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Congenital cardiac

arrhythmias
Wolff-Parkinson-white syndrome

Described in 1930
Most common type of ventricular pre-
excitation
Anomalous atrio-ventricular conduction
pathway (kent pathway)
o Impulse from SA node AV node (delayed)
o Impulse travels through kent pathway
(undelayed)

May result in re-entry circuit consquent


supraventricular tachycardia

In 20% -> A-fib impulses conducted quickly


to ventricles risk of V-fib & sudden death.
Symptoms

Due to tachycardia:
o Palpitations
o Dizziness
o Fainting
o Fatigue with exertion
o Anxiety

First appearance in teens or in 20s


Events begin suddenly can last a few seconds or hours
Events triggered by exercise, alcohol , caffeine and other
stimuli in some people.
Diagnosis
Through EKG:
Findings:
o Normal P wave
o Shortened P-R interval
o Delta wave

EKG findings can vary from day to day/ hour to


hour
o Depending on adrenaline release
o I.e. stress, caffeine intake , exercise ..
o Leading to passage of impulse through kent
pathway
o When impulses go through AV node -> normal
EKG.
Treatment
Patients who dont experience tachycardia dont need treatment
o Sometimes, conduction through kent pathway stops with age.
o Except athletes, advised to follow up to assess risk of sudden
death

Stopping tachycarida:
o Valsalva, carotid massage
Medications
Radiofrequency ablation
Surgery (more invasive)
Long QT syndrome
Disorder of myocardial repolarization
o Typical cause = ion channel defect
Risk of sudden death due to torsades de pointes

Includes:
Romano-Ward syndrome:
o Autosomal dominant
o Pure cardiac phenotype (NO deafness)

Jervell & Lange Nielson Syndrome:


o Autosomal recessive
o Sensorineural defect
ECG findings
Brugada syndrome
Disorder characterized by
sudden death associated with
one of several ECG patterns
Incomplete right bundle-branch
block and ST-segment elevations
in the anterior precordial leads.
Polymorphic ventricular
tachycardia, which may
degenerate into ventricular
fibrillation and cause cardiac
arrest.
The Brothers
Named for the Spanish cardiologists Pedro Brugada
and Josep Brugada.
Genetics
Genetically determined and has an autosomal dominant
pattern of transmission in about 50% of familial cases
The typical patient:
Young, male
Healthy
Normal general medical and cardiovascular physical
examinations.
Alterations in the SCN5A gene, of which nearly 300
mutations have been described. Other genes have also
been reported.
Mutations in sodium ion channels of the myocytes
Signs and symptoms
Generally asymptomatic
Most are unaware
Detected by ECG pattern changes.
It's possible to have a Brugada sign, or pattern, without having Brugada
syndrome. However, signs and symptoms that could mean you have
Brugada syndrome include:
Fainting (syncope)
Irregular heartbeats or palpitations
Extremely fast and chaotic heartbeat (sudden cardiac arrest)
EKG
Brugada syndrome has three different ECG
patterns:
Type 1
Type 2
Type 3
Type 1
Type 1 has a coved type
ST elevation with at
least 2 mm (0.2 mV) J-
point elevation and a
gradually descending ST
segment followed by a
negative T-wave.
Type 2
Type 2 has a saddle-back
pattern with a least 2 mm
J-point elevation and at
least 1 mm ST elevation
with a positive or biphasic
T-wave. Type 2 pattern
can occasionally be seen
in healthy subjects.
Type 3
Type 3 has either a coved
(type 1 like) or a saddle-
back (type 2 like) pattern,
with less than 2 mm J-
point elevation and less
than 1 mm ST elevation.
Type 3 pattern is not rare
in healthy subjects.
Treatment
Implantable cardioverters-
defibrillators (ICDs)
Exposing them to
complications related to
device implantation and
the potential for
inappropriate shocks.
References
http://emedicine.medscape.com/article/163751-
overview#a1
http://www.mayoclinic.org/diseases-
conditions/brugada-
syndrome/basics/definition/con-20034848
http://www.uptodate.com/contents/wolff-
parkinson-white-syndrome-beyond-the-basics
http://www.mayoclinic.org/diseases-
conditions/long-qt-
syndrome/basics/definition/con-20025388