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|Number 17-11: The Ventral Cardiac ‘Flop’–A CMR Sign in Congenital Absence of the Pericardium|
NUMBER 17-11: THE VENTRAL CARDIAC 'FLOP' - A CMR SIGN IN CONGENITAL ABSENCE OF THE PERICARDIUM
Gabriella Captur, MD PhD, MRCP, MSc†; Chinwe Obianyo MD*, Rebecca Hughes*†, Charlotte Manisty*†, 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
Movie 1 (4 chamber supine) and Movie 2 (4 chamber prone)
Movie 3 (2 chamber) and Movie 4 (3 chamber)
Movies 4 and 5: basal mid short axis
In the supine position (Figure A, axial breath-held T1-weighted turbo-spin echo) there was levo-positioning of the heart and posterior left ventricular (LV) displacement with the apex pointing towards the posterior axillary line (white asterix) and rightward displacement of the descending thoracic aorta. 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 (Figure B, black asterix) highlighting the great mobility of the heart with a pericardial defect. Electrocardiography (ECG) in this supine position (Figure 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 (Figure D, arrowheads)
Other CMR signs convinced us that this was a case of partial as opposed to complete left sided absence of the pericardium. Firstly, axial T1-weighted imaging in the supine (Figure E) and prone positions (Figure F), showed the presence of the pericardium overlying the right ventricle (black arrows point to a dark stripe sandwiched between bright epicardial and pericardial fat). No pericardium was visible overlying the LV in the supine position but in the prone position, a pericardial stripe could be seen over the LV apex and terminating posteriorly (red arrowheads) thus suggesting partial as opposed to complete absence. A thin rim of epicardial fat was visible around the LV in both views (white arrows).
On LV short axis cines (Figures G and H), the pericardium was visible only overlying anterior and inferior portions of the LV, and was absent over the lateral wall, indicating a congenital partial left absence of pericardium(1).
CMR also demonstrated the absence of anterior and posterior superior pericardial recesses around the ascending aorta at the level of the pulmonary artery bifurcation (Figure I, white arrows). Lung tissue was insinuated between the aorta and pulmonary artery, indicating the absence of pericardium at this level (Figure J). Left and right ventricular function were within normal limits, as were cavity sizes. There was no inducible ischaemia during adenosine stress perfusion and no previous infarction or scarring on late gadolinium enhancement imaging. No coronary artery anomalies or defects of the atrial or ventricular septa were noted.
The patient was commenced on aspirin and statin for primary prevention given the moderate coronary artery disease identified. No specific management was required for the congenital partial absence of pericardium.
Eliciting this additional CMR sign (the ventral cardiac ‘flop’) by prone position scanning, may be useful in corroborating diagnostic certainty of congenital absence of the pericardium at the time of CMR scanning. This may help technicians scanning the patient, and doctors on site supervising the scan, more robustly identify cases of absent pericardium, allowing them to perform targeted acquisitions before the patient leaves the department. Prone position scanning is useful but not mandatory for diagnosing absent pericardium. Not mandatory, in so far as the diagnosis can often be made on the basis of the other imaging signs present.
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Case was prepared by Associate Editor: Dr. Sylvia Chen
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