Partial Congenital Absence of the Pericardium

Abstract

A 45-year-old male was admitted to the hospital with recurrent, stabbing, nonexertional chest pain. Chest x-ray showed an increased cardiothoracic ratio ( Figure 1A), and ECG demonstrated sinus rhythm with incomplete right bundle-branch block and poor R-wave progression ( Figure 1B). Echocardiography showed normal left ventricular dimensions and function; however, the right ventricle appeared grossly dilated with moderate to severe tricuspid regurgitation with no evidence of intracardiac shunts. These findings were confirmed by right heart catheterization, which also showed normal pulmonary and right atrial pressures without a pressure gradient over the pulmonic valve. Catheterization of the left heart showed normal ventricular pressures and function as well as normal coronary arteries. The patient was referred for cardiac magnetic resonance imaging (CMR) with suspected right ventricular cardiomyopathy. CMR showed cardiac displacement into the left hemithorax ( Figure 2A). There was severe tricuspid regurgitation with marked dilatation of both the right atrium (6ϫ8 cm) and ventricle (end-diastolic volume, 343 mL). Right ventricular function was mildly reduced (ejection fraction, 46%) without localized wall motion abnormalities. Left ventricular volumes and function were normal; however, there was marked indentation of the mid-lateral wall that resulted in herniation of the apical portion of the left ventricle without associated wall-motion abnormalities ( Figure 2B). In addition, there was an extension of lung tissue between the inferior surface of the heart and the diaphragm as a result of the unusually posterior displacement of the left ventricle ( Figure 3). Late enhancement images were normal, which made a diagnosis of right ventricular cardiomyopathy less likely.Both displacement of the heart and interposition of lung parenchyma between heart and diaphragm are typical features of congenital absence of the pericardium, and herniation of the left ventricular apex suggests partial absence of the pericardium distal to the indentation of the left ventricular wall. Because of the severity of tricuspid regurgitation, the patient underwent tricuspid valve surgery, and at operation the partial absence of the pericardium was confirmed. The pericardial defect was repaired with a Goretex patch, and the tricuspid valve was replaced with a 33-mm biological (Perimount) prosthesis because a large defect in the anterior leaflet rendered it unsuitable for repair. Postoperative CMR ( Figure 4) demonstrated successful restoration of normal left ventricular anatomy and normalization of right ventricular volumes (end-diastolic volume, 176 mL) with only mild tricuspid regurgitation. The patient recovered quickly from surgery and remains well.Lateral and posterior displacement of the heart and interposition of lung parenchyma between heart and diaphragm are typical features of congenital absence of the pericardium. 1-3 Although CMR is useful in determination of anatomic abnormalities, the lack of visibility of the pericardium on...

Pericardial Disease: Value of CT and MR Imaging

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Complete Absence of Precordial R Waves Due to Absence of Left‐Sided Pericardium

“…However, more frequently than a prominent R wave in aVR, PRWP in patients with pericardial defects is associated with the ECG pattern of an incomplete right bundle‐branch block, which is explained by the displacement of the heart in the left hemithorax 19 …”

Ausência parcial congênita do pericárdio

“…8 Thus, in the complete forms, there is no indication for surgical treatment due to absence of risk, and pericardium repair is reserved for symptomatic cases or for those at risk of incarceration and syncope. 9,10 Finally, the importance of a joint evaluation using different cardiovascular imaging methods when perfor ming a systematic assessment of atypical clinical cases is emphasized, so that differential diagnosis can be used to achieve the the best possible therapeutic management.…”