Ebstein’s anomaly: A Case Report
DOI:
https://doi.org/10.3823/2104Keywords:
Ebstein’s anomaly; congenital heart disease; Tricuspid insufficiency; iatrogenesis., Ebstein's anomaly, congenital heart disease, Tricuspid insufficiency, iatrogenesis.Abstract
Introduction: Ebstein's anomaly is a rare malformation that corresponds to less than 1% of all congenital heart anomalies. It consist in the caudal displacement of the tricuspid valve with retrograde flow to the right atrium due to valvular insufficiency and is characterized by a variable spectrum of severity, being higher in the neonatal period. Objective: to report a case of Ebstein's anomaly wich early diagnosis avoid iatrogenic acts. Case report: a newborn at term, appropriate weight for gestational age, female, was born of natural childbirth, with Apgar score 8/9 from pregnancy without complications. At birth, not in need of resuscitation in the delivery room, but presented heart murmur and fall of saturation, being supported and then forwarded to the NICU. Not present hemodynamic instability. The chest x-ray showed increased cardiac area with increased right atrium. The Transthoracic Echocardiogram showed mild right ventricular dilatation and important of the right atrium, tricuspid valve dysplasia with low implantation of posterior leaflet of tricuspid insufficiency presence important to Doppler, being diagnosed with Ebstein's anomaly. Evolved with progressive improvement of the frame and saturation above 95% on room air to pulse oximetry. Patient follows in outpatient follow-up in use of inotropic and diuretic, remaining asymptomatic. Conclusion: the recognition of the gravity of the picture is relevant to the proper management in order to prevent iatrogenic ducts, which can lead to complications or permanent sequelae.
References
Caliani JA, Simões LCN. Doença de Ebstein. In: Croti UA, Mattos SS, Pinto Jr. VC, Aiello VD, Moreira VM. Cardiologia e cirurgia cardiovascular pediátrica. 2ª ed. São Paulo:Roca; 2012. p. 749-60.
MacLellan-Tobert SJ, Porter CBJ. Ebstein´s anomaly of the tricuspid valve. In: Gar-son AJ, Bricker JT, Fisher DJ, Neish SR. The Science and Practice of Pediatric Cardiology. 2nd Ed. Lea & Febiger, Cidade: Philadelphia Editora, 1998: 1303.
Edwards WD. Embryology and pathologic features of Ebstein’s anomaly.Prog Pediatr Cardiol. 1993;2:5–15.
Jaquiss RD, Imamura RD. Management of Ebstein’s Anomaly and Pure Tricuspid Insufï¬ciency in the Neonate. Semin Thorac Cardiovasc Surg. 2007;19(3):258-63.
Giuliani ER, Fuster V, Brandenburg RO, Mair DD. Ebstein’s anomaly: the clinical features and natural history of Ebstein’s anomaly of the tricuspid valve. Mayo Clin Proc. 1979;54:163–73.
Shiina A, Seward JB, Edwards WD, Hagler DJ, Tajik AJ. Two-dimensional echocar-diographic spectrum of Ebstein’s anomaly: detailed anatomic assessment. J Am Coll Cardiol. 1984;3:356 –370.
Rivera IR, Silva MAM, Fernandes JMG, Thomaz ACP, Soriano CFR, Souza MGB. Cardiopatia Congênita no Recém-Nascido: da Solicitação do Pediatra à Avaliação do Cardiologista. Arq Bras Cardiol. 2007;89(1):6-10.
McElhinney BD, Salvin WJ, Colan DS, Thiagarajan R, et al. Improving outcomes in fetus and neonates with congenital displacement (Ebstein’s malformation) or dys-plasia of the tricuspid valve. Am J Cardiol 2005;96:582-6.
Atik E. Ebstein’s Anomaly. Arq. Bras. Cardiol. 2011;97(5):363-4.
da Silva JP, Silva LF, Moreira LFP, Lopes LM, Franki SM, Lianza AC, et al. A ténica do cone para correção da anomalia de Ebstein: Resultados imediatos e em longo prazo. Arq Bras Cardiol. 2011;97(3):199-208.
