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Congenital absence of the pericardium

A 71-year-old male presented with atypical chest pain and dilatation of the right ventricle on echocardiography and was referred for cardiac magnetic resonance imaging (CMR) for suspected arrhythmogenic right ventricular cardiomyopathy. CMR showed marked posterior and leftward displacement of the heart, with the apex extending to the left lateral chest wall. Spin echo imaging showed thin pericardium around the atria and great vessels, but no pericardium was detected around the ventricles (Fig. 1). Lung tissue was interpositioned between the heart and the left hemidiaphragm (Fig. 2). Both ventricles had normal morphology and function.

Fig. 1
figure1

Spin echo study showing pericardium around the right atrium (black arrow), but lack of pericardium around the right ventricle (white arrow)

Fig. 2
figure2

Still frames from steady state free precession cine loop showing lung tissue interposition between the inferior myocardial wall and the diaphragm (white arrow). Original cine loops published online (Movie 1)

Patients with congenitally absent pericardium may be asymptomatic or may present with chest pain or dyspnoea [1]. Electrocardiography may show right bundle branch block or clockwise rotation. Chest X-ray may show the left lateral position of the heart and the abnormal interposition of lung tissue between the aorta and pulmonary artery or between the inferior myocardial wall and the left hemidiaphragm. On echocardiography, the right ventricle may appear enlarged, as the beam traverses the ventricle closer to the basis of the heart and tangentially [2].

The diagnosis can be confirmed by computed tomography or CMR by showing (partial) absence of the pericardium. CMR may show excessive cardiac mobility. In incomplete defects, a rim of residual pericardium may cause a left ventricular crease, with (fatal) herniation as a rare potential risk, for which preventive closure has been suggested. Complete forms only need intervention in case of symptoms [3, 4].

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Written informed consent was obtained from the patient.

References

  1. 1.

    Gatzoulis MA, Munk MD, Merchant N, et al. Isolated congenital absence of the pericardium: clinical presentation, diagnosis and management. Ann Thorac Surg. 2000;69:1209–15.

  2. 2.

    Payvandi MN, Kerber RE. Echocardiography in congenital and acquired absence of the pericardium. Circ. 1976;53:86–92.

  3. 3.

    Gassner I, Judmaier W, Fink C, et al. Diagnosis of congenital pericardial defects, including a pathognomic sign for dangerous apical ventricular herniation, on magnetic resonance imaging. Br Heart J. 1995;74:60–6.

  4. 4.

    Scheuermann-Freestone M, Orchard E, Francis J, et al. Partial congenital absence of the pericardium. Circulation. 2007;116:e126–9.

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This article is distributed under the terms of the Creative Commons Attribution Noncommercial License which permits any noncommercial use, distribution, and reproduction in any medium, provided the original author(s) and source are credited.

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Correspondence to J. Kodde.

Electronic supplementary material

Below is the link to the electronic supplementary material.

Steady state free precession short axis cine loop showing lung tissue interposition between the inferior myocardial border and the diaphragm. This movie also shows increased cardiac mobility. (AVI 1044 kb)

Steady state free precession short axis cine loop of a healthy person, showing the normal position of the heart, directly lying on the left hemidiaphragm. (AVI 1168 kb)

Movie 1

Steady state free precession short axis cine loop showing lung tissue interposition between the inferior myocardial border and the diaphragm. This movie also shows increased cardiac mobility. (AVI 1044 kb)

Movie 2

Steady state free precession short axis cine loop of a healthy person, showing the normal position of the heart, directly lying on the left hemidiaphragm. (AVI 1168 kb)

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Open Access This is an open access article distributed under the terms of the Creative Commons Attribution Noncommercial License (https://creativecommons.org/licenses/by-nc/2.0), which permits any noncommercial use, distribution, and reproduction in any medium, provided the original author(s) and source are credited.

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Kodde, J., Groothuis, J.G.J., Beek, A.M. et al. Congenital absence of the pericardium. Neth Heart J 19, 486–487 (2011) doi:10.1007/s12471-011-0111-7

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Keywords

  • Chest Pain
  • Lung Tissue
  • Cardiac Magnetic Resonance
  • Cardiac Magnetic Resonance Imaging
  • Clockwise Rotation