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Number 17-10 The ventral cardiac ‘flop’–A CMR sign in congenital absence of the pericardium
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The ventral cardiac ‘flop’–A CMR sign in congenital absence of the pericardium

 

Gabriella Captur, MD PhD, MRCP, MSc*†; Chinwe Obianyo, MD*†; Rebecca Hughes MD MRCP; James Sneddon, MD*†; James C Moon, MBBS, MRCP*†

 

*Institute of Cardiovascular Science, University College London, London, UK

CMR Unit, The Barts Heart Centre, St Bartholomew’s Hospital, London, UK

 

Clinical History

A 64-year old lady presented with chest pain. She was referred for a coronary angiography with ventriculography, which showed right coronary artery stenosis but also suggested an unusual cardiac orientation. 

 

CMR Findings

Cardiovascular magnetic resonance (CMR) subsequently revealed congenital partial left sided absence of the pericardium. 

 

 

In the supine position (A, axial breath-held T1-weighted turbo-spin echo) there is levo-positioning of the heart and posterior left ventricular displacement with the apex pointing towards the posterior axillary line (white asterix). We rescanned the patient in the prone position and demonstrated a novel, potentially useful CMR sign in suspected cases of congenital absence of the pericardium: the heart ‘flops’ ventrally (B, black asterix). Electrocardiography (ECG) in the supine position (C) showed right axis deviation, poor R wave progression and leftward displacement of the transition zone (arrowheads) in the precordial leads (the normal transition zone is generally between V3 and V4). In the prone position the heart’s ventral ‘flop’ normalizes the transition zone across the precordial ECG leads (D, arrowheads). 

 

Conclusion

Here we present a quick and simple CMR sign–the ventral cardiac ‘flop’–that may be useful in confirming a suspected case of congenital absence of the pericardium at the time of CMR scanning.

 

Perspective

Congenital absence of the pericardium(1) is a rare cardiac condition, most often asymptomatic, thought to arise from premature atrophy of the common cardiac veins supplying the pleuropericardial membranes(2, 3) resulting in partial or complete, pericardial agenesis. It may also be associated with other congenital heart defects (this patient had none).

 

 

Click here to view the entire case on CloudCMR

Case was prepared by Associate Editor: Dr Sylvia Chen

References

1. Kim H-J, Cho Y-S, Cho G-Y, Choi S Il. Congenital absence of the pericardium. J. Cardiovasc. Ultrasound 2014;22:36–39.

2. Verde F, Johnson PT, Jha S, Fishman EK, Zimmerman SL. Congenital absence of the pericardium and its mimics. J. Cardiovasc. Comput. Tomogr. 2013;7:11–7.

3. Broadbent JC, Callahan J a, Kincaid OW, Ellis FH. Congenital deficiency of the pericardium. Dis. Chest 1966;50:237–44.

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