A 57-year-old male presented with recurrent palpitations. He was diagnosed with rheumatic mitral stenosis, right posterior septal accessory pathway and atrial flutter. An electrophysiological study after percutaneous balloon mitral valvotomy showed that the palpitations were due to atrial flutter with right bundle branch aberrancy. The right posterior septal pathway was a bystander because it had a higher refractory period than the atrioventricular node.

1. © RADCLIFFE CARDIOLOGY 2022
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Intervention
CASE REPORT
In India, the prevalence of rheumatic heart disease in the general
population is 1.5–2.0 per 1,000.1,2
However, based on echocardiography,
the prevalence of rheumatic heart disease is likely to be greater.3
The
prevalence of Wolff–Parkinson–White (WPW) syndrome or the WPW
pattern in the general population ranges from 0.1 to 0.3%, with a higher
prevalence among men than women.4
The coexistence of rheumatic
mitral stenosis and WPW syndrome is so rare that a search of the literature
using Google Scholar and PubMed yielded 17 cases between 1960 and
2021. Among these, balloon mitral valvotomy and radiofrequency ablation
were performed simultaneously in only two cases.5,6
Case Report
A 57-year-old male presented with a three-decade history of recurrent
episodes of palpitation with alarming jugular venous pulsation, but
without syncope. Each episode spontaneously reverted to the normal
sinus rhythm 6–8 hours after the onset. Cardiac auscultation raised the
suspicion of underlying rheumatic mitral stenosis. A 12-lead ECG was
suggestive of a WPW pattern with a right posterior septal accessory
pathway (Figure 1A). A chest X-ray in the posterior–anterior view was
consistent with cardiac auscultation (Figure 1B). A transoesophageal
echocardiogram confirmed rheumatic mitral stenosis (Figure 2A and
Supplementary Material Video 1). The pliable mitral valve area was
0.8 cm2
and the mean gradient was 17 mmHg at a heart rate of 87 BPM.
The coronary angiogram was normal.
An electrophysiologist, cardiothoracic surgeon, cardiac anaesthetist and
cardiologist suggested mitral valvotomy followed by ablation of
the accessory pathway in a single procedure if possible to avoid repeated
septal puncture. Informed consent was obtained for the procedure.
The day before the procedure, the patient developed an episode of
palpitation during the clinical round. A 12-lead ECG revealed atrial flutter
with right bundle branch aberrancy on metoprolol succinate (Figure 2B).
The patient’s blood pressure was 124/80 mmHg. Oral verapamil was
initiated and the atrial flutter reverted to normal sinus rhythm.
On the day of the procedure, the first balloon mitral valvotomy was
performed from a right femoral approach using a 23–26 mm Accura
balloon (Vascular Concepts) after transeptal access using an 8 Fr SL-1
sheath and a BRK-0 needle (St Jude Medical). A transeptal puncture was
performed after proper needle tip position was confirmed by fluoroscopy
(right anterior oblique, left anterior oblique and 90° lateral views) and
transoesophageal echocardiography (bicaval and short axis views;
Figure 3A). The mean left atrial pressure prior to the valvotomy was
31 mmHg. The balloon was inflated to 26 mm in the right anterior oblique
20° position under fluoroscopy (Figure 3B) because the patient was 160
cm tall. The mitral valve area increased to 2.2 cm2
without any additional
mitral regurgitation, and the mean left atrial pressure decreased to 12
mmHg without any mitral valve gradient. Immediate transthoracic
echocardiography showed that the mitral valve gradient had decreased
to 7/2 mmHg with negligible mitral regurgitation.
The left atrial wire was reintroduced into the left atrium before the
stretched balloon was removed from the left atrium for the
Abstract
A 57-year-old male presented with recurrent palpitations. He was diagnosed with rheumatic mitral stenosis, right posterior septal accessory
pathway and atrial flutter. An electrophysiological study after percutaneous balloon mitral valvotomy showed that the palpitations were due to
atrial flutter with right bundle branch aberrancy. The right posterior septal pathway was a bystander because it had a higher refractory period
than the atrioventricular node.
Keywords
Rheumatic mitral stenosis, right posterior septal accessory pathway, atrial flutter, right bundle branch, radiofrequency ablation, percutaneous
balloon mitral valvotomy
Disclosure: The authors have no conflicts of interest to declare.
Patient Consent: Written informed consent was obtained from the patient for publication of this case report.
Received: 6 August 2021 Accepted: 16 September 2021 Citation: Journal of Asian Pacific Society of Cardiology 2022;1:e03. DOI: https://doi.org/10.15420/japsc.2021.07
Correspondence: Ramachandra Barik, Department of Cardiology, All India Institute of Medical Sciences, Bhubaneswar, Odisha, Pin-751019, India.
E: cardioramachandra@gmail.com
Open Access: This work is open access under the CC-BY-NC 4.0 License which allows users to copy, redistribute and make derivative works for non-commercial
purposes, provided the original work is cited correctly.
Trio of Rheumatic Mitral Stenosis, Right Posterior Septal
Accessory Pathway and Atrial Flutter: A Case Report
Jogendra Singh ,1
Dibyasundar Mahanta ,1
Rudra Pratap Mahapatra ,2
Debasis Acharya 1
and Ramachandra Barik 1
1. Department of Cardiology, All India Institute of Medical Sciences, Bhubaneswar, India;
2. Department of Cardiothoracic Surgery, All India Institute of Medical Sciences, Bhubaneswar, India

