Echocardiographic evaluation of patients with subacute sclerosing panencephalitis

Objective: Subacute sclerosing panencephalitis is a slowly progressive, inflammatory and neurodegenerative disease caused by virus infection in the central nervous system. Since there are a limited number of studies in the literature evaluating the cardiovascular functions of patients with SSPE, the present study evaluates the patients with SSPE using tissue Doppler echocardiography and compares them between the control group in order to shed some light on the subject. Methods: The study is a prospective observational study. 49 patients (17 female, 32 male) with SSPE were included in the study. Patients were divided into two groups: Stage 2 (n=29) and Stage 3 (n=20). Echocardiographic data were compared with a control group of 26 which is the same average age. All children underwent a detailed echocardiography, which contained an M-mode, pulse Doppler and tissue Doppler imaging. Results: Sinus tachycardia ( >100 beats/min in children) was detected in nineteen (38.7%) patients. There were not significant differences between parameters of systolic and diastolic function of the heart. Stage 2 group, EF: 69.9±6.4; SF: 39.2±5.58; and MPI (mitral): 0.38±0.03 and MPI (tricuspid): 0.39±0.10. And in the Stage 3 group, EF: 68.5±7.0, SF: 37.8±5.34, MPI (mitral): 0.37±0.09 and MPI (tricuspid): 0.38±0.12. In the control group EF:70.96±5.54; SF:39.96±5.05 and MPI(mitral): 0.35±0.06 MPI (tricuspid):0.36±0.04 and statistically meaningful differences were not found between patients and control groups (p >0.05). Conclusion: Cardiac functions may be preserved and cardiac functions constitute no significant risks of mortality in the advanced stages of patients with Subacute sclerosing panencephalitis, which is a group of chronic and bedridden patients

Comparison of the Early Results of Preterm Infants who Underwent the Surgical Ligation of Patent Ductus Arteriosus with two Different Surgical Approaches

INTRODUCTION: Patent ductus arteriosus (PDA) is the most common congenital heart disease in preterm infants. Surgical ligation is still the gold standard technique, especially in low birth weight patients. To reduce surgical trauma and possible complications, the anterior mini-thoracotomy (AMT) technique has been defined. However, conventional lateral thoracotomy (LT) is still the method preferred by many centers today. In our study, we aimed to compare the early results of preterm infants who underwent PDA ligation with two different surgical techniques in a newly established center. METHODS: A total of 26 patients (12 males, 14 females; median gestational age at surgery 32 weeks [IQR: 29-37]) who underwent surgical PDA ligation in preterm infants between January 2018 and February 2020 were retrospectively analyzed. PDA closure was performed through the left anterior mini-thoracotomy approach in 15 of the patients and left lateral thoracotomy in 11 patients. The early outcomes of the two groups were compared. RESULTS: The median weight at operation was 1000 gr. (IQR: 720-1200) in AMT group and 1200 gr. (IQR: 1000 – 2800) in the LT group. The difference between weights at operation was found statistically significant (p: 0.03). The operation time of the AMT group was found to be statistically significantly shorter. (p: 0.03) DISCUSSION AND CONCLUSION: Anterior mini-thoracotomy technique provides an effective surgical closure in the low-weight preterm infant at least lateral thoracotomy technique. Based on the results of our series and our opinion, it should be considered the first choice surgical method, especially in very low and extremely low-weight preterm infants

Beta talassemi majorlu hastalarda böbrek fonksiyonlarının incelenmesi

TEZ3047Tez (Uzmanlık) -- Çukurova Üniversitesi, Adana, 1998.Kaynakça (s. 56-59) var.vii, 59 s. ; 30 cm.

Comparison of Three Different Multiple Organ Dysfunction Scores for Predicting Mortality after Neonatal Cardiac Surgery

Infants who undergo cardiac surgery frequently have complications that may advance to multiple organ failure and result in mortality. This study aims to compare three different multiple organ dysfunction scoring systems: the Neonatal Multiple Organ Dysfunction (NEOMOD) score, the modified NEOMOD score, and the Pediatric Logistic Organ Dysfunction-2 (PELOD-2) score in predicting postoperative 30-day mortality in neonates undergoing cardiac surgery. This retrospective cohort study was conducted between January 2019 and February 2021 in a single unit on neonates operated on due to congenital heart disease in the first 28 days of life. Patients who underwent off-pump surgeries were excluded from the study. The NEOMOD, modified NEOMOD, and PELOD-2 scores were calculated for each of the first 3 days following surgery. A total of 138 patients were included. All scores had satisfactory goodness-of-fit and at least good discriminative ability on each day. The modified NEOMOD score consistently demonstrated the best prediction among these three scores after the first day, reaching its peak performance on day 2 (area under curve: 0.824, CI: 0.75–0.89). Our findings suggest that NEOMOD and modified NEOMOD scores in the first 72 h could potentially serve as a predictor of mortality in this population

