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.

One-Year Follow-Up Results of MIS-C Patients with Coronary Artery Involvement: A Multi-center Study.

Multisystem inflammatory syndrome (MIS-C) in children is a rare complication of SARS-CoV-2 infection. Knowing the course of the affected or unaffected coronary arteries in the patients under follow-up is important in terms of defining the long-term prognosis of the disease and determining the follow-up plan. This is a multicenter and retrospective study. The data were obtained from nine different centers. Between May 2020 and August 2022, 68 of 790 patients had coronary artery involvement. One-year echocardiographic data of 67 of 789 MIS-C patients with coronary artery involvement were analyzed. Existing pathologies of the coronary arteries were grouped as increased echogenicity, dilatation and aneurysm according to Z scores, and their changes over a 1-year period were determined. The data of all three groups are defined as frequency. SPSS Statistics version 22 was used to evaluate the data. In our study, aneurysm was observed in 16.4%, dilatation in 68.7% and increased echogenicity in 13.4% of the patients. All of the patients with involvement in the form of increased echogenicity recovered without sequelae by the end of the first month. No progression to aneurysm was observed in any of the patients with dilatation. No new-onset involvement was observed in patients with previously healthy coronary arteries during the convalescent period. In addition, from the sixth month follow-up period, there was no worsening in the amount of dilatation in any of the patients. At least 94% of the patients who completed the 12th month control period returned to normal.

Single coronary artery and coronary artery-pulmonary artery fistula in a variation of the Shone complex.

We aim to share the diagnostic methods and relevant findings of a rare case involving a 1-year-old girl with a variation of Shone syndrome, presenting with the coexistence of a single coronary artery and a fistula between the coronary artery and the right pulmonary artery. The patient had previously undergone aortic arch reconstruction surgery and during the preoperative evaluation, cardiac computed tomography angiography (CCTA) revealed the additional presence of a single coronary artery originating from the right, which fistulized into the right pulmonary artery distally. This finding was further confirmed by conventional angiography. This case report highlights the importance of CCTA in identifying and directing the treatment of additional anomalies that may accompany rare congenital abnormalities.

A rare ventriculoarterial connection: double outlet of both ventricles.

Ventriculoarterial connection is one of the important points of the segmental approach to congenital cardiac malformations. Double outlet of both ventricles is a rare form where both great arterial roots override the interventricular septum. In this article, we aimed to draw attention to this very rare form of ventriculoarterial connection by presenting an infant case diagnosed using echocardiography, CT angiography, and 3-dimensional modelling.

A unique bizarre subtype of mixed total anomalous pulmonary venous connection.

Mixed total anomalous pulmonary venous connection is a rare CHD with a wide variation in pulmonary venous anatomy and drainage. We present a ten-day-old newborn with a rare bizarre subtype of mixed total anomalous pulmonary venous connection.

Clinical effects of major aortopulmonary collateral arteries in term neonates diagnosed with transposition of the great arteries.