2. Pre-excitation Associated with Rheumatic Mitral Stenosis
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electrophysiological study and for possible radiofrequency ablation
(Figure 3C). The electrophysiologist proceeded with the ablation plan.
Because the patient had baseline pre-excitation through the right
posterior septal path and atrial flutter with orthodromic conduction with
right bundle branch aberration, one decapolar catheter in the coronary
sinus and a quadripolar catheter in the right ventricle were used to study
the effective refractory period (ERP) of the accessory pathway rather than
using the routine four electrophysiology catheters (three quadripolar [high
right atrial, His bundle, right ventricle apex] and one decapolar catheter in
the coronary sinus; Figure 4C). The supra-His conduction time was 65 ms
and the infra-His conduction time was 9 ms. The baseline ECG was
suggestive of a right posterior septal pathway. Right ventricular pacing
showed eccentric conduction up to 450 ms, which suggested a retrograde
pathway ERP of 450 ms. On pacing the integrated pathway, the ERP was
found to be 450 ms. Due to the weak nature of the accessory pathway,
ablation was not performed. On rapid atrial pacing, atrial flutter with right
bundle branch aberrancy was induced, similar to the clinical tachycardia
observed earlier. Ablation for the atrial flutter was not performed, and the
patient was maintained on metoprolol succinate and an oral anticoagulant
in the anticipation of spontaneous remission of atrial flutter after both
remodelling of the atrium and haemodynamic improvement after balloon
valvotomy.
This patient has remained asymptomatic over a follow-up period of 15
months.
Discussion
Balloon mitral valvotomy is preferred to surgery in the case of pliable
rheumatic stenosis. Of the treatments available for valvular AF, less is known
about the efficacy of radiofrequency ablation because of the lack of a
significant number of randomised control trials.7
Arrhythmia-related death in
asymptomatic pre-excitation is as low as 0.05 to 0.9 per 1,000.8
Therefore,
the treatment of rheumatic mitral stenosis with bystander involvement of an
accessorypathwayandanERPthatisgreaterthanthatoftheatrioventricular
node is not a challenge. Ablation of the accessory pathway is not indicated
if the bystander pathway has a high ERP.9
In the present case, the patient
had recurrent episodes of palpitation, but the right posterior septal
accessory pathway did not contribute to these, which is quite an unusual
scenario and unlike the case reported by Jagadheesan et al.10
Figure 1: ECG and Chest X-Ray
Figure 2: Transesophagial Echocardiography and ECG During Palpitation
Report confirmed by
I
II
IR
aVR
V1
V2
V3
V4
V5
V5
aVL
aVP
A B
A: A 12-lead ECG showing the Wolff–Parkinson–White pattern with a possible right posterior septal accessory pathway because the R/S ratio is <0.5 in V1 and V2 and <1 in the inferior leads. B: Chest
X-ray in the posterior–anterior view showing mitralisation of the left heart border, double atrial shadow on the right side and a horizontal left bronchus.
A: There was significant mitral stenosis and the mean mitral valve gradient of 16 mmHg prior to the balloon mitral valvotomy. B: Atrial flutter with 2:1 atrioventricular block and right bundle branch
aberrancy was evident during the electrophysiological study by pacing the atria with a decapolar catheter in the coronary sinus at cycle length of 200 ms.

3. Pre-excitation Associated with Rheumatic Mitral Stenosis
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Patients with rheumatic mitral stenosis who routinely seek help for
fibrillation or flutter are in the 30- to 50-year age group, but the
coexistence of a bystander right posterior septal accessory pathway, as in
the present case, is unusual.11
The incidence of AF in a patient with an accessory pathway is 10–38%, but
the association with common atrial flutter is not known.12
Our patient had
orthodromic conduction of atrial flutter through the atrioventricular node
because the coexisting right posterior septal pathway had an ERP of ≥450
ms. Neither the atrial flutter nor right posterior septal pathway were
ablated, with the expectation that, during follow-up, both would become
non-functional over time because of left atrial remodelling and favourable
haemodynamic changes.13
It is well established that mitral stenosis causes
AF, and the incidence of AF is higher in older age groups. It is also known
that the results of balloon mitral valvotomy are worse in older patients
because of persistent AF.14
Therefore, it has been suggested that balloon
mitral valvotomy is performed at an early age for favourable atrial
remodelling to reduce the occurrence of AF or atrial flutter.15
Conclusion
Palpitations caused by atrial flutter with right bundle branch aberrancy in a
patient with rheumatic mitral stenosis and a right posterior septal accessory
pathway with an ERP higher than that of the atrioventricular node are rare.
Whether left atrial remodelling after percutaneous balloon mitral valvotomy
further reduces atrial flutter requires additional investigation in larger
studies with a longer follow-up period.
Figure 3: Transsepetal Puncture for Balloon Mitral Valvotomy and Electrophysiology Study in One Go
A: Septal puncture using fluoroscopy and transesophageal echocardiography guidance. B: A 23–26 mm Accura balloon was inflated to 26 mm in the right anterior oblique view.
Clinical Perspective
• Severe rheumatic mitral stenosis associated with a right
posterior septal accessory pathway is rare.
• It is very unusual that recurrent palpitations are caused by atrial
flutter with right bundle branch aberrancy; rather, palpitations
are likely caused by right posterior septal accessory pathway-
mediated Wolff–Parkinson–White syndrome because the right
posterior septal accessory pathway has a lower effective
refractory period than the atrioventricular node.
• Treating both conditions with a single intervention (i.e. by
balloon mitral valvotomy and radiofrequency ablation) is rare.
• The almost complete resolution of atrial flutter 15 months after
balloon mitral valvotomy in this patient is an interesting finding.
Figure 4: Electrophysiology Study to
Map the Accessory Pathway
Electrophysiological study using one quadripolar catheter for right ventricle pacing and one
decapolar catheter in the coronary sinus.

4. Pre-excitation Associated with Rheumatic Mitral Stenosis
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