Tag Archives: GUIDELINES

Revised Jones Criteria for Acute Rheumatic Fever – 2015 guideline

Acute rheumatic fever remains a serious healthcare concern for the majority of the world’s population despite its decline in incidence in Europe and North America. This statement reviews the historic Jones criteria used to diagnose acute rheumatic fever in the context of the current epidemiology of the disease and updates those criteria to also taking into account the use of Doppler echocardiography in the diagnosis of carditis as a major manifestation of acute rheumatic fever.

1. Epidemiology:

1. It is reasonable to consider individuals to be at low risk for ARF if they come from a setting or population known to experience low rates of ARF or RHD (Class IIa; Level of Evidence C).
2. It is reasonable that where reliable epidemiological data are available, low risk should be defined as having an ARF incidence <2 per 100 000 school-aged children (usually 5–14 years old) per year or an allage prevalence of RHD of ≤1 per 1000 population per year (Class IIa; Level of Evidence C).
3. Children not clearly from a low-risk population are at moderate to high risk depending on their reference population (Class I; Level of Evidence C).

2. Clinical Manifestations of ARF:

Generally, the clinical profile of ARF in low- and middle-income countries closely resembles that of high-income countries. Universally, the most common major manifestations during the first episode of ARF (the “major criteria” for diagnosis) remain
carditis (50%–70%) and arthritis (35%–66%). These are followed in frequency by chorea (10%–30%), which has been demonstrated to have a female predominance, and then
subcutaneous nodules (0%–10%) and erythema marginatum (<6%), which remain much less common but highly specific manifestations of ARF.

3.Carditis: Diagnosis in the Era of Widely Available Echocardiography:

Classically, as discussed in the 1992 AHA revised Jones criteria statement, carditis as a major manifestation of ARF has been a clinical diagnosis based on the auscultation of typical murmurs that indicate mitral or aortic valve regurgitation, at either valve or both valves. Numerous studies over the past 20 years have addressed the role of echocardiography (compared with purely clinical assessment) in the diagnosis of ARF. More than 25 studies have reported echocardiography/Doppler evidence of mitral or aortic valve regurgitation in patients with ARF despite the absence of classic auscultatory findings. This writing group concludes the following:

1. Echocardiography with Doppler should be performed in all cases of confirmed and suspected ARF (Class I; Level of Evidence B).
2. It is reasonable to consider performing serial echocardiography/ Doppler studies in any patient with diagnosed or suspected ARF even if documented carditis is not present on diagnosis (Class IIa; Level of Evidence C).
3. Echocardiography/Doppler testing should be performed to assess whether carditis is present in the absence of auscultatory findings, particularly in moderate- to high-risk populations and when ARF is considered likely (Class I; Level of Evidence B).
4. Echocardiography/Doppler findings not consistent with carditis should exclude that diagnosis in patients with a heart murmur otherwise thought to indicate rheumatic carditis (Class I; Level of Evidence B).

Evolving role of echogardiography in acute rheumatic fever

Evolving role of echogardiography in acute rheumatic fever

4.Specific doppler criteria for diagnosis of rheumatic valvulitis

Pathological mitral regurgitation (all 4 criteria met)
1.Seen in at least 2 views
2.Jet length ≥2 cm in at least 1 view
3.Peak velocity >3 m/s
4.Pansystolic jet in at least 1 envelope

Pathological aortic regurgitation (all 4 criteria met)
1.Seen in at least 2 views
2.Jet length ≥1 cm in at least 1 view
3.Peak velocity >3 m/s
4.Pan diastolic jet in at least 1 envelope

Morphological Findings on Echocardiogram in Rheumatic Valvulitis

Acute mitral valve changes
Annular dilation
Chordal elongation
Chordal rupture resulting in flail leaflet with severe mitral regurgitation
Anterior (or less commonly posterior) leaflet tip prolapse
Beading/nodularity of leaflet tips

Chronic mitral valve changes: not seen in acute carditis
Leaflet thickening
Chordal thickening and fusion
Restricted leaflet motion
Calcification

Aortic valve changes in either acute or chronic carditis
Irregular or focal leaflet thickening
Coaptation defect
Restricted leaflet motion
Leaflet prolapse

5.Evidence of preceding Streptococcal infection:

Because other illnesses may closely resemble ARF, laboratory evidence of antecedent group A streptococcal infection is needed whenever possible, and the diagnosis is in doubt when such evidence is not available.

