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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 6  |  Issue : 2  |  Page : 92-95

An unusual case of dense mitral annular calcification in a young with plethora of mitral annular premature ventricular complexes


Department of Cardiology, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India

Date of Submission02-Sep-2021
Date of Acceptance08-Feb-2022
Date of Web Publication16-May-2022

Correspondence Address:
Dr. Debasish Das
Department of Cardiology, All India Institute of Medical Sciences, Bhubaneswar - 751 019, Odisha
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/hm.hm_57_21

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  Abstract 


We report a rare case of dense mitral annular calcification in a 26-year-old male presenting with recurrent palpitation for the last year with a plethora of mitral annular premature ventricular complexes (PVCs). PVCs had right bundle branch block morphology, were notched in inferior leads with negative QRS complexes in aVL suggestive of likely origin from the lateral mitral annulus. Patients with PVCs from mitral annulus are usually advised to undergo cardiac magnetic resonance imaging to localize the site of subtle mitral annular calcification and plan subsequent delivery of radiofrequency lesions at the same site during radiofrequency ablation. Dense calcification of the lateral mitral annulus is extremely rare to encounter in such a young age of below 30 years without the presence of any predisposing risk factors for calcification. Our case is the first illustration of dense mitral annular calcification secondary to the plethora of mitral annular PVCs in a symptomatic young person below the age of 30 years. PVCs-induced excessive local excursion with wear and tear phenomenon and secondary dystrophic calcification may be the plausible explanation behind this interesting presentation.

Keywords: Mitral annular calcification, premature ventricular complexes, palpitation


How to cite this article:
Das D, Das T, Pramanik S. An unusual case of dense mitral annular calcification in a young with plethora of mitral annular premature ventricular complexes. Heart Mind 2022;6:92-5

How to cite this URL:
Das D, Das T, Pramanik S. An unusual case of dense mitral annular calcification in a young with plethora of mitral annular premature ventricular complexes. Heart Mind [serial online] 2022 [cited 2022 Jul 1];6:92-5. Available from: http://www.heartmindjournal.org/text.asp?2022/6/2/92/345286




  Introduction Top


Degenerative mitral annular calcification is a well-known phenomenon in the elderly but idiopathic calcification of mitral annulus at a very young age without the presence of concomitant risk factors such as chronic kidney disease, rheumatic heart disease, hypertension, or aortic stenosis is not well-described in the literature. Although premature ventricular complexes (PVCs) are a common cause of palpitation in young persons, mitral annular PVCs are extremely rare to encounter in routine clinical practice. PVC originating from the mitral annulus constitutes the least prevalent type of PVCs causing palpitation in the young, while PVCs arising from the right ventricular outflow tract constitute the most common category. Our case is the first illustration of dense mitral annular calcification in a young person with plethora of mitral annular PVCs.


  Case Report Top


A 26-year-old male presented with frequent palpitation for the past 2 years without any history of presyncope, syncope, exertional angina, or shortness of breath in the past. He was nondiabetic, nonhypertensive, nonsmoker, and euthyroid with a normal lipid profile. All the serum chemistries were within the normal limit. Electrocardiogram (EKG) revealed mitral annular PVC with the right bundle branch block (RBBB) morphology suggestive of the left ventricle (LV) origin, notched late QRS complexes in inferior leads suggestive of mitral annular origin, with negative QRS in a VL suggestive of lateral mitral annulus origin [Figure 1]. EKG wise PVCs were precisely from the lateral mitral annulus. Interestingly, echocardiography showed no regional wall motion abnormality with normal left ventricular ejection fraction with the presence of dense calcification in lateral mitral annulus [Figure 2] and [Figure 3]. Although mitral annular PVCs require cardiac magnetic resonance imaging (MRI) evaluation to look for subtle mitral annular calcification routinely before radiofrequency ablation for precise location of ablation focus, we subjected the patient for routine echocardiography which revealed dense calcification of the lateral mitral annulus which was the site of origin mitral annular PVCs. Our case is the first illustration of dense mitral annular calcification in a case of recurrent mitral annular PVCs. We did a Holter analysis to look for PVC burden which came out to be 11.5%; because of PVC burden <15%, we decided to manage the patient with metoprolol 50 mg twice daily with review Holter analysis after 3 months. He did not have PVC-induced left ventricular systolic dysfunction mandating urgent radiofrequency ablation. In the event of refractory PVC with a PVC burden of more than 15% in the future, we would like to do transseptal three-dimensional (3D) ablation of the same.
Figure 1: Mitral Annular premature ventricular complexes with notched QRS in inferior leads and negative aVL

