Is this Type 2 Brugada syndrome/ECG pattern?

Introduction

Types 2 and 3 Brugada have been merged into Type 2.  The diagnoses have been perplexing for years because they have been based on a "saddleback" morphology.  However, saddleback morphology is a very common normal variant: "saddleback" morphology is rarely Brugada pattern and even more rarely Brugada syndrome.

Case 1

A young athlete presented with syncope.  Here was his ECG:
There is an rSr' with ST elevation and postive T-wave, creating a "Saddleback" in both V1 and V2

Is this Type 2, or Type 3, Brugada pattern?

Brugada syndrome is a condition which can lead to polymorphic ventricular tachycardia, ventricular fibrillation, and sudden death.  Identifying it before death is important.

Brugada syndrome requires BOTH 1) Brugada ECG pattern and 2) clinical criteria.

Here is an example of type I Brugada pattern during fever that disappeared after antipyresis.  It can be transitory and uncovered in a variety of situations (see below).

The recognition and management of Type 1 Brugada pattern ECG is not entirely straightforward, but, in my opinion, it is far easier than the recognition and management of patients with possible Type 2/3 Brugada (Type 3 has morphology has now been incorporated into type 2).   Here one of the Brugada brothers outlines the management of possible Brugada syndrome, but does not discuss type 2 in enough detail for me to understand it.

Many articles and web sites simply state that a Type 2 Brugada pattern has a "saddleback" in V2.   They discuss it no further.  This is not helpful, because a saddleback is a very common pattern in V2 (up to 7% in of patients with no apparent heart disease) and is a normal variant in the vast majority of cases, especially in athletes.

Saddleback is called saddleback because of an r'-wave (rSr') with an upright T-wave, forming a saddle.  This is also known as right ventricular conduction delay, and, if the QRS is wide enough (but not greater than 120 ms), it is incomplete right bundle branch block (I-RBBB).

How does a non-electrophysiologist go about suspecting Type 2 Brugada?

Again, one must distinguish between the syndrome and the ECG morphology.  The morphology alone is not enough for the diagnosis.  There must also be the right clinical context (see below) or positive electrophysiologic testing.

How do we recognize type 2 Brugada morphology on the ECG?  Fortunately, there was a consensus paper on this topic written by Bayes de Luna and the Brugada brothers.  Here is the full text of it.

There is also a very nice paper (full text html link): Differential diagnosis of rSr' pattern in Leads V1-V2: Comprehensive Review and Proposed Algorithm.  This is by the same authors of the new criteria for type 2 Brugada, so it is fairly authoritative.  (Here is a link to the full text pfd).

Here is a differential diagnosis of rSr', modified from the rSr' paper:

A. Benign Patterns:  -r' is of fast inscription, unlike in RBBB or Brugada, both of which have a wider R'.  Caused by:
   1. Higher placement of leads V1, V2
   2. Normal variant, with late activation of the posterobasal LV
   3. Incomplete RBBB, with delayed conduction through right bundle.  No associated adverse outcomes.  However, there is a higher incidence of subsequent development of complete RBBB.
   4. Athletes: 35-50% due to physiologic RV enlargement
   5. Pectus Excavatum, due to change of heart location in chest

B. Pathological Patterns: -r' often taller then -r, with slower ascent/descent
   1. Type 2 Brugada pattern
   2. RV enlargement, hypertrophy from a variety of pathologies
   3. Arrhythmogenic RV dysplasia (ARVD)
   4. WPW
   5. Hyperkalemia
   6. Na channel blockers (anti-dysrhythmics, TCAs)



Let's go back to our case, with more detail:

A young athlete had a painful condition for which he was seen in the ED.  After an episode of pain, he had a syncopal spell with complete loss of consciousness and postural tone, with spontaneous awakening.  There was a prodrome which seemed vasovagal in character.  He had started having lower abdominal discomfort and pressure, associated with some diaphoresis, then started feeling dizzy and passed out.  He spontaneously regained consciousness.  He had no prior history of syncopal spells.   There was no family history of premature sudden cardiac arrest in first degree relatives. There was no history of exertional syncope or post-exertional syncope.  He had a normal exam in the ED.

