A century Saga

It was the late 1915 when the little son of a merchandise family in the city of Shkodra died at age of 6 months after some days of high fewer. The medical personnel of the time couldn't help them, and had no explanation for the cause of death. The parents get depressed, but they wanted to have children. So that they tried again and again. From 1915 till 1945 they give to birth 15 children, of whom 12 died in ages between 6 months and 6 years old, majority of them during periods with high fever like during measles. Only 3 of them survived, 2 boys and a girl, which growth normally and have had siblings. One day one of these sibling, a 50 years old man, come to our clinic for a routine cardiovascular check up. His only complain was "some times I fell my heart beating irregularly for some seconds" but no syncope. 

He didn't smoke, no high blood pressure, no diabetes, no dislipidemia. 

The resting ECG meet the criteria of Brugada Syndrome, and in these settings we monitored the patient with a 24 h Holter of rhythm and there get registered about 200 PVC with the tendency R on T. 

Echocardiogram was normal. 

The patient was referred in an Inherited Arrhythmia Clinic in Italy where even his children get screened. 

The case was considered not a high risk patient for Sudden Cardiac Death (SCD) and didn't meet the criteria for ICD implantation, ( IIa ). We recommended him to not use alcohol and some drugs that can aggravate his disease, and to do frequent follow up cardiology visits. 

Brugada Syndrome is the most probable cause of deaths in young age of the other members of this family. 

Nje Sage shekullore
Dy shoke te vecante                                                                                                              Tetor 2017

Ishte viti 1915 kur djali 6 muajsh i nje familjeje tregetare shkodrane vdiq pas nje periudhe disa ditore me temperature te larte. Personeli shendetesor i kohes nuk arriti ti ndihmonte dhe nuk munden ti jepnin shpjegim per shkakun e vdekjes se femijes. Prinderit megjithese te merzitur, nuk hoqen dore nga deshira e tyre per te patur femije. Keshtu qe ato provuan perseri dhe perseri. Ne harkun kohor 1915-1945 ato linden 15 femije, nga te cilet 12 vdiqen ne moshen  6 muajsh deri 6 vjec , ne pergjithesi gjate episodeve me temperature te larte. Vetem 3 prej tyre mbijetuan, 2 djem dhe 1 vajze , te cilet u rriten normalisht dhe lane pasardhes. Nje dite, nje nga keto pasardhes, burre 50 vjec, erdhi ne kliniken tone per nje check up kardiologjik. E vetmja e ankese e tij ishin rrahje te crregullta te zemres, me kohezgjatje pak seconda, pa episode te fikti. Ai nuk pinte duhan, nuk kishte diabet, pa dislipidemi, pa hypertension. 

EKG-ja e qetesise plotesonte kriteret e Syndromes Brugada. Eko-ja e zemres ishte brenda normes. Ne keto kushte pacienti u monitorua me Holter Ritmi 24 oresh ku u konstatuan rreth 200 ekstrasistola ventrikulare me tendency R mbi T.

Pacienti u referua ne nje Klinike te Aritmive te Trasheguara ne Itali, ku iu krye edhe screening i femijeve. Rasti u konsiderua si jo me rrezik te larte per te zhvilluar Vdekje te Papritur dhe nuk i plotesonte kriteret absolute per te vendosur defibrillator te implantueshem (ICD) (IIa). Ne i rekomanduam pacientit qe te mos perdori alkol si dhe te shmangte nje seri medikamentesh qe e perkeqesojne semundjen e tij , si dhe te ishte nen kontroll te vazhdueshem nga mjeku kardiolog. 

Sindromi Brugada eshte shkaku me i mundshme i vdekjeve ne moshat e reja tek kjo familje. 

Mashkull 40 vjec paraqitet me ankesen e palpitacioneve te theksuara kur perdor produkte qe permbajne kafeine dhe ne efort fizik.           

EKG = Ritem sinusal, bllok i deges se djathte inkomplet, HR 70 bpm

EKO Doppler e zemres konstatoi bashke ekzistencen e dy anomalive te lindura te zemres: Ebstein Anomaly dhe Yamaguchi Cardiomyopathy. 

Anomalia Ebstein ka nje incidence 1 ne 200 000 lindje te gjalla, nderkohe qe incidenca e Yamaguchi cardiomyopathy eshte rreth 1 ne 100 000 lindje dhe me e shpeshte ne popullin japonez. Gjetja e njekohshme tek nje pacient e qe te dy ketyre patologjive te rralla nuk eshte pershkruar asnjehere ne literaturen mjekesore. 

Meqenese ky pacient ka mundesi qe te shvilloje aritmi komplekse, u monitorua me Holter te Ritmit te zemres. Ne te nuk u konstatuan aritmi madhore dhe pacientit iu rekomandua nderprerja e pijeve energjetike dhe me permbajtje kafeine si dhe evitimi i sforcimeve fizike dhe sporteve kompetitive. 

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