Atrial Standstill in association with

Arrhythmogenic Right Ventricular Cardiomyopathy : A

case report
Fariz Dwiky, MD ; Mohammad Iqbal, MD, PhD
Declaration of Interest
• Authors have nothing to declare
• No conflict of interest exists
• No funding was received for this work
 INTRODUCTION
Surface ECG
-No spontaneous atrial activity
~Junctional bradycardia without atrial
-Atria cannot be electrically stimulated activity

Atrial Standstill

Classification
Congenital: ~genetic defects of cardiac
-Partial or total
sodium channel SCN5A
-Intermittent or permanent

Issa ZF, Miller JM, Zipes DP. Sinus Node Dysfunction. In: Clinical Arrhythmology and Electrophysiology: A Companion to Braunwald’s Heart Disease. 3rd ed. Elsevier; 2019:238-254.
Bellmann B, Roser M, Muntean B, et al. Atrial standstill in sinus node disease due to extensive atrial fibrosis: impact on dual chamber pacemaker implantation. Europace. 2016;18(2):238-245.
Gering LE, Knilans TK, Surawicz B, Tavel ME. Atrial Rhythms. In: Chou’s Electrocardiography in Clinical Practice. 6th ed. Saunders Elsevier; 2008:345-360.
 Characteristic
Ventricular arrhythmias and structural
Inherited desmosomal cardiomyopathy abnormalities of the RV (and LV more manifest
during the latter stages of the disease)

Arrhythmogenic right
ventricular cardiomyopathy
(ARVC)
Pathological hallmark
Desmosomes: found throughout the cardiac Progressive loss of myocardium and its
system, including the atria replacement by fibro-fatty tissue 
Evidence for direct atrial involvement with ARVC May lead to the lethal tachyarrhythmias or
remains limited bradyarrhythmias

Issa ZF, Miller JM, Zipes DP. Ventricular Tachycardia in Arrhythmogenic Right Ventricular Cardiomyopathy. In: Clinical Arrhythmology and Electrophysiology: A Companion to Braunwald’s Heart Disease. 3rd ed. Elsevier; 2019:942-967.
Zghaib T, Bourfiss M, van der Heijden JF, et al. Atrial Dysfunction in Arrhythmogenic Right Ventricular Cardiomyopathy. Circ Cardiovasc Imaging. 2018;11(9).
Liang E, Wu L, Fan S, et al. Bradyarrhythmias in Arrhythmogenic Right Ventricular Cardiomyopathy. Am J Cardiol. 2019;123(10):1690-1695.
 In this case
Reporting a rare finding
It is still unclear how common atrial Initial diagnosis: junctional bradycardia
arrhythmia in ARVC patients with atrial paralysis and has undergone
pacing procedure
During follow-up: fit into the criteria of
ARVC diagnosis

Are these findings

related?

Rujirachun P, Wattanachayakul P, Charoenngam N, Winijkul A, Ungprasert P. Prevalence of atrial arrhythmia in patients with arrhythmogenic right ventricular cardiomyopathy. J Cardiovasc Med. 2020;21:368-376.

Initial chief complaint
Epigastric discomfort ~1 week
Atorvastatin 1x40 mg for high
cholesterol ~1 week
No history of hypertension,
diabetes, heart diseases, or
smoking
42-year-old
Female
Referred into our tertiary institution
No nausea, vomiting,
(+) On and off bipedal edema No associated PND or
palpitation, chest pain,
and fatigue ~1 year orthopnea
dyspnea, or syncope
Conscious and alert
BP 120/71 mmHg Other physical examination
findings unremarkable
Family history unremarkable HR of 64x/min regular
Normal heart and lung
RR of 20x/min, afebrile examination
SpO2 of 98% RA
 Patient initially came to a nearby
hospital

ECG
Junctional rhythm at 56 bpm with
incomplete RBBB and notable inverted
T wave in precordial leads

Lab results were unremarkable

Troponin I <0.1
LDL of 145
ECG in First Hospital (23/09/2022)

Patient was admitted with

Lab Result from First Hospital (23/09/2022)
Initial diagnosis: CAD
Hb 12.4/ Ht 38/ Leuko 8750/ Tr 164.000/ Trop I < 0,1/ Ur 23/ Cr 1.1/
and Referred to a higher facility
Total Cholesterol 195/ LDL 145/ HDL 35/ TG 48/ GDS 102/ Na 134/ K
(Private Hospital) for further 3.6
examination and treatment
ECG was repeated in second hospital and showing similar
result

Repeat laboratory examination showing unremarkable

result with normal troponin

ECG in Second Hospital (27/09/2022)

