Neonatal Outcomes of Critical Congenital Heart Defects: A Multicenter Epidemiological Study of Turkish Neonatal Society : Neonatal Outcomes of CCHD.

Critical congenital heart disease (CCHD) is one of the leading causes of neonatal and infant mortality. We aimed to elucidate the epidemiology, spectrum, and outcome of neonatal CCHD in Türkiye. This was a multicenter epidemiological study of neonates with CCHD conducted from October 2021 to November 2022 at national tertiary health centers. Data from 488 neonatal CCHD patients from nine centers were entered into the Trials-Network online registry system during the study period. Transposition of great arteria was the most common neonatal CHD, accounting for 19.5% of all cases. Sixty-three (12.9%) patients had extra-cardiac congenital anomalies. A total of 325 patients underwent cardiac surgery. Aortic arch repair (29.5%), arterial switch (25.5%), and modified Blalock-Taussig shunt (13.2%). Overall, in-hospital mortality was 20.1% with postoperative mortality of 19.6%. Multivariate analysis showed that the need of prostaglandin E1 before intervention, higher VIS (> 17.5), the presence of major postoperative complications, and the need for early postoperative extracorporeal membrane oxygenation were the main risk factors for mortality. The mortality rate of CCHD in our country remains high, although it varies by health center. Further research needs to be conducted to determine long-term outcomes for this vulnerable population.

A Novel Marker for Predicting Fulminant Myocarditis: Systemic Immune-Inflammation Index.

In myocarditis, the search for effective and appropriate prognostic biomarkers can help clinicians identify high-risk patients in a timely manner and make better medical decisions in clinical practice. The prognostic value of systemic immune-inflammatory index (SII), an innovate biomarker of inflammation, in fulminant myocarditis in children has not been assessed. This study aims to (1) determine the effect of SII and other inflammatory markers on the prognosis of patients with myocarditis, and (2) characterize other factors affecting adverse outcomes in myocarditis. All patients aged between 1 months and 18 years who admitted to Pediatric Emergency Department between January 1, 2015 and October 1, 2021 and were diagnosed with myocarditis were retrospectively analyzed. 106 Eligible subjects were enrolled (67% male, 12.5 years (IQR 6-16). Fulminant myocarditis developed in 16 (15%) of the patients. The median SII was 1927 (1147.75-3610.25) in the fulminant myocarditis group and 351 (251.75-531.25) in the non-fulminant group (p < 0.001). In estimation of fulminant myocarditis, AUC was 0.87 for WBC [95% confidence interval (CI) 0.72-1.00, p = 0.002], 0.94 for ANC (95% CI 0.85-1.00), p = 0.000), 0.92 for SII (95% CI 0.82-1.00, p = 0.000). Spearman's correlation analysis showed a significant negative correlation between SII and LVEF (r = 0.576, p < 0.001). The highest AUC values were associated with ANC, SII, and WBC levels to predict fulminant myocarditis. SII, a readily available biomarker from routine blood parameters, allows early recognition of negative outcomes and can independently predict the prognosis of myocarditis in children.

Ultrasonographic evaluation of endotracheal tube position in newborns with CHD.

Many studies have been conducted to determine the most reliable technique for evaluating the position of the endotracheal tube in patients receiving mechanical ventilation support. In this study, we aimed to determine the endotracheal tube position by ultrasonography in intubated patients with a diagnosis of critical CHD followed in the neonatal ICU. METHODS: In this prospective observational clinical study, we performed point-of-care ultrasound for endotracheal tube localisation in 65 intubated newborns with critical CHD. After routine radiography, each patient underwent point-of-care ultrasound examination with a portable ultrasonography device for endotracheal tube end-carina measurement. Endotracheal tube end-carina measurements on chest radiographs were compared with ultrasound images. RESULTS: The mean gestational age and birth weight were 37.8 ± 2.19 weeks and 2888 ± 595 g, respectively. Ultrasound images were obtained after an average of 2.08 ± 1.6 hours from the radiographs. The average ultrasound time allocated to each patient was 5 minutes. The mean endotracheal tube tip-to-carina distance on chest X-ray and ultrasound were optimally 1.33 ± 0.64 cm and 1.43 ± 0.67 cm, respectively. There was no significant difference between chest X-ray and ultrasound measurements in endotracheal tube end-carina distance values evaluated by the Bland-Altman method (mean difference 0.10 cm, p = 0.068). There was a linear correlation between the endotracheal tube tip-carina distance in ultrasound and radiography evaluation (r2 = 0.60, p < 0.001). CONCLUSION: It has been concluded that critical CHDs are frequently accompanied by vascular anomalies, and the endotracheal tube tip-carina distance measurement can be used by determining the carina section as a guide point in the ultrasonographic evaluation of the endotracheal tube location in this patient population.

