Management of atrial fibrillation in rheumatic heart

disease
Jayaprakash Shenthar, MD, DM, FACC, FRCP (Lond)
From the Electrophysiology Unit, Department of Cardiology, Sri Jayadeva Institute of Cardiovascular
Sciences and Research, Bangalore, India.

Rheumatic heart disease (RHD) is the underlying cause of a signif- often younger, are women, and have fewer comorbidities. This re-
icant proportion of atrial fibrillation (AF) in the low- and middle- view critically looks at specific areas such as stroke prevention
income countries, while nonvalvular AF is the most common cause with reference to direct oral anticoagulants, cardioversion, rate
of AF in high-income countries. RHD is also common among African and rhythm control strategies, and the role of nonpharmacological
Americans, migrants, and the indigenous population of high- methods in rheumatic AF management. Future recommendations
income countries. The onset of AF in RHD patients is a clinical must be cognizant of local health care systems and resourcing
marker of worse outcomes and is associated with significant considering the geographic distribution of the disease.
morbidity and mortality. Despite RHD being a major cause of
morbidity and mortality in the young in many parts of the world,
KEYWORDS Rheumatic heart disease; Atrial fibrillation; Rheumatic
it is often neglected by policymakers, the media, and even the med-
atrial fibrillation; Atrial fibrillation management; Valvular heart dis-
ical fraternity. Stroke risk assessment using various risk scores has
ease; Valvular heart disease management
not been systematically evaluated in rheumatic AF patients. Rate
control may not be ideal for symptom control in rheumatic AF pa-
tients considering the young age and an active lifestyle. There is (Heart Rhythm O2 2022;3:752–759) © 2022 Heart Rhythm Society.
limited information regarding the nonpharmacological manage- Published by Elsevier Inc. This is an open access article under the CC
ment of rheumatic AF. The current management guidelines based BY-NC-ND license (http://creativecommons.org/licenses/by-nc-
on nonvalvular AF do not apply to rheumatic AF patients who are nd/4.0/).

Introduction guidelines based on nonvalvular AF have not been systemat-

Rheumatic heart disease (RHD) contributes to a significant ically studied and do not apply to rheumatic AF.
proportion of AF in low- and middle-income countries This review aims to look at the available literature on rheu-
(LMICs), while nonvalvular atrial fibrillation (AF) is the matic AF and suggest possible management strategies based
most common cause of AF in high-income countries on the current evidence (Central Illustration).
(HICs). RHD is the delayed sequelae of acute rheumatic fever
(ARF). While ARF afflicts children between 5 and 15 years Epidemiology
of age, RHD is usually diagnosed between 20 and 50 years In 2015, there were an estimated 33.4 million cases of RHD,
of age.1 Whereas RHD and its antecedent ARF have dimin- causing 319,400 deaths and 10.5 million disability-adjusted
ished in HICs, it continues unabated in the LMICs and among life years globally. The median age at death is 28 years, and
vulnerable groups in the HICs.2 RHD is a condition of global case fatality at 24 months was highest in low-income countries
health importance and is estimated to affect over 33 million (21%) and significantly lower in middle-income countries
people, mostly in LMICs where the disease is endemic.2 (12%–17%).5 Oceania, South Asia, and central sub-Saharan
RHD poses a significant health burden among African Amer- Africa have the highest age-standardized mortality due to
icans and immigrants from developing countries living in RHD.3 A recent meta-analysis of 83 studies from 42 countries
multiethnic urban America. In HICs, the condition is aggres- revealed the global prevalence of AF in RHD to be 32.8%, with
sive in immigrants and requires more interventions.3 Existing substantial heterogeneity (4.3%–79.9%) based on the coun-
international guidelines for the management of AF provide try’s development level.6 RHD remains a significant cause of
limited assistance in managing rheumatic AF.4 Current AF in Africa, China, the Middle East, and India, where it is pre-
sent in nearly one-third of patients with AF.7 The prevalence of
AF in RHD increases with age and varies from 7.6% in children
and adolescents to 39.7% in adults.6
Address reprint requests and correspondence: Dr Jayaprakash Shenthar,
Electrophysiology Unit, Department of Cardiology, Sri Jayadeva Institute of
Patients with severe valvular disease have a higher preva-
Cardiovascular Sciences and Research, 9th Block Jayanagar, Bannerghatta lence of AF than those with mild/moderate disease. AF is
Road, Bangalore 560069, India. E-mail address: [email protected]. more prevalent in mixed mitral valve (MMVD) disease,

