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.

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.

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.

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.

Easy Fenestration Technique for Extracardiac Fontan Operation.

During an extracardiac conduit type of Fontan operation, fenestration is not always technically easy, particularly when the right atrium is small and located posteriorly. We describe here an easy technique for performing an anastomosis of a polytetrafluoroethylene ringed graft to the atrium for fenestration in the setting of an extracardiac conduit Fontan operation. The surgical technique starts with a purse string suture that is customized to fit the atrium and utilizes a ringed graft that is inserted into the right atrium through the purse string, which is then tied.

The use of "aortic plication" for coronary malperfusions in the arterial switch operation.

During an arterial switch operation, reimplantation of the transferred coronary artery is required for malperfusion. However, this reimplantation increases the mortality risk. We describe here a simple technique in patients with malperfusion of the coronary arteries during the arterial switch operation. The surgical technique consists of an aortic plication on the opposite side of the kinking coronary artery.

The effect of corrective surgery on serum IGF-1, IGFBP-3 levels and growth in children with congenital heart disease.

The aim of this study is to evaluate growth and insulin-like growth factor 1 (IGF-1) and insulin-like growth factor binding protein 3 (IGFBP-3) levels in infants with congenital heart disease (CHD) pre- and postoperatively over a period of a year. Anthropometric values and serum levels of IGF-1 and IGFBP-3 of 40 infants with CHD (20 cyanotic and 20 acyanotic) were compared with 32 healthy controls. Acyanotic infants and infants with pulmonary hypertension (PH) presented significantly more growth failure. Preoperatively, serum IGF-1 and IGFBP-3 levels were lower in the acyanotic group than the cyanotic and the control groups (p = 0.22; p < 0.01). The upward trend in IGF-1 and IGFBP-3 levels in this year-long study demonstrated that the values in the third month and the first year were higher than the preoperative values (p < 0.05). The parallel increase of weight gain and IGF-1, IGFBP-3 levels were the best evidence that these parameters are good nutritional indicators. Timing the corrective surgery before chronic malnutrition or PH develops is an important issue to maintain a normal growth for children with CHD.

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.

Effect of obesity on inflammatory markers and renal functions.

AIM: To examine the relationship between inflammation criteria and body mass index in otherwise-healthy obese schoolchildren and to evaluate the effect of obesity on renal functions. METHODS: Sixty-five otherwise-healthy obese children (median age 10.8 y, range 7.1-16.5 y; median body mass index 26.8 kg/m(2), range 19.9-38.7 kg/m(2)) and 20 healthy controls (median age 12.4 y, range 10.1-17.1 y; median body mass index 18.8 kg/m(2), range 17.3-23.1 kg/m(2)) were included. Blood and urine samples were taken from every child. RESULTS: Children in the obese and control groups had similar age and sex distributions (p>0.05). Inflammatory mediators were higher in obese children (p<0.05). A significant positive correlation was found between glomerular filtration rate and body mass index in the whole study group (r=0.39, p=0.001). A positive correlation was found between body mass index standard deviation and inflammatory mediators and glomerular filtration rate. No significant difference existed regarding protein and microalbumin excretion in the urine. CONCLUSION: Inflammatory mediators increased significantly in obese children, and the glomerular filtration rate increased as the body mass index increased. To prevent obesity-related complications in adulthood, it is important to take measures to prevent development of obesity during childhood.