Is There a Role for Laboratory Parameters in Predicting Coronary Artery Involvement in Kawasaki Disease?

BACKGROUND: Kawasaki disease (KD) may cause cardiac and coronary complications. Since definite markers to accurately predict coronary involvement is not present, we aimed to analyze the role of hematological indices [neutrophil-to lymphocyte ratio (NLR), platelet-to lymphocyte ratio (PLR), lymphocyte-to monocyte ratio (LMR), and mean platelet volume (MPV)-to lymphocyte ratio (MPVLR)], prognostic nutritional index (PNI) and systemic immune-inflammation index (SII) in predicting coronary involvement of KD. Patients The medical records of 134 KD patients admitted between January 2008 and December 2019 were investigated. Also, 268 age-matched healthy controls (HCs) were included in the study. METHODS: KD patients were divided into two groups: KD with coronary artery lesions (KD-CALs) and KD without CALs. Logistic regression analysis was performed to determine parameters that may predict coronary involvement in children with KD. RESULTS: Among KD patients, 39 (29.1%) had CALs. When compared with HCs, the median levels of WBC, neutrophils, monocytes, eosinophils, platelets, MPV and, the values of NLR, PLR, MPVLR, SII were significantly higher; whereas lymphocyte count, PNI, platelet distribution width (PDW), LMR were markedly lower in the KD group (p˂0.001 for all, except for p=0.010 for eosinophil count). The CALs group's SII, PLR, and PNI values were significantly lower than those without (p=0.030, p=0.032, and p ˂0.001; respectively). Multivariable regression analysis revealed that PNI, SII, and gender (male) were associated with CALs in KD. CONCLUSION: Our analysis revealed that male sex, lower PNI, and lower SII levels were independently associated with CALs in children with KD.

Balloon atrial septostomy through azygos vein in two cases of D-TGA with left atrial isomerism.

Balloon atrial septostomy is a palliative procedure that is performed in D-transposition of great arteries when surgery is not immediately available. Although D-TGA and left isomerism association are rare, it is an important condition as the BAS procedure approach is unique. In this case report, we present two cases of D-TGA with left isomerism where BAS was performed due to restrictive atrial septal defect and lack of immediate availability of the paediatric cardiac surgeon.

Role of systemic immune-inflammatory index in early diagnosis of sepsis in newborns with CHD.

OBJECTIVE: Congenital heart diseases (CHD) are the most common causes of birth defects that have increased the risk of infections. Neonatal sepsis is a life-threatening condition and early diagnosis can be life-saving. We aimed to evaluate the potential role of the systemic immune-inflammatory index in the early diagnosis of neonatal sepsis. METHODS: A retrospective cohort study was conducted on 166 newborns with a diagnosis of neonatal sepsis who were admitted to our hospital with CHD between January 2017 and June 2021. Haematological indices including neutrophil/lymphocyte ratio, platelet/lymphocyte ratio, and systemic immune-inflammatory index were calculated for all patients at the time of diagnosis of neonatal sepsis (sepsis). The sepsis values of these indices were compared with the admission values (pre-sepsis) of the patients. RESULTS: The mean gestational age and birth weight of the patients were 38.36 ± 1.42 weeks and 3057.75 ± 484.68 g. It was found that absolute neutrophil count, systemic immune-inflammatory index, neutrophil/lymphocyte ratio, but not platelet/lymphocyte ratio were significantly increased at the time of sepsis. The receiver operating characteristic curve showed that systemic immune-inflammatory index, neutrophil/lymphocyte ratio, and absolute neutrophil count have predictive ability to define neonatal sepsis among newborns with CHD. The systemic immune-inflammatory index produced an area under the curve receiver operating characteristic curve of 0.76 (70% sensitivity, 70.5% specificity). To discriminate neonatal sepsis, the cut-off values of systemic immune-inflammatory index, neutrophil/lymphocyte ratio, and absolute neutrophil count were 517.19, 2.62, and 9210/mm3, respectively. CONCLUSION: As an easily accessible and reliable indicator, systemic immune-inflammatory index may be used in combination with the other parameters in the early diagnosis of neonatal sepsis.

