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.

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.

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.

Potential role of salivary cortisol levels to reflect stress response in children undergoing congenital heart surgery.

AIM: This study aimed to provide baseline information on the potential role of salivary cortisol in reflecting the stress response in children undergoing congenital heart surgery. PATIENTS AND METHODS: Children underwent congenital cardiac surgery, aged between one and seventeen years were included. Saliva samples were collected pre- and postoperatively by the health caregiver immediately after the children woke up (07:00-09:00 am) and at 06:00 pm in the evening. Salivary cortisol levels were compared with the reference index values from a large database. RESULTS: Median baseline preoperative morning salivary cortisol levels were significantly lower than the reference values in both < 5-year-old females (p = 0.01) and males (p = 0.04) and in males between 11 and 20 years of age (p = 0.01). Median baseline preoperative evening salivary cortisol levels were significantly higher than the reference value in < 5-year-old females (p = 0.01) and between 5 and 10 years of age (p = 0.04) and in between 11- and20-year-old males (p = 0.01). Median postoperative morning salivary cortisol levels were significantly lower than the reference value in both < 5-year-old females (p = 0.01) and males (p = 0.04) and females between 5 and 10 year of age (p = 0.04). Median postoperative evening salivary cortisol levels were significantly higher than the reference value in < 5-year-old females (p = 0.01) and between 5- and 10-year-old females (p = 0.04). CONCLUSION: Diurnal variability of salivary cortisol levels in children undergoing congenital heart surgery may be different from normal reference values both in preoperative and postoperative periods that can be a predictive indicator of anxiety on pre- and postoperative period for children that undergoing cardiac surgery.

Minimally invasive permanent pacemaker implantation immediately after birth: from delivery room to heart surgery.

INTRODUCTION: Congenital atrioventricular block is diagnosed in uterine life, at birth, or early in life. Atrioventricular blocks can be life threatening immediately at birth so urgent pacemaker implantation techniques are requested. Reasons can be cardiac or non-cardiac, but regardless of the reason, operations are challenging. We aimed to present technical procedure and operative results of pacemaker implantation in neonates. MATERIALS AND METHODS: Between June 2014 and February 2021, 10 neonates who had congenital atrioventricular block underwent surgical operation to implant permanent epicardial pacemaker by using minimally invasive technique. Six of the patients were female and four of them were male. Mean age was 4.3 days (0-11), while three of them were operated on the day of birth. Mean weight was 2533 g (1200-3300). RESULTS: Operations were achieved through subxiphoidal minimally skin incision. Epicardial 25 mm length dual leads were implanted on right ventricular surface and generators were fixed on the right (seven patients) or left (three patients) diaphragmatic surface by incising pleura. There were no complication, morbidity, and mortality related to surgery. CONCLUSION: Few studies have characterised the surgical outcomes following epicardial permanent pacemaker implantation in neonates. The surgical approach is attractive and compelling among professionals so we aimed to present the techniques and results in patients who required permanent pacemaker implantation in the first month of life.

Images of a large fistulous communication between the right ventricle and left anterior descending coronary artery.

Coronary artery fistulas are relatively rare congenital or iatrogenic heart defects that can present with or without symptoms. Symptomatic patients manifest as myocardial ischaemia, arrhythmia, or heart failure. We present a asymptomatic child with a large left anterior descending coronary artery to right ventricular fistula.

A rare indication of permanent pacemaker implantation in children: congenital long QT syndrome.

Congenital Long QT Syndrome (LQTS) is a dangerous arrhythmic disorder that can be diagnosed in children with bradycardia. It is characterised by a prolonged QT interval and torsades de pointes that may cause sudden death. Long QT syndrome is an ion channelopathy with complex molecular and physiological infrastructure. Unlike the acquired type, congenital LQTS has a genetic inheritance and it may be diagnosed by syncope, stress in activity, cardiac dysfunction, sudden death or sometimes incidentally. Permanent pacemaker implantation is required for LQTS with resistant bradycardia even in children to resolve symptoms and avoid sudden death.

Validation of Renal Risk Score Models for Coronary Artery Bypass Surgery in Diabetic Patients.

