Lipoprotein aferezi homozigot ailevi hiperkolesterolemi hastalarında ventriküler repolarizasyonu etkiler

Yıl 2019, , 340 - 347, 30.09.2019

Firdevs Ayşenur Ekizler , Serkan Cay , Burak Acar , Bahar Tekin Tak Elif Hande Ozcan Cetin , Ahmet Temizhan

https://doi.org/10.18663/tjcl.568661

Öz

Amaç:
Homozigot ailevi hiperkolesterolemisi (HoAH)
olan hastalar erken yaşlarda kardiyovasküler hastalığa yakalanma riski altındadırlar
ve genellikle genç yaşta ani kardiyak ölüm (SCD) nedeniyle kaybedilirler. Bu
hastalarda ömrü uzatmak amacı ile tercih edilen tedavi yöntemi lipoprotein
aferezidir (LA). LA’nın kısa ve uzun dönem klinik faydalarından açıklamak için
çeşitli mekanizmalar önerilmiştir. Bu çalışmada, HoAH’si olan hastalarda tek
seans LA'nın, ventriküler repolarizasyon parametreleri üzerine olan etkisini
araştırdık.

Gereç ve Yöntemler:
LA uygulanan 11 HoAH hastası çalışmaya dahil edildi. Hastaların ortalama yaşı
30.1 ± 5,5 yıl ve hastaların %63,6’sı erkek idi. Tüm hastalara double
filtrasyon plazmaferez uygulandı.  İşlem
öncesi ve sonrasında hastaların ventriküler repolarizasyon parametrelerinden
OT, QTc aralığı, Tp-e aralığı, Tp-e / QT ve Tp-e / QTc oranı incelendi.

Bulgular:
Tek seans lipoprotein aferezi LDL'yi anlamlı bir şekilde düşürdü (10.04 ±
1.91'den 4.16 ± 1.21 mmol / L, P <001). İşlem sonrası plazma kalsiyum ve
magnezyum seviyeleri anlamlı derecede azaldı. QTc 443.8 ± 23.3 ms'den 412.3 ±
20.0 ms'ye (P <0.001) düştüğü saptandı. Tp-e aralığı ve Tp-e / QTc oranları
önemli ölçüde azaldı [85 (70-89) ms ile 63 (58-71) ms; P = 0.003 ve 0.19
(0.16-0.20) - 0.15 (0.13-0.16); P = 0,003, 
sırasıyla].

Sonuç:
Verilerimiz, tek bir lipoprotein aferez seansının bile elektrokardiyografik
repolarizasyon indekslerini iyileştirdiğini göstermiştir.

Anahtar Kelimeler

Aferez, aritmi, çift filtrasyon plazmaferez, homozigot ailesel hiperlipidemi, repolarizasyon dispersiyonu

Lipoprotein apheresis affects ventricular repolarization in patients with homozygous familial hypercholesterolemia

Öz

Aim:  Patients
with Homozygous Familial
Hypercholesterolemia (HoFH) prone to experience premature
cardiovascular disease and often die from sudden cardiac death (SCD) at a young
age. Lipoprotein
apheresis (LA) is the treatment of choice to prolong survival. Several
mechanisms has been suggested to be responsible for the known short and
long-term clinical benefits of this procedure. This study was conducted to
assess the effect of single LA on ventricular repolarization parameters in
patients with HoFH.

Material and Methods: Eleven patients (mean
age 30.1 ± 5.5 years, male 63.6%) with HoFH on chronic LA treatment were
enrolled in this preliminary study. Double filtration plasmapheresis (DFPP) was
performed in all patients. To examine the effects of a single
session of LA, on ventricular repolarization, the OT, QTc
interval, the T peak-to-end (Tp-e) interval, Tp-e/QT and the Tp-e/QTc ratio
were specifically calculated.

Results:
The single session of LA reduced total LDL (from 10.04 ± 1.91 to 4.16 ± 1.21
mmol/L, P<001). The heart rate did
not change significantly after LA session. Plasma levels of
calcium and magnesium was significantly decreased after the procedure. The QTc
decreased from 443.8 ± 23.3 ms to 412.3
± 20.0 ms (P <0.001). The Tp-e interval and the Tp-e/QTc ratio decreased significantly [85
(70-89) ms vs. 63 (58-71)ms; P =0.003,
and 0.19
(0.16-0.20) vs 0.15
(0.13-0.16); P =0.003, respectively].

Conclusion: Our
data suggest that even a
single session of LA improved electrocardiographic repolarization indexes.

Anahtar Kelimeler

apheresis, arrhythmia, dispersion of repolarization, double filtration plasmapheresis, homozygous familial hypercholesterolemia

Kaynakça

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