Ventricular Tachycardia in Kearns–Sayre
Syndrome: A Case Report
Hamid BARAKPOUR, M.D., Zahra EMKANJOO, M.D., Hamid BONAKDAR, M.D.,
Abolfath ALIZADEH, M.D., Amir Farjam FAZELIFAR, M.D., Majid HAGHJOO, M.D.,
Mohammad Ali SADR–AMELI, M.D.
Rajaie Cardiovascular Research and Medical Centre, Pacemaker and Electrophysiology Department, Tehran, Iran
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Kearns-Sayre Sendromu’nda Ventriküler Taşikardi:
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Rajaie Cardiovascular Research and Medical Centre, Pacemaker and Electrophysiology Department, Tehran, Iran
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earns–Sayre is a rare syndrome described
in 1958 (1), which is often related to deletion of mitochondrial DNA. This syndrome is
noted by the combination of progressive ophthalmoplegia, sensory neural deafness, pigmentary retinopathy and cardiac conduction disorders. Sudden cardiac death in patients with KSS
has been reported as high as 20% in one study
(2) and this is of major concern given that 57%
of patients with KSS have clinical manifestations of cardiac disease (3). This is the history of
one of Iranian leading horsemen who has challenged this syndrome all his life.
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The patient was born in 1977. He had a normal birth but his problems started within the
first 2 years with repeated vomiting. He used to
get bouts of vomiting up to twice per week often
with gaps of 1–2 months. Later years his vomiting was associated with headache which was often throbbing and bifrontal. He was also noticed
to be of short stature and fairly thin. His complete endocrinological investigations at the age
of 15 were normal except for raised bilirubin
which was thought to be due to Gilbert,s disease.
He also had an ECG which showed RBBB and
left axis deviation. He was given some dietary
advice. At the age of 16 he was noted to have decreased hearing and mild ptosis. An ENT specialist confirmed bilateral sensory motor hearing loss. Other endocrinological results showed
his serum and RBC folate was low. At the age of
20 he had a sudden syncopal attack while sitting
and he was noted to have complete heart block.
A pacemaker VDD was inserted for him. Full
assessment by a cardiologist in 1998 revealed no
other cardiac problem. He then started to have
mild unsteadiness especially at night and his
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headaches became more frequent. He had no
family history of neurological or cardiac diseases except for mild epilepsy in his mother. At the
age of 24 he had a sudden episode of numbness
in the left upper limb lasting for around 3 minutes associated with slight blurred vision. Later he was seen by an ophthalmologist because
of decreased vision at night that also found evidences of pigmentary retinopathy, ophthalmoplegia and made a diagnosis of Kearns–Sayre
syndrome.
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Ventricular Tachycardia in Kearns–Sayre Syndrome