Here is the ECG again:

Is it type 2 Brugada pattern?
Is it type 2 Brugada syndrome?


The short answer is NO.

1. It is not type 1 Brugada.
2. The takeoff/downstroke of the ST segment in V1 and V2 is not flat enough ("beta" angle not wide enough) for it to be type 2 Brugada.  A pronounced r'-wave is common in athletes.

Here is the long answer

Below I summarize the paper I mentioned above, entitled (with full text link): Current electrocardiographic criteria for diagnosis of Brugada pattern: a consensus report

Diagnosis of Brugada Syndrome requires both:

1. Brugada pattern ECG (either Brugada Type 1, or the newly defined Brugada Type 2)
Findings may be dynamic and are sometimes concealed; findings may be observed only in certain circumstances such as fever, intoxication, electrolyte imbalance, presence of sodium channel medications/drugs, or vagal stimulation.
2. At least one of the following:
(a) survivor of cardiac arrest,
(b) witnessed/recorded polymorphic ventricular tachycardia (VT),
(c) history of nonvagal syncope,
(d) familial antecedents of sudden death in patients younger than 45 years without acute coronary
syndrome
(e) Type 1 Brugada pattern in relatives.

Type 1 Morphology

Here is Type 1 pattern in V1 and/or V2, which is quite recognizable:
R' wave must have amplitude of at least 2 mm
Corrado index (see below) = 1.5/0.5 = 3.0. Thus it is greater than 1.0.
Criteria for Type 1 Morphology:
1. R'-wave at least 2 mm in V1 or V2
2. But no distinct R'-wave because the ST segment takes off at an angle from the peak
3. The ST segment is convex upward ("coved"). [They use terminology of "concave downward"]
4. The peak at the high takeoff does not correspond with the J-point.  It is BEFORE the J-point, as measured in other leads (such as lead II across the bottom).
5. Gradual downsloping of ST segment such that at 40 ms after the takeoff, the decrease in amplitude is less than 4 mm (in this example, it is less than 1 mm).  In normal RBBB, the decrease in amplitude is much greater (see this example).
6. ST is followed by a symmetrically negative T-wave
7. "The duration of QRS is longer than in RBBB," and "there is a mismatch between V1 and V6." This criterion is perplexing and not well explained.
8. The downsloping should be such that the Corrado index is greater than 1.0 (see example above).
This is the ratio: [ST elevation at the J-point] divided by [ST elevation at 80 ms after the J-point].

In athletes, the index is less than 1.0 due to a horizontal or upsloping elevated ST segment, as here:
Corrado index less than 1 in an athlete, as ST segment slopes up


Criteria for Type 2 Brugada:

In the consensus paper we are discussing here, types 2 and 3 Brugada have been merged into one type called type 2. They specify the criteria to be used (below).

Here is an example from the article:
First, there must be:
a) An RSr' with a typical saddleback pattern in V1 and/or V2.
b) V1 may have either an upright, flat, or inverted T-wave (in our case above it is inverted).
c) T-wave in V2 is usually but not always positive.
d) Minimum ST segment ascent of 0.5 mm.  There could be no saddle without an ascent.

Once these are fulfilled, there should be, in lead V2:

1.  High take-off of the descending limb of the r' at least 2 mm above the isoelectric line (in our case, it is greater than 2 mm).   The r'-wave is thus not distinct, as it is in benign causes of rSr'

2.  Mismatch between QRS duration in leads V1 and V6 (longer in lead V1).  This helps to distinguish from RBBB, in which the QRS duration is equal in V1 and V6.

3. As with Type 1, the peak of the r'-wave does not correspond to the J-point in other leads.

4. The base of the triangle outlined should be longer than 3.5 mm.  This confirms that the slope of the ST segment is flat enough for the diagnosis.  I explain this in an annotated version here:
Explanation
1. Draw a horizontal line from top of r' wave (black line 1)
2. Draw a horizontal line 5 mm below this (green line 2)
3. Extend the downsloping r'-ST segment (black line 3) until it intersects the green line
4. Measure the base.  