2666-5018/© 2022 Heart Rhythm Society. Published by Elsevier Inc. This is an open access article https://doi.org/10.1016/j.hroo.2022.09.020
under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Shenthar Management of Rheumatic Atrial Fibrillation 753

is mainly seen in LMICs, whereas NRVD is more common in

KEY FINDINGS HICs.11 RVD is seen in patients in their 20s and 30s with
- Atrial fibrillation in rheumatic heart disease is under- fewer comorbidities. NRVD patients present in their 60s
recognized but is prevalent in many countries around and 70s and more often have hypertension, diabetes, chronic
the world. kidney disease, and coronary artery disease as comorbid-
ities.1,11,12 NRVD patients have more myocardial disease,
- It causes significant morbidity and mortality, mainly in causing a stiffer atrium and left ventricle, leading to diastolic
the young. dysfunction, compared with a more pliable atrium and ventri-
- There are no current guidelines for managing rheumatic cles in RHD patients.11 The stroke risk for RVD patients with
atrial fibrillation. AF is far higher than for age-matched control subjects. AF
patients with NRVD tend to be older, and rate control is
- Randomized trials have excluded patients with rheu- preferred, which may not be the best option in younger rheu-
matic atrial fibrillation, especially mitral stenosis. matic AF patients. The European Heart Rhythm Association
has classified valvular heart disease. Type 1 refers to AF pa-
followed by isolated mitral stenosis (MS) and mitral regurgi- tients needing therapy with vitamin K antagonists (VKAs)
tation (MR).6 Patients who have undergone surgical valve (moderate-to-severe MS and mechanical prosthesis), and
replacement have a higher prevalence of AF than patients type 2 requires treatment with VKAs or direct oral anticoag-
who have undergone valvuloplasty.8 In RHD patients with si- ulants (DOACs), taking into consideration the CHA2DS2-
nus rhythm, AF develops in about 20% over a median of 72 VASc (congestive heart failure, hypertension, age 75 years,
months, with an average annual event rate of 3.5% per year. diabetes mellitus, prior stroke or transient ischemic attack or
However, patients with an enlarged left atrium (LA) (47 thromboembolism, vascular disease, age 65-74 years, sex)
mm) have an average annual AF development rate of 6.0% score risk factor.13
per year.9
Management of AF
The cornerstones for the management of AF in RHD involve
Differences between rheumatic vs 3 goals: (1) control symptoms and prevent or reverse tachy-
nonrheumatic valvular AF cardiomyopathy by rate or rhythm control, (2) prevent stroke
Valvular heart disease affects approximately 3% of the pop- and peripheral embolism by therapeutic anticoagulation, and
ulation in the United States and causes up to 30,000 deaths (3) improve survival by appropriate timing of valve interven-
annually. RHD affects roughly 5% of the population world- tions.
wide and causes over 300,000 deaths annually.10 There are
several differences between rheumatic valvular disease
(RVD) vs nonrheumatic valvular disease (NRVD) Stroke prevention in rheumatic AF
(Table 1). It is essential to understand the differences, as MS was estimated to be responsible for 25% of all deaths
each group’s risk stratification and treatment strategy are from a systemic embolism in the presurgical and preanticoa-
different. RVD can present as MMVD, MS, or MR. NRVD gulant therapy era.14 Nearly 80% of patients with MS and
usually presents as MR due to mitral valve prolapse or, less systemic embolism have AF on electrocardiography. One-
commonly, as MS due to severe mitral annular calcification, third of embolic events occur within 1 month of the onset
and MMVD is uncommon. AF is more often seen in patients of AF, and two-thirds occur within 1 year.15 In patients
with RVD and less common in NRVD (52% vs 16%).11 RVD with RHD and prior embolism, the recurrence rate of 15 to

