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    A 10-year-old child who underwent coronary artery bypass graft with the indication of familial hypercholesterolemia
    (Dr Behcet Uz Cocuk Hastaliklari Ve Cerrahisi, 2018) Demircan, Tulay; Kizilca, Ozgur; Yilmaz, Nuh; Zihni, Cuneyt; Oncu, Bahattin; Kir, Mustafa; Unal, Nurettin
    Homozygous familial hypercholesterolemia (HoFH) is a result of a defect in low-density lipoprotein (LDL) receptor gene located on the short arm of chromosome 19 which leads to inadequate clearance of lipoproteins from blood circulation. Homozygous and heterozygous types are available. In homozygous types coronary lesions emerge due to early atherosclerosis in the first decade of life, and untreated patients are lost generally when they are 20 years of age. Our patient was admitted to our outpatient clinic with the complaint of exertional chest pain six months after diagnosis of familial hypercholesterolemia. Coronary angiography was performed because of the patological exercise stress test. Significant coronary artery stenosis was detected. Then the patient successfully underwent coronary bypass grafting. This case is presented to highlight that coronary lesions may develop at an early disease stage of the patients with hyperlipidemia.
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    Evaluation of left ventricular functions by speckle-tracking echocardiography in coarctation patients
    (Wiley, 2021) Demircan, Tulay; Kizilca, Ozgur; Yilmaz, Nuh; Zihni, Cuneyt; Kir, Mustafa; Unal, Nurettin
    Background/Aim Two-dimensional speckle-tracking echocardiography (2D-STE) is a novel method that allows the assessment of regional myocardial function. The aim of our study was to use 2D-STE to assess left ventricular deformation in patients with coarctation of the aorta (CoA). Methods In this prospective study, patients with CoA (n = 42) and healthy controls (n = 39) were recruited. Children with CoA who visited the outpatient clinic between 2013 and 2014 were included. The data were compared with those obtained from the sex- and age-matched controls. Results The mean age of the patients was 5.8 +/- 4.5 years. Global longitudinal strain based on all three apical views and total global strain values did not appear to be different between the patient and the control groups (P = .59, P = .51, P = .15, P = .38). Hypertension was detected in 14 (33.3%) patients with CoA. There were significant differences between the global longitudinal strain values of the normotensive CoA subgroup and the hypertensive CoA subgroup (P < .05). Conclusions In our study, we found that 2D-STE total strain analysis of patients with CoA was not different from comparative healthy controls. However, we determined that 2D-STE parameters were lower in the hypertensive CoA subgroup compared to the normotensive CoA subgroup.
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    Holt-Oram Sendromlu Bir Olgu
    (2021) Demircan, Tulay; Kızılca, Özgür; Yılmaz, Nuh; Kır, Mustafa; Ünal, Nurettin
    Holt-Oram sendromu üst ekstremite anomalileri ve doğumsal kalp hastalıkları ile karakterize, otozomal domiant katılımlı bir sendromdur. Doğumsal kalp defektlerinden en sık atriyal septal defekt (ASD) eşlik eder. Beş yaşında kız olguya; 2 yaşında iken ASD, baş parmağının trifalankslı olması ve babasında da ASD ve el anomalisi olması nedeni- yle Holt-Oram sendromu tanısı konuldu. İlk planda ASD’nin transkateter kapatılması planlandı fakat transözafagiyal ekokardiyografi ile rimlerinin uygun olmadığına karar verilerek ASD’si cerrahi olarak kapatıldı. Bu makale, üst eks- tremite anomalisi olan hastalarda doğumsal kalp defektlerininde eşlik edebileceğinin anımsanması ve ender görülen bir sendrom olan Holt-Oram sendromunun akla getirilmesi amacı ile sunuldu.