Effect of cardiac surgery on survival in patients with trisomy 18: A single-center experience.

Background: In this study, we aimed to compare the results of operated patients diagnosed with trisomy 18 with those who were followed with medical treatment alone. Methods: Between May 2014 and January 2022, a total of 18 patients (6 males, 12 females; median age: 39 days; range, 32 to 79 days) diagnosed with trisomy 18 were retrospectively analyzed. Patient data were obtained from the pediatric cardiovascular surgery digital database. The patients were divided into two groups: those who underwent surgery (n=10) and those who were followed with medical treatment (n=8). Results: Cardiac pathology was detected in all 18 patients included in the study. Three (30%) patients in the surgical group and two (25%) patients in the medical treatment group were discharged and followed with medical treatment. One of the three patients discharged after surgery died during follow-up. The median survival in surgical and medical treatment groups was 150 (range, 75 to 308) days and eight (range, 3 to 51) days, respectively (p=0.009). While patients in the medical treatment group died due to multi-organ failure, those in surgical group died due to sepsis, heart failure, and respiratory failure. Conclusion: Although cardiac surgery contributed positively to survival in patients with trisomy 18, the mortality rate was still high due to non-cardiac causes. We believe that a multidisciplinary approach would contribute positively to the treatment of this patient group with multi-organ failure and would aid in prolonging their life span.

Risk factors for and incidence of hospital-acquired infections after cardiac surgery in children with congenital heart disease: a single center experience.

BACKGROUND: The epidemiology of hospital-acquired infections (HAIs) has been less well studied in critically ill children in pediatric cardiothoracic intensive care units. This study aimed to investigate independent risk factors for and incidence of HAIs after cardiac surgery in children with congenital heart disease (CHD). METHODS: Our study included 574 patients who underwent congenital heart surgery and were followed up in the cardiothoracic intensive care unit between September 2016 and December 2020. All patients were divided into four groups according to age: 0-1 months, 1-6 months, 6-12 months, and 1-18 years, and into two subgroups according to HAI development. RESULTS: The patients` median age and weight at surgery were 3.28 (interquartile range [IQR]): 0.43-8.1) months and 4.34 (IQR: 4.34-6.69) kg, respectively. HAIs and infection-related deaths were observed in 223 and 21 patients, respectively. Age at surgery, weight at surgery, concomitant syndromes and immunodeficiency status, presence of cyanotic heart disease, intubation, and use of antibiotics during hospitalization were statistically significant between the two groups with and without infection (p < 0.05). In logistic regression analysis, surgical weight < 5 kg (odds ratio [OR]: 2.55; 95% confidence interval [CI]: 1.56-4.17; p < 0.001), preoperative mechanical ventilation (OR: 2.0; 95% CI: 1.26-3.12; p=0.003), complexity of cardiac surgery according to the risk-adjusted congenital heart surgery classification score 3 (OR: 3.13; 95% CI: 1.24-7.92; p=0.016), presence of an concomitant syndrome (OR: 1.56; 95% CI: 1.02-2.88; p=0.040), age (OR: 1.01; 95% CI: 1.01-1.04; p=0.044) were independent risk factors for HAIs after cardiac surgery in children with CHD. CONCLUSIONS: In this study, younger age, presence of an associated syndrome, preoperative mechanical ventilation, and weight less than 5 kg were found to be independent risk factors for HAI after cardiac surgery in children with CHD.

Comparison of serum N-terminal pro-brain natriuretic peptide levels, conventional echocardiography, exercise parameters, and dyssynchrony measurements in Fontan patients.