BACKGROUND: Transposition of the great arteries is a severe CHD that affects term neonates. The presence of major aortopulmonary collateral arteries in neonatal transposition of the great arteries patients is rare. This study investigated the clinical and haemodynamic implications of the presence of major aortopulmonary collateral arteries in neonatal transposition of the great arteries patients who underwent an arterial switch operation. MATERIALS AND METHODS: The study was a retrospective analysis conducted on neonates diagnosed with transposition of the great arteries who underwent arterial switch operation within the period from 1 May 2020 to 1 January 2023 at two high-patient-volume paediatric cardiac surgery centres in Turkey. The patients' demographic characteristics, echocardiographic features, and clinical data were analysed. Additionally, the possible clinical effects of the presence of major aortopulmonary collateral arteries were statistically evaluated. RESULTS: Two hundred cases of neonatal transposition of the great arteries were included in this study, with 55% of the cases male. All the patients underwent arterial switch operation. The median age at the time of arterial switch operation was 5 days (interquartile range 3-7), with a median weight of 3,100 g (interquartile range 2,900-3,400). The median pre-operative saturation level was 76% (interquartile range 70-82%). Prior to arterial switch operation, 32 patients underwent balloon atrial septostomy.In all the patients, the interatrial septum was checked to determine if the atrial septum was intact. A patent foramen ovale (≤ 3 mm) was found in 112 patients, and a non-restrictive atrial septal defect (> 3 mm) was found in 88. Forty-eight patients had ventricular septal defects, and 72 had coronary anomalies. Major aortopulmonary collateral arteries were found in 4 patients pre-operatively and in 12 patients after arterial switch operation (echocardiography, n = 8; angiography, n = 4). Of the patients with post-operative detection of cumulative number of major aortopulmonary collateral arteries were on post-operative day 1 in 2 patients, on post-operative day 3 in 5 patients, on post-operative day 7 in 6 patients, and on post-operative day 14 in 11 patients.Transcatheter closure was performed in 3 cases due to recurrent extubation failure. Major aortopulmonary collateral artery shrinkage was observed in one case under medical treatment. The length of paediatric cardiac intensive care unit stay (10 days versus 8 days; p < 0.005), mechanical ventilator time (4 days versus 2 days; p = 0.02), and inotrope use time (5 days versus 3 days; p = 0.04) were higher in the major aortopulmonary collateral artery cases than patients without major aortopulmonary collateral artery. CONCLUSION: Major aortopulmonary collateral arteries are frequent in transposition of the great arteries patients and may have clinical effects. The presence of major aortopulmonary collateral arteries should be investigated in patients who do not have a favourable post-operative course after arterial switch operation.

Evaluation of perfusion index and left ventricular output changes in low cardiac output syndrome after arterial switch operation.

INTRODUCTION: Transposition of great arteries is one of newborns' most common cyanotic CHDs, and its treatment is arterial switch operation in the first days of life. Low cardiac output syndrome may develop in the early postoperative period. In this study, we evaluated perfusion index and left ventricular output blood flow changes in patients who underwent arterial switch operation and developed low cardiac output syndrome. METHODS: This study was conducted prospectively in newborns with transposition of great arteries who underwent arterial switch operation between 1st August 2020 and 1st August 2022. Low cardiac output syndrome score and left ventricular output were investigated. Initially, 6th, 12th, 18th, and 24th hour perfusion index and left ventricular output values of patients with and without low cardiac output syndrome were recorded. The results were evaluated statistically. RESULTS: A total of 60 patients were included in the study. Sex distribution was equal. The median age at the time of surgery was 5 days (interquartile range 3-7 days), and the median weight was 3.1 kg (interquartile range 2.9-3. 4). Low cardiac output syndrome was detected in 30% (n = 18) of cases. The median perfusion index of patients who developed low cardiac output syndrome was significantly lower at the 12th, 18th, and 24th hours (p < 0.05) (0.99 versus 1.25, 0.86 versus 1.21, and 0.96 versus 1.33, respectively). Similarly, the median left ventricular output of patients who developed low cardiac output syndrome was significantly lower at 12th, 18th, and 24th hours (p < 0.05) (95 versus 110 ml/kg/min, 89 versus 109 ml/kg/min, and 92 versus 112 ml/kg/min, respectively). There was a significant correlation between perfusion index values and left ventricular output at all measurements (r > 0.500, p < 0.05). CONCLUSION: Perfusion index and left ventricular output measurements decreased in newborns who developed low cardiac output syndrome after arterial switch operation, especially at 12th and 18th hours. Serial perfusion index and left ventricular output measurements can be instructive in predicting low cardiac output syndrome development.

Comparison of the effects of conventional method and primary sutureless techniques on early postoperative rhythm problems in patients with total abnormal pulmonary venous return anomaly.