Any one of the following can serve as evidence of preceding infection:
Increased or rising anti-streptolysin O titer or other streptococcal antibodies (anti-DNASE B) (Class I, Level of Evidence B). A rise in titer is better evidence than a single titer result.
A positive throat culture for group A β-hemolytic streptococci (Class I, Level of Evidence B).
A positive rapid group A streptococcal carbohydrate antigen test in a child whose clinical presentation suggests a high pretest probability of streptococcal pharyngitis (Class I, Level of Evidence B).

6. Diagnosis of Acute rheumatic fever:

For all patient populations with evidence of preceding GAS infection

Diagnosis: initial ARF: 2 Major manifestations or 1 major plus 2 minor manifestations
Diagnosis: recurrent ARF: 2 Major or 1 major and 2 minor or 3 minor

Major and minor criteria for diagnosis of Acute rheumatic fever

Major and minor criteria for diagnosis of Acute rheumatic fever


Flow charts for diagnosis of rheumatic fever

Flow charts for diagnosis of rheumatic fever

 

7.Rheumatic Fever Recurrences

As stated in the 1992 guidelines, patients who have a history of ARF or RHD are at high risk for “recurrent” attacks if reinfected with group A streptococci. Such an attack is considered
a new episode of ARF, but one in which the complete set of Jones criteria, even as revised, may not be completely fulfilled.

The guideline recommendations for diagnosing rheumatic fever recurrences are:
1. With a reliable past history of ARF or established RHD, and in the face of documented group A streptococcal infection, 2 major or 1 major and 2 minor or 3 minor manifestations may be sufficient for a presumptive diagnosis (Class IIb; Level of Evidence C).
2. When minor manifestations alone are present, the exclusion of other more likely causes of the clinical presentation is recommended before a diagnosis of an ARF recurrence is made (Class I; Level of Evidence C).

8.“Possible” Rheumatic Fever

In some circumstances, a given clinical presentation may not fulfill these updated Jones criteria, but the clinician may still have good reason to suspect that ARF is the diagnosis.
This may occur in high-incidence settings. In such situations the clinicians should use their discretion and clinical acumen to make the diagnosis that they consider most likely and manage the patient accordingly.

1. Where there is genuine uncertainty, it is reasonable to consider offering 12 months of secondary prophylaxis followed by reevaluation to include a careful history and physical examination in addition to a repeat echocardiogram (Class IIa; Level of Evidence C).
2. In a patient with recurrent symptoms (particularly involving the joints) who has been adherent to prophylaxis recommendations but lacks serological evidence of group A streptococcal infection and lacks echocardiographic evidence of valvulitis, it is reasonable
to conclude that the recurrent symptoms are not likely related to ARF, and discontinuation of antibiotic prophylaxis may be appropriate (Class IIa; Level of Evidence C).

Summary:

Jones criteria needed revision to meet current technological advances and clinical needs. Strict application of echocardiography/Doppler findings may be used to fulfill the major criterion of carditis, even in the absence of classic auscultatory findings, providing that ambient loading conditions are taken into consideration. In addition, monoarthritis or polyarthralgia could be accepted as fulfilling the major criterion of arthritis, but only in moderate- to high-risk populations. For low-risk populations, monoarthritis is not included, and polyarthralgia remains a minor criterion. Similarly, the requirement for the presence of fever can be fulfilled with oral, tympanic, or rectal temperature documented at 38°C in moderate- to high-risk populations, but only at ≥38.5°C in others.

Refernce:

1. http://circ.ahajournals.org/content/early/2015/04/23/CIR.0000000000000205.abstract

Implantable cardioverter defibrillator (ICD) – the others

Implantable cardioverter defibrillator (ICD) – the others

New ACC/AHA/HRS Expert consensus document for implantable cardioverter defibrillator (ICD) implantation in patients who are excluded or not well represented
in clinical trials.

A guideline has been published for ICD implantation. This new document is a good attempt at helping cardiologists in deciding about ICD.
There are some good points about the document.

Overview of the guideline:

1. The doument considers the published studies in ICD and makes recommendations.
2. Since the guideline considers only patients who are excluded or not well represented in clinical trials, there is no class of recommendations or level of evidence.
Rather there are categories like recommended, not recommended, can be done etc
3. Patients have been divided into different populations and recommendations are made for each patient population.