Lab Result from 2nd Hospital

Hb 14.5/ Ht 45/ Eritrosit 5.37/ MCV 83.1/ MCH 27.0/ MCHC 32.5/ Leu 6480/
Tr 212.000/ Diffcount 1/5/65/21/8/ NLR 3.10/ GDS 78/ Ur 25/Cr 0.60/ Na 145/
K 3.7/ Cl 107/ Ca 1.1/Total Ca 9.3/Mg 1.89/ APTT 23.3/PT 10.4/INR 1.02/
Troponin T <40 (normal)/ HBsAg NR
 EP Study Baseline
Baseline cycle 928 ms PR interval 109 ms
length
AA interval - QRS duration 337 ms

PA interval - QT interval -

H V H V AH interval - QTc value -

HV interval 47 ms SACT -

Electrophysiology Results :
Baseline ECG shows Junctional Bradycardia
No electrical activation at right atrium
Pacing with high output at all area right atrium cannot capture the atrial tissue
Normal HV interval
Procedure done, no acute complication occured

Final Diagnosis :
Junctional bradycardia with atrial paralysis

Recommendation :
Single chambers His Bundle Pacing
 Referred to our institution for that
procedure

Repeat ECG and laboratory

showing similar result with
previous examinations

Initial management
Dopamine 5 mcg/kg/min
Atorvastatin 1x40 mg

ECG in our Hospital (28/09/2022)

Single Chamber LBB area
Pacing of placement was
done Lab Result from our Hospital
Hb 14.8/ Ht 44.5/ Eritrosit 5.23/ MCV 85.1/ MCH 28.3/ MCHC 33.3/ Leu 5980/ Tr
219.000/ Diffcount 0/5/0/56/30/9 NLR 1.87/ GDS 118/ Ur 18.9/Cr 0.64/ Na 139/ K
3.7/ Cl 107/ Ca 4.83/Mg 1.89/ Troponin I 0.01 (normal)
 Follow-up ECG post pacing

Follow-up TTE
Dilated LA and RA with eccentric LVH
Normal LV systolic function with abnormal septal motion
Bicuspid aortic valves; mild TR; low probability of PH
Reduced RV systolic function (TAPSE 15)
RVOT PLAX of 35 mm and PSAX of 40/31 mm
Pulsed-wave doppler: Absent A wave
TDI at mitral annulus: presence of E’ wave without A’ wave
Suggesting findings of ARVC fulfilling its criteria according to 2010 Task Force Criteria
 Follow-up TTE
Dilated LA and RA with eccentric LVH
Normal LV systolic function with abnormal
septal motion
Bicuspid aortic valves; mild TR; low probability
of PH
Reduced RV systolic function (TAPSE 15)
RVOT PLAX of 35 mm and PSAX of 40/31 mm
Pulsed-wave doppler: Absent A wave
TDI at mitral annulus: presence of E’ wave
without A’ wave
Suggesting findings of ARVC fulfilling its criteria
according to 2010 Task Force Criteria
 CASE PRESENTATION

Follow-up TTE
Dilated LA and RA with eccentric LVH
Normal LV systolic function with abnormal
septal motion
Bicuspid aortic valves; mild TR; low probability
of PH
Reduced RV systolic function (TAPSE 15)
RVOT PLAX of 35 mm and PSAX of 40/31 mm
Pulsed-wave doppler: Absent A wave
TDI at mitral annulus: presence of E’ wave
without A’ wave
Suggesting findings of ARVC fulfilling its criteria
according to 2010 Task Force Criteria
 Cardiac MRI
27/01/2023

Conclusion :
Normal LV volume, normal LV
systolic function
Normal RV volume, normal low RV
function, with Dyskinetic apical RV, RA RV
mid RV free wall
Bicuspid AV, trivial MR, mild TR
Subendocardial fibrosis at mid apical
RV wall
Mid wall fibrosis at basal mid
anteroseptal LV

Impressions :
Fulfilled 1 major criteria of ARVC
with fibrosis at apical RV wall.
Biatrial standstill with enlargement.
 Subendocardial Fibrosis
Midwall Fibrosis

Cardiac MRI
27/01/2023
 In this case

Rare occurrence of atrial dysfunction (Atrial Standstill) on top of the latest finding of ARVC
suspicion in our patient

Exclusive atrium pathology may be possible in the presence of ARVC

• More possible in cases with findings of identified cause of AS, in which there were none in the patient except for
the probability of some kind of cardiomyopathy causing the atrial dysfunction

Reports of SCN5A mutations might link AS and ARVC

-Mutations will cause desmosomal abnormalities throughout the myocardium  fibro-fatty
changes in the ventricle and atrium
 Conclusion

• This report showed a very rare case of AS and ARVC in coexistence. Limited availability of atrium imaging
data with CMR in patients with ARVC makes structural analysis of the atria difficult. The thin-walled
nature of the atria also makes biopsy unwise.
• Genetic testing can be done to confirm if the AS is related to the ARVC by the same genetic mutations or
merely just a consequences of ventricular remodelling that caused atrial remodelling in the long run.
• In the end, it must be kept in mind that AS can coexists with ARVC since relatively little attention has
been focused on atrial pathologies in patients with ARVC.
Thank You