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.

The Effects of Thyroid Hormone Levels on Patent Ductus Arteriosus Closure in Newborns.

Objective: Although the role of thyroid hormones in functional and anatomical closure of patent ductus arteriosus (PDA) is well known, their effects on the medical or surgical closure of PDA in newborns remain unclear. This study aimed to assess the correlation between thyroid function tests and PDA closure through medical or surgical interventions in newborns. Methods: This retrospective study was conducted on 65 newborns diagnosed with hemodynamically significant PDA (hs-PDA), with a premature rate of 81.5% (n=53). The subjects were divided into two groups according to the nature of the ductal closure as medically responsive "MR-PDA" or surgically treated "ST-PDA". The groups were compared in terms of thyroid hormone levels and other clinical parameters. Results: Thirty-three (51%) of all 65 patients had PDA and responded to medical treatment. Gestational week, birth weight, and mode of delivery were similar between the medical and surgical treatment groups (p>0.05). Free thyroxine levels were significantly lower in the MR-PDA group than in the ST-PDA group (p=0.01). Conclusions: Because hs-PDA is associated with increased morbidity and mortality in the neonatal period, especially in premature infants, we hypothesize that thyroid hormone levels may play a role in the closure of hs-PDA.

An analysis of cardiology consultation requests in infants presented to the pediatric emergency department of a tertiary children's hospital.

INTRODUCTION: Cardiological emergencies are reported to constitute almost 15% of all emergency department visits. This study aimed to characterize the main signs and symptoms of the infants that necessitated pediatric cardiology consultation and to analyze the characteristics of patients diagnosed with a cardiological disorder. MATERIAL AND METHODS: Patients aged 1 month to 1 year who were consulted to the pediatric cardiology service during a 4-year period were retrospectively evaluated. Patients' age, sex, nationality, complaints at PED, physical examination findings, reason for echocardiography (echo) and final diagnosis were recorded from the hospital medical record system for further analysis. Patients were divided into two groups according to the severity of the echo findings (patients with significant cardiovascular issues and patients without significant cardiovascular issues). RESULTS: Of the 200 patients included in the study, 19 were in the significant cardiovascular issues, and 181 were in the without significant cardiovascular issue group. The leading complaints of the patients who were consulted to cardiology were cyanosis (22.5%), seizure (22.5%), cough (22%), and fever (19.5%). In emergency presentations, jaundice (16%), nutritional problems (21%), and cardiomegaly (21%) on x-rays were higher in patients with significant cardiovascular issues (p < 0.05). CONCLUSION: In conclusion, congenital heart disease is usually diagnosed in the neonatal period, but some patients may be missed due to a variety of symptoms and findings. Infants with feeding problems and jaundice, especially those with cardiomegaly on chest radiographs, should be carefully evaluated for underlying serious congenital heart disease.

Evaluation of risk factors, functionalities, and quality of life in patients with pediatric acute arterial ischemic stroke.

BACKGROUND: This study aimed to evaluate the etiology and prognosis of patients followed up for pediatric acute arterial ischemic stroke. METHODS: The clinical characteristics and etiology of patients aged 1 month-18 years who had acute arterial ischemic stroke between January 2010 and December 2020 were retrospectively evaluated. At last follow-up, the patients` functionality (Barthel Index, Functional Independence Measure), quality of life (SF-36 questionnaire), and motor outcomes (Gross Motor Function Classification System) were recorded prospectively/crosssectionally. RESULTS: Forty children (25 boys) with a median current age of 112.5 months (range: 3.6-294) were included in the study. The most frequent etiology was prothrombotic disorders, and the most important factor associated with long-term mortality was valvular heart disease. Of the 27 (67.5%) surviving patients, 29.6% had positive motor outcomes and 29.6% were independent according to the Barthel Index. In terms of quality of life, SF-36 scores were highest in the pain scale and lowest in emotional role difficulty. CONCLUSIONS: Determining the etiology and evaluating prognosis are important to plan effective treatment and rehabilitation for pediatric acute arterial ischemic stroke.