INTRODUCTION: Little data are available concerning the methods used in the long-term follow-up of Fontan patients. We analyzed the association between serum N-terminal pro-brain natriuretic peptide levels, conventional echocardiography findings, exercise parameters, and dyssynchrony measurements in patients who underwent Fontan surgery. METHODS: This study included 28 patients who underwent Fontan surgery (mean age 12.8 ± 4.36 years) and 27 healthy controls (mean age 12.5 ± 3.76 years). Echocardiography examinations and exercise tests were performed in both groups. The systemic ventricle was examined via echocardiography, dyssynchrony measurement was performed, the systemic ventricular myocardial performance index was calculated, and serum N-terminal pro-brain natriuretic peptide levels were measured for all subjects. RESULTS: Lower cardiac output, stroke volume, maximal work, chronotropic index, maximal oxygen uptake, and higher N-terminal pro-brain natriuretic peptide levels were observed in the Fontan group than in the control group (p < 0.05). A negative correlation was found between physical exercise parameters and N-terminal pro-brain natriuretic peptide levels and dyssynchrony measurements. CONCLUSION: Measurements of exercise capacity, serum N-terminal pro-brain natriuretic peptide levels, and dyssynchrony measurement were more valuable than conventional methods for assessing patients' clinical and functional status. Dyssynchrony measurements provided better information about ventricular status than did conventional echocardiography studies. While patients' systolic function determined by conventional echocardiography was normal, dyssynchrony measurements showed the opposite result. The negative relationship between serum N-terminal pro-brain natriuretic peptide levels, dyssynchrony measurements, and exercise capacity suggests that these parameters should be investigated further in Fontan patients.

Frequency and Predictors of Acute and Persistent Femoral Artery Occlusion in Infants with Congenital Heart Disease: A Study Using Ultrasonography for Arterial Access and the Diagnosis of Arterial Occlusion.

Femoral arterial access is challenging in infants. Furthermore, after cardiac catheterization, femoral arterial occlusion (FAO) can be underestimated and easily missed on physical examination. Ultrasound is recommended for femoral arterial access and the correct diagnosis of FAO; however, few studies have reported its effectiveness.To investigate the frequency and risk factors of acute loss of the arterial pulse (ALAP) and persistent femoral arterial occlusion (PFAO) in infants with congenital heart disease who underwent ultrasound-guided femoral arterial access (US-GFAA) and were diagnosed with FAO by ultrasound.We obtained data related to patient characteristics, access variables of US-GFAA, and ultrasonography findings of the femoral artery from our pediatric cardiac catheterization database between August 2017 and August 2022. We divided the patients into groups based on the presence of ALAP and PFAO. We identified ALAP in 99 (19%) patients and PFAO in 21(4%) of 522 patients in the study. The median patient age was 132 days (interquartile range: 75-202 days). The logistic regression analysis identified younger age, aortic coarctation, previous catheterization of the same femoral artery, larger sheath size (5F), and longer duration of cannulation as independent risk factors for ALAP and younger age as an independent risk factor for PFAO (all p < 0.05). This study showed that younger age at procedure was a risk factor for both ALAP and PFAO, while aortic coarctation, previous arterial catheterization, use of a larger sheath and longer duration of cannulation were risk factors for ALAP in infants. The majority of FAO is reversible and secondary to arterial spasm, and the of FAO increases inversely with patient age.

Comparison of Surgical Techniques Used in Ventricular Septal Defect Closure.

INTRODUCTION: We compared transatrial closure, tricuspid valve septal detachment, and tricuspid valve chordal detachment techniques for ventricular septal defect (VSD) closure. METHODS: Patients who had VSD closure with three different techniques in our clinic between September 2016 and December 2020 were retrospectively reviewed. A total of 117 patients were included in the study. The patients were divided into three groups: group 1, classical transatrial closure; group 2, closure with tricuspid valve septal detachment; and group 3, closure with tricuspid valve chordal detachment. The groups were evaluated by serial transthoracic echocardiography (preoperative, postoperative 1st day, postoperative 1st month). Cardiac rhythm checks and recordings were performed. RESULTS: No residual VSD was observed in early or late periods in any of the groups whose VSD closure was performed with the three different techniques. No severe tricuspid regurgitation (TR) was detected during the early and late postoperative periods of all operating procedures. When the groups were compared in terms of early/late TR after the operation (without TR+trace amount of TR and mild TR+moderate TR were compared), no statistically significant difference was found (P>0,05; P=0,969 and P>0,05; P=0,502). CONCLUSION: In this study, we found no statistically significant difference between three VSD closure techniques in terms of early TR, late TR, residual VSD, and permanent atrioventricular complete block during postoperative period. We hope that our results will be supported by the results of researches that are being made about this subject in large series.

