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Armanni-Ebstein kidney — Clinical Guidelines

Overview

Ebstein anomaly (EA) is a congenital heart defect characterized by an abnormally positioned and malformed tricuspid valve, leading to right ventricular atrialization, hypoplasia, and functional impairment. It often results in symptoms such as cyanosis, tricuspid regurgitation, arrhythmias, and heart failure 1.

Diagnosis

Key Diagnostic Criteria: Apical displacement of the tricuspid valve, right ventricular atrialization, and tricuspid regurgitation 1.

Recommended Tests: Echocardiography for detailed anatomical assessment, including evaluation of valve position and function 1 3 4 6 13.

Additional Imaging: Cardiac MRI for comprehensive assessment of biventricular function and anatomy 4 7 13.

Electrocardiogram (ECG) Findings: QRS duration and fractionation can indicate right ventricular dysfunction and atrialization 19.

Management

Surgical Interventions: Cone repair for tricuspid valve reconstruction to improve right ventricular function and tricuspid valve competence 5 13 18.

Valve Replacement: Use of bioprosthetic or mechanical valves when tricuspid valve repair is insufficient 21.

Percutaneous Interventions: Transcatheter tricuspid valve-in-valve implantation for failed surgical repairs 10 17.

Medical Management: Focus on symptom control, anticoagulation (if mechanical valve), and management of arrhythmias 2 12.

Special Populations

Pregnancy: Prenatal management with NSAIDs may be considered to improve outcomes in severe cases with circular shunt, though evidence is limited 9 15.

Pediatrics: Cone reconstruction shows promising results in pediatric patients with improved biventricular function 18.

Comorbidities: Left ventricular dysfunction should be monitored preoperatively and postoperatively, with surgical intervention aimed at preserving LV function 23.

Key Recommendations

Perform comprehensive echocardiography and cardiac MRI preoperatively to assess biventricular function and anatomy (Evidence: Moderate 4 6 7 13).

Consider cone repair as a primary surgical technique for Ebstein anomaly to enhance right ventricular rehabilitation and tricuspid valve function (Evidence: Moderate 5 13 18).

Evaluate and manage potential left ventricular involvement preoperatively and postoperatively (Evidence: Moderate 23).

Use transcatheter interventions for patients with failed surgical repairs, particularly those requiring tricuspid valve replacement (Evidence: Weak 10 17).

In severe fetal cases with circular shunt, consider prenatal NSAID therapy cautiously, acknowledging limited evidence (Evidence: Weak 9 15).

