Odd Acoustic Window and Elongated Ventricles: Echocardiographic Diagnosis of Congenital Absence of the Pericardium

Abstract

A 14-year-old boy with a heart murmur was referred to the authors' department because structural heart disease could not be ruled out by standard echocardiographic views. The best apical four-chamber view was obtained with the patient turned to a right lateral decubitus position and the transducer shifted almost to the posterior axillary line. A biplane chest x-ray also showed a counterclockwise heart axis deviation. Magnetic resonance imaging confirmed the suspected congenital absence of the pericardium.

Case series, contemporary review and imaging guided diagnostic and management approach of congenital pericardial defects

“…One of the first findings appreciated on echocardiography in patients with the complete absence of the pericardium is that the standard views may not apply 12 29. On the traditional left parasternal view, more of the right ventricle is seen due to the left shift of the heart and, as a result, the patient may be falsely diagnosed with RV dilatation (figure 3A).…”

“…The paradoxical movement can be cancelled in the right lateral position since the right-sided pericardium restricts the abnormal shift of the heart 20. Cardiac hypermobility and abnormal ventricular septal wall ‘rocking motion’ are described in most patients 29. The typical findings with the complete absence of the pericardium are a ‘teardrop’ shape of the heart, a bulbous LV and elongated atria in the apical four-chamber view, because of the absence of normal tethering applied by the normal pericardium 12.…”