Chest Pain may be the First Symptom of Malignant Tumors in Children: Report of Two Cases

The majority of chest pain in children is idiopathic or as­sociated with locomotor system pathologies. Albeit car­diac and other thoracic malign tumors may cause chest pain, malignancies usually show rapid clinical onset and are presented with multiple systemic symptoms. It is not a usual finding for chest pain being the sole initial symp­tom. Herein, we report two children who were presented with chest pain as an initial symptom and diagnosed with fibrosarcoma and lymphoma afterwards. Therefore, we want to emphasize that cardiac and pericardial malignant tumors should be remembered in differential diagnosis of chest pain in children

Lethal Very Long-Chain Acyl-Coa Dehydrogenase Deficiency with A Novel Mutation

WOS: 000472585000023Very long chain acyl-CoA dehydrogenase deficiency is an autosomal recessive genetic disorder in which the first step in the mitochondrial beta-oxidation of fatty acids for 14-20 carbons is defective. Clinical presentation is heterogeneous ranging from the severe neonatal form presenting with hypo-ketotic hypoglycemia, liver dysfunction and rapidly fatal cardiomyopathy with episodes of hypo-ketotic hypoglycemia in infants. Herein we report a patient with novel homozygous missense mutation c.1391C>A in exon 14 with a severe neonatal onset type who presented with hypoketotic hypoglycemia, cardiomyopathy and hepatomegaly.Çok uzun zincirli açil-KoAdehidrogenaz eksikliği, yağ asitlerinin mitokondriyalbeta-oksidasyonunda ilk basamak olan 14-20 karbonlu yağ asitoksidasyonunun defektif olduğu otozomal resesif geçiş gösteren genetik birhastalıktır. Klinik başvuru şekli hipoketotik hipoglisemi, karaciğer yetmezliği vehızlı seyirli fatal kardiyomyopatinin eşlik ettiği form ile hafif hipoketotikhipoliseminin eşlik ettiği infant form arasında heterojendir. Burada,hipoketotik hipoglisemi, hepatomegali ve kardiyomyopati ile başvuran, exon14’de c.1391C>A yeni tanımlanmış homozigot missense mutasyon saptanan,ciddi yenidoğan başlangıçlı bir çok uzun zincirli açil-KoA eksikliği vakasısunulmaktadır

Evaluation of echocardiography and holter electrocardiography findings in patients with mucopolysaccharidosis

Giriş: Mukopolisakkaridozlar (MPS) glikozaminoglikanların (GAG) yıkımını sağlayan enzimlerin fonksiyonel eksikliği nedeniyle ortaya çıkan kalıtsal lizozomal depo hastalıklarıdır. Kalp kapaklarında kalınlaşma, fonksiyon kaybı, iletim bozuklukları, koroner arter ve diğer damarların tutulumu görülebilir. Elektriksel açıdan iletken olmayan GAG’ların iletim bozukluklarına yol açtığı varsayılmaktadır. Kardiyak nedenli ölümlerin bir kısmından ritim bozuklukları sorumlu tutulmaktadır. Bu çalışmada Holter elektrokardiyografi (EKG) ile hastaların ritim durumunun araştırılması amaçlandı. Gereç ve Yöntem: Bu çalışmada 2010-2011 yılları arasında mukopolisakkaridoz tanısı ile takip edilmekte olan 17 hasta ve 17 kontrol olgu alındı. Olguların hepsine ekokardiyografik inceleme yapıldı. Bütün olgularda yüzey EKG ve 24 saatlik Holter EKG ile ritim durumu incelendi. Bulgular: MPS tanısı alan hastaların sekizi tip VI, dördü tip II, biri tip I, ikişer hasta tip III ve tip IV tanısı almıştı. Yaş ortalaması hasta grubunda 5,82±2,24 yıl idi. Kapak tutulumu %82 oranında saptandı. Mitral kapak (%73) birinci sırada, aort kapağı (%21) ikinci sıradaydı. Hasta grubunda maksimum kalp hızı 161/dk, ortalama kalp hızı 108/dk, minimum kalp hızı 81/dk, kontrol grubunda ise sırasıyla 151/dk, 96/dk, 70/dk olarak bulundu. Hasta grubunun kalp hızları kontrol grubunun kalp hızlarına göre yüksekti (p<0,05). Hasta grubunda sol ventrikül diyastol sonu çapı yüksek bulundu. Olgularda koroner iskemi veya aritmi saptanmadı. Sonuç: MPS’lerde kardiyak tutulum sıktır. Bilindiği üzere en sık tutulan kapak mitral, ikinci sırada aort kapağıdır. Bu çalışma, bildiğimiz kadarıyla Holter EKG inceleme yapılan ilk çalışma olmakla birlikte çocukluk yaş grubunda, özellikle erken dönemde enzim yerine koyma tedavisi alan hastalarda aritminin önemli bir sorun oluşturmadığını göstermektedir. Hasta grubunda ortalama kalp hızının yüksek bulunması kapak yetmezliği nedeniyle oluşan artmış volüm yüküne bağlanmıştır.Introduction: Mucopolysaccharidosis (MPS) are lysosomal storage disorders characterized with the deficiency of enzymes involving in the destruction of glycosaminoglycans (GAG). Cardiac findings include cardiac valve thickening and dysfunction, conduction abnormalities, coronary artery and other vessels involvement. It is thought that GAGs, which are non-conductive electrically, causes failure in cardiac conduction. Arrhythmias are considered to be responsible from deaths of cardiac origin. In this study cardiac rhythm and structures were evaluated using Holter electrocardiography (ECG) and transthoracic echocardiography. Materials and Methods: Seventeen patients who were followed with the diagnosis of MPS and 17 healthy controls were enrolled in this study in 2010 and 2011. All cases were examined with transthoracic echocardiography. Cardiac rhythms were evaluated using a 24 hour Holter ECG. Results: The mean age was 5.82±2.24 in patient group. Valve involvement was observed in 82%. The mitral valve was the most commonly (73%) affected followed by aortic valve (21%). There was significant heart rate variations between patients and control group, in favour of patient group (p<0.05). The left ventricular end-diastolic diameter in patient group was found higher. Coronary ischemia or arrhythmias were not detected. Conclusions: Cardiac involvement in MPSs is frequent. To our knowledge this is the first study evaluating cardiac rhythms of children with MPS by 24 hour Holter ECG screening. Our findings suggested that arrhythmia is not an important medical problem in pediatric patients, especially in whom enzyme replacement therapy have been commenced at early ages. The high heart rate in patient group was considered as a result of increased cardiac volume due to valvular insufficiency

