Congenital absence of the pericardium: case presentation and review of literature

Abbas, Amr E.; Appleton, Christopher P.; Liu, Patrick T.; Sweeney, John P.

doi:10.1016/j.ijcard.2003.10.021

Cited by 106 publications

(73 citation statements)

References 15 publications

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“…[1][2][3] Although CMR is useful in determination of anatomic abnormalities, the lack of visibility of the pericardium on CMR images does not prove its congenital absence and may lead to an erroneous diagnosis of pericardial absence in up to 10% of patients. 4 In the present case, the features of left ventricular indentation, cardiac displacement, and lung interposition between heart and diaphragm rather than direct visualization of the pericardium suggested the diagnosis, which was confirmed by surgery. In patients with absence of the pericardium, ECG often shows incomplete right bundle-branch block with poor R-wave progression as a result of the leftward displacement of the heart, 4,5 as seen in the present case.…”

supporting

confidence: 50%

Partial Congenital Absence of the Pericardium

Scheuermann-Freestone

Orchard

Francis

et al. 2007

Circulation

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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...

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supporting

confidence: 50%

Partial Congenital Absence of the Pericardium

Scheuermann-Freestone

Orchard

Francis

et al. 2007

Circulation

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“…Imaging is often necessary to confirm the presence, severity, and extent of fluid; to characterize the nature of fluid (transudates vs exudates); to rule out pericardial inflammation; to determine the hemodynamic effect on the heart; and ultimately to guide pericardiocentesis. Although transthoracic echocardiography is the preferred first-line modality for this evaluation, CT and MR imaging often allow for a more comprehensive location of the defect (Fig 5) (67,68). Since herniation is often intermittent in time, positional changes to the left lateral decubitus position, for example, can be helpful in diagnosing pericardial defects.…”

Section: Review: Ct and Mr Imaging Of Pericardial Diseasementioning

confidence: 99%

Pericardial Disease: Value of CT and MR Imaging

Bogaert

Francone²

2013

Radiology

189

151

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“…Almost always, neonatal and infantile patients are being diagnosed at the time of surgery for other congenital anomalies usually a congenital diaphragmatic hernia [2]. Reported presenting features of complete pericardial absence in adults include atypical chest pain of varying severity, palpitations, shortness of breath or incidental discovery at the time of cardiothoracic surgeries [4]. Due to vague and atypical symptoms, congenital absence of pericardium can mimic other conditions such as acute coronary syndromes, valvular heart disease like mitral prolapse, pulmonic stenosis, tumours of the lung or heart.…”

Section: Discussionmentioning

confidence: 99%

“…Defects can either be partial or complete. The most common type is a left sided pericardial absence which accounts for 2/3 of all congenital pericardial defects [4]. Complete absence of the entire pericardium (left and right sided) is consi-dered very rare and accounts for only a fraction of the cases reported.…”

Section: Introductionmentioning

confidence: 99%

Congenital complete absence of pericardium presenting with chest pain: MRI characteristics

Nellaiyappan

Demo²,

Omar³

et al. 2016

Int Arch Med

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Congenital absence of the pericardium is a very rare anomaly which has an incidence of 1 in 14000 cases in autopsy studies. Defects in the pericardium are either partial or complete with complete defects having a much benign course than partial defects. Partial defects are associated with complications like strangulation of a herniated cardiac chamber or coronary artery stenosis from external compression. Absence of the left pericardium is the most frequently encountered type of defect which accounts for 2/3 of the cases. We herein report a case of complete absence of the entire pericardium which is an extremely rare subtype with associated Cardiac magnetic resonance images.

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Congenital absence of the pericardium: case presentation and review of literature

Cited by 106 publications

References 15 publications

Partial Congenital Absence of the Pericardium

Partial Congenital Absence of the Pericardium

Pericardial Disease: Value of CT and MR Imaging

Congenital complete absence of pericardium presenting with chest pain: MRI characteristics

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