The broad categories considered are:

1. ICD Implantation in the Context of an Abnormal Troponin that Is Not Due to a Myocardial Infarction
2. ICD Implantation Within 40 Days of a Myocardial Infarction
3. ICD Implantation Within 90 Days of Revascularization
4. ICD Implantation <9 Months from the Initial Diagnosis of Nonischemic Cardiomyopathy

We will discuss the recommendations in each category

1. ICD Implantation in the Context of an Abnormal Troponin that Is Not Due to a Myocardial Infarction

First group of patients are those having elevated troponin levels but not fulfilling the  definition of MI (see other causes of elevated troponin e.g.kidney disease,
acute pulmonary embolus, heart failure, myocarditis, chest trauma, or tachyarrhythmia)and satisfying standard ICD indications for primary and
secondary prevention. ICD is recommended is such patients.
The idea is to define whether the elevated cardiac markers are due to MI or not. If not due to MI then go for ICD implatation early and no need to wait
for 40 days, like in a post MI setting.

2. ICD Implantation Within 40 Days of a Myocardial Infarction

 

1-Implantation of an ICD within the first 40 days following acute MI in patients with                       preexisting systolic ventricular dysfunction (who would have qualified for a
primary prevention ICD) is not recommended.
2-In patients who, within 40 days of an MI, require nonelective permanent pacing, who               also would meet primary prevention criteria for implantation of an ICD, and recovery of       left ventricular function is uncertain or not expected, implantation of an ICD  is                          recommended.
The basis of such recommendations has been explained by the writing group
“This reflects the fact that implantation of
a pacemaker or ICD is associated with some risk, especially
infection. If the likelihood that a patient requiring PPM implantation
early post-MI will ultimately require a second
procedure to extract the PPM and leads and replace it with
an ICD system 40 days later, it would seem inappropriate
not to implant an ICD rather than a PPM.”
3-Patients within 40 days of an MI who subsequently present sustained or hemodynamically significant ventricular tachyarrhythmias.

In this scenario the following recommendations are made
(i)In patients who, within 40 days of an MI, develop sustained (or hemodynamically significant) ventricular tachyarrhythmias >48 hours after an MI and
in the absence of ongoing ischemia, implantation of an ICD is recommended.

(ii)In patients who, within 40 days of an MI, develop sustained (or hemodynamically significant) VT >48 hours after an MI that can be treated by ablation,
implantation of an ICD can be useful.

(iii)In patients who, within 40 days of an MI, develop sustained (or hemodynamically significant) ventricular tachyarrhythmias where there is
clear evidence of an ischemic etiology with coronary anatomy amenable to revascularization (and appropriately treated), implantation
of an ICD is not recommended.
4- Patients within 40 days of MI who present with syncope that is thought to be due to ventricular arrhythmia – implantation of an ICD can be done.
In this recommendation quite a liberal one as there is no need to document ventricular arrhythmia. ICD can be done for suspected ventricular arrhythmia ( on the
basis of clinical history, documented NSVT or EP study). There are no studies that have specifically addressed whether ICD implantation is beneficial  in the setting of syncope thought to be due to a ventricular tachyarrhythmia in the first 40 days after MI. However, the consensus of the writing group is that syncope in the setting of a recent MI is a potentially serious issue, and ICD implantation can be useful if syncope is thought to be due to a ventricular tachyarrhythmia (by clinical history, documented NSVT, or EP study), regardless of timing in relationship to an MI (either <40 days or >40 days after MI).
5- Elective ICD replacement for battery depletion can be done in first 40 days after MI (in patients who have been previously implanted with ICD).
6- ICD implantation in patients within 40 days of an MI who have been listed for heart transplant or implanted with a left ventricular assist device is not recommended.