Lactobacillus rhamnosus Sepsis Associated with Probiotic Therapy in a Term Infant with Congenital Heart Disease.

BACKGROUND: Congenital heart diseases (CHD) are the most common birth defects. Necrotizing enterocolitis (NEC) is an important cause of morbidity and mortality in premature infants, and probiotics can be used to protect NEC. CASE REPORT: We present a term infant with aortic coarctation who developed sepsis with Lactobacillus rhamnosus GG after probiotic use, successfully treated with ampicillin. The baby unfortunately died of acute cardiac arrest on the 90th day of life. CONCLUSION: Probiotic-associated sepsis may develop in infants with various risk factors such as central catheterization, long-term mechanical ventilation and in those at risk for NEC.

Transcatheter radiofrequency pulmonary valve perforation in newborns with pulmonary atresia/intact ventricular septum: Echocardiographic predictors of biventricular circulation.

OBJECTIVE: We report a single-institute experience of neonatal transvenous radiofrequency pulmonary valve perforation for pulmonary atresia/intact ventricular septum, with echocardiographic predictors of biventricular circulation. METHODS: Data were reviewed retrospectively for all neonates who underwent primary transvenous pulmonary valve perforation for pulmonary atresia/intact ventricular septum between January 2008 and November 2018 at our institution. We compared patients who need systemic-to-pulmonary shunt or ductal stenting with patients who did not need. RESULTS: During the study period, 31 patients with pulmonary atresia/intact ventricular septum underwent successful radiofrequency pulmonary valve perforation and balloon dilation of the pulmonary valve. There was no procedure-related mortality. Sixteen patients (52%) needed systemic-to-pulmonary shunt or ductal stenting after initial procedure. Among the survivors (follow-up time of 1 to 11.5 years), 15 patients had a biventricular circulation and 6 patients had 1 and 1/2 ventricular circulation. Two patients are awaiting for Fontan operation. Both the TV/MV annulus ratio (>0.85) and tricuspid valve z-score (>-1) were found to be a good predictor of a biventricular outcome in our cohort. CONCLUSIONS: Percutaneous radiofrequency pulmonary valve perforation and balloon valvotomy is an effective and safe primary treatment strategy for neonates with pulmonary atresia/intact ventricular septum. Ductal stenting or systemic-to-pulmonary shunt may be required in the majority of patients who had smaller right heart components. Preselection of patients according to tricuspid valve z-score and TV/MV annulus ratio allows predicting biventricular circulation.

Tricuspid annular plane systolic excursion and mitral annular plane systolic excursion cardiac reference values in 1300 healthy children: Single-center results.

BACKGROUND: Tricuspid annular plane systolic excursion (TAPSE) and mitral annular plane systolic excursion (MAPSE) are two echocardiographic parameters which provide reliable information about the longitudinal myocardial functions of the right and left ventricles in both adult and pediatric population. The aim of this study was to determine the TAPSE and MAPSE reference values in healthy children aged 0-18 years. METHODS AND RESULTS: This prospective study included 1300 healthy children evaluated with two-dimensional echocardiography. In addition to routine echocardiographic examination, the right and left ventricular systolic functions were assessed by TAPSE and MAPSE measurements. Statistical analyses were carried out in the groups of subjects stratified according to age and body surface area. The mean and standard deviation values and z-scores of TAPSE and MAPSE were developed in each group. CONCLUSIONS: The determination of reference values for TAPSE and MAPSE in healthy children will be of guidance in the evaluation of both healthy children and those with congenital or acquired heart diseases in which the right and left ventricular systolic functions are affected. The reference values obtained will contribute to the clinical practice and the future studies.