Table 1 Differences between rheumatic AF and nonrheumatic valvular atrial fibrillation

Parameter Rheumatic AF Nonrheumatic valvular AF
Presentation Mitral stenosis, mitral regurgitation, Mitral regurgitation, mitral stenosis
mixed mitral valve disease
Age, y 20-50 .60
Geographic distribution Low- and middle-income countries High-income countries
Pathology Fibrosis and commissural fusion Myxomatous valve (mitral regurgitation)
Calcification of annulus and leaflets
(mitral stenosis)
Comorbidities Lesser Higher
Atrial fibrillation Common (52%) Less common (16%)
Left atrial volume Larger Moderate enlargement
CHA2DS2-VASc Score Untested, not applied Applicable
As presented in Pressman et al.11
AF 5 atrial fibrillation; CHA2DS2-VASc 5 congestive heart failure, hypertension, age 75 years, diabetes mellitus, prior stroke or transient ischemic attack or
thromboembolism, vascular disease, age 65-74 years, sex category.
754 Heart Rhythm O2, Vol 3, No 6PB, December 2022

Rheumatic Heart Disease with Atrial fibrillation

Approach to Stroke Prevention

Direct Oral Anticoagulants Vitamin K antagonists

Mixed Mitral Mild Aortic Moderate Severe

Mitral Regurgitation Mitral Valve Mitral Stenosis
Valve Stenosis Disease (MVA < 1.5 cm sq.)
Disease (MVA >
Bio-prosthetic
1.5 cm sq.)
Valve Mechanical Valve
Prosthesis

Figure 1 Stroke prevention in rheumatic atrial fibrillation. MVA 5 mitral valve area.

40 events per 100 patient-years is the highest reported in AF between 2.0 and 3.0 was highest in Western Europe, at
of any etiology.14 There is no relation between the occurrence 67%, and lowest in India, at 35%. The TTR was between
of embolism and mitral orifice dimensions, presence or 51% and 62% in Western countries compared with 32% to
absence of heart failure (HF), or patient symptoms. Embo- 40% in India, China, and Southeast Asia.7 It is also of
lism may be the first manifestation of MS, and it can occur concern that Asians have higher rates of intracranial hemor-
even in patients with mild MS before symptom develop- rhage with VKAs than Caucasians.22 In a large longitudinal
ment.14 In rheumatic mitral valve disease patients, thrombus study of Korean patients by Kim and colleagues23 that
can occur in areas outside the LA appendage (LAA)16 and included 27,824 MS and AF patients, though there was an in-
even in the right atrial appendage.17 crease in the use of VKAs over time, the thromboembolic
Well-studied risk stratification scores in nonvalvular AF stroke rate plateaued out. The increased intracranial hemor-
have not been validated for stroke risk assessment in rheumatic rhage rates in Asian patients indicate the necessity of an alter-
AF. A recent study of validation of CHA2DS2-VASc and native anticoagulant strategy.23
HAS-BLED (hypertension, abnormal renal or liver function, All randomized trials of DOACs have excluded patients
stroke, bleeding, labile international normalized ratio, elderly, with moderate-to-severe MS and mechanical heart valves.21
drugs or alcohol) scores in valvular AF patients showed that In a retrospective observational Korean study of 2230 MS
both scores were only modestly predictive of thromboembo- with AF patients comparing DOACs with VKAs, showed
lism and bleeding events.18 Less than 80% of eligible valvular that patients on DOACs had significantly lower thromboem-
AF patients are on oral anticoagulation, of whom ,30% have bolic rates than VKAs. The all-cause mortality was signifi-
a therapeutic international normalized ratio (INR).19 While all cantly lower in the DOAC group, with no significant
patients with rheumatic MS with AF invariably require antico- difference in the incidence of intracranial hemorrhage be-
agulation, the data are not very clear for other rheumatic tween the 2 groups.24 The limitations of this study are its
valvular lesions. MS patients in sinus rhythm with dense left retrospective nature, and inability to ascertain the severity
atrial spontaneous contrast have increased cardioembolic of MS, which may have biased the result. The European So-
risk and may benefit from oral anticoagulation.20 ciety of Cardiology guidelines recommend against the use of
According to recent guidelines, VKAs are the only treat- DOACs in patients with AF and moderate-to-severe MS
ment with established safety in AF patients with rheumatic (mitral valve area ,1.5 cm2) (Class 3, Level of Evidence
mitral valve disease (Figure 1).4 Disadvantages of VKAs C).4 The American College of Cardiology/American Heart
are, a narrow therapeutic window requiring frequent INR Association guidelines recommend only VKAs in the previ-
monitoring and dose adjustments, need for adequate time in ous subset.25
therapeutic range, teratogenicity, and food and drug interac- The INVICTUS (INVestIgation of rheumatiC AF Treat-
tions.21 Time in therapeutic range (TTR) of .70% (based on ment Using VKAs, rivaroxaban, or aspirin Studies) registry
the Rosendaal method or the percentage of therapeutic INRs) is an observational registry of 17,000 patients from 23 coun-
is necessary for effective stroke prevention. A low TTR is tries with rheumatic MS and AF and a randomized noninfer-
associated with higher stroke risk, and a very high TTR is iority trial of rivaroxaban vs VKAs. The final analysis had
associated with increased bleeding risk.4 In the 4531 patients with mean age of 50.5 years, 72.3% women,
Randomized Evaluation of Long-Term Anti- coagulation and a mean duration of follow-up of 3.1 6 1.2 years. Patients
Therapy in AF registry, the proportion of INR values with a CHA2DS2-VASc score of at least 2 with a mitral valve
Shenthar Management of Rheumatic Atrial Fibrillation 755

