The Lifesaving Impact of Transcatheter Interventions in the Early Post-Fontan Palliation Period.

Despite advancements in postoperative outcomes after Fontan surgery, there remains a risk of suboptimal outcomes and significant morbidity in the early postoperative period. Anatomical obstructions in the Fontan pathway can lead to prolonged pleural effusion or ascites, cyanosis, and low cardiac output syndrome (LCOS). Transcatheter interventions offer an alternative to early re-surgery for treating these complications. Over a 13-year period, early catheter angiography, performed within 30 days post-index procedure, was administered to 41 patients, identifying anatomical issues that necessitated re-intervention in 39 cases. This led to transcatheter interventions in 37 (10.4%) of the 344 Fontan surgery patients. The median age was 4.8 years (IQR: 4-9.4), and the median weight was 16.5 kg (IQR: 15-25.2), with females comprising 51.4% (19/37) of this group. The primary indications for the procedures were persistent pleural effusion or ascites in 27 patients (66%), LCOS in 8 patients (20%), and cyanosis in 6 patients (14%). Among the 37 undergoing transcatheter intervention, 30 were treated solely with this method and discharged, three died in ICU follow-up, and four required early re-surgery. No procedural mortality was observed. Our findings demonstrate that transcatheter interventions, including stent implantation, balloon angioplasty, and fenestration dilation, are safe and effective in the early post-Fontan period. Therefore, they should be considered an integral part of the management strategy for this patient group.

Utility of Balloon Occlusion Testing in Determining Fontan Suitability Among Patients with Elevated Pulmonary Artery Pressure and Additional Antegrade Pulmonary Blood Flow.

In individuals with a single ventricle undergoing evaluation before Fontan surgery, the presence of excessive pulmonary blood flow can contribute to increased pulmonary artery pressure, notably in those who had a Glenn procedure with antegrade pulmonary flow. 28 patients who had previously undergone Glenn anastomosis with antegrade pulmonary blood flow (APBF) and with elevated mean pulmonary artery (mPAP) pressure > 15 mmHg in diagnostic catheter angiography were included in the study. After addressing other anatomical factors that could affect pulmonary artery pressure, APBF was occluded with semi-compliant, Wedge or sizing balloons to measure pulmonary artery pressure accurately. 23 patients (82% of the cohort) advanced to Fontan completion. In this group, median mPAP dropped from 20.5 (IQR 19-22) mmHg to 13 (IQR 12-14) mmHg post-test (p < 0.001). Median PVR post-test was 1.8 (IQR 1.5-2.1) WU m2. SpO2 levels decreased from a median of 88% (IQR 86%-93%) pre-test to 80% (IQR 75%-84%) post-test (p < 0.001). In five patients, elevated mPAP post-test occlusion on diagnostic catheter angiography led to non-completion of Fontan circulation. In this group, median pre- and post-test mPAP were 23 mmHg (IQR 21.5-23.5) and 19 mmHg (IQR 18.5-20), respectively (p = 0.038). Median post-test PVR was 3.8 (IQR 3.6-4.5) WU m2. SpO2 levels decreased from a median of 79% (IQR 76%-81%) pre-test to 77% (IQR 73.5%-80%) post-test (p = 0.039). Our study presents a specialized approach for patients initially deemed unsuitable for Fontan due to elevated pulmonary artery pressures. We were able to successfully complete the Fontan procedure in the majority of these high-risk cases after temporary balloon occlusion test.

Surgical Treatment of Pediatric Refractory Ventricular Tachycardia Originating From a Left Ventricular Rhabdomyoma.

Cardiac tumors are very rare in children, and echocardiography is very important in their detection. The clinical presentation can vary greatly depending on arrhythmia or obstruction. One of the most important factors determining the surgical approach is the clinical process. In this case report, we report the surgical treatment of a rhabdomyoma that caused refractory ventricular tachycardia.

