
  • Assessment for development of bacterial endocarditis with careful history and examination for signs of bacterial endocarditis, such as fever, weight loss, anorexia, night sweats, splinter hemorrhages, clubbing, Roth spots, Janeway lesions, Osler nodes, splenomegaly, and hematuria
  • Assessment of signs of worsening heart failure, such as weight gain, ankle edema, and rales
  • Assessment for arrhythmias by history of symptoms, such as faintness or syncope
  • Periodic chest radiographs to assess for worsening cardiomegaly
  • Evaluation and adjustment of medications

Transfer

Transfer to a cardiothoracic surgery unit if necessary.

Complications

  • Congestive heart failure
  • Sudden cardiac death
  • Bacterial endocarditis
  • Brain abscess
  • Paradoxical embolism
  • Transient ischemic attacks
  • Stroke

Prognosis

  • Prognosis depends on the severity of the disease and treatment options available.
  • Poor prognostic signs include the following:
    • Male sex
    • Earlier age at presentation8
    • Cardiothoracic ratio of more than 0.65 on chest radiographs
    • Septal leaflet attachment ratio (ie, ratio of distance between AV ring and distal attachment of septal leaflet to length of septal leaflet) of more than 0.45
    • Increasing ratio of combined area of right atrium and atrialized right ventricle to that of the functional right ventricle—grade 1, which is less than 0.5, to grade 4, which is more than 1.5 (increase in the relative risk of 2.7 for each increment in the grade)
    • NYHA class - Also has been linked with mortality rate in some studies9
  • Pregnancy seems to be well tolerated with adequate supervision.10,7

Miscellaneous

Medicolegal Pitfalls

  • Failure to diagnose Ebstein anomaly in patients with tricuspid regurgitation and right heart failure is a potential pitfall.
  • Failure to identify and treat various arrhythmias associated with Ebstein anomaly may cause problems.
  • Failure to refer the patient for electrophysiological studies is a potential pitfall.
  • Failure to provide endocarditis prophylaxis advice is a potential pitfall.
  • Failure to refer the patient to a cardiothoracic surgeon in a timely fashion is a potential pitfall.

References

  1. Muñoz-Castellanos L, Espinola-Zavaleta N, Kuri-Nivón M, Keirns C. Ebstein's Anomaly: anatomo-echocardiographic correlation. Cardiovasc Ultrasound. Nov 23 2007;5:43. [Medline].

  2. Armengol Rofes AJ, Serrano Durán M, Albert Brotons DC, Sánchez López C, Casaldáliga Ferrer J, Girona Comas JM. [Ebstein's anomaly of the tricuspid valve. Apropos 35 cases]. An Esp Pediatr. Feb 1996;44(2):139-44. [Medline].

  3. Khositseth A, Khowsathit P. Factors affecting mortality in Ebstein's anomaly of the tricuspid valve. J Med Assoc Thai. Nov 1999;82 Suppl 1:S10-5. [Medline].

  4. Correa-Villaseñor 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].

  5. Attie F, Casanova JM, Zabal C, Buendía A, Miranda I, Rijlaarsdam M. Ebstein's anomaly. Clinical profile in 174 patients. Arch Inst Cardiol Mex. Jan-Feb 1999;69(1):17-25. [Medline].

  6. Brown ML, Dearani JA, Danielson GK, Cetta F, Connolly HM, Warnes CA, et al. Effect of operation for Ebstein anomaly on left ventricular function. Am J Cardiol. Dec 15 2008;102(12):1724-7. [Medline].

  7. Brown ML, Dearani JA, Danielson GK, Cetta F, Connolly HM, Warnes CA, et al. Functional status after operation for Ebstein anomaly: the Mayo Clinic experience. J Am Coll Cardiol. Aug 5 2008;52(6):460-6. [Medline].

  8. Celermajer DS, Bull C, Till JA, Cullen S, Vassillikos VP, Sullivan ID. Ebstein's anomaly: presentation and outcome from fetus to adult. J Am Coll Cardiol. Jan 1994;23(1):170-6. [Medline].

  9. Hong YM, Moller JH. Ebstein's anomaly: a long-term study of survival. Am Heart J. May 1993;125(5 Pt 1):1419-24. [Medline].

  10. Riesgo CA, Gómez LG, Higareda SH, Vargas JR. [Ebstein anomaly and pregnancy]. Ginecol Obstet Mex. Aug 2008;76(8):461-7. [Medline].

  11. Ammash NM, Warnes CA, Connolly HM, Danielson GK, Seward JB. Mimics of Ebstein's anomaly. Am Heart J. Sep 1997;134(3):508-13. [Medline].

  12. Attie F, Rosas M, Rijlaarsdam M, et al. The adult patient with Ebstein anomaly. Outcome in 72 unoperated patients. Medicine (Baltimore). Jan 2000;79(1):27-36. [Medline].