Experiences of two centers in percutaneous ventricular septal defect closure using konar multifunctional occluder

BACKGROUND: Transcatheter closure of perimembraneous ventricular septal defect still poses a challenge due to the adjacent structures of the tricuspid and aortic valves and the risk of atrioventricular block. We report our experience at 2 centers using the KONAR-MF (multifunctional occluder) ventricular septal defect device, which gained its CE mark in May 2018. METHODS: A retrospective study was carried out on all patients who underwent transcatheter ventricular septal defect closure with the KONAR-MF (multifunctional occluder) ventricular septal defect device at 2 centers. RESULTS: A total of 47 patients were identified. The median age and weight of the patients were 25.8 months and 11 kg. The ventricular septal defects that were closed in 5 cases were post-operative hemodynamically significant residual ventricular septal defects. Forty-eight devices were used in the 47 cases. As for the location of the ventricular septal defect, 40/48 (83.33%) ventricular septal defects were perimembranous and 8/48 (16.66%) were muscular. The percutaneous device closure was successful in 46 procedures (95.8%). Device embolization was observed in 2 patients, and a significant residual shunt was observed in 2 cases. In the follow-up, there was no enhancement in the residual shunt in the remaining cases. Temporary atrioventricular block occurred in 2 cases during the procedure and improved after long sheath withdrawal. CONCLUSION: Soft, flexible, and low-profile KONAR-MF (multifunctional occluder) occluders ensure easy and safe implantation, and small sheath sizes allow for their use in small infants. Although near and mid-term follow-ups did not indicate any permanent atrioventricular block, a larger sample of patients and a longer follow-up period is necessary to understand long-term outcomes

Comparación de la técnica de palpación y guía por ultrasonido para el cateterismo de la arteria femoral en niños sometidos a cirugía cardíaca

Objetivo: El objetivo de este estudio fue comparar la técnica de palpación y ecoguiado para el cateterismo de la arteria femoral en pacientes pediátricos operados de cardiopatías congénitas. Materiales y métodos: Este estudio prospectivo, aleatorizado y controlado incluyó a 40 niños ASA III-IV que se sometieron a cirugía cardíaca congénita. Los pacientes se dividieron en 2 grupos; Grupo de cateterismo guiado por ecografía y grupo de cateterismo guiado por palpación. Resultados: El diámetro de la arteria femoral fue significativamente más corto, el tiempo de acceso y el número de intentos fueron significativamente menores y la tasa de éxito del primer intento fue significativamente mayor en grupo estadounidense. La tasa de complicaciones fue significativamente mayor en el grupo P. El número de cateterismos fallidos fue mayor en el grupo P. El costo total requerido para el procedimiento fue significativamente menor en el grupo de EE. Conclusiones: Encontramos que el cateterismo arterial guiado por ultrasonido aumenta la tasa de éxito y el número de cateterismos exitosos, al tiempo que reduce el tiempo total del procedimiento, la incidencia de complicaciones y el costo. Por tanto, creemos que el uso de guía ecográfica en cateterismo arterial en cirugía cardiaca pediátrica sería una mejor opción