IS Case 131: Ebstein's Anomaly
Imaging Sciences URMC 2008
Publication Date: 2009-05-21
Patient is a newborn infant with known Ebstein's anomaly and hemodynamic instability.
When massive cardiomegaly is identified on a neonatal chest film few disease processes must be considered:
- Ebstein's anomaly: Apical displacement of the tricuspid leaflets causing "atrialization" of a portion of the right ventricle. The massive shadow is caused by enlarged right atrium secondary to obstruction at the level of the dysfunctional tricuspid valve.
- Pulmonary atresia with intact ventricular septum: No forward flow from right side of heart so the right atrium massively enlarges with some enlargement of right ventricle.
- Tumor: Cardiac mass such as rhabdomyoma or mediastinal masses.
- Peripheral AV fistula with high output cardiac failure.
- Diaphragmatic hernia without air in bowel.
The diagnosis of Ebstein's anomaly is made by echocardiography, but a massive cardiac silhouette with paucity of pulmonary vascular markings on chest x-ray should lead to a differential including the top two options above.
Surgical correction may be considered in Ebstein's anomaly in the following scenarios:
- NYHA Class III-IV Heart Failure
- NYHA Class I-II Heart Failure with worsening symptoms and/or cardiothoracic ration >0.65
- Arrhythmias refractory to other therapy (common cause of death in these patients)
- Polycythemia with HgB >16 g/dl
- Cyanosis with arterial oxygen saturation of <80%
- Paradoxical embolism (shunting)
- Donnelly L. Fundamentals of Pediatric Radiology. WB Saunders Co, 2001:71-97.
- Augustin N, Schreiber C, Wottke M, Meisner H. [Ebstein's anomaly: when should a patient have operative treatment?]. Herz. 1998 Aug;23(5):287-92. [PMID: 9757375]
- # Attie F, Casanova JM, Zabal C, et al. Ebstein's anomaly. Clinical profile in 174 patients. Arch Inst Cardiol Mex. 1999 Jan-Feb;69(1):17-25. [PMID: 10367089]