Surgical repair of the anomalous aortic origin of the coronary arteries: a single-center experience.

OBJECTIVE: Anomalous aortic origin of the coronary artery is a rare congenital cardiac anomaly. The aim of this study was to present our experience with patients who underwent surgery for the anomalous aortic origin of the coronary artery. METHODS: This was a retrospective review of our experience with patients who had anomalous aortic origin of the coronary artery from 2019 to 2022. RESULTS: Seven patients were managed for anomalous aortic origin of the coronary artery, including five males and two females. The median age of the patients were 16 years (IQR, 14.5-26 years). Five patients had anomalous aortic origin of the right coronary artery and two patients had anomalous aortic origin of the left coronary artery. Five patients were treated surgically, one patient refused surgical treatment despite myocardial ischaemia symptoms, and the other one was not operated because she had no symptoms. Two patients underwent pulmonary root anterior translocation and left main coronary artery unroofing procedure, one patient underwent right coronary artery unroofing procedure, one patient underwent pulmonary artery lateral translocation procedure, and the last patient underwent right coronary artery osteal translocation procedure. The post-operative mortality or myocardial infarction was not observed in any patient. Patients were followed for a median of 10 months (IQR, 6.75-20.5 months) after repair. CONCLUSION: The data suggest that surgical repair of anomalous aortic origin of the coronary artery can be performed confidently and can be very effective for relieving myocardial ischaemia symptoms. Different surgical techniques can be used in anomalous aortic origin of the coronary artery according to the course and origin of the coronary arteries. To the our knowledge, pulmonary root anterior translocation and coronary artery unroofing procedure were performed for the first time in the literature.

Surgical management of cardiac cystic echinococcosis in a paediatric patient: a case report.

Cystic echinococcosis, a zoonotic parasitic disease, is endemic to many countries worldwide. This slowly progressing disease is seen rarely in the paediatric age group. In terms of cyst localization, cardiac involvement is infrequent. We report the case of a successful surgical and medical management of a paediatric hydatid disease patient with involvement of the heart.

A novel Fontan Y-graft for interrupted inferior vena cava and azygos continuation.

OBJECTIVES: To evaluate the hemodynamicdynamic advantage of a new Fontan surgical template that is intended for complex single-ventricle patients with interrupted inferior vena cava-azygos and hemi-azygos continuation. The new technique has emerged from a comprehensive pre-surgical simulation campaign conducted to facilitate a balanced hepatic flow and somatic Fontan pathway growth after Kawashima procedure. METHODS: For 9 patients, aged 2 to18 years, majority having poor preoperative oxygen saturation, a pre-surgical computational fluid dynamics customization is conducted. Both the traditional Fontan pathways and the proposed novel Y-graft templates are considered. Numerical model was validated against in vivo phase-contrast magnetic resonance imaging data and in vitro experiments. RESULTS: The proposed template is selected and executed for 6 out of the 9 patients based on its predicted superior hemodynamic performance. Pre-surgical simulations performed for this cohort indicated that flow from the hepatic veins (HEP) do not reach to the desired lung. The novel Y-graft template, customized via a right- or left-sided displacement of the total cavopulmonary connection anastomosis location resulted a drastic increase in HEP flow to the desired lung. Orientation of HEP to azygos direct shunt is found to be important as it can alter the flow pattern from 38% in the caudally located direct shunt to 3% in the cranial configuration with significantly reversed flow. The postoperative measurements prove that oxygen saturation increased significantly (P-value = 0.00009) to normal levels in 1 year follow-up. CONCLUSIONS: The new Y-graft template, if customized for the individual patient, is a viable alternative to the traditional surgical pathways. This template addresses the competing hemodynamic design factors of low physiological venous pressure, high postoperative oxygen saturation, low energy loss and balanced hepatic growth factor distribution possibly assuring adequate lung development. DATE AND NUMBER OF IRB APPROVAL: 25 October 2019, 280011928-604.01.01.