BACKGROUND: Total abnormal pulmonary venous return anomaly is a CHD characterised by abnormal pulmonary venous flow directed to the right atrium. In this study, we aimed to compare the effects of these techniques on early rhythm problems in total abnormal pulmonary venous return anomaly cases operated with conventional or primary sutureless techniques. METHOD: Seventy consecutive cases (median age 1 month, median weight 4 kg) who underwent total abnormal pulmonary venous return anomaly repair with conventional or primary sutureless technique between May 1 2020 and May 1 2022 were evaluated. The rate, diagnosis, and possible risk factors of postoperative arrhythmias were investigated. The results were evaluated statistically. RESULTS: When the total abnormal pulmonary venous return anomaly subgroup of 70 cases was evaluated, 40 cases were supracardiac, 18 cases were infracardiac, 7 cases were cardiac, and 5 cases were mixed type. Twenty-eight (40%) cases had a pulmonary venous obstruction. Primary sutureless technique (57%, supracardiac n = 24, mixed = 3, infracardiac = 13) was used in 40 patients. Median cardiopulmonary bypass time (110 versus 95 minutes) and median aortic clamp time (70 versus 60 minutes), median peak lactate (4.7 versus 4.8 mmol/l) in the first 72 hours, and median peak vasoactive inotropic score in the first 72 hours of the primary sutureless and conventional technique used cases value (8 versus 10) were similar. The total incidence of arrhythmias in the conventional group was significantly higher than in the primary sutureless group (46.7% versus 22.5%, p = 0.04). Supraventricular early beat was observed in 3 (7.5%), sinus tachycardia was seen in 6 (15%), junctional ectopic tachycardia was seen in 1 (2.5%), intra-atrial reentry tachycardia was seen in 1 (2.5%), usual supraventricular tachyarrhythmia was seen in 2 cases (5%) in the primary sutureless group. In the conventional group, supraventricular early beat was observed in six of the cases (20%), sinus tachycardia in five (16.7%), junctional ectopic tachycardia in four (13.3%), intra-atrial reentry tachycardia (10%) in three, and supraventricular tachyarrhythmia in seven cases (23.3%). In the first 30 days, there was a similar mortality rate (10% versus 10%), with four patients in the primary sutureless group and three in the conventional group. The median follow-up period of the cases was 8 months (interquartile range (IQR) 6-10 months). In the follow-up, arrhythmias were detected in two cases (one supraventricular tachyarrhythmia and one intra-atrial reentry tachycardia) in the primary sutureless group and three cases (two supraventricular tachyarrhythmia, one intra-atrial reentry tachycardia) in the conventional technique. All cases were converted to normal sinus rhythm with cardioversion and combined antiarrhythmic therapy. CONCLUSION: Different arrhythmias can be observed in the early period in patients with operated total abnormal pulmonary venous return anomaly. Although a higher rate of rhythm problems was observed in the early period in the conventional method compared to the primary sutureless technique, no significant effect was found on mortality and morbidity between the groups.

The comparison of three different acute kidney injury classification systems after congenital heart surgery.

BACKGROUND: We aimed to compare the frequency of acute kidney injury (AKI) and its effects on mortality and morbidity with different classification systems in pediatric patients who had surgery under cardiopulmonary bypass for congenital heart disease. METHODS: This study included children younger than 18 years old who were followed up in the pediatric cardiac intensive care unit between September 1 and December 1, 2020, after congenital heart surgery with cardiopulmonary bypass. Each case was categorized postoperatively in terms of AKI using Pediatric-Modified Risk, Injury, Failure, Loss, and End-Stage (pRIFLE), Acute Kidney Injury Network (AKIN), and Kidney Disease: Improving Global Outcomes (KDIGO). Hospital mortality (developed within the first 30 days postoperatively) and morbidity (longer than 7 days intensive care unit stay) were compared by three model classes. Results were evaluated statistically. RESULTS: One hundred patients were included in the study. The median age was 3 months (1 day-180 months). Acute kidney injury was diagnosed in 49% of the cases according to the pRIFLE classification. It was diagnosed in 31% of the patients by AKIN classification. It was diagnosed in 41% of the patients with the KDIGO criteria. Morbidity was observed in 25% (n = 25) of all cases. The morbidity predictor was 0.800 for pRIFLE, 0.747 for AKIN and 0.853 for KDIGO by receiver operating characteristics analysis. All three categories predicted morbidity significantly (P < 0.001). Mortality was 10% (n = 10) for all groups. The mortality predictor was 0.783 for pRIFLE, 0.717 for AKIN and 0.794 for KDIGO by receiver operating characteristics analysis, and all three categories predicted mortality significantly (P < 0.001). CONCLUSIONS: Regardless of the three methods used, AKI was commonly detected in pediatric patients undergoing congenital heart surgery. pRIFLE classification diagnosed more patients with AKI than AKIN and KDIGO. The KDIGO and pRIFLE classifications were better in predicting hospital mortality.

