|Year : 2018 | Volume
| Issue : 3 | Page : 189-190
Pericardial agenesis as a rather unusual cause of palpitations: We only see what we know
Chrysanthos Grigoratos1, Giancarlo Todiere2, Giovanni Donato Aquaro2, Andrea Barison1
1 Fondazione G. Monasterio CNR-Regione Toscana; Institute of Life Sciences, Scuola Superiore Sant'Anna, Pisa, Italy
2 Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy
|Date of Web Publication||26-Sep-2018|
Via Moruzzi 1, Pisa, 56124
Source of Support: None, Conflict of Interest: None
Cardiac palpitations secondary to ventricular ectopic beats are a frequent clinical indication for a cardiac magnetic resonance (CMR) scan. CMR has already demonstrated its additive diagnostic value in patients with frequent arrhythmias even when echocardiogram appears normal. Hereby, we describe a case of a middle-aged male patient referred to our laboratory because of frequent ventricular ectopic beats and an inconclusive echocardiogram due to an extremely poor acoustic window. A diagnosis of pericardial agenesis (PA) was made explaining patient symptoms and arrhythmias previously detected. Furthermore, at the case report description, PA prevalence, associated cardiac pathologies, and novel CMR diagnostic criteria are being described.
Keywords: Cardiac magnetic resonance, palpitations, pericardial agenesis, ventricular ectopic beats
|How to cite this article:|
Grigoratos C, Todiere G, Aquaro GD, Barison A. Pericardial agenesis as a rather unusual cause of palpitations: We only see what we know. J Cardiovasc Echography 2018;28:189-90
|How to cite this URL:|
Grigoratos C, Todiere G, Aquaro GD, Barison A. Pericardial agenesis as a rather unusual cause of palpitations: We only see what we know. J Cardiovasc Echography [serial online] 2018 [cited 2022 Jan 16];28:189-90. Available from: https://www.jcecho.org/text.asp?2018/28/3/189/242247
| Introduction|| |
Cardiac palpitations secondary to ventricular ectopic beats are frequently a clinical indication for a cardiac magnetic resonance (CMR) scan. CMR has already demonstrated its additive diagnostic value in patients with frequent arrhythmias even when echocardiogram appears normal. Normal CMR query in these patients is either arrhythmogenic right ventricular cardiomyopathy or an ischemic or nonischemic left ventricular cardiomyopathy.
| Case Report|| |
A 66-year-old male patient, symptomatic for palpitation, underwent a 24-h electrocardiography Holter monitoring, showing frequent ventricular ectopic beats. Transthoracic echocardiography was deemed incomplete due to a very poor acoustic window and was therefore referred for a CMR scan.
Scout images [Figure 1]a immediately showed a levorotation of the heart, raising the suspicion of pericardial agenesis (PA). Axial and short-axis cine stack [Figure 1]b and [Figure 1]c and fast spin echo inversion recovery images [Figure 1]d and [Figure 1]e confirmed the suspicion revealing a partial absence of pericardial layers in the left lateral wall. No evidence of cardiac herniation was seen. CMR protocol was then modified to assess the presence of associated congenital abnormalities described in scientific literature. Aortic valve was tricuspid, and no evidence of atrial or ventricular septal defect or patent ductus arteriosus was seen with normal Qp/Qs, normal biventricular dimensions and systolic function and no myocardial fibrosis [Figure 1]f. The extreme rarity of PA accounts for the absence of studies and clear diagnostic criteria. The biggest population of PA patients studied with CMR (9 patients) by Macaione et al. demonstrated the diagnostic value of whole-heart volume change (WHVC), expressed as the systodiastolic change of volume measured in axial cines, showing that a >13% threshold has an absolute diagnostic accuracy with sensitivity and specificity of 100%. WHVC of our patient was 25%, additional evidence of PA diagnosis.
|Figure 1: Scout images (a) showing cardiac levorotation; axial (b) and short-axis (c) cine stack and fast spin echo inversion recovery images (d and e) confirming suspicion and revealing a partial absence of pericardial layers in the left lateral wall without evidence of cardiac herniation; T1-weighted gradient echo late gadolinium enhancement sequences (f) showing the absence of myocardial fibrosis|
Click here to view
PA is an extremely uncommon congenital cardiac disease with a prevalence of <1:10000. It can be complete or partial, and it may be associated with other congenital abnormalities such as atrial septum defect, patent ductus arteriosus, or bicuspid aortic valve. CMR represents the noninvasive diagnostic gold standard of this unusual pathology albeit a high level of suspicion is necessary to avoid underdiagnosis.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Macaione F, Barison A, Pescetelli I, Pali F, Pizzino F, Terrizzi A, et al.
Quantitative criteria for the diagnosis of the congenital absence of pericardium by cardiac magnetic resonance. Eur J Radiol 2016;85:616-24.
Nasser WK. Congenital diseases of the pericardium. Cardiovasc Clin 1976;7:271-86.
Peebles CR, Shambrook JS, Harden SP. Pericardial disease – Anatomy and function. Br J Radiol 2011;84:S324-37.