Rheumatic Heart Disease with Atrial fibrillation

No Valve Intervention Required

Rhythm Control Strategy Rate Control Strategy

1. Symptomatic 1. Minimal/No symptoms
2. Young, active 2. Older age
3. No/ Minimal Co- 3. Co-morbidities
morbidities 4. LA Size > 50 mm
4. LA size < 50 mm 5. Normal LV function

Failed
Cardioversion

Intravenous Ibutilide/ Verapamil/Diltiazem

Direct Current Cardioversion Beta-blockers
Amiodarone
Digoxin (heart failure)
Recurrent
Sinus A
Uncontrolled Ventricular
rate/Adverse effect

Flecainide + AV blockers AV node ablation

Amiodarone +
Permanent pacemaker
implantation

Figure 2 Suggested management strategy of rheumatic atrial fibrillation not requiring valve intervention. AF 5 atrial fibrillation; AV 5 atrioventricular; LA 5
left atrial; LV 5 left ventricular.

area of ,2 cm2, LA spontaneous echo contrast, or LA intervention is unnecessary, then a decision to follow a rate or
thrombus were randomized to standard doses of rivaroxaban rhythm control strategy is considered (Figure 2). If valve
or dose-adjusted VKAs. There was a significantly higher intervention is necessary, the decision on rate or rhythm con-
incidence of death in the rivaroxaban arm than in the VKA trol strategy is decided at the time of valvular intervention
arm, with no significant group difference in the rate of major (Figure 3).
bleeding.26 The results of the INVICTUS registry support
current guidelines, which recommend VKAs for the preven-
tion of stroke in patients with RHD with AF. Rate control in rheumatic AF
The DAVID-MS (DAbigatran for Stroke PreVention In Acute rate control is necessary for symptomatic hemodynam-
Atrial Fibrillation in MoDerate or Severe Mitral Stenosis) ically stable patients for symptom alleviation. AF in MS or
trial is another ongoing noninferiority trial comparing dabi- MMVD patients results in an increased gradient across the
gatran vs VKAs in the prevention of stroke or systemic em- mitral valve. The preferred strategy is to reduce the resting
bolism.27 ventricular rate to ,80 beats/min using intravenous atrioven-
The optimal anticoagulation strategy for patients with bio- tricular (AV)-blocking drugs.29 AV-blocking drugs such as
prosthetic valves and AF is uncertain. A recent meta-analysis beta-blockers, calcium-channel blockers (diltiazem, verap-
included 4 randomized controlled trials and 6 observational amil), digoxin, or amiodarone may be used for rate control.
studies of 6405 patients with bioprosthetic valves and AF. The short-acting intravenous beta-blocker esmolol is the
It showed that DOACs were equivalent to VKAs in prevent- initial treatment of choice in the acute setting in patients
ing stroke and all-cause mortality, resulting in lesser major with stable hemodynamics. Nondihydropyridine calcium-
bleeding than VKAs.28 However, even though the recent Eu- channel blockers (diltiazem, verapamil) are an alternative
ropean Society of Cardiology guidelines suggest that for patients with contraindications to beta-blockers. Intrave-
DOACs may be used in patients with bioprosthetic valves, nous amiodarone is preferred in the intensive care unit for
it does not make a firm recommendation. rate control because of the lesser negative inotropic effect
Along with stroke prevention, the management of rheu- and safety in patients with structural heart disease or HF.30