What is the Significance of Elevated Troponin I in Children and Adolescents? A Diagnostic Approach.

Troponin is a marker that displays cardiac injury quickly and accurately. In adults, troponin elevation is usually associated with coronary artery disease and requires urgent cardiac catheterization. In healthy children, myocardial injury is rare and may develop due to many different causes. Therefore, troponin elevation in children and adolescents does not usually require emergency cardiac catheterization. The aim of this study is to assess the most common causes of troponin elevation in children and adolescents and to show which diagnostic tests are helpful in assessing pediatric patients with elevated troponin. Patients who had been diagnosed with troponin I elevation (> 0.06 ng/ml) at Dr. Sami Ulus Maternity, Children's Health and Disease Training and Research Hospital between 2007 and 2018 were retrospectively evaluated. Patients undergoing cardiac surgery and those with severe congenital heart disease were excluded from the study. The medical records of the patients were examined and age, gender, diagnostic tests, and diagnosis were evaluated. During the study period, the records of 972 patients were obtained. 213 patients were excluded from the study because of heart surgery, congenital heart disease, and neonatal asphyxia or sepsis. Of the remaining 759 patients, 58% were male, 42% were female, and the median age was 4 years (3 days to 17 years). The most frequent causes are myopericarditis (n: 164), drug intoxications (n: 85), carbon monoxide poisoning (n: 74), perimyocarditis (n: 65), and intensive inhalation ß agonist use in acute asthma and lower respiratory tract infections (n: 70). Patients diagnosed with myocarditis and myopericarditis were admitted with a complaint of chest pain, and the diagnosis was made by history, physical examination, ECG, and echocardiographic findings. Unlike adults, troponin I elevation may be associated with many cardiac and non-cardiac pathologies in children. The most common pathologies in cardiac etiology are myopericarditis and perimyocarditis and can be diagnosed by history, physical examination, ECG, and echocardiography. Cardiac catheterization is not necessary except for rare cardiac pathologies and does not alter the prognosis.

Anxiety, depression, and behavioural rating scales in children with non-cardiac chest pain.

INTRODUCTION: Chest pain is the second most common reason for referral to paediatric cardiologists after benign heart murmurs. Aetiology frequently depends on non-cardiac reasons. In addition, individuals may experience non-cardiac chest pain which is idiopathic or of unknown origin. The aim of this study is to examine psychological symptoms in children and adolescents with medically unexplained chest pain. METHODS: A total of 76 patients (ages 8-18 years) were included in the study, who were referred to the paediatric cardiology department with the complaint of chest pain but did not have any detected cardiac aetiology or any other organic causes of chest pain. The control group was composed of 51 healthy volunteers. Self-evaluation scales were given to both groups which included Beck Anxiety Inventory and Children's Depression Inventory. Also parents of both groups completed the Conner's Parent Rating Scale for assessment of Attention-deficit/hyperactivity disorder. RESULTS: Anxiety scores of the non-cardiac chest pain group were significantly higher compared to controls. No significant differences were found between patients and controls in terms of attention-deficit/hyperactivity disorder and depression scores. In patient group, patterns were similar for boys and girls and for children and adolescents; except girls scored significantly higher than boys in children's depression inventory. CONCLUSIONS: In children and adolescents, non-cardiac chest pain is associated with increased levels of anxiety. These results show the importance of psychiatric evaluation in non-cardiac chest pain patients. Larger controlled studies are needed to determine the prevalence and impact of attention-deficit/hyperactivity disorder and depression in children and adolescents with non-cardiac chest pain.

First cardiac manifestation of hypotonia-cystinuria syndrome.

Hypotonia-cystinuria syndrome is a very rare autosomal recessive contiguous gene deletion syndrome of PREPL and SLC3A1 at 2p21 with neuromuscular and neuroendocrinologic presentation. We report a two-year-six-month-old affected female infant and her five-month-old affected brother with a novel homozygous deletion in SLC3A1 and PREPL gene. Both of siblings had mild facial dysmorphism, hypotonia, feeding problems, failure to thrive, developmental delay. She also had dilated cardiomyopathy which differ from other reported patients. Therefore cardiomyopathy may also be considered one of the features of hypotonia-cystinuria syndrome. With this case report, we present cardiac manifestation of hypotonia-cystinuria syndrome for the first time. Because of two siblings had hyperechogenic bowel in prenatal sonography, it might be a prenatal marker for HCS.