3. ICD Implantation Within 90 Days of Revascularization.

The ICD indications can be primary and secondary or patients requiring permanent pacing
1-ICD is recommended for primary prevention within 90 days of revascularization
a. In patients who have indication for ICD implantation for primary prevention of sudden cardiac death,and who have undergone revascularization
that is unlikely to result in an improvement in LVEF >0.35, and who are not within 40 days after an acute MI.
2-ICD is recommended for secondary prevention within 90 days of revascularization
a. In patients with abnormal left ventricular function and previous indication for ICD for            secondary prevention of sudden cardiac death (resuscitated from cardiac arrest due to        ventricular tachyarrhythmia) and have abnormal left ventricular function,
implantation of an ICD is recommended.
b. In patients with normal left ventricular function and previous indication for ICD for                  secondary prevention of sudden cardiac death  (resuscitated from cardiac arrest due to        ventricular tachyarrhythmia) that is unlikely related to myocardial ischemia/injury
c. ICD not indicated in patients whose cardiac arrest or VT/VF was due to acute myocardial        ischemia or injury.
3-ICD recommendations for patients with indication for permanent pacemaker

ICD is recommended in patients with indication for permanent  pacemaker + indication for ICD.
This indication is likely to come-up for further discussion in view of recent developments. The FDA has approved Medtronic CRT-D and CRT devices for patients with AV-block,NYHA I,II, III heart failure and LVEF<50% (based on data from BLOCK-HF trial)(http://www.medscape.com/viewarticle/823485).
So many of the patients with the above indication for ICD will receive CRT-D.

4-ICD is recommended in patients who develop  sustained VT/VF not related to myocardial ischemia ,syncope, patients needing pulse generator replacement, patients listed for heart transplant or implanted with ventricular assist devices, who are not within 40 days of acute myocardial infarction

4. ICD Implantation <9 Months from the Initial Diagnosis of Nonischemic Cardiomyopathy

ICD recommended in:
1-Implantation of an ICD for primary prevention is not recommended within the first 3 months after initial diagnosis of NICM.
2-If recovery of left ventricular function is unlikely, implantation of an ICD for primary prevention can be useful between 3 and 9 months after initial diagnosis of NICM.

3-ICD is indicated in patients having sustained VT/VF, syncope, indication for permanent pacing, listed for heart transplant, implanted with ventricular assist devices

Indications for putting an atrial lead:

1. In patients with symptomatic sinus node dysfunction, an atrial lead is recommended.
2. In patients with sinus bradycardia and/or AV conduction disturbances limiting the use and/or up-titration of necessary beta-blocker or other negative chronotropic drug therapy, an atrial lead is recommended.
3. In patients with sinus rhythm who have a documented second- or third-degree AV block, but who are not otherwise candidates for cardiac resynchronization therapy, an atrial lead is recommended.
4. In patients with bradycardia-induced or pause-dependent ventricular tachyarrhythmia (such as patients with long QT syndrome and torsades de pointes) an atrial lead can be useful.
5. In patients with a documented history of atrial arrhythmias (but not in permanent atrial fibrillation), an atrial lead may be considered.
6. In patients with hypertrophic cardiomyopathy and a significant resting or provocable left ventricular outflow tract gradient, an atrial lead may be considered.

Atrial lead not indicated in:

1. In patients with no documented history of atrial arrhythmias who have no other reason for requiring an atrial lead, an atrial lead is not recommended.
2. In patients with permanent or longstanding persistent atrial fibrillation in whom efforts to restore or maintain sinus rhythm are not planned, an atrial lead is not recommended.
3. In patients with conditions likely to result in VF (rather than monomorphic or polymorphic VT) without a bradycardia-induced or pause-dependent mechanism of
initiation and no other indication for an atrial lead, an atrial lead is not recommended. Conditions likely to result in VF – idiopathic ventricular fibrillation, Brugada syndrome, catecholaminergic polymorphous ventricular tachycardia, and short QT syndrome

This article adequately covers patients who really need an ICD, but have been excluded from trials. I have made a simplified version of the document which is easy to
understand. Your comments and questions are welcome

 

 

Note:

1. The diagnostic criteria for acute MI, established by the joint ESC/ACC/AHA/WHF Task  Force, are the following:

An appropriate rise and/or fall in cardiac biomarkers with at
least one value above the 99th percentile upper reference level, together with evidence of myocardial ischemia and with at least ONE of the following:
 1. Electrocardiographic evidence of new ischemia (ST
segment shift or development of left bundle branch
block [LBBB])
2.  Evolution of pathologic Q waves on the electrocardiogram
3.  Imaging evidence of new regional wall motion abnormality
or new loss of viable myocardium
4.  Ischemic symptoms
(J Am Coll Cardiol 2012;60:1581–98.)
2. BLOCK HF trial (N Engl J Med 2013;368:1585-93)