If greater than 3.5 mm, then meets criteria (this is equivalent to a 35 degree beta angle)


I have done this with lead V1 of our ECG:
The distance from the S-wave to the almost vertical line is less than 3.5 mm, so does not meet criteria.  
How about in lead V2?
This drawing is incorrect, and only to show how it should NOT be done.
Both lines must be drawn from where the downsloping begins at the top of the r' wave.

The data for this comes from this study comparing patients with known high risk type 2 Brugada syndrome to athletes with rSr'.  The full text can be found here.

The criteria as studied in these comparison populations were approximately 90% sensitive and 90% specific in this study.


Case Progress:

This was recorded later:
All the suspicious findings are gone. There is only normal variant ST elevation (early repol)



The findings of Brugada can be unmasked by recording V1 and V2 at higher interspaces (see this paper).

This was done here:
V1, V2 recorded one interspace higher, does not uncover type 1 Brugada pattern.  It does show a pronounced rSr'
Recording V1 and V2 one or two interspaces higher, over the area of the heart that is most likely to produce the Brugada pattern, should make the pattern more apparent.  If it did so, that would be very suggestive of Brugada.
Notice that there is T-wave inversion in V2 (but not Brugada pattern!).  
High lead placement is one of the causes of "abnormal" T-wave inversion in V2 and beyond. 

This recording also suggests that the initial ECG was recorded too high, as it is very similar to that first ECG



Additional etiologies were considered for his syncope given he is a collegiate athlete including HOCM, catecholaminergic polymorphic VT, and arrhythmogenic right ventricular dysplasia. Transthoracic echo did not show any evidence of HOCM or right ventricular dysplasia.  EF was normal. He had no known family history of sudden cardiac death at a young age.

The electrophysiologist was confident that this was not Brugada, that a procainamide challenge test could be done but that this would normally be done only for "unexplained" syncope, not for typical vasovagal syncope.  The patient was discharged with no exercise limitations.  He left open the possibility of an exercise test to rule out catecholaminergic polymorphic VT (CPVT), which occurs during stress and exercise.  However, without an exertional component, the probability of CPVT was very low.

Case Conclusion

In fact, this ECG is either:
1) a typical athlete's mimic of type 2 Brugada, or 
2) a too-high recording, or 
3) BOTH.  


Case 2

Here is a patient who presented with recurrent pre-syncope and palpitations.

Here is lead V2 blown up:
The base of the triangle is about 3.5 mm, and it meets the other criteria.
The electrophysiologist was worried enough about type 2 Brugada that he placed an implantable loop recorder.


Case 3

This 40-something patient presented with dizziness and chest pain.  The dizziness seemed to be vertigo more than pre-syncope.


Here the lines are drawn:
The base is greater than 3.5 mm, and other criteria for type 2 Brugada Pattern are present.
In this case, the patient underwent a stress test for his chest pain, but the diagnosis of type 2 Brugada was dismissed because of the absence of clinical criteria.  Dizziness due to vertigo was not enough.

There is Brugada Pattern, but not Brugada syndrome




Here is a comprehensive article inherited dysrhythmias: Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes

A Male in his 50’s with epigastric pain

This was sent by a recent residency graduate who works at a hospital without a cath lab.  As you might imagine, our graduates have learned to scrutinize ECGs for subtle findings:

This middle-aged male with a history of smoking and alcohol use who had not had medical contact in many years presented with sharp, severe, "indigestion" in his epigastrium.  Medics gave him nitro and aspirin without improvement.  He received more nitro and also a "GI cocktail" in the ED without improvement.

Here is his initial ECG:
There is subtle scooped ST depression in III and aVF, and subtle ST elevation in aVL  (and also I).  The T-wave in aVL is very large compared to the QRS.  There is also a "down-up" (reverse biphasic) T-wave in V2.  This finding is very suspicious for posterior MI.
Since lateral and posterior MI are in one vascular territory (the circumflex), these findings are highly suspicious for circumflex occlusion.

Remember that circumflex occlusion results in diagnostic ST elevation (1 mm in two consecutive leads) in only about 50% of cases.  The lateral wall is often referred to as being "electrocardiographicaly silent".