Can we predict risk for cardiac involvement in paediatric inflammatory multi-system syndrome?

INTRODUCTION: Increasing recognition of paediatric inflammatory multi-system syndrome is a cause of concern. This study aimed to evaluate children with paediatric inflammatory multi-system syndrome and compare the clinical and laboratory features of children with and without cardiac involvement. MATERIAL AND METHODS: We conducted a prospective single-centre study including 57 (male 37, 65%) patients with paediatric inflammatory multi-system syndrome at a tertiary care hospital between November, 2020 and March, 2021. The mean age was 8.8 ± 4.5 years (range, 10 months-16.7 years). RESULTS: The most frequent symptoms were fever (100%), abdominal pain (65%) and diarrhoea (42%). SARS-CoV-2 PCR and serology tests were positive in 3 (5%) and 52 (91%) patients, respectively. Eight patients required intensive care support. Nineteen patients (33%) had cardiac involvement (valvular regurgitation in 15, left ventricular systolic dysfunction in 11 and coronary artery dilation in 1). The presence and duration of cough and intensive care admissions were significantly higher in children with cardiac involvement than those without it. The cut-off values of troponin T, pro-brain natriuretic peptide and interleukin 6 for predicting cardiac involvement were 11.65 ng/L (95% confidence interval, 0.63-0.90; sensitivity, 0.63; specificity, 0.84; area under the curve: 0.775, p = 0.009), 849.5 pg/mL (95% CI, 0.54-0.86; sensitivity, 0.63; specificity, 0.63; area under the curve: 0.706, p = 0.009) and 39.8 pg/mL (95% CI, 0.54-0.85; sensitivity, 0.63; specificity, 0.60; area under the curve: 0.698, p = 0.023), respectively. CONCLUSIONS: Cardiac involvement in children with paediatric inflammatory multi-system syndrome is common. The risk of cardiac involvement can be predicted by troponin T, pro-brain natriuretic peptide and interleukin 6 levels.

Evaluation of different lead types and implantation techniques in pediatric populations with permanent pacemakers: Single-center with 10 years' experience.

BACKGROUND: Permanent pacemaker (PM) implantation is performed for various indications and by different techniques in children; however, many problems with lead performance are encountered during follow-up. This study aims to evaluate the possible effects of different lead types and implantation techniques on pacing at early and midterm in children with a permanent PM. PATIENTS AND METHODS: Pediatric patients who underwent permanent PM system implantation at our tertiary cardiac surgery center between January 1, 2010 and January 1, 2020 were evaluated retrospectively. Patients were categorized in the epicardial pacing lead (EP), transvenous pacing lead (TP), and transvenous bipolar lumenless (Select Secure [SS]) lead groups according to the lead implantation technique and lead type with the same manufacturer. Groups were evaluated statistically for demographic features, pacing type and indication for implantation, lead electrical performance, lead failure, complications, and outcome. RESULTS: Over 10 years, 323 lead implantations were performed on 167 patients (96 males, median age 68 months [5 days-18 years]). Of 323 leads, 213 (66%) were EP, 64 (20%) were TP, and 46 (14%) were SS. Of the total, 136 of the leads were implanted in atria, and 187 were implanted in ventricles. Primary pacing indications were postoperative complete atrioventricular (AV) block (n = 95), congenital AV block (n = 71), sinus node dysfunction (n = 13), and acquired complete AV block (n = 1). Additional cardiac diseases were present in 115 patients (69%). No statistically significant difference was observed in gender, syndrome, or pacing indication (P > .05). Atrial and ventricular capture, threshold, sensing, and lead impedance measurements were not significantly different at the initial and follow-up periods (P > .05). The median follow-up duration was 3.3 years (6 months-10 years). Twenty lead failures were determined in 15 patients (EP: 14 lead failures in 10 patients; TP: two lead failures in two patients; and SS: four lead failures in three patients) during follow-up, and no statistically significant difference was found between groups (P = .466). The 5-year lead survival was 98% for TP, 95% for EP, and 90% for SS; the 10-year lead survival was 90% for TP, 70% for EP, and 70% for SS. There was no mortality related to chronic pacing or due to the procedure of implantation. CONCLUSIONS: Despite improvements in technology, lead failure is still one of the most critical problems during these patients' follow-up. Early to midterm lead survival rates of all three lead types were satisfactory.

