Magnetic Resonance Imaging Differentiated Partial From Complete Absence of the Left Pericardium in a Case of Leftward Displacement of the Heart

Abstract

solated congenital absence of the pericardium is a rare malformation and can vary from partial to complete absence, occurring more often on the left than the right side. 1 The features of complete absence of the left pericardium on chest X-ray and echocardiography, which are mainly related to the displacement of the whole heart, have been described by many investigators. [2][3][4][5][6][7][8] In partial absence of the pericardium, the characteristic features are quite different from those of complete absence of the pericardium because partial absence is occasionally complicated with herniation of a cardiac structure. [9][10][11] We present a case of partial absence of the left pericardium that was diagnosed by delineating the pericardium with magnetic resonance imaging (MRI), although the chest X-ray and echocardiographic features indicated a complete absence. Case ReportA 44-year-old man was referred for the evaluation of atypical chest pain. The pain eased spontaneously after several minutes, but was triggered in the left lateral position and he felt no distress. His apical impulse was prominent and displaced leftward to the anterior axillary line in the fifth intercostal space and a grade I/VI systolic murmur was present in the pulmonary valve area. Electrocardiography revealed right-axis deviation (QRS axis: 106°) and Circulation Journal Vol.68, April 2004incomplete right-bundle branch block. The chest X-ray in the standing posteroanterior view demonstrated leftward displacement of the heart without tracheal deviation. The chest X-rays in the right and the left lateral positions showed unusual cardiac mobility in the leftward direction (Fig 1). In transthoracic echocardiography, the apical window was displaced laterally in the usual left lateral position. Each echocardiogram recorded at expiration is shown (Fig 2). The 2-dimensional echocardiogram in the Magnetic Resonance Imaging Differentiated Partial From Complete Absence of the Left Pericardium in a Case of Leftward Displacement of the HeartTetsuhiro Yamano, MD; Takahisa Sawada, MD; Kenzo Sakamoto, MD; Takeshi Nakamura, MD; Akihiro Azuma, MD; Masao Nakagawa, MDA 44-year-old man was referred to hospital for the evaluation of atypical chest pain. His chest X-ray showed leftward displacement of the heart. During echocardiography, the apical window displaced laterally in the usual left lateral position and characteristic motions of the interventricular septum and left ventricular posterior wall were recognized with postural alterations. We presumed a complete absence of the left pericardium. Magnetic resonance imaging (MRI), however, demonstrated a partial left-sided pericardium. The diagnosis was corrected to partial absence of the left pericardium and we have carefully followed up this case without surgical prophylactic intervention. It is very important to differentiate partial from complete absence of the pericardium, because only in patients with partial absence of the pericardium is there a risk of fatal myocardial strangulation. The features of the c...

Paroxysmal atrial fibrillation due to left atrial appendage herniation and review of the literature

“…Isolated congenital absence of the pericardium includes a range of congenital pericardial defects from a small foramen in the pericardium to a complete absence of the entire pericardium and is an unexpected finding at surgery or postmortem. With the widespread use of advanced imaging, nonoperative diagnosis may redefine the prevalence of this condition [5]. Herniation of the heart through the partial defect can be appreciated, forming an indentation on the heart or a myocardial crease on MRI [6].…”

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“…On chest MRI taken after surgery, the layer of pericardium which is reported to be depicted as a dark line adjacent to the myocardium [2][3][4][5], was not apparent on either side of the heart (Fig. 3).…”

Multimodality Imaging of Pericardial Diseases

“…In surgical and pathologic series, the reported prevalence is approximately 0.002% to 0.004% (52). The defects, classified as partial or complete, are most commonly located on the left side of the heart but can be located anywhere in the pericardium.…”