References

1 Coacci S, Sanders SP, Carreon CK. Ebstein Anomaly and the Range of Right-Sided Heart Defects. Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society 2026. link 2 Afoke J, Stephens EH, Allison TG, Chopko TC, Chaubey S, Briggs C et al.. Shifting Paradigms: Exercise Testing as a Metric of Long-Term Success in Surgery for Ebstein Anomaly. The Annals of thoracic surgery 2026. link 3 Guo X, Zhao B, Li Y, Zhou X. Study on the Correlation Between Ventricular Function Evaluated by Z-Score of Atrioventricular Annular Plane Systolic Excursion and Pulmonary Artery Abnormality and Retrograde Ductus Arteriosus Flow in Fetuses With Ebstein Anomaly. Journal of clinical ultrasound : JCU 2026. link 4 Gupta A, Da Silva L, Wang L, Diaz-Castrillon CE, Dobson CP, Da Silva J et al.. Assessing Left Ventricular Pathology in Patients with Ebstein Anomaly Using Cardiovascular Magnetic Resonance: Looking Past the Right Heart. Pediatric cardiology 2025. link 5 Ashraf SF, Da Silva JP, Castro-Medina M, Viegas M, Alsaied T, Seese L et al.. The cone repair allows right ventricle rehabilitation with excellent tricuspid valve function following the Starnes procedure. The Journal of thoracic and cardiovascular surgery 2025. link 6 Meng H, Wan LY, Qu R, Liu QQ, Li MZ, Li YD et al.. Biventricular function after Ebstein anomaly repair from a single-center echocardiography study. Hellenic journal of cardiology : HJC = Hellenike kardiologike epitheorese 2025. link 7 Carney M, Gupta A, Christopher A, Olivieri L, Da Silva J, Diaz Castrillon C et al.. Large Right Atrial Size on Cardiac MRI is Associated with Post-operative Right Ventricular Dysfunction After the Cone Operation for Ebstein Anomaly. Pediatric cardiology 2025. link 8 Goo HW, Park SH. Complimentary Cardiac Computed Tomography Ventricular Volumetry-Derived Metrics of Severity in Patients with Ebstein Anomaly: Comparison with Echocardiography-Based Severity Indices. Pediatric cardiology 2024. link 9 Mustafa HJ, Aghajani F, Bairmani ZA, Khalil A. Transplacental non-steroidal anti-inflammatory drugs versus expectant management in fetal Ebstein anomaly with circular shunt: Systematic review and meta-analysis. Prenatal diagnosis 2024. link 10 Odemis E, Yenidogan I, Aydin S. Sequential percutaneous tricuspid and pulmonary valve implantation in a young child operated previously for Ebstein Anomaly. Cardiology in the young 2023. link 11 Holst KA, Dearani JA, Qureshi MY, Wackel P, Cannon BC, O'Leary PW et al.. From Safety to Benefit in Cell Delivery During Surgical Repair of Ebstein Anomaly: Initial Results. The Annals of thoracic surgery 2022. link 12 Egbe A, Miranda W, Connolly H, Dearani J. Haemodynamic determinants of improved aerobic capacity after tricuspid valve surgery in Ebstein anomaly. Heart (British Cardiac Society) 2021. link 13 Neijenhuis RML, Tsang VT, Marek J, Issitt R, Bonello B, Von Klemperer K et al.. Cone reconstruction for Ebstein anomaly: Late biventricular function and possible remodeling. The Journal of thoracic and cardiovascular surgery 2021. link 14 Kronberg K, Horn M, Mellert F, Elsässer A. Transcatheter tricuspid valve-in-valve replacement in two patients with Ebstein anomaly: technical considerations. Clinical research in cardiology : official journal of the German Cardiac Society 2021. link 15 Freud LR, Wilkins-Haug LE, Beroukhim RS, LaFranchi T, Phoon CK, Glickstein JS et al.. Effect of In Utero Non-Steroidal Anti-Inflammatory Drug Therapy for Severe Ebstein Anomaly or Tricuspid Valve Dysplasia (NSAID Therapy for Fetal Ebstein anomaly). The American journal of cardiology 2021. link 16 Freeman A, Byard RW. Ebstein Anomaly and Sudden Childhood Death. Journal of forensic sciences 2018. link 17 Taggart NW, Cabalka AK, Eicken A, Aboulhosn JA, Thomson JDR, Whisenant B et al.. Outcomes of Transcatheter Tricuspid Valve-in-Valve Implantation in Patients With Ebstein Anomaly. The American journal of cardiology 2018. link 18 Anderson HN, Dearani JA, Said SM, Norris MD, Pundi KN, Miller AR et al.. Cone reconstruction in children with Ebstein anomaly: the Mayo Clinic experience. Congenital heart disease 2014. link 19 Egidy Assenza G, Valente AM, Geva T, Graham D, Pluchinotta FR, Sanders SP et al.. QRS duration and QRS fractionation on surface electrocardiogram are markers of right ventricular dysfunction and atrialization in patients with Ebstein anomaly. European heart journal 2013. link 20 Rao MP, Panduranga P, Al-Mukhaini M, Al-Jufaili M. Ebstein anomaly in an adult presenting with wide QRS tachycardia: diagnostic and therapeutic dilemmas. The American journal of emergency medicine 2012. link 21 Brown ML, Dearani JA, Danielson GK, Cetta F, Connolly HM, Warnes CA et al.. Comparison of the outcome of porcine bioprosthetic versus mechanical prosthetic replacement of the tricuspid valve in the Ebstein anomaly. The American journal of cardiology 2009. link 22 Adachi I, Ho SY, McCarthy KP, Uemura H. Coronary blood supply of the inferior wall of the right ventricle in hearts with Ebstein malformation: relevance to vertical plication. The Journal of thoracic and cardiovascular surgery 2008. link 23 Brown ML, Dearani JA, Danielson GK, Cetta F, Connolly HM, Warnes CA et al.. Effect of operation for Ebstein anomaly on left ventricular function. The American journal of cardiology 2008. link 24 Wu Q, Huang Z, Pan G, Wang L, Li L, Xue H. Early and midterm results in anatomic repair of Ebstein anomaly. The Journal of thoracic and cardiovascular surgery 2007. link 25 Vettraino IM, Huang R, Comstock CH. The normal offset of the tricuspid septal leaflet in the fetus. Journal of ultrasound in medicine : official journal of the American Institute of Ultrasound in Medicine 2002. link 26 Eidem BW, Tei C, O'Leary PW, Cetta F, Seward JB. Nongeometric quantitative assessment of right and left ventricular function: myocardial performance index in normal children and patients with Ebstein anomaly. Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography 1998. link70004-5) 27 Suneja A, Nalini, Agarwal N, Gupta S, Dev G, Jain N. Fetal Ebstein anomaly--a rare cause of non-immune hydrops. Indian journal of pediatrics 1996. link 28 Kastler B, Livolsi A, Zhu H, Roy E, Zollner G, Dietemann JL. Potential role of MR imaging in the diagnostic management of Ebstein anomaly in a newborn. Journal of computer assisted tomography 1990. link 29 Taguchi K, Matsumura M, Ishikawa M, Isono M, Hamanaka Y, Sueda T et al.. Deterioration of Ebstein disease after closure of atrial septal defect. The Japanese journal of surgery 1982. link

Armanni-Ebstein kidney