Part of the reason for this is that the QRS axis is often perpendicular to lead aVL, so there is often very little QRS voltage in aVL: when there is low QRS voltage, there is also low T-wave voltage.  So one must rely an subtle findings that do not reach 1 mm if one is to make the diagnosis.

Often the findings of ST elevation in aVL are best seen in the reciprocal ST depression in inferior leads.

Here is another case for contrast, in which ST elevation in aVL with reciprocal ST depression in lead III is a false positive:
 
http://hqmeded-ecg.blogspot.com/2011/12/subtle-lateral-st-elevation-false.html
This was a false positive cath lab activation.
Here there is a lot of voltage in aVL, so you can't blame subtle ST elevation on lack of QRS voltage. 


Case continued:

The physician was worried about all these findings but was reluctant to activate.  The first troponin I returned at 0.031 ng/mL (less than 99% cutoff of 0.32).

At one hour, with the patient still in pain, he repeated the ECG:
There are some subtle changes: mostly a more upright T-wave in III and V2

The physician started a nitro drip and heparin and consulted a cardiologist at a referral institution.

The patient's pain increased while waiting for transport and another ECG was recorded:
No significant change

Just after leaving, the second troponin I returned at 0.90 ng/mL (positive).  The physician called the referral hospital to recommend immediate cath given a now objective diagnosis of NonSTEMI and with pain refractory to medical therapy.

On arrival, he did not go immediately to the cath lab.

Later that night he did go.  The circumstances surrounding that delayed decision are uncertain.

He had a 100% mid circumflex occlusion with otherwise clean coronaries.  It was stented.

I do not have the peak troponin or echo results.

Learning points:

1. Many occlusions do not reach 1 mm of ST Elevation.  These NonSTEMIs with occlusion can be recognized and need immediate cath lab activation.

2. The ACC/AHA recommends immediate cath for patients with ACS who have uncontrolled symptoms.

A Male in his 50’s with epigastric pain

This was sent by a recent residency graduate who works at a hospital without a cath lab.  As you might imagine, our graduates have learned to scrutinize ECGs for subtle findings:

This middle-aged male with a history of smoking and alcohol use who had not had medical contact in many years presented with sharp, severe, "indigestion" in his epigastrium.  Medics gave him nitro and aspirin without improvement.  He received more nitro and also a "GI cocktail" in the ED without improvement.

Here is his initial ECG:
There is subtle scooped ST depression in III and aVF, and subtle ST elevation in aVL  (and also I).  The T-wave in aVL is very large compared to the QRS.  There is also a "down-up" (reverse biphasic) T-wave in V2.  This finding is very suspicious for posterior MI.
Since lateral and posterior MI are in one vascular territory (the circumflex), these findings are highly suspicious for circumflex occlusion.

Remember that circumflex occlusion results in diagnostic ST elevation (1 mm in two consecutive leads) in only about 50% of cases.  The lateral wall is often referred to as being "electrocardiographicaly silent".

Part of the reason for this is that the QRS axis is often perpendicular to lead aVL, so there is often very little QRS voltage in aVL: when there is low QRS voltage, there is also low T-wave voltage.  So one must rely an subtle findings that do not reach 1 mm if one is to make the diagnosis.

Often the findings of ST elevation in aVL are best seen in the reciprocal ST depression in inferior leads.

Here is another case for contrast, in which ST elevation in aVL with reciprocal ST depression in lead III is a false positive:
 
http://hqmeded-ecg.blogspot.com/2011/12/subtle-lateral-st-elevation-false.html
This was a false positive cath lab activation.
Here there is a lot of voltage in aVL, so you can't blame subtle ST elevation on lack of QRS voltage. 


Case continued:

The physician was worried about all these findings but was reluctant to activate.  The first troponin I returned at 0.031 ng/mL (less than 99% cutoff of 0.32).

At one hour, with the patient still in pain, he repeated the ECG:
There are some subtle changes: mostly a more upright T-wave in III and V2

The physician started a nitro drip and heparin and consulted a cardiologist at a referral institution.

