Jugular phlebogram in congenital absence of the pericardium

The purpose of this study was to emphasize the diagnostic value of the jugular phlebogram in congenital absence of the pericardium. Phonocardiographic study was performed in seven patients with complete absence of the left pericardium and in four with partial pericardial defect (left sided in three, right sided in one). Associated heart lesions were absent in all patients. The characteristic features of the jugular phlebogram in these patients were decreased depth of x descent and tall v wave followed by deep y descent (M-shaped pattern). These jugular abnormalities were more prominent in complete absence of the left pericardium than in partial pericardial defect. The x descent was completely obliterated in two patients with complete left pericardial defect. Loss of decrease in pericardial pressure during ventricular ejection and altered cardiac position as a result of the absence of the pericardium may be responsible for these jugular abnormalities. Jugular venous M-shaped pattern may be one of the useful physical and phonocardiographic indicators for the diagnosis of congenital absence of the pericardium.