Subclinical left ventricular systolic and diastolic dysfunction in type 1 diabetic children and adolescents with good metabolic control.

OBJECTIVE: Cardiac dysfunction is a well-known consequence of diabetes mellitus. This study was designed to assess whether type 1 diabetic children and adolescents with good metabolic control have early echocardiographic signs of subclinical left ventricular dysfunction and whether diabetes duration has any influence, using conventional and nonconventional echocardiographic tools. METHODS: A total of 100 patients with type 1 diabetes mellitus and 80 gender- and age-matched healthy controls were included. The cases underwent standard conventional transthoracic echocardiography, tissue Doppler imaging, and two-dimensional speckle tracking echocardiography. None of the diabetic patients had signs of renal, retinal, or neurological complications of the disease, and all were good metabolic control (mean HbA1c <7.5%). RESULTS: There was no difference among groups in relation to age, sex, body mass index, and blood pressure. Conventional echocardiographic parameters were similar between diabetic and nondiabetic subjects except increased mitral valve peak A-wave and significantly lower mitral E/A ratio in diabetics. Diabetic patients had more advanced diastolic dysfunction with TDI analysis. In the diabetic group, left ventricular global longitudinal, circumferential, and radial strain and strain rate were significantly lower compared with the controls. There was a positive correlation between diabetes duration and cardiac dysfunction. CONCLUSION: The results of this study showed that the diabetic children and adolescents with good metabolic control had diastolic dysfunction when assessed with either conventional or tissue Doppler echocardiography. Also diabetic patients had subclinical LV systolic dysfunction with a normal LVEF which can be detected with 2D speckle tracking echocardiography.

Maternal and neonatal micronutrient levels in newborns with CHD.

OBJECTIVE: It is suggested that folic acid and/or multivitamins, taken periconceptionally, have a role in the prevention of many congenital anomalies. The aim of this study was to determine the serum micronutrient levels in mother-infant pairs with CHD compared with those with healthy newborns and their mothers. METHODS: Serum levels of folic acid, homocysteine, zinc, vitamin A, vitamin D, and vitamin B12 were measured from 108 newborns with CHD (study group) and 103 healthy newborns (control group). The mothers' micronutrient levels were also measured simultaneously. RESULTS: When compared with healthy newborns, for both maternal and neonatal data, homocysteine and zinc levels were higher and vitamin D levels were lower in the study group. In multivariate analysis, only maternal high zinc levels were associated with CHD in the newborns (p=0.02, OR: 0.9, 95% CI 0.8-0.9). The results did not change when analysed for truncal anomalies including truncus arteriosus, tetralogy of Fallot, and d-transposition of great arteries. There were positive correlations between maternal and neonatal levels of micronutrients, except vitamin B12. CONCLUSION: We thought that high homocysteine and zinc levels and low vitamin D levels in mother-infant pairs might have a role in the aetiopathogenesis of CHD. Large-scale, prospective studies are needed to clarify the role of micronutrients in CHDs.

Persistent left superior vena cava accompanying congenital heart disease in children: Experience of a tertiary care center.