BACKGROUND: Coronary artery bypass grafting is applicable with very low mortality and morbidity rates around the world. However, exposure to even one of the risk factors increases mortality and morbidity significantly. There are three acute kidney injury definitions, and classification methods are applicable (Kidney Disease: Improving Global Outcomes (KDIGO); Risk, Injury, Failure, Loss of kidney function and End-stage kidney disease (RIFLE);" (for accuracy) and Acute Kidney Injury Network (AKIN)), for understanding and grading of renal impairment. With these definitions, it became possible to take measures at an early stage and start the management process. Methods for assessing renal impairment after coronary artery bypass grafting (CABG) specifically in patients with diabetes mellitus require further investigation. We compared these three acute kidney injury definitions for prediction of outcomes in diabetic patients undergoing coronary artery bypass grafting procedure. METHODS: Between January 2010 and December 2013, a total of 617 consecutive patients with diabetes mellitus undergoing coronary artery bypass grafting (CABG) with cardiopulmonary bypass in our institution were included in the study. RESULTS: We considered 617 CABG operations on diabetes mellitus patients for this study from January 2010 to December 2013. The three scores provided good discriminative capacity in the global patient sample, with the area under the ROC curve (AUC) being higher, RIFLE (0.803, 95% CI: 0.724-0.882). The goodness of fit was good for all scales. CONCLUSIONS: Especially in on-pump CABG patients with diabetes mellitus, we can use AKIN, RIFLE, and KDIGO scoring systems to predict early diagnosis for acute kidney injury (AKI). In our analysis, the KDIGO criterion was superior to AKIN and RIFLE with regard its prognostic power.

Analysis of the Early Results of 693 Patients Undergoing Valvular Reoperation Between 1993 and 2011.

BACKGROUND: The study aim was to identify factors affecting early mortality in valvular reoperations. METHODS: Between January 1993 and December 2011, a total of 693 patients who had undergone valvular reoperations due to problems with previously implanted mechanical and biological valves, new valve degeneration or valve failure after a reconstructive procedure was included in the study. Factors affecting early mortality were identified by the examination of preoperative and perioperative data, using multivariate analysis. RESULTS: The average age of the patients was 44.9 years. For all patients, overall hospital mortality was 15.9%, while hospital mortality rates were 12.9% and 35.3% for elective operation and urgent/emergency treatment, respectively. Factors affecting early mortality in the multivariate analysis were longer total perfusion time (>120 min, p = 0.001), emergency or urgent treatment (p = 0.001), and the presence of preoperative renal failure (p = 0.001). CONCLUSIONS: Mortality for elective patients in valvular reoperations was within an acceptable range. Total mortality was dependent on a high mortality level of emergency/urgency of the cases. The use of a well-defined protocol in valvular reoperations may decrease hospital mortality.

Predictors of Early Extubation After Pediatric Cardiac Surgery: A Single-Center Prospective Observational Study.

This prospective, observational, single-center study aimed to determine the perioperative predictors of early extubation (<24 h after cardiac surgery) in a cohort of children undergoing cardiac surgery. Children aged between 1 month and 18 years who were consecutively admitted to pediatric intensive care unit after cardiac surgery for congenital heart disease between January 2012 and June 2014. Ninety-nine patients were qualified for inclusion during the study period. The median duration of mechanical ventilation was 20 h (range 1-480), and 64 patients were extubated within 24 h. Four of them failed the initial attempt at extubation, and the success rate of early extubation was 60.6 %. Older patient age (p = .009), greater body weight (p = .009), absence of preoperative pulmonary hypertension (p = .044), lower RACHS-1 category (OR, 3.8; 95 % CI 1.35-10.7; p < .05), shorter cardiopulmonary bypass (p = .008) and cross-clamp (p = .022) times, lower PRISM III-24 (p < .05) and PELOD (p < .05) scores, lower inotropic score (p < .05) and vasoactive-inotropic score (p < .05), and lower number of organ failures (OR, 2.26; 95 % CI 1.30-3.92; p < .05) were associated with early extubation. Our study establishes that early extubation can be accomplished within the first 24 h after surgery in low- to medium-risk pediatric cardiac surgery patients, especially in older ones undergoing low-complexity procedures. A large prospective multiple institution trial is necessary to identify the predictors and benefits of early extubation and to facilitate defined guidelines for early extubation.

Surgical Treatment of Isolated Interrupted Aortic Arch in a Young Female Diagnosed during Pregnancy.

Interrupted aortic arch (IAA) is a very rare pathology characterized by luminal discontinuity between ascending and descending aorta. IAA is commonly treated in pediatric ages, but the surgery is rarely used for adult patients. In this case report, we aimed to present a successful surgery in a young woman in whom IAA was diagnosed during pregnancy.

