Thursday, September 16, 2021

Syncope in a young man

 Written by Pendell Meyers

Let's say a young person presented with exertional syncope. They are now at baseline, asymptomatic, normal vital signs, and they have this ECG at triage:

What do you think?

Below are two other variations of this patient's resting ECG from different time periods:

Answer: Arrhythmogenic Right Ventricular Cardiomyopathy

See the end of the post for review and details on ARVC and it's ECG findings, but suffice to say that this patient has sinus rhythm, small epsilon wave in some of his ECGs, and R waves in V1-V3 with TWI.

Here is a close up of V1-V3:

Notice the very subtle micro-voltages at the J point.  These are epsilon waves.

As a teenager, this patient was playing basketball when he suddenly collapsed and became unresponsive. EMS found him in ventricular fibrillation. He returned neurologically intact. During his hospitalization he received an ICD. Genetic testing confirmed arrhythmogenic right ventricular cardiomyopathy/dysplasia with a mutation in his desmoglein-2 gene. During hospitalization he was started in mexiletine for frequent uniform PVCs which seemed to significantly decrease ectopy burden. He was restricted from activity and later also metoprolol was added.

Over the years he has had several ICD shocks, several medication adjustments, but is otherwise doing well. 

ARVD Review below, reproduced from this post: 

Young man with syncope while riding a bike [Arrhythmogenic Right Ventricular Dysplasia (ARVD)]

ARVD, also known as arrhythmogenic RV cardiomyopathy, is estimated to have a prevalence of 1 in 5000 adults and is responsible for approximately 11% of sudden death in young adults and 22% in a study of athletes in northern Italy.  The diagnosis is not easy (see below).

There is a 2010 publication by the Task Force in Diagnosis of ARVD: Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the task force criteria. 

There are 6 categories of criteria
1) Imaging
2) Pathologic
3) ECG Repolarization
4) ECG Depolarization
5) Arrhythmias
6) Family History.  

ECG and historical Highlights of this publication are (Suspect ARVD with):
1) High risk syncope with no other etiology; Family History
2) Depolarization abnormalities (Major criteria):
        a) Epsilon Waves
        b) Localized prolongation (greater than 110 ms) of the QRS complex in right precordial leads (V1-V3)
3) Repolarization abnormalities in patients of age at least 14 years (because younger patients often have juvenile T-waves)
        a) Minor: Inverted T-waves in right precordial leads V1-V2 
        b) Major: Inverted T-waves in right precordial leads V1-V3 or beyond (major criteria) 
4) Arrhythmias
        a) Major criterion:
                i) VT of LBBB morphology with superior axis (negative or indeterminate QRS in leads II, III, aVF and positive in lead aVL) (major criteria)
        b) Minor criteria:
                i) VT of LBBB morphology with inferior axis (positive QRS in leads II, III, aVF and negative in lead aVL) (minor criteria)
                ii) More than 500 PVCs per hour
5) Finally, it is a progressive disease and patients without ECG abnormalities may develop them over time.

Here is an example of an epsilon wave (image C).  And another example.  Here are some great examples from the post on RV dysplasia (translated by Google translate!) on Pierre Taboulet's great French site:  #1#2#3

Here's a great example on Wave Maven.

Here is an explanation of the importance of leads V1 and V2.

Here is another nice example.  I've taken the liberty of blowing up part of the ECG at this link for better viewing.  Look closely at V1-V2:
There are Epsilon waves (small waves at the end of the QRS) and also a slight prolongation of the QRS at the very end. 

Some excellent references on ARVD:

This is a case report with lots of good info, from 2019, but you can only read it if you have a subscription to American J Cardiol:

Holshouser JW and Littmann L.  Usefulness of the Electrocardiogram in Establishing the Diagnosis and Prognosis of Arrhythmogenic Right Ventricular Cardiomyopathy

Other References, from the above article:
RNW Hauer, MGPJ Cox, JA GroenewegImpact of new electrocardiographic criteria in arrhythmogenic cardiomyopathy
Front Physiol, 3 (2012), p. 352
eCollection 2012
FI Marcus, WJ McKenna, D Sherrill, C Basso, B Bauce, DA Bluemke, H Calkins, D Corrado, MG Cox, JP Daubert, G Fontaine, K Gear, R Hauer, A Nava, MH Picard, N Protonotarios, JE Saffitz, DM Sanborn, JS Steinberg, H Tandri, G Thiene, JA Towbin, A Tsatsopoulou, T Wichter, W ZarebaDiagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the task force criteria
Circulation, 121 (2010), pp. 1533-1541
WC Roberts, N Kondapalli, SA HallUsefulness of total 12-lead QRS voltage for the diagnosis of arrhythmogenic right ventricular cardiomyopathy in patients with heart failure severe enough to warrant orthotopic heart transplantation and morphologic illustration of its cardiac diversity
Am J Cardiol, 122 (2018), pp. 1051-1061
JE Madias, R Bazaz, H Agarwal, M Win, L MedepalliAnasarca-mediated attenuation of the amplitude of electrocardiogram complexes: a description of a heretofore unrecognized phenomenon
J Am Coll Cardiol, 38 (2011), pp. 756-764

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