The haemodynamic effects of umbilical cord milking in term infants: a randomised controlled trial.

Introduction: Umbilical cord milking (UCM) is a method that allows for postnatal placental transfusion. The benefits of UCM have been demonstrated in some studies, but knowledge about its haemodynamic effects in term infants is limited. The aim of this study was to evaluate the haemodynamic effects of UCM in term infants. Methods: In this prospective, randomised controlled study, 149 healthy term infants with a birth week of ≥37 weeks were randomly assigned to either the UCM or immediate cord clamping (ICC) group. Blinded echocardiographic evaluations were performed in all the neonates in the first 2-6 h. Results: Superior vena cava (SVC) flow measurements were higher in the UCM group compared to the ICC group (132.47 ± 37.0 vs. 126.62 ± 34.3 mL/kg/min), but this difference was not statistically significant. Left atrial diameter (12.23 ± 1.99 vs. 11.43 ± 1.78 mm) and left atrium-to-aorta diastolic diameter ratio (1.62 ± 0.24 vs. 1.51 ± 0.22) were significantly higher in the UCM group. There were no significant differences in other echocardiographic parameters between the two groups. Conclusion: We found no significant difference in the SVC flow measurements in term infants who underwent UCM versus those who underwent ICC. This lack of significant difference in SVC flow may be explained by the mature cerebral autoregulation mechanism in term neonates.

New approach in stage 1 surgery for hypoplastic left heart syndrome: preliminary outcomes.

OBJECTIVES: We present the short-term results of an alternative method in stage 1 surgery for hypoplastic left heart syndrome. METHODS: Data of 16 consecutive patients who were treated with the novel method in our clinic between February 2019 and March 2021 were analysed retrospectively. Preoperative data and postoperative follow-up were recorded. RESULTS: Of the 16 operated patients, 12 were diagnosed with hypoplastic left heart syndrome, while four were diagnosed with hypoplastic left heart syndrome variants. Seven patients died during early postoperative period. One patient died at home waiting stage 2 surgery. Three patient underwent stage 2 surgery. Pulmonary artery reconstruction was performed in one patient due to left pulmonary artery distortion. CONCLUSIONS: We believe that our method can be an effective alternative in the surgery of hypoplastic left heart syndrome and its variants. It is hoped that with increasing number of studies and more experience better outcome will be achieved.

Risk factors and frequency of acute and permanent femoral arterial occlusion in neonates with CHD who undergo ultrasound-guided femoral arterial access.

OBJECTIVE: We investigated frequency and risk factors of acute loss of the arterial pulse and permanent femoral arterial occlusion in neonates with CHD who underwent ultrasound-guided femoral arterial access. METHODS: We divided the patients into groups according to the presence of acute loss of the arterial pulse and permanent femoral arterial occlusion. We obtained data related to patient characteristics and access variables of ultrasound-guided femoral arterial access from our database of cardiac catheterisation between August, 2017 and May, 2021. We used an echocardiography-S6, 12-MHz linear probe, 21-gauge needle, 0.018"guidewire, and a 4F sheath for arterial access. RESULTS: Ultrasound-guided femoral arterial access was obtained in 323 (98.8%) of the 327 neonates. We identified acute loss of the arterial pulse in 130 (40.2%) patients and permanent femoral arterial occlusion in 19 (5.9%) patients. Median weight was 3.05 (Interquartile range (IQR): 2.80-3.40) kg, first attempt success rate was 88.2%, and median access time was 46 sec (IQR: 23-94). Logistic regression analysis identified coarctation of the aorta (odds ratio: 2.46; 95% CI: 1.30-4.66; p = 0.006) as independent risk factor for acute loss of the arterial pulse, but did not identify any independent risk factors for permanent femoral arterial occlusion. CONCLUSIONS: This study showed coarctation of the aorta as an independent risk factor for acute loss of the arterial pulse, but did not identify any independent factors for permanent femoral arterial occlusion in neonates with CHD. Although most cases of acute loss of the arterial pulse resolve in the early period, the frequency of permanent femoral arterial occlusion remains high despite effective treatment.