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Figure 2: Mitral annular calcification

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Figure 3: Mitral annular calcification

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  Discussion Top


Most PVCs or idiopathic ventricular tachycardia have a right ventricular outflow tract or the left ventricular inferoseptal origin.[1],[2] Some PVCs originate from the left ventricular outflow tract[3],[4] or a left ventricular epicardial site.[5],[6],[7] Small number of PVCs have been reported to originate from the anterolateral portion of mitral annulus close to aortomitral continuity.[8],[9],[10]

Mitral annular PVCs show a characteristic notching pattern in the late phase of QRS complex in inferior leads. Septal or Posteroseptal PVCs by the rule have an early transition in V2 as demonstrated in [Figure 4]. PVC from aortomitral continuity in this regard constitutes a different entity and has two characteristic features; the presence of qR complexes in V1 and pattern break pattern in precordial leads which means QRS complex will be positive in V1, will become negative in V2, and again will be positive in V2. In the above electrocardiogram (ECG) [Figure 1] of our patient, ECG has RBBB morphology suggestive of LV origin, notched QRS complexes in inferior leads are characteristics of mitral annulus origin, and negative avL is finally suggestive of origin from the lateral mitral annulus. Interestingly, echocardiography demonstrated also the presence of dense calcification in the lateral mitral annulus. The mechanism of ectopic causing focal calcification is not well known, but one part of the heart, if vigorously contracts for a long time secondary to premature complexes undergoes local wear and tear phenomenon with development of subsequent dystrophic calcification as also noted at the site of insertion of accessory pathways where vigorous contraction occurs secondary to electromechanical stimulation of myofibers by accessory fibers. Our case has typical lateral mitral annular PVCs with evidence of dense lateral mitral annular calcification in routine echocardiography in a young male with a normal renal profile with normal serum calcium and phosphorus with no echocardiographic evidence of rheumatic heart disease in the form of valve thickening or aortic stenosis. We also ruled out hyperparathyroidism in the aforesaid patient. Dystrophic calcification occurs in myocardial scar postmyocardial infarction which is well delineated in cardiac MRI and the point of radiofrequency ablation is precisely localized from the site of the scar by cardiac MRI before dense 3D mapping during radiofrequency ablation. Mitral annular calcification is usually observed as a chronic degenerative process at the base of the mitral valve. Calcification occurs on the fibrous support of mitral valve. It is visualized as an echo dense structure lumpy in appearance with involving the mitral annulus. Mitral annulus calcification (MAC) is commonly observed as a chronic, degenerative process of the base of the mitral valve. MAC is a chronic, degenerative process of the fibrous support of the mitral valve. MAC is usually visualized on echocardiography as an echo-dense structure with a lumpy appearance involving the mitral valve annulus, with associated acoustic shadowing. In cardiovascular health study, mitral annular calcification was quantified as mild (focal in nature), moderate (marked echo density one third to one half of the ring circumference), or severe (echo density involving more than half of the ring or into left ventricular inflow tract). Spiral computed tomography and electron-beam computed tomography better quantifies, predicts the location, and extension of mitral annular calcification. Factors behind mitral annular calcification includes age-related degenerative process of calcification, female sex, associated atherosclerosis, increased stress on the mitral valve in aortic stenosis, hypertension and hypertrophic cardiomyopathy, abnormal calcium phosphorus metabolism in chronic kidney disease, and metabolic disorders such as Marfan syndrome and Hurler syndrome. Many often – mitral annular calcification is an incidental finding in patients undergoing echocardiographic evaluation in cardiovascular or pulmonary disease. Mitral annular calcification is associated with an increased risk of cardiovascular disease, death, and all-cause death. There is inconsistent association of mitral annular calcification with risk of stroke. It can present as mitral stenosis and exacerbates preexisting mitral regurgitation. The presence of mitral annular calcification is associated with a higher prevalence of atrioventricular block, bundle branch block, intraventricular conduction delay, and atrial fibrillation. Severe mitral annular calcification complicates mitral valve replacement. Patients with mitral annular calcification carries a high risk of cardiac rupture at the atrioventricular junction or left ventricular free wall, injury to the left circumflex artery during debridement with a higher risk of paraprosthetic mitral regurgitation with less debridement. Severe mitral annular calcification is considered a contraindication for mitral clip placement for severe mitral regurgitation. Severe MAC has been considered a contraindication for percutaneous mitral valve intervention with the MitraClip. Mitral annular calcification serves as an independent predictor for the need of permanent pacemaker implantation following transcatheter aortic valve replacement.[11] Many associations of mitral annular calcification with cardiovascular disease have been described as enumerated above but our case is the first illustration of dense mitral annular calcification in a symptomatic patient with a plethora of mitral annular PVCs. Local wear and tear-induced dystrophic calcification may be the plausible explanation behind the paradoxical presence of dense mitral annular calcification in a young with a plethora of annular PVCs.
Figure 4: Mitral Annular premature ventricular complexes from the anterior, posterior, and posteroseptal site showing notch in the late phase of QRS complexes

