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Table of Contents   
CASE REPORT  
Year : 2022  |  Volume : 15  |  Issue : 1  |  Page : 67-69
Sinoatrial nodal disease presenting with tachy-bradycardia syndrome in a fetus of anti-SSA/SSB-positive mother


Sowmi Fetal and Pediatric Heart Centre, Tirunelveli, Tamilnadu, India, 2Department of Pediatric Cardiology, Amrita Institute of Medical Sciences, Kochi, Kerala, India

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Date of Submission14-Jan-2021
Date of Decision13-May-2021
Date of Acceptance21-May-2021
Date of Web Publication14-Jun-2022
 

   Abstract 


Tachy-bradycardia syndrome (TBS) has been reported rarely in the fetus. We present here an unusual dominant involvement of sinoatrial node in a fetus presenting with TBS of anti-Sjögren'syndrome-related antigen A (SS-A) and antigen B (SS-B) autoantibodies positive mother. Prenatal imaging findings, Doppler hemodynamics, and outcomes are described.

Keywords: Atrial arrhythmia, autoimmune associated tachy-bradycardia syndrome, fetus

How to cite this article:
Karmegaraj B. Sinoatrial nodal disease presenting with tachy-bradycardia syndrome in a fetus of anti-SSA/SSB-positive mother. Ann Pediatr Card 2022;15:67-9

How to cite this URL:
Karmegaraj B. Sinoatrial nodal disease presenting with tachy-bradycardia syndrome in a fetus of anti-SSA/SSB-positive mother. Ann Pediatr Card [serial online] 2022 [cited 2022 Jul 7];15:67-9. Available from: https://www.annalspc.com/text.asp?2022/15/1/67/347423





   Introduction Top


Tachy-bradycardia syndrome (TBS) is defined as an atrial arrhythmia formed by the alternation of various atrial tachycardias with sinus bradycardia or sinoatrial block.[1] It has been reported in fetuses with the missense variant p.(Gly482Arg) in HCN4 gene.[2] Atrial ectopics and junctional ectopic tachycardia were reported in fetuses with autoimmune atrioventricular block.[3] An unusual dominant involvement of sinoatrial node in a fetus presenting with TBS of anti-SSA/SSB-positive mother is presented in this report.


   Case Report Top


A 22-year-old primigravida was referred for fetal echocardiography at 24 weeks' gestation given suspicion of fetal tachyarrhythmia on screening. The four-chamber view showed moderate pericardial effusion, hyperechoic endocardium, papillary muscles, and both atrioventricular valve annuli [Figure 1]a and [Figure 1]b. The heart was otherwise structurally normal. There was ventricular diastolic dysfunction as evident in the left ventricle inflow–outflow Doppler (presystolic flow in ascending aorta) [Figure 2]a and [Figure 2]b] with no atrioventricular valve regurgitation.
Figure 1: (a) Four-chamber view showing hyperechoic endocardium, papillary muscle, and pericardial effusion. (b) Sagittal view showing hyperechoic atrioventricular valves

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Figure 2: (a) Left ventricle inflow–outflow Doppler showing presystolic forward flow into the aorta (yellow arrow). This finding suggests increased stiffness of the left ventricle due to endocardial inflammation resulting in a significant increase in left ventricular pressure during left atrial contraction leading to presystolic forward flow into the aorta. (b) Left ventricle inflow–outflow Doppler showing forward flow into the aorta (yellow arrow) during the isovolumetric contraction time

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Left ventricle inflow–outflow Doppler showed periods of fetal sinus bradycardia [Figure 3] alternating with frequent conducted atrial ectopics triggering episodic atrial tachyarrhythmia. Left brachiocephalic vein-aorta Doppler showed the beginning and termination of the tachyarrhythmia. It was a 1:1 long VA tachyarrhythmia (VA interval 600 ms, AV interval 267 ms) triggered by an atrial ectopic, and spontaneous termination of the tachyarrhythmia followed by an atrial ectopic and bradycardia [Figure 4]. Hence, a diagnosis of TBS was made. There was no evidence of atrioventricular block. Maternal serum SSA and SSB antibody levels were elevated 145 U (normal <20) and 181 U (normal <20), respectively. There was no family history of sinus node dysfunction.
Figure 3: Left ventricle inflow–outflow Doppler showing sinus bradycardia with atrioventricular interval of 183 ms

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Figure 4: Left brachiocephalic vein-aorta Doppler demonstrating the beginning of the tachyarrhythmia triggered by an atrial ectopic (pink arrow) and termination of the tachyarrhythmia followed by an atrial ectopic (blue arrow) and bradycardia. It is a long ventriculoatrial tachyarrhythmia (atrioventricular interval 267 ms; ventriculoatrial interval 600 ms) with 1:1 atrioventricular conduction

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In utero course

Transplacental therapy with oral dexamethasone at a dose of 4 mg/day was given initially for 7 days. Reassessment after 1 week showed absent pericardial effusion and improved ventricular diastolic function [Figure 5]. However, fetal bradycardia, conducted atrial ectopics, and episodic atrial tachycardia persisted. Hence, oral dexamethasone dose was increased to 8 mg/day. Reassessment after a week showed no further improvement.
Figure 5: (a) Four chamber view and (b) Sagittal view of the fetal heart showing disappearance of pericardial effusion after steroid therapy. (c) Left ventricle inflow–outflow Doppler showing disappearance of presystolic forward flow into the aorta suggestive of improved diastolic function and persistence of tachycardia–bradycardia