Comparison of Surgical Techniques Used in Ventricular Septal Defect Closure

ABSTRACT Introduction: We compared transatrial closure, tricuspid valve septal detachment, and tricuspid valve chordal detachment techniques for ventricular septal defect (VSD) closure. Methods: Patients who had VSD closure with three different techniques in our clinic between September 2016 and December 2020 were retrospectively reviewed. A total of 117 patients were included in the study. The patients were divided into three groups: group 1, classical transatrial closure; group 2, closure with tricuspid valve septal detachment; and group 3, closure with tricuspid valve chordal detachment. The groups were evaluated by serial transthoracic echocardiography (preoperative, postoperative 1st day, postoperative 1st month). Cardiac rhythm checks and recordings were performed. Results: No residual VSD was observed in early or late periods in any of the groups whose VSD closure was performed with the three different techniques. No severe tricuspid regurgitation (TR) was detected during the early and late postoperative periods of all operating procedures. When the groups were compared in terms of early/late TR after the operation (without TR+trace amount of TR and mild TR+moderate TR were compared), no statistically significant difference was found (P>0,05; P=0,969 and P>0,05; P=0,502). Conclusion: In this study, we found no statistically significant difference between three VSD closure techniques in terms of early TR, late TR, residual VSD, and permanent atrioventricular complete block during postoperative period. We hope that our results will be supported by the results of researches that are being made about this subject in large series.

Management of pediatric cardiac transplantation candidates with pulmonary hypertension and high pulmonary vascular resistance.

BACKGROUND AND OBJECTIVES: Right ventricular failure is an important cause of mortality and morbidity after orthotopic heart transplantation (OHT). The right ventricle of the donor may fail to accommodate to the high pulmonary vascular resistance (PVR) of the recipient. Pulmonary hypertension (PH) due to chronic heart failure with PVRi > 4 Wood units.m2, transpulmonary gradient > 15 mmHg adversely affect the outcome of OHT. In this study we aimed to evaluate management strategies in our pediatric cardiac transplantation candidates with PH and high PVR prior to OHT. METHOD: Twenty-six cardiac transplantation candidates (age: 10.2 ± 4.6, 1-17 years) underwent cardiac catheterization for the determination of PVR and pulmonary arterial pressure. They were admitted to the hospital and received 1-3 days of intravenous (IV) vasodilator therapy; 0.5-3 µg/kg/min nitroglyserin and/or 0.5-3 µg/kg/min nitroprusside, 5-15 µg/kg/min dobutamin and/or dopamin to keep systolic blood pressure above 80 mmHg. RESULTS: Thirteen patients had dilated cardiomyopathy (CMP), 11 had restrictive CMP, one had hypertrophic CMP and one had congenital heart disease (CHD). Nineteen of the 26 patients underwent OHT. Mean pulmonary arterial pressure of the patients ranged between 11 and 82 mmHg (30.4 ± 16 mmHg) and PVRi between 0.41-21.4 Wood units.m2 (5.3 ± 5.7). Nine patients had PVRi above 4 Wood units.m < sup > 2 < /sup > . Six of these patients had IV treatment for longer than three days and some received specific anti-PH treatment. Eventually they underwent a pulmonary vasoreactivity test with IV iloprost and six had PVRi < 4 Wood units.m < sup > 2 < /sup > . Five of them underwent OHT. CONCLUSION: Cardiac transplantation candidates with PH and high PVR should be evaluated after conditioning with vasodilator and inotropic treatment. Specific treatment for PH and vasoreactivity testing may help selected patients reenter the transplantation list.