Repair of complete atrioventricular canal defects: Early and midterm results and comparison of the left anterior leaflet augmentation technique with traditional technics.

BACKGROUND: Complete atrioventricular septal defects (CAVSD) include a variable spectrum of congenital malformations with different forms of clinical findings. We examined early and midterm outcomes, the need for reoperation, postoperative residual AV valve regurgitation, and other risk factors after various CAVSD repairs. METHODS: Between 2014 and 2018, we have performed 89 isolated CAVSD repairs. We retrospectively reviewed the patients' medical records. Patients were divided into three groups according to their repair techniques modified one patch repair (MP) (n = 16); two patch repair (TP) (n = 49); and left anterior leaflet augmentation (ALA) technique (n = 24). RESULTS: Eighty patients (89.8%) survived hospital discharge. Early mortality rates were three (18.8%) in the group MP, five (10.2%) in the group TP, and one (4.2%) in the group ALA. Thirteen patients died during the follow-up period. Late mortality rates were three (18.8%) in the group MP, four (16.6%) in the group ALA, and six (12.2%) in the group TP. The mean follow-up time was 35.9 ± 22.97 months (range: 0.3-77 months). The morbidity and mortality results were similar between-group TP and ALA but worse in the MP group. Low body weight (<4 kg) and younger age at surgery (<4 months) were found to be risk factors on mortality by univariate and multivariate analysis. Surgical technic was not found to be an independent risk factor. CONCLUSION: In our series, TP and ALA techniques had satisfactory results in early and midterm periods. Younger age and small bodyweight might increase early mortality and the need for reoperation.

Catheter ablation of focal atrial tachycardia in children using three-dimensional electroanatomic mapping system: a 6-year single-centre experience.

OBJECTIVE: This study demonstrates the clinical and electrophysiological details of catheter ablation conducted in children with focal atrial tachycardia using three-dimensional electroanatomic mapping systems. PATIENTS AND METHODS: Electrophysiological procedures were performed using the EnSite™ system. RESULTS: Between 2014 and 2020, 60 children (median age 12.01 years [16 days-18 years]; median weight 41.5 kg [3-98 kg]) with focal atrial tachycardia and treated with catheter ablation were evaluated retrospectively. Tachycardia-induced cardiomyopathy was developed in 15 patients (25%). Most of the focal atrial tachycardia foci were right-sided (75%), and more than one focus was found in four patients. Radiofrequency ablation was performed in 47 patients (irrigated radiofrequency ablation in seven cases), cryoablation in 9, and radiofrequency ablation and cryoablation in the same session in 4 patients. The median procedural time was 163.5 minutes (82-473 minutes). Fluoroscopy was used in 29 of (48.3%) patients (especially for left-side substrate) with a mean time of 8.6 ± 6.2 minutes. The acute success rate was 95%. The procedure failed in three patients, and recurrence was observed in 3.5% of patients (2/57) during a median follow-up of 17 months (2-69 months). The second ablation was performed in four cases, of which three were successful. Overall success rate was 96.6% with no major complications observed, except in one patient with minimal pericardial effusion. CONCLUSION: Catheter ablation seems to be an effective and safe treatment in focal atrial tachycardia. Electroanatomic mapping system can facilitate the ablation procedure and minimise radiation exposure.