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.

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.

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.

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.

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.

Comparison of the diameter of coronary arteries between small for gestational age (SGA) and appropriate for gestational age (AGA) newborn infants.

AIM: For the small for gestational age (SGA) infants born at the normal birth time but whose birth weights were below the 10th percentile and for the appropriate for gestational age (AGA) infants born in normal birth weights according to gestational week, the diameters of right and left coronary arteries were echocardiographically measured in three separate time intervals with their body weights, heights and body surface area parameters. The aim of this study is to compare the progression of coronary artery diameters of SGA infants with AGA infants over time and the effects of coronary artery diameters on the clinical and hemodynamic parameters of SGA babies. MATERIAL AND METHODS: Term 55 SGA babies were compared with 200 AGA infants at birth, first and sixth months of ages for both growth parameters and coronary artery diameters measurements. RESULTS: In comparison of SGA and AGA groups at birth, first and sixth months of ages on body weight, height and body surface area, aortic annulus and left coronary artery and right coronary artery diameters, it was seen that SGA group could not catch up the AGA group in all time periods on both anthropometric and coronary artery diameters data even if they have an increased growth (p<.05). Although SGA infants appeared to be far away from SGA criteria in body weight measurements at 6th months. These infants maintained a significant deficit in height and coronary artery measurements according to the AGA group (p<.05). CONCLUSIONS: In the evaluation of growth indices and coronary artery diameters at birth, first and sixth months of ages in SGA babies, it is clear that impaired fetal growth of these babies has lasting-effects on coronary artery diameters and cardiac structure.

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.

Effects of hemodialysis access type on right heart geometry in adolescents.

INTRODUCTION: This study aimed to investigate the complication frequency and the changes in right heart geometry with different access types in the pediatric population. METHODS: We included 32 consecutive patients aged between 10 and 19 and who underwent hemodialysis sessions via permanent hemodialysis catheter (nHC = 18) or arterio-venous fistula (nAVF = 14) between January 2013 and March 2018. We recorded and compared the complication frequency and the changes in echocardiography findings with different access types. FINDINGS: Demographic data were similar in both groups. Number of new access creation (nHC = 15 vs nAVF = 1) and all complications (nHC = 19 vs nAVF = 6) were significantly higher in hemodialysis catheter group and the statistical analysis showed the superiority of arterio-venous fistula group in comparison of event-free survival (event-free patients; nAVF = 8 (57%), nHC = 3 (16%); p = 0.02). Control echocardiography showed impressive delta-change in right atrium diameter (p = 0.04), right ventricular end-diastolic volume (p = 0.004), right ventricular end-systolic volume (p < 0.001), and right ventricular free wall thickness (p = 0.009) in arterio-venous fistula group, but no significant difference between two groups in terms of delta-change of right ventricular ejection fraction (p = 0.35), fractional area change (p = 0.21), and tricuspid annular plane systolic excursion (p = 0.13) parameters. CONCLUSION: Arterio-venous fistula has lower risk of complications, but overloading stress on right heart chambers triggers remodeling process and geometrical changes, which can be early pieces of evidence of delayed right heart dysfunction in pediatric hemodialysis patients.

Predictive Value of Vasoactive-inotropic Score for Mortality in Newborns Undergoing Cardiac Surgery.