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  Conclusion Top


Our case is the first description of dense mitral annular calcification in a symptomatic young patient with a plethora of mitral annular PVCs. PVCs-induced excessive local excursion with wear and tear and secondary dystrophic calcification may be the plausible explanation behind the paradoxical presence of dense mitral annular calcification in a young with a plethora of annular PVCs.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal his identity, but anonymity cannot be guaranteed.

Declaration of ethical approval

The Institutional Ethical Committee approval has been obtained.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Sirichand S, Killu AM, Padmanabhan D, Hodge DO, Chamberlain AM, Brady PA, et al. Incidence of idiopathic ventricular arrhythmias: A population-based study. Circ Arrhythm Electrophysiol 2017;10:e004662.  Back to cited text no. 1
    
2.
Anderson RD, Kumar S, Parameswaran R, Wong G, Voskoboinik A, Sugumar H, et al. Differentiating right and left sided out flow tract ventricular arrythmias. Circ Arrythm Electrophysiol 2019;12: e007392.  Back to cited text no. 2
    
3.
Gouda S, Wichterle D, Peichl P, Kautzner J. Idiopathic left ventricular outflow tract ectopy: A single focus with extremely divergent breakouts. BMC Cardiovasc Disord 2014;14:161.  Back to cited text no. 3
    
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Yamada T. Idiopathic ventricular arrhythmias: Relevance to the anatomy, diagnosis and treatment. J Cardiol 2016;68:463-71.1  Back to cited text no. 4
    
5.
Scanavacca M, Lara S, Hardy C, Pisani CF. How to identify & treat epicardial origin of outflow tract tachycardias. J Atr Fibrillation 2015;7:1195.  Back to cited text no. 5
    
6.
Mar PL, Barmeda M, Stucky MA, Devabahktuni SR, Garlie J, Miller JM, et al. Unique features of epicardial ventricular arrhythmias/premature ventricular complexes ablated from coronary venous system in veteran population. Indian Pacing Electrophysiol J 2020;20:97-104.  Back to cited text no. 6
    
7.
Carrigan TP, Patel S, Yokokawa M, Schmidlin E, Swanson S, Morady F, et al. Anatomic relationships between the coronary venous system, surrounding structures, and the site of origin of epicardial ventricular arrhythmias. J Cardiovasc Electrophysiol 2014;25:1336-42.  Back to cited text no. 7
    
8.
Santos H, Valente B, Cunha P, Portugal G, Ferreira R, Oliveira M. The aorto mitral continuity challenge. Ann Clin Case Rep 2020;5:1-5.  Back to cited text no. 8
    
9.
Wissner E, Saguner AM, Metzner A, Chmelesky M, Tsyganov A, Deiss S, et al. Radiofrequency ablation of premature ventricular contractions originating from the aortomitral continuity localized by use of a novel noninvasive epicardial and endocardial electrophysiology system. HeartRhythm Case Rep 2016;2:255-7.  Back to cited text no. 9
    
10.
Dar T, Yarlagadda B, Parikh V, Reddy YM, Sheldon SH. Intramural anterolateral mitral annular idiopathic ventricular tachycardia successfully ablated from the atrium. HeartRhythm Case Rep 2019;5:384-7.  Back to cited text no. 10
    
11.
Abramowitz Y, Jilaihawi H, Chakravarty T, Mack MJ, Makkar RR. Mitral annulus calcification. J Am Coll Cardiol 2015;66:1934-41.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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