The relationship between placental transfusion, and thymic size and neonatal morbidities in premature infants - A Randomized Control Trial

OBJECTIVE: To compare the effect of umbilical cord milking and early cord clamping on thymic size, and neonatal mortality and morbidity in preterm infants. METHODS: This single-center, prospective, double-blind, randomised controlled study was conducted at Baskent University, Konya Education and Research Centre, Konya, Turkey, between October 2015 and April 2016. Pregnant women who delivered before 32 weeks of gestation were randomised to receive umbilical cord milking (group 1) or early cord clamping (group 2). Ultrasonographic evaluation was performed in each newborn by an experienced radiologist within the first 24 hours of life. Thymic si ze was estimated in l ine with literatu re. SPSS 15 was u sed for a ll data analyses. RESULTS: There were 38 subjects in group 1 and 37 in group 2. There were as many infants in the two groups (p>0.05) The haemoglobin levels was higher in group 1, but not significantly (p=0.213). The absolute neutrophil count in group 1 was significantly lower (p= 0.017) than group 2. In terms of neonatal mortaility and morbidity, there were no significant differences between the groups (p>0.05). CONCLUSIONS: Umbilical cord milking was not associated with thymic size during the the first 24h of life.

Hybrid approach: an alternative prior to corrective surgery in a patient with arch hypoplasia and complete atrioventricular septal defect.

The hybrid approach is mostly preferred in patients with hypoplastic left heart syndrome or univentricular physiology. Here, the hybrid approach is applied as a palliative procedure prior to corrective surgery in a patient with complete atrioventricular septal defect associated with arcus hypoplasia and results are discussed according to the literature.

Severe hypernatremia associated catheter malposition in an intensive care patient.

We present a catheter related severe hypernatremia in a 2-month-old baby who was admitted to the pediatric intensive care. Imbalance of plasma sodium is commonly seen in pediatric intensive care patients. The water and sodium balance is a complex process. Especially, brain and kidneys are the most important organs that affect the water and sodium balance. Other mechanisms of the cellular structure include osmoreceptors, Na-K ATPase systems, and vasopressin. Hypernatremia is usually an iatrogenic condition in hospitalized patients due to mismanagement of water electrolyte imbalance. Central venous catheterization is frequently used in pediatric intensive care patients. Complications of central venous catheter placement still continue despite the usage of ultrasound guidance. Malposition of central venous catheter in the brain veins should be kept in mind as a rare cause of iatrogenic hypernatremia.

Inferior Vena Cava Oxygen Saturation during the First Three Postnatal Days in Preterm Newborns with and without Patent Ductus Arteriosus.

BACKGROUND: Inferior vena cava (IVC) oxygen saturation as an indicator of mixed venous oxygenation may be valuable for understanding postnatal adaptations in newborn infants. It is unknown how this parameter progresses in critically ill premature infants. AIMS: To investigate IVC oxygen saturation during the first three days of life in preterm infants with and without patent ductus arteriosus (PDA). STUDY DESIGN: Case-control study. METHODS: Twenty-seven preterm infants were admitted to the Neonatal Intensive Care. Preterm infants with umbilical venous catheterization were included in the study. Six umbilical venous blood gas values were obtained from each infant during the first 72 hours of life. Preterm infants in the study were divided into two groups. Haemodynamically significant PDA was diagnosed by echocardiography in 11 (41%) infants before the 72(nd) hour of life in the study group and ibuprofen treatment was started, whereas 16 (59%) infants who didn't have haemodynamically significant PDA were included in the control group. RESULTS: In the entire group, the highest value of mean IVC oxygen saturation was 79.9% at the first measurement and the lowest was 64.8% at the 72(nd) hour. Inferior vena cava oxygen saturations were significantly different between the study and control groups. Post-hoc analysis revealed that the first and 36(th) hour measurements made the difference (p=0.01). CONCLUSION: Inferior vena cava oxygen saturation was found to be significantly different between preterm infants with and without PDA. Further studies are needed to understand the effect of foetal shunts on venous oxygenation during postnatal adaptation in newborn infants.

"Shunt index" can be used to predict clinically significant patent ductus arteriosus in premature neonates in early post-natal life.