OBJECTIVE: Vasoactive-inotropic Score (VIS) was developed to quantify the amount of inotropic support provided in the postoperative period. We investigated the predictive value of (VIS) for mortality in neonates with congenital heart disease (CHD). STUDY DESIGN: Prospective cohort. PATIENTS: 119 newborns who underwent cardiac surgery. SETTING: Tertiary NICU-CHD center of Ankara from November 2016 to January 2019. INTERVENTION/MEASUREMENT: VIS values were calculated by a standard formula for the first 72 postoperative hours, and the maximum score was recorded. PRIMARY OUTCOMES: Duration of mechanical ventilation, NICU length of stay, and mortality. RESULTS: At surgery, the median (IQR) age was 15 d (9-31). The patients were divided into two groups according to mortality; Group 1 (Non-survivors) (n=36) and Group 2 (Survivors) (n=83). Higher VIS score was correlated to longer duration of mechanical ventilation (P=0.009, r=0.33), and was higher among patients who died (P=0.003). Area under the curve (AUC) was 0,83 (P<0.001, CI: 95% 0.7-0.9) for VIS to identify mortality. At a cut-off value of 15.5, sensitivity and negative predictive values of VIS for mortality were 73.6% and 85.3%, respectively. The higher VIS (>15.5) was independently associated with increased odds for mortality (OR: 8.1, 95% CI: 1.8-35.7, P=0.005). CONCLUSIONS: In newborns with CHD, a higher VIS within 72 hours after cardiac surgery is associated with increased duration of mechanical ventilation, and mortality. VIS may be useful for prediction of mortality at early postoperative period.

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.

[Idiopathic dilated cardiomyopathy in children: Prognostic indicators]. / Çocuklarda idiyopatik dilate kardiyomiyopati: Prognostik belirteçler.

OBJECTIVE: Dilated cardiomyopathy (DCM) is a disorder featuring left ventricular dysfunction, heart failure, and a poor prognosis. The etiology is still unclear, despite diagnostic and therapeutic developments. This study was an evaluation of factors affecting the life span of a group of idiopathic DCM patients. METHODS: A total of 79 patients from between October 2005 and October 2017 with a diagnosis of idiopathic DCM were evaluated retrospectively. Demographic characteristics, clinical information, left ventricular function, treatment, and follow-up of the patients were reviewed based on hospital records. Age, gender, parental consanguinity, cardiomegaly on telecardiography, reduced ejection fraction (EF) and shortening fraction (SF), degree of mitral regurgitation, and intracardiac thrombosis were determined to affect prognosis. RESULTS: The patients were aged 20+-60 months, and the male/female ratio was 1.02/1. The patients most frequently presented with heart failure signs and symptoms (n=59, 74.7%). The most common physical examination findings were a murmur (n=53, 67.1%) and tachycardia (n=48, 60.8%). Cardiomegaly was observed on telecardiography in 73.4% of the patients. The EF and SF values were 35.7+-12.6% and 17.3+-6.5%, respectively. In all, 42 (53.2%) patients had mitral regurgitation of grade 2 or higher. The duration of follow-up was between 1 and 156 months (20+-34.9 months). Intracardiac thrombosis was detected in 4 (5.1%) patients. The mortality rate was 36.7%. When the prognostic factors were compared according to survival time, it was determined that survival was reduced in cases of parental consanguinity, low EF, and cardiomegaly. CONCLUSION: The most important negative markers affecting the length of survival of DCM patients were parental consanguinity, cardiomegaly detected on telecardiography, and a reduced EF level.

A pediatric case of Ortner`s syndrome caused by heritable pulmonary arterial hypertension and review of the literature.

Kaya Ö, Yoldas T, Karademir S, Örün UA, Sari E. A pediatric case of Ortner`s syndrome caused by heritable pulmonary arterial hypertension and review of the literature. Turk J Pediatr 2019; 61: 963-966. An 11-year-old male, who complained of hoarseness and fatigue on effort presented to our institution for evaluation. Left vocal cord paralysis and severe pulmonary hypertension was diagnosed. The patient had an enlarged pulmonary artery due to pulmonary hypertension which was responsible for compression to left vocal cord paralysis causing hoarseness. Ortner`s syndrome or cardiovocal syndrome is known as hoarseness due to left vocal cord paralysis secondary to cardiac pathologies. Although hoarseness of voice is frequently encountered in the otorhinolaryngology clinics, pulmonary hypertension related hoarseness is an unusual presentation in childhood.