Cardiac resynchronization therapy in paediatric patients with congenital heart disease: single centre with 10 years of experience.

OBJECTIVES: In recent years, cardiac resynchronization therapy (CRT) has also started to be performed in the paediatric and CHD population. This study aimed to evaluate the efficacy of CRT in children with CHD. PATIENTS AND METHODS: Patients with CHD who underwent CRT treatment in our paediatric cardiology clinic between January, 2010 and January, 2020 were included in the study. Demographic findings, 12-lead electrocardiograms, echocardiograms, clinical characteristics, management strategies, and outcomes were reviewed systematically. RESULTS: The study population consisted of 18 CHD patients who had been treated with CRT for 10 years in our institution. The median age was 11 years (2.2-18 years) and the median weight was 39 kg (10-81 kg). Systemic ventricle was left ventricle in 13 patients, right ventricle in 4 patients, and 1 patient had single-ventricle physiology. CRT implantation indications were as follows: dysfunction after permanent pacemaker in 11 patients, dysfunction after left bundle branch block in 4 patients, and systemic ventricular dysfunction in 3 patients. CRT implantation techniques were epicardial (n = 13), hybrid (n = 4), and transvenous (n = 1) methods. QRS duration significantly decreased after CRT implantation (160 versus 124 m/second, p < 0.05). Median systemic ventricle ejection fraction (EF) significantly increased after the procedure (30 versus 50%, p < 0.05). Fourteen patients (78%) were responders, two patients (11%) were superresponders, and two patients (11%) were non-responders after the CRT treatment. One patient deceased during follow-up. Median follow-up duration was 40 months (6-117 months). CONCLUSION: When electromechanical dyssynchrony occurs in paediatric cases with CHD and developing heart failure, patients should be evaluated in terms of CRT to improve ventricular function. Alternative CRT therapy will be beneficial in these cases that do not improve clinically despite optimal medical treatment.

Electrocardiographic properties of Topsy-Turvy heart.

Topsy-turvy heart (TTH) is an exceedingly rare cardiovascular malformation characterized by clockwise rotation of the entire heart around its longitudinal axis. There is superoinferior ventricle positioning without the twisting of atrioventricular connections. Electrocardiography (ECG) is a simple noninvasive tool to aid the diagnosis of heart disease. In this case series, we discuss 12-lead ECG findings of three patients with TTH.

Early results of neopulmonary valve creation technique using right atrial appendage tissue.

INTRODUCTION: Various valve repair techniques have been described for prevention of pulmonary insufficiency (PI) during right ventricular outflow tract (RVOT) reconstruction with transannular patch. Herein, we present the early results of an alternative technique of neopulmonary valve reconstruction using right atrial appendage (RAA) tissue. METHODS: Between October 2019 and December 2019, 12 patients with tetralogy of fallot (TOF) (n = 10), TOF-absent pulmonary valve (n = 1) and intact ventricular septum-pulmonary atresia (n = 1) underwent RVOT reconstruction with neopulmonary valve that was created using RAA tissue. Median age and weight of patients were 9.9 months (5 months-14 years) and 9.8 kg (6.2-47), respectively. RESULTS: No mortality or major events developed. Postoperative echocardiography revealed none/trace PI in 11 patients and mild PI in 1 patient. Median right ventricle/left ventricle ratio was 0.46 (0.35-0.65) and RVOT gradient was 20 mm Hg (0-30). Median cardiopulmonary bypass and aortic clamping times were 121 minutes (81-178) and 94 minutes (59-138), respectively. Operative times revealed statistically significant learning curve pattern in terms of cardiopulmonary bypass (r2 = .568; P = .005) and aortic clamping times (r2 = .635; P = .002). Median ventilation time, intensive care unit stay, and the length of hospital stay were 6 hours (2-48), 1 days (1-5), and 7 days (4-10), respectively. Longer perfusion time was not correlated with postoperative times. At median 6 months, echocardiography showed none/trivial PI in 11 patients and mild PI in one patient. CONCLUSION: Early results showed that neopulmonary valve reconstruction using the RAA tissue may provide a reasonable alternative for RVOT reconstruction with transannular patch. But long-term results are needed.