Evaluation of midterm outcomes after Shone's complex surgery: Analysis of reoperation and mortality risk factors.

OBJECTIVE: The midterm results of patients who underwent biventricular repair surgery for Shone's complex were examined, and mortality and reoperation risk factors were evaluated. METHODS: This retrospective study included 34 patients with Shone's complex who underwent mitral valve (MV) surgery between 2005 and 2020. RESULTS: A total of 19 patients (56%) had coarctation, 10 (29%) patients had subaortic stenosis, 9 (26.5%) patients had a hypoplastic aortic arch (AA), and 9 (26.5%) patients had aortic valve (AV) stenosis. Twenty-four (70.6%) patients had bileaflet AV. Associated left-sided in-flow stenotic lesions included parachute MV in 19 (56%) patients and supramitral ring in 18 (53%) patients. The estimated freedom from reoperation rate on the 6th month, 1 year and 2 years after surgery was 84.4%, 79.5%, and 71.5%, respectively. The overall mortality rate was 20.6% (seven patients) with a median follow-up of 10 months (0-41). The estimated survival rate on the 6th month, 1 year, and 3 years after surgery was 83.8%, 79.4%, and 79.4 respectively. Bicuspid aortic valve (p = .017) (HR (95% CI) = 0.130 (0.025-0.695) and hammock mitral valve (p = .038) (HR (95% CI) = 11,008 (1,146->100) were associated with mortality. CONCLUSION: The presence of a bicuspid aortic valve hammock mitral valve might have an effect on negative effect on the outcome.

The choice of palliative arterial switch operation as an alternative for selected cases in a single center: Experience and mid term results.

INTRODUCTION AND OBJECTIVE: There are various management options for newborns with single ventricle physiology, ventriculoarterial discordance and subaortic stenosis, classically involving the early pulmonary banding and aortic arch repair, the restricted bulboventriculer foramen enlargement or the Norwood and the Damus-Kaye-Stansel procedures. The aim of this study is to evaluate our preferred technique and comment on the midterm results of our clinical experience with palliative arterial switch operation (pASO) for a certain subset of patients. METHOD: We hereby retrospectively evaluate the charts of patients who went through pASO, as initial palliation through Fontan pathway, starting from 2014 till today. RESULTS: Ten patients underwent an initial palliative arterial switch procedure. Eight of 10 patients survived the operation and discharged. Seven of 10 patients completed Stage II and 1 patient reached the Fontan completion stage and the other six of ten (6/10) patients are doing well and waiting for the next stage of palliation. There are two mortalities in the series (2/10) and one patient lost to follow-up (1/10). CONCLUSION: In our opinion, the pASO can be considered as an alternative palliation option for patients with single ventricle physiology, transposition of the great arteries and systemic outflow tract obstruction despite longer cross clamp times compared to other methods, but It not only preserves systolic and diastolic ventricular function, but also provides a superior anatomic arrangement for following stages.

The impact of COVID-19 pandemic on congenital heart surgery practice: An alarming change in demographics.

BACKGROUND: The aim of this study is to investigate the effect of COVID-19 outbreak on congenital cardiac surgery practice in a single center. METHODS: The first case of COVID-19 in our country was seen on March 11th, 2020. The patients operated between March 11th, 2019-and March 10th, 2020 were taken as the pre-COVID group, and those operated between March 11th and May 11th, 2020 were taken as the COVID group. The data was retrospectively collected, and the two periods were compared. RESULTS: Monthly average number of cases which was 52 patients/month (626 patients in 12 months) before COVID decreased to 35 patients/month (70 patients in 2 months) during COVID periods (P < .01). During the pre-COVID period the median postoperative length of hospital stay was 3 (IQR: 1-5) days. During the COVID period, this decreased to 1 (IQR: 1-3) day (P < .01). During the pre-COVID period, the hospital expenses of 17% (8/47) of the foreign nationals were covered by their homeland. The remaining 83% (39/47) were paid from the asylum seekers' fund. The proportion of foreign nationals operated significantly decreased during the COVID period ([7%; 47/632 vs 1%; 1/70]; P = .04). No significant difference was observed in terms of STAT mortality scores and categories and postoperative results of the operations performed between the two periods. CONCLUSIONS: Congenital cardiac surgery practice can be safely maintained with restricted case volume during the pandemic period. It is alarming that patients in the deprived areas cannot access pediatric cardiac surgery and possibly other health services because of closure of the borders between countries.