BACKGROUND: This study aimed to examine the differences between arterial and inferior caval vein oxygen saturation, fractional oxygen extraction, and the shunt index, which were calculated in the diagnosis of patent ductus arteriosus. METHODS: Twenty-seven preterm infants were included in this study and were divided into two groups according to patent ductus arteriosus. Among them, 11 (41%) infants had haemodynamically significant patent ductus arteriosus and 16 (59%) did not have significant patent ductus arteriosus. Synchronous arterial and venous blood gases were measured during the first post-natal hours after the insertion of umbilical catheters. The differences between arterial and inferior caval vein oxygen saturation, inferior body fractional oxygen extraction, and the shunt index were calculated. Echocardiography was performed before the 72nd hour of life in a selected group of patients who had haemodynamically significant patent ductus arteriosus. Ibuprofen treatment was administered to patients with patent ductus arteriosus. Echocardiography was performed on the 72nd hour of life in preterm infants without any clinical suspicion of patent ductus arteriosus. RESULTS: The early measured differences between arterial and inferior caval vein oxygen saturation and inferior body fractional oxygen extraction were found to be lower and the shunt index was found to be higher in the haemodynamically significant patent ductus arteriosus group than in the group without haemodynamically significant patent ductus arteriosus. CONCLUSION: We found that the shunt index, calculated in the first hours of life as ≥63%, predicted haemodynamically significant patent ductus arteriosus with a sensitivity of 78% and specificity of 82% in preterm newborns.

Anomalous pulmonary venous connection to the azygous vein: surgical approach of a case with 3D computed tomography findings.

Echocardiographic evaluation of anomalous pulmonary venous connection (APVC) may be difficult especially in patients with complex congenital heart diseases. Angiography may also be hazardous in sick patients. For this reason, sometimes we need noninvasive and highly demonstrative procedures for the diagnosis and management of APVC. We have presented a patient, operated with the diagnosis of partial APVC to the azygous vein. In this patient the diagnosis was suspected by echocardiography and confirmed by using three-dimensional computed tomography with angiography.

Evaluation of right ventricular functions and B-type natriuretic peptide levels by cardiopulmonary exercise test in patients with pulmonary regurgitation after repair of tetralogy of Fallot.

Impairment of right ventricular functions, especially due to chronic pulmonary regurgitation (PR), is a well-known entity in patients with tetralogy of Fallot (ToF) after repair. The aim of this study was to examine the relation between B-type natriuretic peptide (BNP) levels and right ventricular dysfunction by cardiopulmonary exercise test (ET) in patients after repair of ToF. Twenty-five patients with a mean age of 14.1 +/- 4.4 years at follow-up who underwent repair of ToF at a mean age of 4.9 +/- 5.1 years and 29 age- and sex-matched healthy children at a mean age of 13.1 +/- 2.8 years were enrolled in this study. Plasma BNP levels were measured at baseline and at maximal exercise. The volume of right ventricle (RV) and the degree of PR were assessed by two-dimensional echocardiography and color Doppler. Plasma BNP levels were significantly higher in patients with ToF than in controls (28.3 +/- 24.1 vs 7.4 +/- 2.3 pg/mL, p = 0.0001). Exercise was associated with increased plasma BNP levels in both groups. A greater increase in BNP was noted in patients with ToF than in controls (37.6 +/- 27.5 vs 11.3 +/- 4.5 pg/mL, p = 0.0001). Forced vital capacity (FVC%) (84.9 +/- 16.9 vs 98.4 +/- 18.2, p = 0.01) and forced expiratory volume during the 1st second (FEV1%) (91.5 +/- 19.3 vs 103.8 +/- 16.1, p = 0.02) were decreased, exercise duration (ED) (10.1 +/- 1.9 vs 11.4 +/- 1.7 min, p = 0.02), maximum heart rate (HRmax) (171.2 +/- 18.9 vs 186.4 +/- 13.9 /min, p = 0.004), and maximum oxygen uptake (VO(2)max) (1.56 +/- 0.53 vs 2.1 +/- 0.6 L/min, p = 0.007) were lower in patients with ToF. There were significant correlations between the degree of PR and ED (r =-0.3, p = 0.009), HRmax (r =-0.4, p = 0.001), and VO(2)max (r =-0.4, p = 0.001). The correlations were significant both before and after exercise, being more pronounced after exercise between BNP level and the degree of PR (r = 0.6, p = 0.0001). As a result, the severity of PR has a negative influence on right ventricular functions and there is significant relation between right ventricular functions and exercise capacity after repair of tetralogy of Fallot.