Regional cervical plexus blockage for carotid endarterectomy in patients with cardiovascular risk factors.

BACKGROUND: Carotid artery disease is not rare in cardiac patients. Patients with cardiac risk factors and carotid stenosis are prone to neurological and cardiovascular complications. With cardiac risk factors, carotid endarterectomy operation becomes challenging. Regional anesthesia is an alternative option, so we aimed to investigate the operative results of carotid endarterectomy operations under regional anesthesia in patients with cardiac risk factors. METHODS: We aimed to analyze and compare outcomes of carotid endarterectomy under regional anesthesia with cardiovascular risk groups retrospectively. Between 2006 and 2014, we applied 129 carotid endarterectomy ± patch plasty to 126 patients under combined cervical plexus block anesthesia. Patients were divided into three groups (high, moderate, low) according to their cardiovascular risks. Neurological and cardiovascular events after carotid endarterectomy were compared. RESULTS: Cerebrovascular accident was seen in 7 patients (5.55%) but there was no significant difference between groups (P > .05). Mortality rate was 4.76% (n = 6); it was higher in the high risk group and was not statistically significant (P = .180). Four patients required revision for bleeding (3.17%). We did not observe any postoperative surgical infection. CONCLUSION: Carotid endarterectomy can be safely performed with regional cervical anesthesia in all cardiovascular risk groups. Comprehensive studies comparing general anesthesia and regional anesthesia are needed.

Lung ultrasound in the evaluation of pulmonary edema in newborns with critical congenital heart disease.

BACKGROUND: Newborns with critical congenital heart disease (CCHD) with increased pulmonary blood flow (PBF) are at high risk for congestive heart failure. In this study, we aimed to evaluate the presence and degree of pulmonary edema in newborns with CCHD using lung ultrasound (LUS) during the perioperative period. METHODS: Prospective clinical trial, 44 newborn patients with CCHD were evaluated in this prospective clinical trial. LUS was repeatedly performed to determine the course of pulmonary edema during the perioperative period. LUS was performed simultaneously with chest radiography (CXR), which was the main part of patient management. The primary outcome of this study was to identify whether a correlation existed between LUS and CXR findings. The secondary outcomes were to determine the relationship between LUS and the need for respiratory support, diuretic use, vasoactive inotropic score (VIS), and pro-B-type natriuretic peptide (pro-BNP) levels during the perioperative period. RESULTS: The mean gestational age of the patients was 38.3 ± 1.7 weeks, with a mean birth weight of 3026 ± 432 g. In the preoperative period, both LUS and CXR images were consistent with clinical signs of pulmonary edema. On the first postoperative day, pulmonary edema increased compared to the preoperative period but gradually decreased by the 6th day of surgery (p < 0.05). Positive correlations were observed between the LUS and CXR findings at all study points (p < 0.05). The LUS findings exhibited trends parallel to those of VIS, serum pro-BNP levels, need for respiratory support, and diuretic requirements. As expected, these trends were more pronounced in CCHDs where PBF increased. CONCLUSION: In CCHD, serial lung ultrasound (LUS) assessments, particularly in cases with increased PBF, can provide valuable guidance for managing patients during the perioperative period.

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.

Hybrid coronary revascularization for the treatment of left main coronary artery disease in high-risk patients.

OBJECTIVE: Hybrid coronary revascularization is an alternative for treatment for high-risk patients with coronary artery disease. We evaluated the efficacy of staged hybrid coronary revascularization for the treatment of unprotected left main coronary artery disease in high-risk patients. METHODS: Patients with left main or proximal left anterior descending coronary artery stenosis who are not good candidates for percutaneous coronary intervention and who had suitable lesions in the right coronary and circumflex arteries were considered for staged hybrid therapy if they had poor left ventricular functions (ejection fraction <0.40) and comorbid illnesses. From January 2008 through December 2010, 11 patients (8 men, 3 women; mean age: 66.1 ± 9.1 years) were treated with off-pump coronary artery bypass grafting combined with staged percutaneous coronary intervention. Nine patients had left main coronary artery stenosis together with circumflex or right coronary artery stenosis, and 2 patients had proximal left anterior descending artery stenosis and right coronary artery stenosis. RESULTS: After off-pump coronary artery bypass grafting, procedure-related complications did not occur, and there was no in-hospital death. Coronary re-angiography after a median of 16 days revealed patent and functioning left internal mammarian artery grafts in all patients. Applying subsequent percutaneous transluminal coronary angioplasty and occasional stenting (n = 14), a total of 14 lesions were treated successfully. Procedure related complications did not occur. All patients remained angina-free, and no stress electrocardiographic changes were recorded. CONCLUSION: Our preliminary results indicate that a "staged hybrid" approach to the treatment of left main coronary artery disease in high-risk patients is safe and effective. Hybrid coronary revascularization enables complete revascularization and may be an alternative method of treating left main coronary artery disease in selected high-risk patients.