Modified Closed Coronary Transfer is a Good Alternative to the Trap-Door Method During Arterial Switch Operation: a Retrospective Propensity-Matched Comparison.

OBJECTIVE: To compare the early and long-term results of patients in whom was performed modified closed coronary transfer with the results of patients in whom was performed trap-door transfer techniques by utilizing propensity-matching analysis to provide optimal identical patient matching for the groups. METHODS: From August 2015 to December 2017, 127 consecutive patients underwent arterial switch operation due to simple and complex transposition of the great arteries, with or without additional arch and complex coronary pattern, by a single surgical team included into the study. Of these, in 70 patients it was performed modified closed coronary transfer technique and in 57 patients it was performed trap-door style coronary transfer technique. The patients were divided into two groups in terms of coronary transfer method. In the final model, after propensity matching, 47 patients from each group having similar propensity score were included into the study. RESULTS: There was no significant difference between the groups regarding patient characteristics. Cross-clamp time and operation time were significantly lower in the modified technique group compared with the other group (P=0.03 and P=0.05, respectively). When compared the early and late postoperative outcomes, there was no significant difference between the groups. Postoperative echocardiographic findings were mostly similar between the groups. CONCLUSION: The patients in whom was performed our modified technique demonstrate overall good outcomes and the current technique ensures shorter arterial cross-clamp and operation times. It may be an alternative method to the trap-door technique for the coronary transfer during the arterial switch operation.

The poor long-term outcomes of owl's eye pulmonary reconstruction technique after arterial switch operation.

OBJECTIVES: This study aims to compare the early- and long-term outcomes of patients who undergo owl's eye pulmonary artery (PA) reconstruction to those of patients who undergo conventional PA reconstruction. METHODS: From January 2016 to January 2017, 64 consecutive patients underwent an arterial switch operation. The patients were divided into 2 groups in terms of neo-PA reconstruction method: 30 patients who underwent neo-PA reconstruction by owl's eye technique were defined as group 1 and 34 patients who underwent neo-PA reconstruction by the conventional approach were defined as group 2. In the final model, after propensity matching, 23 patients from each group with similar propensity scores were included in the study. RESULTS: There was no significant difference between the groups regarding patient characteristics and operative findings. In the early period, the duration of intensive care unit and hospital stays and the rate of mild neo-pulmonary stenosis (neo-PS) were significantly higher in the owl's eye group (P = 0.04, 0.04 and 0.03). In the late period, the rate of severe neo-PS and reintervention was significantly higher in the owl's eye group (P = 0.02 and 0.04). Furthermore, the rates of 3-year freedom from pulmonary reintervention and freedom from moderate-severe neo-PS were significantly lower in group 1 (P = 0.04). In addition, the owl's eye reconstruction was the only factor independently related to moderate-severe neo-PS in the long term (hazard ratios = 11.2, P = 0.02). CONCLUSIONS: We have abandoned the owl's eye method for neo-PA reconstruction of the neo-PA because of serious complications. According to our series and the literature, reconstruction of the neo-PA with an oversized, pantaloon-shaped fresh autologous pericardial patch is still superior to the other techniques.