BACKGROUND: This study focuses on determining concomitant persistent left superior vena cava (SVC) in patients with congenital heart disease (CHD). METHODS: Between 2005 and 2012, a total of 2.663 patients with CHD, 88 (3.3%) of whom were diagnosed with persistent left SVC, were evaluated retrospectively. The demographic characteristics of patients, clinical and radiographic findings, echocardiography, cardiac catheterization, and angiography results obtained from the patients' records were reviewed. RESULTS: The median age of the patients was 9.5 months, and 46 of the 88 (52.3%) patients were female. The most common concomitant CHD were ventricular septal defect, double outlet right ventricle (DORV), and tetralogy of Fallot (TOF). When the patients were compared according to their CHD, persistent left SVC frequency was significantly higher in those with DORV (P<.001), TOF (P=.04), patent ductus arteriosus (P=.01), and atrial septal defect (P=.03). Eighty-three of the 88 (94.3%) patients with persistent left SVC had right SVC, and 5 (5.7%) had absence of the right SVC. Twenty-seven of the 83 (32.5%) patients with double SVC had connected normal innominate vein. In all cases, right aortic arch association was seen in 14 (15.9%) patients. Eighty-four (95.4%) of the patients were diagnosed by echocardiography prior to catheter angiography. Persistent left SVC drained to the coronary sinus in all cases. CONCLUSION: Increased awareness about the association of certain CHD with persistent left SVC and a careful echocardiographic examination can facilitate the diagnosis of persistent left SVC. In addition, precise prior diagnosis of persistent left SVC can prevent complications during surgery.

Massive Pericardial Effusion and Tamponade Can Be a First Sign of Familial Mediterranean Fever.

Cardiac involvement is a rare initial presentation of familial Mediterranean fever (FMF). We described 2 children with massive pericardial effusion and cardiac tamponade, who were later diagnosed as having FMF based on clinical and laboratory findings. Therefore, in children presenting with massive pericardial effusion, FMF should be considered as one of the differential diagnoses. In addition, massive pericardial effusion is a serious clinical condition that requires emergency therapeutic approach including pericardiocenthesis. For medical treatment, colchicine is the first line therapy, but in resistant cases, other anti-inflammatory drugs can be used for extra anti-inflammatory effect.

Regression of symptomatic multiple cardiac rhabdomyomas associated with tuberous sclerosis complex in a newborn receiving everolimus.

UNLABELLED: Cardiac rhabdomyoma is the most common primary cardiac tumor, is considered to be a hamartoma of developing cardiac myocytes. Cardiac rhabdomyoma is associated with tuberous sclerosis complex (TSC) in 50-86% of cases. Mutations in TSC-1/TSC-2 genes result in increased mammalian target of rapamycin (mTOR) pathway activation responsible for the hamartomatous lesions of tuberous sclerosis complex. Therapy with mTOR inhibitors is currently under investigation as a treatment option for tumors associated with TSC. In this report we present a case with multiple symptomatic rhabdomyomas associated with tuberous sclerosis complex, deemed to be ineligible for surgical removal, treated with everolimus (mTOR inhibitor). CONCLUSION: As we observed in our patient, in cases with inoperable symptomatic rhabdomyomas associated with TSC, everolimus, an mTOR inhibitor, may be the treatment of choice, which should be confirmed with additional studies.

A rare association: inferior vena cava anomalies and congenital heart diseases.

The numerous variations of abnormalities of the inferior vena cava (IVC) result in anomalies such as isolated left IVC, double IVC and more than 60 types of malformation. These anomalies are rare and recognized incidentally during surgical or radiological procedures. They may lead to clinical complications during abdominal surgery, and predispose to venous thrombosis. Although they have no definite relationship to other congenital cardiac lesions, identification of these anomalies are important for pre-operative planning and post-operative follow-up. This report presents two cases of congenital IVC anomalies accompanied by congenital heart diseases.

Double outlet right ventricle with intact ventricular septum.

Double outlet right ventricle is a cardiac malformation in which both the aorta and pulmonary artery arise from the right ventricle. A double outlet right ventricle with intact ventricular septum is extremely rare. A nine-day-old boy born at 38 weeks with a birth weight of 3200 g was referred due to cyanosis and murmur. The history of the patient was nonsignificant. On the physical examination, oxygen saturation of the patient on room air was 84%, and cardiac activity and a 2/6 systolic murmur at the right sternal border were found. On electrocardiography, deep q's were determined in D2, D3, and aVF. On echocardiography, left atrial isomerism, dextrocardia, wide, high venosum atrial septal defect, double outlet right ventricle with intact ventricular septum, malposition of the great arteries, and moderate pulmonary stenosis were observed. Catheter angiography was performed for diagnostic confirmation. Herein, a newborn with cyanosis and murmur diagnosed as double outlet right ventricle with intact ventricular septum is reported.