Isolated congenital absence of the pericardium: Clinical presentation, diagnosis, and management

Background. Congenital absence of the pericardium (CAP) is a rare clinical entity. Methods. We identified from the two hospital databases all patients with is olated CAP, reviewed their data, and invited them for prospective clinical evaluation with electrocardiography, chest x-ray findings (CXR), echocardio graphy, and magnetic resonance imaging (MRI). Results. Ten patients (3 males, 7 females) presented at a median age of 21 years (range, 2-53 years) with paroxysmal stabbing chest pain, largely none xertional (9), and heart murmur with an abnormal CXR (1). Three patients ha d partial and 7 had complete CAP (all 7 had marked lateral displacement of the cardiac apex). CXR combined with MRI were key to establishing the diagn osis; a "tongue" of lung tissue interposing between the main pulmonary arte ry and aorta was the most consistent diagnostic feature. Four patients unde rwent pericardioplasty, 3 for debilitating symptoms and 1 for left atrial a ppendage herniation, followed by improvement or resolution of symptoms. At a mean of 10.5 years from presentation all patients were alive. No complica tions were seen in the nonsurgical group. Conclusions. Isolated CAP has a common presentation pattern with periodic s tabbing chest pain mimicking coronary artery disease. CXR and MRI are requi red for definitive diagnosis. Symptomatic patients with the complete form m ay benefit from pericardioplasty. (C) 2000 by The Society of Thoracic Surge ons.