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.
Highlights
Ebstein’s anomaly is a rare malformation that corresponds to less than 1% of all congenital heart anomalies
Ebstein’s anomaly is a rare heart disease that variable morphology corresponds to less than 1% of all congenital cardiac anomalies
The aim of this study is to report an Ebstein’s anomaly case which early diagnosis avoided iatrogenic ducts
Summary
Ebstein’s anomaly is a rare malformation that corresponds to less than 1% of all congenital heart anomalies It consists in the caudal displacement of the tricuspid valve with retrograde flow to the right atrium due to valvular insufficiency and it is characterized by a variable spectrum of severity, being higher in the neonatal period. There are cases in which there is relation with family history, the most is sporadic[1,2] It is characterized by malformations and low deployment of septal and posterior leaflets of the tricuspid valve, showing an area of “atrialized” right ventricle[3]. The etiology of this anomaly can be explained by the rupture of the papillary muscle of the tricuspid valve. The typical presentation of Ebstein’s anomaly in the neonatal period is cyanotic newborn with cardiomegaly to x-ray[4]
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