대한흉부영상의학회 Korean Society of Thoracic Radiology

Discussion

Diagnosis With Brief Discussion

Diagnosis

Congental Abscence of the Left Pericardium

Radiologic Findings

Chest PA shows leftward displacement of the heart with prominent pulmonary trunk. Lucency between the ascending aorta and pulmonary trunk is noted.

Chest CT with lung window setting demonstrates abnormal interposition of lung between the ascending aorta and pulmonary trunk which has rotated clockwise away from the mediastinum. CT at the level of heart demonstrates abrupt termination of the right pericardium at the site of indentation of the cardiac contour (arrow) where the heart bulges into the left chest.

Brief Review

Congenital absence of the left pericardium results from maldevelopment of the pleuropericardial membrane, which is likely due to an inadequate blood supply resulting from premature involution of the left duct of Cuvier (common cardinal vein). Both partial and complete absence are quite rare and were seen in less than 0.01% of 34,000 patients undergoing cardiovascular surgery at one institution. Defects occurring on the right are exceedingly rare, presumably because the right duct of Curvier develops into the SVC, thus ensuring closure of the right pleuropericardial membrane. Although are usually asymptomatic, the most common complaint is vague chest pain, possibly as a result of adhesion or abnormal traction of great vessels. Deaths have been reported in partial absence of pericardium due to herniation of the left ventricle through the defect. Established radiographic findings of complete absence of left pericardium include an unusual cardiac silhouette with elongation of the left heart border, leftward shift and clockwise rotation of the heart, a radiolucent cleft between the ascending aorta and the main pulmonary artery, and the lucency separating the heart and left hemidiaphragm. The first two of the above findings related to the abnormal mobility of the heart, while the last two findings result from interposition of lung that is normally restricted by the left pericardium. The CT findings are homologous to those on the chest radiograph. The normal pericardium is seen on CT in 95% to 100% of cases, thus, nonvisualization of the pericardium strongly suggests its absence when ancillary findings are present.

References

1. Baim Rs, MacDonald IL, Wise DJ, Lenkei SC. Computed Tomography of Absent Left Pericardium. Radiology 1980; 135: 127-128.
2. Gutierrez FR, Shackelford GD, McKnight RC, Levitt RG, Hartmann A. Diagnosis of Congenital Absence of Left Pericardium By MR Imaging. J Comput Assist Tomogr 1985;9:551-553.
3. Pickhardt PJ. Congenital Absence of the Pericardium Confirmed by Spontaneous Pneumothorax. Clin Imaging 1998;22:404-407.

Keywords

Pericardium, Congenital,