Ectopia cordis is a rare congenital malformation in which the heart is located partially or totally outside the thoracic cavity. The estimated prevalence of ectopia cordis is 5.5-7.9 per million births and it comprises 0.1% of congenital heart diseases. Ectopia cordis is associated with other congenital heart diseases and various tissue and organ disorders. Common cardiac anomalies associated with ectopia cordis include ventricular septal defect, atrial septal defect, pulmonary stenosis, right ventricular diverticulum, double right ventricular outflow tract and tetralogy of Fallot. Extracardiac anomalies associated with ectopia cordis reported in the literature include omphalocele, gastrochisis, cleft lip and palate, scollosis and central nervous system malformations. Here we report a newborn with ectopia cordis who was diagnosed prenatally. (Turk Pediatri Ars 2015; 50: 129-31)
The study concluded that 2D-speckle tracking echocardiography method can determine cardiac involvement earlier than conventional echocardiography in children with chronic kidney disease having preserved ejection fraction.
Objective: The aim was to evaluate the long-term changes in systolic and diastolic functions with tissue Doppler imaging in children with sickle cell anemia. Materials and Methods: In this study, myocardial performance index of ventricles and mitral and tricuspid valve E/A ratios were calculated. Wall motion velocities of the interventricular septum, right and left ventricles, and mitral and tricuspid annulus were assessed during the systole (Sm), the early diastole (Em), and the late diastole (Am) with pulsed Doppler echocardiography. Results: These diagnostic parameters were obtained from 38 patients (mean 12.5 ± 3.2 years old) who were reached after a mean 6.7 years in long-term follow-up. Left ventricular and septal end-systolic and end-diastolic diameters, and right and left ventricular myocardial performance index were higher in patients ( P < .05 and P = .001, respectively). The Em, Am, and Sm velocities of the right ventricle and septum were significantly lower in the patients at the baseline measures ( P < .05) and all these velocities decreased significantly in patients over time compared with their baseline values ( P < .05). In addition, it was observed that myocardial performance index values of the right and left ventricles increased significantly over time ( P < .05). Conclusion: This is the first study reflecting the age-related changes in global systolic and diastolic functions in sickle cell anemia by serial imaging.
Amaç: Kardiyak cerrahi sonrası gelişen aritmiler, sık görülen komplikasyon olmasına rağmen, genellikle önceden bir risk belirlemek olanaksızdır. Bu çalışmanın amacı pediatrik popülasyonda kardiyak operasyon sonrası gelişen ritim bozukluklarının tür ve sıklığını saptamaktır. Yöntem: Ocak 2008-Aralık 2015 tarihleri arasında çocuk kardiyoloji ünitesinde izlenen kardiyak operasyon geçirmiş, 2-17 yaş arası, 124 pediatrik hastanın (59'u kız, 65'i erkek) 24 saatlik holtermonitorizasyon kayıtları retrospektif olarak değerlendirildi. Hastaların demografik özellikleri, preoperatif tanıları ve geçirdikleri cerrahi girişim tipleri kaydedildi. Bulgular: Altmış sekiz hastada (%54.8) aritmi olduğu saptandı. En sık görülen ritim bozuklukları supraventriküler ekstrasistol (SVE) ve ventriküler ekstrasistol (VES) olup her ikisinin de görülme sıklığı %15.3 idi. Bu iki ritim bozukluğunun birlikte görülme oranı % 12.9 bulundu. Kızlarda SVE ve VES görülme oranı biraz daha yüksekti. SVE ve VES riski sırasıyla atriyal septal defekt tamirinde %26.3 ve %7.9, ventriküler septal defekt tamirinde %10.5 ve %15.8, tüm düzeltme yapılan Fallottetralojisinde ise %4.5 ve %9.1 bulundu. Atriyal septal defekt tamiri yapılan bir hastada supraventriküler taşikardi, ventriküler septal defekt tamiri ve tüm düzeltme yapılan Fallottetralojisi olan iki hastada ise sürekli olmayan ventriküler taşikardi izlendi. Sonuç: Kardiyak operasyon geçiren hastaların izleminde aritmi gelişebileceği bilinmeli ve hastalara düzenli holter EKG izlemleri yapılmalıdır. Bu çalışmada en sık görülen postoperatif aritmi tiplerinin SVE ve VES olduğu ve atriyal septal defekt, ventriküler septal defekt ve Fallottetralojisi operasyonlarının aritmiler için risk faktörü olduğu gösterilmiştir.
Although there is increased survival and quality of life for thalassemia patients, which results from a genetic defect in globin synthesis, heart diseases are still important causes of morbidity and mortality related to the iron burden. Therefore, we aimed to evaluate the cardiac functions of thalassemia patients followed up in Hatay State Hospital using echocardiography. Method: A total of 101 patients with 7 thalassemia intermedia and 94 thalassemia major who were followed at Hatay state hospital were examined retrospectively. Of patients, 47 were male and 54 were female. Their ages ranged between 1-17 years (mean 8.6±4.6 years). M-mode echocardiography was used to measure LVEDd (left ventricular enddiastolic diameter) and EF (ejection fraction). The demographic and laboratory values of the patients were obtained from their files. Results: The mean of LVEDd (mm) and EF (%) were evaluated as 34.8±6.1, and 66.36±1.4 with M-mode echocardiography, respectively. When the correlation between age, ferritin and LVEDd and EF were evaluated; it was determined that there was a high positive correlation (r=0.649) between age and LVEDd (p<0.01). A negative correlation was detected between ferritin and EF (r=-0.271) (p<0.01). EF was found to be significantly lower in the group with ferritin <2000. LVEDd was found to be increased in the group with ferritin >2000, although it was not statistically significant. Conclusions: In our study, it was shown that EF was decreased with increasing ferritin and LVEDS was increased with increasing age of patients with thalassemia. The ideal ferritin level which was ensured after suitable chelation therapy should be an important goal to reduce cardiac involvement.
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