SPR Unknown #67 --FINAL

Christine Carqueville

Cynthia Rigsby, M.D.

Children's Memorial Hospital

Abstract

13 year old male with chest pain

Keywords

absence of pericardium, Chest x-ray, CXR, 447


Publication Date: 2004-11-10

History

13 year old male with chest pain

Findings

CXR - Rotation and shift of the heart to the left with bulge in the upper cardiac silhouette.

Diagnosis

Congenital absence of the pericardium

Differential

hilar mass.

Discussion

Congenital absence of the pericardium is most likely secondary to the early atrophy of the left duct of Cuvier, which is responsible for supplying blood to the embryonic pleuropericardial membrane. This membrane forms the left pericardium in normal individuals. Partial or complete absence of the left pericardium accounts for up to 70% of cases, with total absence of the left pericardium being the most common, as seen in this case. Right pericardial defects and total absence of pericardium are rare. Associated congenital anomolies involving the heart and lung include patent ductus arteriosus, atrial septal defect, TOF, and pulmonary sequestration. Other anomalies that could be seen are diaphragmatic hernia, sternal defect, VATER, and omphalocele.

Presentation may include chest pain, systilic murmurs, and an abnormal point of maximal cardiac impulse. Pericardial absence is often a benign condition, but congenital pericardial defects can potentially result in herniation and strangulation of the heart, leading to death.

Complete absence of the left pericardium on a chest x-ray shows the heart to be shifted to the left and rotated clockwise so that the left border of the heart is formed by the right ventricle. The trachea is characteristically midline, and the main pulmonary artery has variable noted prominence along the left upper heart border. Striking prominence of the left atrial appendage along the left heart margin may be seen.

References

  1. Kuhn, J.P., Slovis, T.L., Haller, J.O., CAFFEY'S PEDIATRIC DIAGNOSTIC IMAGING, Elsevier, Inc., 2004, 1383-1385.

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