  13. Augustin N, Schreiber C, Lunge R. Valve preserving treatment of Ebstein's anomaly: perioperative and follow-up results. Thorac Cardiovasc Surg. Oct 2000;48(5):316. [Medline].

  14. Augustin N, Schreiber C, Wottke M, Meisner H. [Ebstein's anomaly: when should a patient have operative treatment?]. Herz. Aug 1998;23(5):287-92. [Medline].

  15. Brickner ME, Hillis LD, Lange RA. Congenital heart disease in adults. Second of two parts. N Engl J Med. Feb 3 2000;342(5):334-42. [Medline].

  16. Chauvaud S. Ebstein's malformation. surgical treatment and results. Thorac Cardiovasc Surg. Aug 2000;48(4):220-3. [Medline].

  17. Friedman WF. Congenital heart disease in infancy and childhood. In: Braunwald E, ed. Heart Disease: A Text Book of Cardiovascular Medicine. 5th ed. Philadelphia, Pa: WB Saunders Company; 1997:934-5.

  18. Gerlis LM, Ho SY, Sweeney AE. Mitral valve anomalies associated with Ebstein's malformation of the tricuspid valve. Am J Cardiovasc Pathol. 1993;4(4):294-301. [Medline].

  19. Kaneko Y, Okabe H, Nagata N, Yasui S, Yamada S, Kobayashi J. Repair of septal and posterior tricuspid leaflets in Ebstein's anomaly. J Card Surg. Jul 1998;13(4):229-35. [Medline].

  20. MacLellan-Tobert SG, Driscoll DJ, Mottram CD, Mahoney DW, Wollan PC, Danielson GK. Exercise tolerance in patients with Ebstein's anomaly. J Am Coll Cardiol. Jun 1997;29(7):1615-22. [Medline].

  21. Perloff JK. Ebstein's anomaly of the tricuspid valve. In: Perloff JK, ed. The Clinical Recognition of Congenital Heart Disease. 3rd ed. Philadelphia, Pa: WB Saunders Company; 1991:235-251.

  22. Ronan Jr JA. Congenital heart disease. In: Chizner MA, ed. Classic Teachings in Clinical Cardiology. 2. First ed. Cedar Grove, NJ: Laennec; 1996:1242-44.

  23. Shaughnessy R. Ebstein's anomaly. In: Crawford MH, DiMarco JP, eds. Cardiology. London: Mosby International; 2001:6.13.1-6.

  24. Weyman AE. Right ventricular inflow tract (congenital anomalies associated with tricuspid regurgitation). In: Weyman AE, ed. Principles and Practice of Echocardiography. 1994. 3rd ed. Philadelphia, Pa: Lea & Febiger:840-2.

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

Contributor Information and Disclosures

Author

Kamran Riaz, MD, Clinical Assistant Professor, Department of Internal Medicine, Section of Cardiology, Wright State University School of Medicine
Kamran Riaz, MD is a member of the following medical societies: American College of Cardiology, American College of Physicians, American Medical Association, American Society of Echocardiography, Ohio State Medical Association, and Royal College of Physicians
Disclosure: Nothing to disclose.

Medical Editor

Park W Willis IV, MD, Sarah Graham Distinguished Professor of Medicine and Pediatrics, University of North Carolina at Chapel Hill School of Medicine
Park W Willis IV, MD is a member of the following medical societies: American Society of Echocardiography
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Marschall S Runge, MD, PhD, Charles and Anne Sanders Distinguished Professor of Medicine, Chairman, Department of Medicine, Vice Dean for Clinical Affairs, University of North Carolina at Chapel Hill School of Medicine
Marschall S Runge, MD, PhD is a member of the following medical societies: American Association for the Advancement of Science, American College of Cardiology, American College of Physicians-American Society of Internal Medicine, American Federation for Clinical Research, American Federation for Medical Research, American Heart Association, American Physiological Society, American Society for Clinical Investigation, American Society for Investigative Pathology, Association of American Physicians, Association of Professors of Cardiology, Association of Professors of Medicine, Southern Society for Clinical Investigation, and Texas Medical Association
Disclosure: Pfizer Honoraria Speaking and teaching; Merck Honoraria Speaking and teaching; Orthoclinica Diagnostica Consulting fee Consulting

CME Editor

Amer Suleman, MD, Consultant in Electrophysiology and Cardiovascular Medicine, Department of Internal Medicine, Division of Cardiology, Medical City Dallas Hospital
Amer Suleman, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Institute of Stress, American Society of Hypertension, Federation of American Societies for Experimental Biology, Royal Society of Medicine, and Society of Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

Chief Editor

Park W Willis IV, MD, Sarah Graham Distinguished Professor of Medicine and Pediatrics, University of North Carolina at Chapel Hill School of Medicine
Park W Willis IV, MD is a member of the following medical societies: American Society of Echocardiography
Disclosure: Nothing to disclose.

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