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Imaging Sciences Interesting Cases

Case 131

Nate Johnson, MD

Clinical Presentation: Patient is a newborn infant with known Ebstein's anomaly and hemodynamic instability.

Imaging Findings: (see below)
Figure 1: Massive cardiothymic silhouette with paucity of pulmonary vascular markings.

Diagnosis: Ebstein's Anomaly

Discussion: When massive cardiomegaly is identified on a neonatal chest film few disease processes must be considered:

  1. 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.
  2. 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.
  3. Tumor: Cardiac mass such as rhabdomyoma or mediastinal masses.
  4. Peripheral AV fistula with high output cardiac failure.
  5. 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:

  1. NYHA Class III-IV Heart Failure
  2. NYHA Class I-II Heart Failure with worsening symptoms and/or cardiothoracic ration >0.65
  3. Arrhythmias refractory to other therapy (common cause of death in these patients)
  4. Polycythemia with HgB >16 g/dl
  5. Cyanosis with arterial oxygen saturation of <80%
  6. Paradoxical embolism (shunting)

References:

  1. Donnelly L. Fundamentals of Pediatric Radiology. WB Saunders Co, 2001:71-97.
  2. 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. [PubMed]
  3. 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. [PubMed]