Congenital partial absence of the pericardium in a young man with atypical chest pain

Abstract

Pericardial defects are infrequent congenital anomalies due to agenesis caused by premature atrophy of the common cardinal vein or Cuvier duct during the 5(th) or 6(th) week of embryonic life. These congenital defects are rare, typically observed as an incidental finding and usually remain asymptomatic. Nevertheless, the more widespread use of modern imaging techniques has contributed to an increase of its incidence in recent years. There is currently no consensus regarding therapeutic options, all of which are based on small retrospective studies that evaluate the risk of developing a life-threatening complication such as herniation and incarceration of the myocardium. We report on a 22-year-old male who presented with sudden onset of sharp chest pain and dyspnea. Computed tomography and cardiac magnetic resonance scan revealed a pericardial defect adjacent to the lateral free wall of the left atrium with associated herniation of the left atrial appendage. The patient was managed conservatively and had an uneventful clinical progress.

Surgical Treatment of Partial Agenesis of the Left Pericardium

“…Among the two, the RM has a better definition of soft tissue (2). Possible changes are characterized by the prominence of the left pulmonary artery, interposition of the lungs in the aorto-pulmonary window and the presence of sternoclavicular pericardial ligament (1,2). In the case that reports the diagnosis was evidenced by MRI.…”

“…Both techniques are able to confirm the diagnosis, assess the extent of injury and the presence of complications, being fundamental to plan therapy. Among the two, the RM has a better definition of soft tissue (2). Possible changes are characterized by the prominence of the left pulmonary artery, interposition of the lungs in the aorto-pulmonary window and the presence of sternoclavicular pericardial ligament (1,2).…”

“…Among patients who have symptoms, the most common are asthenia, malaise, nonspecific chest pain, dyspnea, syncope and dizziness. In addition, partial forms at risk of herniation of the left atrial appendage and/or ventricle may cause entrapment and death (2,4).…”

“…With the evolution of imaging, the incidence tends to increase, due to the greater improvement of these methods in confirming the diagnosis. Magnetic resonance imaging (MRI) is the imaging method with improved accuracy, confirming the diagnosis in most cases (2). This anomaly is three times more prevalent in men, being agenesis of the left pericardium the most common presentation.…”

Complete Absence of The Left Pericardium in On-Pump Coronary Artery Bypass Operation

“…The CALP is infrequent. (1) The number of cases reported in the literature is less than 500 up to now (2). It is three times more common in men compared to women.…”