The patient's pain increased while waiting for transport and another ECG was recorded:
No significant change

Just after leaving, the second troponin I returned at 0.90 ng/mL (positive).  The physician called the referral hospital to recommend immediate cath given a now objective diagnosis of NonSTEMI and with pain refractory to medical therapy.

On arrival, he did not go immediately to the cath lab.

Later that night he did go.  The circumstances surrounding that delayed decision are uncertain.

He had a 100% mid circumflex occlusion with otherwise clean coronaries.  It was stented.

I do not have the peak troponin or echo results.

Learning points:

1. Many occlusions do not reach 1 mm of ST Elevation.  These NonSTEMIs with occlusion can be recognized and need immediate cath lab activation.

2. The ACC/AHA recommends immediate cath for patients with ACS who have uncontrolled symptoms.

A Male in his 50’s with epigastric pain

This was sent by a recent residency graduate who works at a hospital without a cath lab.  As you might imagine, our graduates have learned to scrutinize ECGs for subtle findings:

This middle-aged male with a history of smoking and alcohol use who had not had medical contact in many years presented with sharp, severe, "indigestion" in his epigastrium.  Medics gave him nitro and aspirin without improvement.  He received more nitro and also a "GI cocktail" in the ED without improvement.

Here is his initial ECG:
There is subtle scooped ST depression in III and aVF, and subtle ST elevation in aVL  (and also I).  The T-wave in aVL is very large compared to the QRS.  There is also a "down-up" (reverse biphasic) T-wave in V2.  This finding is very suspicious for posterior MI.
Since lateral and posterior MI are in one vascular territory (the circumflex), these findings are highly suspicious for circumflex occlusion.

Remember that circumflex occlusion results in diagnostic ST elevation (1 mm in two consecutive leads) in only about 50% of cases.  The lateral wall is often referred to as being "electrocardiographicaly silent".

Part of the reason for this is that the QRS axis is often perpendicular to lead aVL, so there is often very little QRS voltage in aVL: when there is low QRS voltage, there is also low T-wave voltage.  So one must rely an subtle findings that do not reach 1 mm if one is to make the diagnosis.

Often the findings of ST elevation in aVL are best seen in the reciprocal ST depression in inferior leads.

Here is another case for contrast, in which ST elevation in aVL with reciprocal ST depression in lead III is a false positive:
 
http://hqmeded-ecg.blogspot.com/2011/12/subtle-lateral-st-elevation-false.html
This was a false positive cath lab activation.
Here there is a lot of voltage in aVL, so you can't blame subtle ST elevation on lack of QRS voltage. 


Case continued:

The physician was worried about all these findings but was reluctant to activate.  The first troponin I returned at 0.031 ng/mL (less than 99% cutoff of 0.32).

At one hour, with the patient still in pain, he repeated the ECG:
There are some subtle changes: mostly a more upright T-wave in III and V2

The physician started a nitro drip and heparin and consulted a cardiologist at a referral institution.

The patient's pain increased while waiting for transport and another ECG was recorded:
No significant change

Just after leaving, the second troponin I returned at 0.90 ng/mL (positive).  The physician called the referral hospital to recommend immediate cath given a now objective diagnosis of NonSTEMI and with pain refractory to medical therapy.

On arrival, he did not go immediately to the cath lab.

Later that night he did go.  The circumstances surrounding that delayed decision are uncertain.

He had a 100% mid circumflex occlusion with otherwise clean coronaries.  It was stented.

I do not have the peak troponin or echo results.

Learning points:

1. Many occlusions do not reach 1 mm of ST Elevation.  These NonSTEMIs with occlusion can be recognized and need immediate cath lab activation.

2. The ACC/AHA recommends immediate cath for patients with ACS who have uncontrolled symptoms.

Chest Pain, LVH with Incomplete LBBB, and ST Elevation

A middle aged male with history of bicuspid aortic valve and aortic stenosis complained of 30 minutes of chest pain and dyspnea.  He described it as tightness and pressure.  It resolved with nitroglycerine.   Vital signs were normal and he appeared well.

Here is the initial ECG:
There is profound LVH with incomplete LBBB, with a QRS of 118 ms.  There is marked ST elevation in precordial leads. Although this can be seen with this degree of LVH and incomplete LBBB, one must entertain the possibility of anterior STEMI.
The ST/S ratio is 5/28 in lead V2, for a ratio of 0.18.   