Short- and midterm outcomes of aortic arch reconstruction: Beating heart versus cardiac arrest.

OBJECTIVES: We aimed to compare the short- and midterm results of perfusion strategies used for arch reconstruction surgery. MATERIAL AND METHODS: One hundred and seventy-three consecutive patients who underwent aortic arch reconstruction surgery for transverse arcus hypoplasia between January 2011 and February 2020 were retrospectively analyzed. The patients were divided into two groups, as beating heart (BH) group and cardiac arrest (CA) group. RESULTS: The CA group comprised 60 (35%) patients and the remaining 113 (65%) patients were in the BH group. The median age of the patients was 30 (interquartile range: 18-95) days. The incidences of acute renal failure (ARF) and delayed sternal closure were higher in the CA group (p = .05, <.001, respectively). Balloon angioplasty was performed in 5 (2%) patients and reoperation was performed in 11 (6%) patients due to restenosis. There were no statistically significant differences between the two groups in terms of reoperation or reintervention rates (p = .44 and .34, respectively). CONCLUSIONS: Both strategies were associated with satisfactory midterm prevention of reintervention and reoperation. Given the lower incidence of ARF and delayed sternal closure in the postoperative period and similar midterm outcomes, we believe that the BH strategy is preferable.

Parameters affecting pleural drainage and management strategy after Fontan operation.

BACKGROUND: Prolonged pleural drainage is a common complication after undergoing the Fontan procedure. Although various protocols have been described, there is no definitive consensus for how to treat this complication. MATERIALS AND METHODS: Our primary aim was to determine the effect of the management strategy protocol on the duration of drainage and length of hospital stay. Our secondary aim was to determine the parameters affecting the need for prolonged drainage after the Fontan procedure. Ninety-two consecutive patients who underwent the Fontan procedure were retrospectively analyzed. A protocol-based postoperative management strategy was adopted in July 2018. Group 1 (n = 48) consisted of patients that underwent the procedure before the protocol was implemented. Group 2 (n = 44) consisted of patients that underwent the procedure after the protocol was implemented. RESULTS: The mean age was 5 years (interquartile range [IQR], 4.0-6.9); the mean body weight was 17.3 kg (IQR, 15.1-21.8). Statistically significant differences were found between the groups in terms of total drainage, duration of pleural drainage, prolonged drainage, and length of hospital stays (LOHS) (P = .05, P = .04, P = .04, P = .04, respectively). The multivariate analysis results showed that the application of the protocol was the only factor impacting prolonged drainage (OR, 2.46, 95% CI lower-upper: 1.03-5.86, P = .04). CONCLUSION: Standardization and strict application of the medical treatment within a specific protocol without being affected by doctor-, nurse-, or patient-based factors increases the success rate of this procedure. After implementing the changes in the medical management strategy, total drainage and duration of pleural drainage and LOHS decreased, and the costs associated with these factors also decreased.

A rare cause of abnormal pulmonary venous drainage: septum primum malposition.