matic AF depends on the need for valve intervention. If valve However, amiodarone should be used with caution, as it
756 Heart Rhythm O2, Vol 3, No 6PB, December 2022

Figure 3 Suggested management strategy for rheumatic atrial fibrillation requiring valve intervention.

may convert AF to sinus rhythm, and in patients with LA AV node ablation with permanent pacemaker implanta-
thrombus, it can potentially result in a stroke. tion is an effective treatment strategy in AF patients resistant
AV-blocking drugs such as nondihydropyridine calcium- to other treatment modalities. The ablate-and-pace approach
channel blockers, beta-blockers, or digoxin are used for offers much better ventricular rate control and regularization
chronic rate control. In a study of digoxin, verapamil, or me- of the R-R intervals. AV nodal ablation in patients with un-
toprolol for rate control in improving symptoms and exercise controlled AF improves the quality of life and exercise toler-
capacity, digoxin produced the least and verapamil the ance and decreases HF episodes and hospital admissions.36
maximum symptomatic improvement.31 RHD patients with Guidelines recommend AV node ablation for rate control in
AF and HF may potentially derive some benefit from digoxin (1) patients with inadequate rate control with pharmacological
use.32 Digoxin controls the resting heart rate due to increased agents, (2) patients with intolerable side effects to drugs, or (3)
vagal tone. Upright exercise decreases vagal tone and en- patients in whom catheter ablation (CA) or surgical ablation of
hances AV conduction due to increased sympathetic tone, re- AF is not indicated, has failed, or is rejected.4 Though there are
sulting in fast ventricular rates.33 Hence, digoxin is no ablate-and-pace trials in patients with rheumatic AF, it can
ineffective in ventricular rate control after exercise and in be considered in a select subset of patients with uncontrolled
young patients with a high sympathetic tone. With the limited ventricular rates or adverse drug effects.
data available, verapamil can be the drug of choice in patients Rate control is the preferred option in older patients, those
with AF with no left ventricular dysfunction or HF. Beta- with minimal symptoms, those with extremely large LA
blockers approved for HF should be preferred in patients (.5.5 cm), and those who fail the rhythm control strategy.
with rheumatic AF with left ventricular dysfunction or HF.
Digoxin should be avoided in patients with rheumatic AF
not in HF. The aim of rate control should be to achieve a Rhythm control in rheumatic AF
resting heart rate of 60 to 80 beats/min and an exercise rate Acute rhythm control
of 110 beats/min. A tight heart rate control will reduce the The primary indication for rhythm control is to reduce AF-
gradient across the mitral valve and reduce symptoms. Con- related symptoms, improve quality of life, and improve left
trol of ventricular rate in MS patients with AF has shown a ventricular function in tachycardiomyopathy patients.4 To
reduction in coagulation system activation and may decrease evaluate the response, an attempt to restore sinus rhythm is
the risk of thrombosis.34,35 a logical first step. Restoration of sinus rhythm in rheumatic
Shenthar Management of Rheumatic Atrial Fibrillation 757