Leite, Maria de Fátima M.P., Gianisella, Roberto B. and Zielinsky, Paulo. Anomalia de Ebstein detectada in utero e sÃndrome de Down: diagnóstico pré-natal de uma combinação rara. Arq. Bras. Cardiol. 2004; 82(4):390-2.
Zielinsky P, Pilla CB. Anomalia de Ebstein Com Valva Tricúspide Imperfurada. Di-agnóstico Pré-Natal. Arq Bras Cardiol. 2000;75(1):59-61.
Rao PS, Jue KL, Isabel-Jones J, Ruttenberg HD. Ebstein’s malformation of the tri-cuspid valve with atresia - Differentiation from isolated tricuspid atresia. Am J Cardiol 1973; 32: 1004-9.
Anderson RH, Shinebourne EA, Macartney FJ, Tynan M. Ebstein’s malformation and related lesions of the tricuspid valve. Paediatric Cardiology 1987: 721-36
Bove EL, Hirsch JC, Ohye RG, Devaney EJ. How I Manage Neonatal Ebstein’s Anomaly. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2009; 12: 63-5.
Silversides CK, Kiess M, Beauchesne L, et al. Canadian Cardiovascular Society 2009 Consensus Conference on the management of adults congenital heart dis-ease: Outflow tract obstruction, coarctation of the aorta, tetralogy of Fallot, Ebstein anomaly and Marfan's syndrome. Can J Cardiol. 2010 Mar;26(3):e80-97.
Lunn RJ. Inhaled nitric oxide therapy. May Clin Proc. 1995;70(3):247–55.
Silva JP, Baumgratz JF, Fonseca LD, Afiune JY, Franchi SM, Lopes LM, et al. A-nomalia de Ebstein. Resultados com a reconstrução cônica da valva tricúspide. Arq Bras Cardiol. 2004;82(3):212-6.
Negoi RI, Ispas AT, Ghiorghiu I, Filipoiu F, Negoi I, Hostiuc M, Hostiuc S, Ginghina C. Complex Ebstein’s Malformation: Deï¬ning Preoperative Cardiac Anatomy and Function. J Card Surg. 2013;28:70–81
Shinkawa T, Palimenakos AC, Gomez-Fifer CA, Charpier JR, Hisrsh JC, Devaney EJ, et al. Management and long-term outcome of neonatal Ebstein anomaly. J Thorac Cardiovasc Surg. 2010;139(2):354-8.
Jost CHA, Connolly HM, Dearani JA, Edwards WD, Danielson GK. Ebstein's Ano-maly. Circulation. 2007;115:277-85.
Radford EPW, Gary DWM, Hijazi SA. et al. ACC/AHA 2008 Guidelines for the Man-agement of Adults With Congenital Heart Disease. Circulation. 2008;118:e714-e833. doi: 10.1161/.Circulation AHA.108.190690.
Krebs VLJ, Trostes EJ. Complicações da ventilação mecânica. Pediatria Moderna, 2000;36:58-60.
Costa ACO , Schettino RC , Ferreira SC. Fatores preditivos para falha de extuba-ção e reintubação de recém-nascidos submetidos à ventilação pulmonar mecânica. Rev Bras Ter Intensiva. 2014;26(1):51-6.
Downloads
Published
Issue
Section
License
Authors who publish with this journal agree to the following terms:
- Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a Creative Commons Attribution License that allows others to share the work with an acknowledgement of the work's authorship and initial publication in this journal.
- Authors are able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgement of its initial publication in this journal.
- Authors are permitted and encouraged to post their work online (e.g., in institutional repositories or on their website) prior to and during the submission process, as it can lead to productive exchanges, as well as earlier and greater citation of published work (See The Effect of Open Access and Benefits of Publishing Open Access).
Â
Â