Dilek Giray

Prognostic markers in congenital diaphragmatic hernia: Left ventricular diameter and pulmonary hypertension

The aim of this study was to investigate whether the outcome of congenital diaphragmatic hernia (CDH) in newborns can be predicted using left ventricular (LV) diameter and estimated pulmonary artery pressure.

Therapeutic hypothermia and myocardium in perinatal asphyxia: a microvolt T-wave alternans and Doppler echocardiography study

OBJECTIVES This is the first study evaluating the predictive value of myocardial performance on arrhythmia and mortality via tissue-Doppler and microvolt T-wave alternans in infants with hypoxic-ischemic encephalopathy treated with therapeutic hypothermia-rewarming. METHODS The study included 23 term newborns having criteria for hypoxic-ischemic encephalopathy, and 12 controls. Tissue-Doppler imaging and T-wave alternans were performed in the first six hours after birth in patients from both groups and after hypothermia-rewarming treatment on the fifth day. RESULTS The basal T-wave alternans values were higher in patients in lead aVF(p < 0.001) which also correlated with existing acidemia (r = 0.517; p = 0.012). Basal T-wave alternans and post‑treatment values of patients were compared in leads V1 (p < 0.001) and aVF (p < 0.001); a significant decrease was found on the fifth day. Moreover, right ventricle diastolic diameter and estimated systolic pulmonary artery pressure of patients in the first 6 hours were higher (p = 0.03, p < 0.001, respectively). Although, the ejection fraction of patients did not decrease, basal values of left and right ventricular systolic and diastolic functions were lower initially, and increased significantly after treatment. CONCLUSION The global cardiac functions and myocardial performance of newborns with hypoxic-ischemia might be improved with therapeutic hypothermia which can be determined by using T-wave alternans and tissue-Doppler measurements. However, further studies are needed to assess whether these measurements are prognostic in determining the myocardial dysfunction and arrhythmias (Tab. 2, Fig. 3, Ref. 26).

Fetal tanı alan ve yenidoğan döneminde supraventriküler taşikardi geliştiren intraperikardiyal sol ventrikül duvarına invaze dev rabdomiyomlu bir olgu

Amac: Fetal kardiyak tumorler oldukca nadir olup gebeliklerin % 0.14’unde gorulur. Prenatal kalp yetersizligine de neden olabilen bu tumorlerin en sik goruleni rabdomiyomlardir ve hastalarin yarisindan fazlasinda tuberoskleroz oldugu gosterilmistir. Bu rapor, fetal donemde sol ventrikule (LV) invaze intraperikardiyal dev rabdomiyomun literaturde bu boyutta ilk bildirilen olgu olmasi nedeniyle sunulmustur. Olgu: Gestasyonun 22. haftasindaki gebeye yapilan fetal ekokardiyografi degerlendirmesinde perikard icinde 45x27 mm boyutunda, LV’yi kismen basiya ugratan kitle saptandi. Ayni zamanda LV icinde interventrikuler septumdan (IVS) koken aldigi dusunulen en buyugu 10x6 mm olculen dort adet kitle saptandi. Dogum sonrasi yapilan ekokardiyografi degerlendirmesi benzerdi ve klinik olarak basi bulgulari olmayan kitlenin LV duvarina invaze oldugu belirlendi. Hastanin postnatal 1. gununde direncli aritmisi gelisti. Izlemde sinus ritmi elde edildi ve 4. ay kontrolunde kitlelerde kuculme oldugu belirlendi. Ayrica govdesinde gelisen hipopigmente lekeler nedeniyle de tetkik edilen hastaya tuberoskleroz tanisi konuldu. Sonuc: Fetal donemde taninabilen ventrikul duvarina invaze rabdomiyom gibi kitlelerin direncli aritmilere neden olabilecegi, rabdomiyomlarin izlemde kuculebilecegi ve tuberoskleroz ile sik birlikteligi akilda tutulmalidir.

Can whole-blood parameters be used in follow-up of children with rheumatic valvular heart disease?

OBJECTIVES The aim of the present study was to investigate the relationships between red blood cell distribution width, platelet distribution width, and mean platelet volume and the presence and severity of valvular involvement in patients with rheumatic heart disease. METHODS Between April, 2012 and December, 2015, 151 patients who were admitted to the Pediatric Cardiology Unit with diagnosis of rheumatic heart disease and 148 healthy children were included to our study. Transthoracic echocardiography for all children was performed, and the values of red blood cell distribution width, platelet distribution width, and mean platelet volume, besides other blood count parameters, erythrocyte sedimentation rate, and C-reactive protein levels were recorded. RESULTS Red blood cell distribution width, platelet distribution width, mean platelet volume, and C-reactive protein levels were significantly higher in patients with rheumatic heart disease when compared with healthy controls (p0.05). CONCLUSION This is the first study in children with rheumatic heart disease that demonstrated significantly increased red blood cell distribution width, platelet distribution width, and mean platelet volume levels, as well as evaluated all three parameters together. Furthermore, red blood cell distribution width values in the chronical period of acute rheumatic fever, due to the positive correlation with the other chronic inflammatory markers, may help make the diagnosis in children.

Familial hypertrophic cardiomyopathy: A case with a new mutation in the MYBPC3 gene

Familial hypertrophic cardiomyopathy is a genetically heterogeneous disease with variable clinical features that is inherited as autosomal dominant with variable penetrance. Recent developments in genetics of hereditary cardiomyopathy have not only enlightened many points about pathogenesis, but have also provided great benefit to diagnostic approaches of clinicians. Heterozygous mutation of c3691-3692insTTCA in MYBPC3 gene was identified in a pediatric patient with diagnosis of hypertrophic cardiomyopathy at clinic. Hypertrophy was observed in sister and father of the patient in echocardiography screening, and it was subsequently determined that they also had same mutation. This mutation has not previously been defined and reported previously in the literature as cause of hypertrophic cardiomyopathy.