Home About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Subscribe Contacts Login 


 
 Table of Contents  
CASE REPORT
Year : 2018  |  Volume : 28  |  Issue : 3  |  Page : 189-190

Pericardial agenesis as a rather unusual cause of palpitations: We only see what we know


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 Publication26-Sep-2018

Correspondence Address:
Chrysanthos Grigoratos
Via Moruzzi 1, Pisa, 56124
Italy
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jcecho.jcecho_15_18

Rights and Permissions
  Abstract 


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 2021 Apr 21];28:189-90. Available from: https://www.jcecho.org/text.asp?2018/28/3/189/242247




  Introduction Top


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 Top


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.[1] 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.[2] 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.[3] 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

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
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.  Back to cited text no. 1
    
2.
Nasser WK. Congenital diseases of the pericardium. Cardiovasc Clin 1976;7:271-86.  Back to cited text no. 2
    
3.
Peebles CR, Shambrook JS, Harden SP. Pericardial disease – Anatomy and function. Br J Radiol 2011;84:S324-37.  Back to cited text no. 3
    


    Figures

  [Figure 1]



 

Top
 
 
  Search
 
Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

 
  In this article
Abstract
Introduction
Case Report
References
Article Figures

 Article Access Statistics
    Viewed1242    
    Printed60    
    Emailed0    
    PDF Downloaded79    
    Comments [Add]    

Recommend this journal


[TAG2]
[TAG3]
[TAG4]