Firdevs Ayşenur EKİZLER, Serkan ÇAY, Burak AÇAR, Bahar TEKİN TAK, Elif Hande ÖZCAN ÇETİN, Ahmet TEMİZHAN

Lipoprotein aferezi homozigot ailevi hiperkolesterolemi hastalarında ventriküler repolarizasyonu etkiler

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

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.

Keywords:

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

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