Modified closed coronary transfer is a good alternative to the trap-door method during arterial switch operation: a retrospective propensity-matched comparison

Abstract Objective: To compare the early and long-term results of patients in whom was performed modified closed coronary transfer with the results of patients in whom was performed trap-door transfer techniques by utilizing propensity-matching analysis to provide optimal identical patient matching for the groups. Methods: From August 2015 to December 2017, 127 consecutive patients underwent arterial switch operation due to simple and complex transposition of the great arteries, with or without additional arch and complex coronary pattern, by a single surgical team included into the study. Of these, in 70 patients it was performed modified closed coronary transfer technique and in 57 patients it was performed trap-door style coronary transfer technique. The patients were divided into two groups in terms of coronary transfer method. In the final model, after propensity matching, 47 patients from each group having similar propensity score were included into the study. Results: There was no significant difference between the groups regarding patient characteristics. Cross-clamp time and operation time were significantly lower in the modified technique group compared with the other group (P=0.03 and P=0.05, respectively). When compared the early and late postoperative outcomes, there was no significant difference between the groups. Postoperative echocardiographic findings were mostly similar between the groups. Conclusion: The patients in whom was performed our modified technique demonstrate overall good outcomes and the current technique ensures shorter arterial cross-clamp and operation times. It may be an alternative method to the trap-door technique for the coronary transfer during the arterial switch operation.

Single-Stage Fontan Operation in a Patient With Coronary Sinus Ostium Atresia.

An eight-year-old boy with tricuspid atresia was found to have atretic coronary sinus ostium during cardiac catheterization. Single-stage extracardiac fenestrated Fontan operation was performed with surgical unroofing of the coronary sinus into the left atrium to avoid the risk of cardiac congestion.

Intraoperative Flow Study Predicted the Postoperative Pulmonary Artery Pressure in the Bidirectional Glenn Operation.

BACKGROUND: The aim of this study was to evaluate the predictability of postoperative pulmonary artery pressure (PAP) using intraoperative flow study in patients undergoing bidirectional Glenn operation. METHODS: Patients who underwent Glenn operation under cardiopulmonary bypass (CPB) were included in the study. During the operation, after the completion of additional procedures under CPB, an intraoperative flow study was performed prior to Glenn anastomosis. After the completion of bidirectional Glenn, the patient was separated from the CPB and PAP was measured. The relationship between this pressure and flow study measurement was analyzed. RESULTS: Nine patients who underwent bidirectional Glenn operation with additional procedures under CPB between July 2018 and January 2019 were included in the study. The median PAP was 9 mm Hg (interquartile range [IQR]: 7-10 mm Hg) in the flow study and 10 mm Hg (IQR: 8-11 mm Hg) after CPB, and the median difference between these pressures was 1 mm Hg (IQR: 1-3 mm Hg). There was a strong correlation between these two measurements (r = 0.732; P = .025). CONCLUSION: The results of this study show that PAP after the Glenn procedure can be estimated using an intraoperative flow study. We believe that this method may be useful in intraoperative decision-making for Glenn operation in single ventricular patients who require extensive pulmonary artery (PA) reconstruction due to limited PA development, branch PA stenosis, or nonconfluent PAs. Also, this method can be used as a sort of intraoperative pulmonary resistance reversibility study in patients with high preoperative pulmonary vascular resistance due to surgically correctable pulmonary venous hypertension.

Midline one-stage complete unifocalization early outcomes from a single center.

