Congenital absence of the left pericardium and complete heart block. Report of a case.

FIGURE 1: Significant features include leftward displacement of the heart without tracheal deviation. In addition, three distinct convexities are noted along the left border of the heart. These are the aortic knob, pulmonary artery segment and the left ventricular contour. T HE RECOGNITION AND DEFINITIVE DIAGnosis of absent pericardium has gained impetus within recent years. Although well described for many years as a curious anomaly found at post-mortem examination , it was not until 1959 that Ellis et al' reported the first case of congenital absence of the pericardium in which the diagnosis was successfully established by diagnostic pneumothorax during life. This case of deficient left pericardium with concomitant complete heart block depicted with angiographic, radiologic, and hemodynamic studies is presented and the proper diagnostic techniques underscored. The differentiation of complete and partial deficiency of the pericardium is stressed in terms of diagnosis, prognosis and treatment .