Abstract
Introduction: Ebstein’s anomaly is a rare congenital heart disorder occurring in ≈1 per 210 000 live births and accounting for <1% of all cases of congenital heart disease. In ebstein anomaly there is downward displacement of the septal and posterior leaflets of tricuspid valve into the RV cavity, resulting in functional hypoplasia of RV.
Case Report: Full term appropriate for gestational age female child born by vaginal delivery, without any antenatal complications, APGAR at birth 8/10, did not require any resuscitation was admitted in NICU in view of cyanosis. Auscultation revealed murmur and saturation was 75-77% in all 4 limbs. Clinical features revealed persistant tachycardia, gallop rhythm, suggestive of congestive cardiac failure. The baby had multiple episodes of supra ventricular tachycardia which responded to anti-arrhythmic agents including intravenous adenosine, intravenous beta blocker, followed by oral beta blocker.
The chest x-ray showed cardiomegaly with oligemic lung fields. ECG had tall peaked P wave with RBBB. In 2 D echo there was right atrial dilatation with apical displacement of septal leaflet of tricuspid valve suggestive of ebstein anomaly. Cardiac CT confirmed ebstein anomaly. Anti-failure medication continued and baby was considered for surgical intervention on oral anti-arrhythmic medications.
Keywords: Congenital heart disease, ebstein’s anomaly, supraventricular tachycardia
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: Garson 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 Insufficiency in the Neonate. Semin Thorac Cardiovasc Surg. 2007;19(3):258-63.
- Shiina A, Seward JB, Edwards WD, Hagler DJ, Tajik AJ. Twodimensional echocardiographic 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 dysplasia of the tricuspid valve. Am J Cardiol 2005;96:582-6.
- Trippel DL, Wiest DB, Gillette PC. Cardiovascular and antiarrhythmic effects of esmolol in children. J Pediatr. 1991;119: 142–147.
- Wiest DB, Trippel DL, Gillette PC, Garner SS. Pharmacokinetics of esmolol in children. Clin Pharmacol Ther. 1991;49: 618–623
- Abrams J, Allen J, Allin D, et al. Efficacy and safety of esmolol vs propranolol in the treatment of supraventricular tachyarrhythmias: a multicenter double-blind clinical trial. Am Heart J. 1985; 110:913–922
Corresponding Author
Dr Shaista Parween
M.D. Pediatrics, Junior Resident, Department of Pediatrics, Grant Govt Medical College, Mumbai
DOI: https://dx.doi.org/10.18535/jmscr/v7i8.56