OBJECTIVES: This study aimed to evaluate the clinical features of patients with septum primum malposition, imaging tools used for diagnosis, and their effects on the surgical approach. MATERIALS AND METHODS: Patients diagnosed with septum primum malposition in our paediatric cardiac centre between 1 January, 2015 and 1 January, 2019 were included in the study. In all patients, the age, reason for admission, transthoracic echocardiography, cardiac multidetector CT angiography findings, and subsequent surgical data were evaluated. RESULTS: Fifteen patients were diagnosed with septum primum malposition during the study period. The median age was 12 months (2 months-10 years). Six patients were left isomeric, and the rest were situs solitus; 80% of the patients (n = 12) had additional secundum atrial septal defect. There was cardiac pathology in 46% of the patients (n = 7) in addition to the abnormal pulmonary venous drainage, ventricular septal defect (n = 3), left ventricularhypoplasia (n = 2), cortriatriatum sinister (n = 2), double outlet right ventricle (n = 1), and atrioventricular septal defect (n = 1). There was bilateral superior caval vein in three patients, right-sided superior caval vein in 11 patients, and left-sided superior caval vein in one patient. All three patients with total abnormal pulmonary venous drainage were left atrial isomeric. There were differences between the results of transthoracic echocardiography and CT angiographies in two patients. The surgical strategy was changed in three patients after the preoperative diagnosis of septum primum malposition. CONCLUSION: Septum primum malposition should be kept in mind during the imaging of complex CHDs specifically during the segmental analysis of the pathologies with heterotaxy syndromes; it should be differentiated from other aetiologies of abnormal pulmonary venous drainage as accurate diagnosis would facilitate the ideal surgery in these complex pathologies requiring a detailed preoperative preparation.

Use of extracorporeal membrane oxygenation in postcardiotomy pediatric patients: parameters affecting survival.

AIM: We aimed to investigate the risk factors affecting survival after extracorporeal membrane oxygenation use in pediatric postcardiotomy patients. METHODS: One hundred thirty-three consecutive patients who underwent surgery for congenital heart disease who needed extracorporeal membrane oxygenation support were retrospectively analyzed. RESULTS: In all, 3,082 patients were operated, of which 140 patients (4.54% of the total number of operations) needed extracorporeal membrane oxygenation. Eighty (60.1%) patients were successfully weaned and 51 (38.3%) patients were discharged. Of the 50 patients discharged during the mean follow-up period of 34.8 (0-192.4) months, 6 (12%) patients died. The extracorporeal membrane oxygenation support was instituted in 29 (21.8%) patients for extracorporeal membrane oxygenation cardiopulmonary resuscitation, in 44 (33.1%) patients due to the inability to be separated from cardiopulmonary bypass, in 19 (14.3%) patients due to respiratory failure, and in 41 patients due to low cardiac output syndrome. Eighty patients (60.2%) were successfully weaned from extracorporeal membrane oxygenation support. The remaining 53 (39.8%) patients died on extracorporeal membrane oxygenation. Mortality was observed in 29 (21.8%) of the 80 patients in the successful weaning group, while the remaining 51 (38.3%) patients were discharged from the hospital. Multivariate analysis showed that double-ventricular physiology increased the rate of successful weaning (odds ratio: 3.4, 95% confidence interval lower: 1.5 and upper: 8, p = 0.004) and prolonged extracorporeal membrane oxygenation durations were a risk factor in successful weaning (odds ratio: 0.9, 95% confidence interval lower: 0.8 and upper: 0.9, p = 0.007). The parameters affecting mortality were the presence of syndrome (odds ratio: 3.8, 95% confidence interval lower: 1.0 and upper: 14.9, p = 0.05), single-ventricular physiology (odds ratio: 5.3, 95% confidence interval lower: 1.8 and upper: 15.3, p = 0.002), and the need for a second extracorporeal membrane oxygenation (odds ratio: 12.9, 95% confidence interval lower: 1.6 and upper: 104.2, p = 0.02). While 1-year survival was 15.2% and 3-year survival was 12.1% in patients with single-ventricular physiology, the respective survival rates were 43.9% and 40.8%. CONCLUSION: Parameters affecting mortality after extracorporeal membrane oxygenation support in pediatric postcardiotomy patient group were the presence of a syndrome, multiple runs of extracorporeal membrane oxygenation, and single-ventricular physiology. Timing of extracorporeal membrane oxygenation initiation, appropriate patient selection, appropriate reintervention or reoperation for patients with correctable pathology, the use of an appropriate cannulation strategy in single-ventricle patients, management of shunt flow, and appropriate interventions to reduce the incidence of complications play key roles in improving survival.