Though 0.18 is less than 0.25, and of course also less than 0.20, it is still quite a high ratio.   A mean maximal ST/S ratio for a cohort of patients with complete LBBB is 0.10 (95% CI: 0.9-0.11).  


Worried about STEMI, a bedside echo was immediately recorded. Here is the parasternal long axis:

There is profound LVH and good wall motion

Here is a still image:
The arrows highlight the wide aortic root

A parasternal short axis was done:


This shows profound LVH with overall good wall motion.  In particular, the anterior wall and septum have good wall motion.  This makes anterior STEMI very unlikely.

The aortic root was not initially noticed, but the physicians were confident there was no STEMI.



The initial troponin I returned elevated at 0.128 ng/mL, at which point another ECG was recorded:
No change, consistent with the interpretation that this is the baseline ECG

The on-call cardiologist was consulted for management of this patient with a positive troponin and worrisome ST segments.  Because of the history of bicuspid aortic valve, the cardiologist was concerned about possible Aortic Dissection and suggested a chest CT.

After a second look at the echo, there was more suspicion of dissection, and a CT was ordered.  Here is one image:
This shows a large aortic root aneurysm that also has a subtle dissection (low density thin lines in the midst of the contrast is a dissection flap)

A repeat ECG was essentially unchanged.

Consistent with massive LVH



The patient went to the Operating Room.

(I am not completely certain that there was no involvement of the coronary cusps, with resulting partial occlusion of the left main and resulting NonSTEMI, but the serial ECGs do not support this).

Learning points:

1) Massive LVH can evolve over time to incomplete, or complete, LBBB, and it can have ST elevation that mimics STEMI.
2) Use ED echo to confirm good wall motion when there is anterior ST elevation due to LVH
3) Don't forget aortic dissection!

Paced rhythm. Is there Ischemic ST elevation?

An elderly male with a dual chamber pacemaker and severe dilated non-ischemic cardiomyopathy presented with dyspnea.  He had not had an angiogram in 10 years.  Although he had severe heart failure, the etiology of acute dyspnea was not readily apparent.  The differential was pneumonia/sepsis, heart failure exacerbation, pulmonary embolism, or possibly ACS.

He had an ECG recorded:
There are P-waves but a ventricular paced rhythm.  The heart rate is 118.
Is there excessive discordant ST elevation in anterior leads?
ST/S ratios have not been studied for paced rhythm, but we have studied them for LBBB, and perhaps this knowledge can be applied to paced rhythm (?)  This is uncertain, but I do it frequently and I think it works. 
If we do apply these rules, the ST/S ratio is highest in V2 and V3.  In these leads, the STE at the J-point is 4 mm in V2 and 4-5 mm in V3, with a 27-28 mm S-wave, for a max ratio somewhere between 0.145 - 0.185.

These ratios are a bit higher than normal for a maximal ST/S ratio, but they are not higher than cutoffs of 0.20 (more sensitive, less specific) or 0.25 (very specific).  

So this is unlikely to represent acute anterior STEMI, but let's compare to a previous ECG:
There is much less STE here.  The ratio is closer to 0.06.

Should you worry that there is an increase in the ST elevation and ST/S ratio?  Normally, yes.

But look at the heart rate: the bottom ECG has a heart rate of 79.  The top one has a rate of 118.

Tachycardia results in increased discordant ST elevation in paced rhythm and in LBBB.  All of this ST segment shift can be attributed to tachycardia.

Outcome:

 Troponins were mildly elevated (up to 0.176 ng/mL).  The patient was diagnosed with acute decompensated heart failure.   As the heart failure was managed, and the heart rate decreased, the ST segments shifted down.  There was no wall motion abnormality.

Exacerbation of heart failure can also exaggerate ST elevation in Paced rhythm and LBBB, as demonstrated in this case.



Learning Points:

1.  Tachycardia can exaggerate the appropriately discordant ST elevation in paced rhythm (and in LBBB)

2. Heart Failure can also exaggerate this ST elevation.