AF patients has several benefits, including increased cardiac rhythm at 3 months of follow-up. At 12 months, 55% in
output, better exercise capacity, reduced heart rate at rest and the amiodarone group and 17.1% in the placebo group main-
exercise, and alleviation of symptoms.37,38 Restoration of si- tained sinus rhythm.39
nus rhythm also increases LAA velocities, resulting in In a pilot study of 50 patients, oral flecainide successfully
reverse remodeling of the LA.34 maintained sinus rhythm in 60% of patients at 1-year follow-
In a study of intravenous loading of amiodarone post– up.42 Further larger randomized studies of flecanide are
balloon valvotomy, amiodarone did not cardiovert any patient necessary to confirm its efficacy. Rheumatic AF patients
to sinus rhythm at 12 hours.39 In another study of oral amiodar- tend to be young, with fewer comorbidities, have a normal
one loading for 1 to 6 weeks after balloon valvotomy, 26% to left ventricular function, and need long-term antiarrhythmic
40% of rheumatic AF patients converted to sinus rhythm.38,40 therapy to maintain sinus rhythm. Flecanide, along with an
Amiodarone’s class III activity is a delayed effect observed AV-blocking drug, is the first option. Those with AF recur-
days to weeks following drug loading; hence, acute intrave- rence on flecainide can be considered for treatment with
nous loading is ineffective in sinus rhythm restoration.41 amiodarone, preferably in a low dose. Most small studies
In a pilot study of 50 rheumatic AF patients, a single oral have shown that maintenance of sinus rhythm was superior
dose of 4 mg/kg of flecanide effectively cardioverted 4% to to rate control on exercise capacity, quality of life, morbidity,
sinus rhythm, and 72% achieved sinus rhythm with direct and mortality.37–39 There are no large randomized rate vs
current cardioversion (DCCV).42 In a study of 165 patients rhythm control studies in rheumatic AF patients.
with rheumatic AF, ibutilide successfully restored sinus A rhythm control strategy should be considered in young,
rhythm in 77%, with a mean conversion time of 7.9 6 4.1 mi- symptomatic patients leading an active lifestyle with mild-
nutes. Torsades de pointes requiring defibrillation developed moderate mitral valve disease or early after valvular interven-
in 1.8% of patients at a mean interval of 55 6 37 minutes, and tions. The patients should preferably have a LA diameter
there were no deaths.43 Use of 4 gm. of intravenous magne- ,5.0 cm and little or no comorbidities.
sium and esmolol along with ibutilide enhances cardiover-
sion than ibutilide alone in patients with nonvalvular AF Catheter ablation
with a reduced incidence of QTc prolongation and dimin- There is minimal data on CA of rheumatic AF. Most of the
ished risk of ventricular tachycardia.44 studies of CA of rheumatic AF are small, with limited
DCCV is effective and safe in restoring sinus rhythm in follow-up data. In an early study of 13 patients, induced atrial
most rheumatic AF patients, especially after valvular inter- arrhythmia was successfully terminated by radiofrequency CA
ventions such as balloon valvotomy.42 To improve cardio- near the coronary sinus ostium by Nair and colleagues.47 A
version success and prevent relapses, DCCV should be careful look at the cycle length and the activation sequence
done after at least 1 month of oral amiodarone loading.45 suggest that the arrhythmias were organized atrial flutter rather
Cardioversion should preferably be done after antiar- than AF.47 A study of 20 rheumatic AF patients compared
rhythmic drug loading to prevent recurrences. Ibutilide simultaneous balloon valvotomy and pulmonary vein isolation
should be considered the first choice for pharmacological car- or DCCV, followed by antiarrhythmic therapy. At 4 years of
dioversion of rheumatic AF. DCCV should be considered if follow-up, 80% of CA group patients were in sinus rhythm
pharmacological cardioversion fails. compared with 10% who were only on antiarrhythmic medica-
tion.48 In a recent retrospective study comparing CA in rheu-
Pharmacological maintenance of sinus rhythm matic AF vs nonvalvular AF, the long-term (23–140 months)
Most studies of rheumatic AF of rhythm control strategy have outcomes of CA for rheumatic AF were modest. Compared
with amiodarone. In the Control of Rate versus Rhythm in with nonvalvular AF, the results were worse in rheumatic
rheumatic Atrial Fibrillation Trial study of 144 rheumatic AF patients (32% vs 56%).49 CA of LA and right atrial re-
AF patients, Vora and colleagues37 performed DCCV after entrant arrhythmias is feasible in patients with RHD with a
oral amiodarone loading. At 1-year follow-up, 70% main- high acute success rate. However, there is a significant inci-
tained sinus rhythm on amiodarone 200 mg maintenance dence of arrhythmia recurrence and late AF development.50
dose. The patients in the study were young (38.6 6 10.3 The limited data in RHD patients shows that CA of atrial
years), with an LA size of ,50 mm, and 72% had undergone arrhythmias, including AF, is associated with modest acute
valvular interventions.37 In a randomized study of 183 pa- success and a high recurrence rate. Hence, CA cannot be
tients who had undergone successful balloon valvuloplasty, considered a primary therapeutic option.
96% maintained sinus rhythm at 1 year on low-dose amiodar-
one (mean of 130 mg/d).38 In a small randomized study to Surgery
evaluate the efficacy of early DCCV along with intravenous James Cox introduced the Cox maze III, the cut-and-sew
loading dose followed by low-dose short duration (100 mg/ approach using multiple incisions in the left and the right atria
d during 6 weeks) amiodarone after balloon valvotomy, to eliminate circulating wavelets of AF while allowing the si-
87% remained in sinus rhythm at a short follow-up period nus impulse to reach the AV node.51 A recent meta-analysis of
of 6 to 9 months.46 In a recent randomized placebo- 4 randomized controlled trials and 4 observational studies
controlled trial of amiodarone, 77.5% in the amiodarone involving 1931 patients evaluated the safety and efficacy of
group and 34.1% in the placebo group remained in sinus concomitant surgical AF ablation during rheumatic mitral
758 Heart Rhythm O2, Vol 3, No 6PB, December 2022