The use of posterior pericardiotomy technique to prevent postoperative pericardial effusion in cardiac surgery.

AIM: The goal was to determine the effectiveness of the posterior pericardiotomy technique in preventing the development of early and late pericardial effusions (PEs) and to determine the role of anxiety level for the detection of late pericardial tamponade (PT). MATERIALS AND METHODS: We divided 100 patients randomly into 2 groups, the posterior pericardiotomy group (n = 50) and the control group (n = 50). All patients undergoing coronary artery bypass grafting surgery (CABG), valvular heart surgery, or combined valvular and CABG surgeries were included. The posterior pericardiotomy technique was performed in the first group of 50 patients. Evaluations completed preoperatively, postoperatively on day 1, before discharge, and on postoperative days 5 and 30 included electrocardiographic study, chest radiography, echocardiographic study, and evaluation of the patient's anxiety level. Postoperative causes of morbidity and durations of intensive care unit and hospital stays were recorded. RESULTS: The 2 groups were not significantly different with respect to demographic and operative data (P > .05). Echocardiography evaluations revealed no significant differences between the groups preoperatively; however, before discharge the control group had a significantly higher number of patients with moderate, large, and very large PEs compared with the pericardiotomy group (P < .01). There were 6 cases of late PT in the control group, whereas there were none in the pericardiotomy group (P < .05). Before discharge and on postoperative day 15, the patients in the pericardiotomy group showed significant improvement in anxiety levels (P = .03 and .004, respectively). No differences in postoperative complications were observed between the 2 groups. CONCLUSION: Pericardiotomy is a simple, safe, and effective method for reducing the incidence of PE and late PT after cardiac surgery. It also has the potential to provide a better quality of life.

Giant Right Atrial Myxoma with Fulminant Progression in an Infant.

Cardiac myxoma is rare in children. Myxomas are exceedingly rare in infancy. Right atrial myxomas were recorded in a small number of case reports involving infants worldwide. We report the case of a 2-month-old infant with giant right atrial myxoma. The case presented to our hospital with respiratory distress, and had pericardial and pleural effusion. Diagnosis of cardiac tumor was made with the aid of computerized tomography scan and echocardiogram. The tumor size was 3.1 × 3.4 × 3.9 cm. The patient worsened rapidly and had sudden cardiac arrest which did not respond to interventions. Postmortem cardiac autopsy confirmed the diagnosis of myxoma on gross examination and histology. This article aims to focus attention to the atypical size and location of this atrial myxoma, causing diagnostic difficulty in this infant.

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.

Carotid patch and cerebrovascular event relation after carotid endarterectomy procedure.

BACKGROUND: Carotid endarterectomy (CEA) is the treatment of choice for patients with symptomatic and asymptomatic high-grade carotid stenosis. In literature there are papers advocating patch plasty for better results. But there is controversy about which patch material is optimal for use. This study compared rate of neurologic events, and perioperative death in patients undergoing CEA with primary closure versus patch closure. METHODS: We compared patch angioplasty to primary closure between August 2008 and December 2012 in 57 operations. Patients grouped according to their closure technique. Early (30-day) stroke and mortality rate were evaluated. Mean age of patients was 67.79±10.19 (range 41-85 years). 15 (26.31%) of patients were female. RESULTS: Primary closure was used in 13 operations, and patch angioplasty was used in 44 (saphenous vein: 5, Dacron: 23, Fabric: 5, Carotid: 11). There were no significant differences among groups' baseline characteristics. Primary closure was associated with significantly more postoperative major stroke compared with patch used closure (P=0.05). None of preoperative variables were associated with postoperative stroke. CONCLUSIONS: On the basis of our results, avoiding primary closure for CEA is defendable. But choice of patch material needs large trials to conclude.