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   Discussion Top


Fetal cardiac failure due to arrhythmias is associated with high fetal and neonatal mortality and affects the long-term neurodevelopmental outcome.[4] Tachyarrhythmias has been reported in fetuses with complete heart block. The reported tachyarrhythmias in the fetal literature include junctional ectopic tachycardia, atrial flutter, and ventricular tachycardia.[5],[6],[7] To the best of our knowledge, immune-mediated TBS is not reported in fetuses so far. Presystolic flow in ascending aorta has been reported in adults with ventricular diastolic dysfunction. It occurs due to impaired relaxation of the left ventricle resulting in decreased filling of left ventricle in early diastole which is compensated by forceful contraction of the left atrium to complete left ventricle filling.[8] Immune-mediated SA node injury has discordances between the echocardiographic and pathologic findings.[9]

Hemodynamic evaluation using Doppler echocardiography helps in understanding the electrophysiological mechanism and to make an accurate diagnosis of fetal arrhythmias.[10] In this case, Doppler echocardiography helped in understanding both the arrhythmia mechanism and cardiac function. Although the cardiac function stabilized after transplacental therapy with oral steroids, the arrhythmia persistent probably due to immune-mediated fibrosis of the sinoatrial node and the atrium. No pharmacological therapy has yet resulted in permanent reversal of fetal third-degree heart block due to maternal lupus. However, transplacental therapy with fluorinated steroids has some efficacy in treating second-degree heart block and cardiac disease beyond the atrioventricular node and beta-agonists have been used to increase fetal heart rate in utero. No controlled experiments regarding the use of plasmapheresis have been performed, and due to the costly and time-consuming process, it does not play a significant role in its management. Intravenous immunoglobulin has shown promise in the treatment specifically associated with fetal cardiomyopathy/endocardial fibroelastosis.[11] Hydroxychloroquine is a highly beneficial drug in the management of women with autoimmune disease and now has a new role for the prophylaxis of recurrent fetal heart block in which it halved the rate of development. The risk of heart block in a fetus exposed to maternal anti-SSA/Ro antibodies is approximately 2% if the mother has never had an affected child and 18% if she has. Hydroxychloroquine 400 mg daily initiated at or before 10 weeks gestation is associated with a recurrence rate less than one-half that of historical control subjects.[12]


   Conclusion Top


Analysis of the arrhythmia mechanism is very important to assess the degree of conduction tissue involvement by the disease process and will aid in prognostication and decision regarding maternal transplacental therapy.

Declaration of patient consent

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

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Flammang D, Loteanu V, Hamani D, Lambiez M, Flammang-Dorie G. Brady-tachy syndrome: Rapid atrial pacing efficacy in preventing atrial fibrillation recurrence assessed by reliable electrograms: The prefib pilot study. Europace 2005;7:482-9.  Back to cited text no. 1
    
2.
Wacker-Gussmann A, Oberhoffer-Fritz R, Westphal DS, Hessling G, Wakai RT, Strasburger JF. The missense variant p.(Gly482Arg) in HCN4 is responsible for fetal tachy-bradycardia syndrome. HeartRhythm Case Rep 2020;6:352-6.  Back to cited text no. 2
    
3.
Dubin AM, Cuneo BF, Strasburger JF, Wakai RT, Van Hare GF, Rosenthal DN. Congenital junctional ectopic tachycardia and congenital complete atrioventricular block: A shared etiology? Heart Rhythm 2005;2:313-5.  Back to cited text no. 3
    
4.
Karmegeraj B, Namdeo S, Sudhakar A, Krishnan V, Kunjukutty R, Vaidyanathan B. Clinical presentation, management, and postnatal outcomes of fetal tachyarrhythmias: A 10-year single-center experience. Ann Pediatr Cardiol 2018;11:34-9.  Back to cited text no. 4
    
5.
Zhao H, Cuneo BF, Strasburger JF, Huhta JC, Gotteiner NL, Wakai RT. Electrophysiological characteristics of fetal atrioventricular block. J Am Coll Cardiol 2008;51:77-84.  Back to cited text no. 5
    
6.
Vigneswaran TV, Sankaran S, Rosenthal E, Simpson JM. Atrial flutter in fetus with immune-mediated complete heart block. Ultrasound Obstet Gynecol 2018;52:680-1.  Back to cited text no. 6
    
7.
Duke C, Stuart G, Simpson JM. Ventricular tachycardia secondary to prolongation of the QT interval in a fetus with autoimmune mediated congenital complete heart block. Cardiol Young 2005;15:319-21.  Back to cited text no. 7
    
8.
Mittal SR. Presystolic flow in ascending aorta in a case of left ventricular diastolic dysfunction. Indian Heart J 2015;67:152-5.  Back to cited text no. 8
    
9.
Llanos C, Friedman DM, Saxena A, Izmirly PM, Tseng CE, Dische R, et al. Anatomical and pathological findings in hearts from fetuses and infants with cardiac manifestations of neonatal lupus. Rheumatology (Oxford) 2012;51:1086-92.  Back to cited text no. 9
    
10.
Sonesson SE, Acharya G. Hemodynamics in fetal arrhythmia. Acta Obstet Gynecol Scand 2016;95:697-709.  Back to cited text no. 10
    
11.
Saxena A, Izmirly PM, Mendez B, Buyon JP, Friedman DM. Prevention and treatment in utero of autoimmune-associated congenital heart block. Cardiol Rev 2014;22:263-7.  Back to cited text no. 11
    
12.
Izmirly P, Kim M, Friedman DM, Costedoat-Chalumeau N, Clancy R, Copel JA, et al. Hydroxychloroquine to prevent recurrent congenital heart block in fetuses of anti-SSA/Ro-positive mothers. J Am Coll Cardiol. 2020;76:292-302.  Back to cited text no. 12
    

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Correspondence Address:
Dr. Balaganesh Karmegaraj
Amrita Institute of Medical Sciences, Kochi, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/apc.apc_9_21

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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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