valve surgery. Concomitant SA during rheumatic MV surgery and implementation of a feasible program for the prevention
does not increase perioperative adverse events more effective and control of RHD should be an integral component of the
in sinus rhythm restoration than MV surgery alone at national health system.
discharge and 1- and 3-year follow-up.52 Surgical AF ablation
has a class IIa recommendation in patients with valvular heart
disease undergoing surgery in the recent AF guidelines.4
Conclusion
RHD is still prevalent in many parts of the world. It affects
mainly the young and underprivileged causing significant
LAA closure morbidity and mortality. Even though rheumatic AF is com-
In a retrospective study of 860 patients, LAA exclusion dur- mon in many countries, all major guidelines have neglected
ing mitral valve replacement was safe and effective in pre- this disease entirely. There are no guideline recommenda-
venting postoperative ischemic stroke in rheumatic AF.53 tions for managing rheumatic AF. There are no large random-
In another observational study of 136 patients of LAA exclu- ized trials of rheumatic AF, and all major trials have excluded
sion during mitral valve surgery, there was a significant inci- patients with RHD. There is an urgent need for large random-
dence of thromboembolic events in patients not prescribed ized trials of patients with rheumatic AF. It is time for the
warfarin therapy at hospital discharge.54 various guideline writing committees to give recommenda-
Percutaneous LAA closure, an established treatment in tions for managing rheumatic AF.
nonvalvular AF, is limited to case reports and has not been
Funding Sources: The author has no funding sources to disclose.
tested systematically in rheumatic AF.55 Considering the
frequent presence of thrombus outside appendage in RHD Disclosures: The author has no conflicts of interest to disclose.
patients, LAA closure alone may not be beneficial in patients Authorship: The author attests he meets the current ICMJE criteria for
with rheumatic mitral valve disease, and all patients need to authorship.
be on oral anticoagulants.
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