OBJECTIVE: This study aims to present our experience with single-stage complete unifocalization and intraoperative flow study for the repair of ventricular septal defect, pulmonary atresia, and major aortopulmonary collateral arteries. METHODS: This study was conducted through retrospective chart review of all the patients who underwent complete single-stage midline unifocalization in a single tertiary-care institution. RESULTS: Twenty-two patients underwent midline single-stage unifocalization. The median age was 11 months (IQR: 5-21 months). The number of collateral arteries unifocalized was between one and three (median two). In-hospital mortality was 5%. Follow-up was complete; and the median follow-up regarding survival was 20 months (IQR: 10-28 months). There were three late deaths, and the estimated survival rate was 80% at 10 months and on. Out of 22 patients, ventricular septal defect was closed in the first surgery in three patients (14%) and the second surgery in four patients (19%). Total seven patients underwent surgical total repair (32%). Additionally, one out of four patients whose ventricular septal defects were closed with a fenestrated patch is under follow-up with a small ventricular septal defect, while two are waiting for ventricular septal defect closure. Therefore, total eight patients (36%) have reached total correction. CONCLUSION: Single-stage unifocalization is a feasible treatment option in ventricular septal defect, pulmonary atresia, and major aortopulmonary collateral arteries. This cohort had unfavorable results regarding the rate of complete repair. The pitfalls encountered were related to problems with meticulous surgical technique, complete unifocalization, and correct implementation of the flow study.

Ross operation early and mid-term results in children and young adults.

OBJECTIVE: The Ross procedure has been cited as the procedure of choice for young patients requiring aortic valve replacement. However, potential for reintervention requirement in both left and right ventricular outflow tracts can be a source of concern. The aim of the present study was to describe our experience with this procedure. METHODS: A retrospective chart review of all the patients who underwent the Ross procedure in a single institution was performed. National death registry records were used for late mortality. RESULTS: Eighteen Ross procedures between May 2003 and May 2018 were performed. The median age of the cohort was 15 [interquartile range (IQR): 12-18] years. The pulmonic conduit was a homograft in 11 patients, Labcor in 5 patients, Contegra in 1 patient, and Medtronic Freestyle Valve in 1 patient. There were three early deaths. The median follow-up of 15 hospital survivors was 11 (IQR: 3-14) years. Any late mortality was not observed. In the two surviving patients with infective endocarditis, there was no recurrent infective endocarditis. Freedom from reintervention was 80% at 8 years and onward. Any risk factors associated with reintervention could not be identified. However, freedom from autograft dilatation at 10 years was 45%. CONCLUSION: Autograft failure is a potential problem in the long-term follow-up of Ross patients. Freedom from reintervention was satisfactory, and the type of pulmonic conduit did not affect the mid-term outcomes. In patients with infective endocarditis, the Ross procedure has a low recurrence rate, but it might have an increased risk of mortality.

Aortic translocation procedure: Early outcomes from a single center.

BACKGROUND/AIM: The data on the Nikaidoh procedure is limited in the literature. This study presents our experiential findings with aortic translocation (Nikaidoh) procedure and its modifications. METHODS: We retrospectively reviewed the data of all the patients who underwent aortic translocation in our institution. RESULTS: Between September 2014 and November 2018, eight patients underwent aortic translocation surgery. The median age was 3.2 years (14 months and 9 years). The diagnosis was transposition of the great arteries (TGA), ventricular septal defect (VSD), and left ventricular outflow tract obstruction (LVOTO) in five patients (63%); double-outlet right ventricle (DORV), VSD, and LVOTO in two patients (25%) and DORV and remote VSD in one patient (12%). Additionally, two patients had tricuspid straddling. In terms of surgical procedures, six patients underwent standard Nikaidoh procedures, while one patient underwent double root translocation and one patient underwent a half-turned truncal switch operation. Hospital mortality was recorded for one patient (12.5%). Median intensive care and hospital stay duration was 3 days (interquartile range [IQR]: 2-5 days) and 11 days (IQR: 8-17 days). Median follow-up duration was 10 months (IQR: 10-24 months). One of the patients, who underwent standard Nikaidoh procedure, died in the late follow-up. CONCLUSIONS: Aortic translocation procedure is an option for TGA, VSD, and LVOTO patients with a distal VSD or tricuspid straddling and DORV patients with a small VSD. Avoiding the use of a conduit through modifications, such as double root translocation and half-turned truncal switch operation, may decrease the risk of long-term recurrent interventions.

Giant mycotic pseudoaneurysm of the aorta protruding over the sternal notch in a child.

Aortic mycotic pseudoaneurysms are rare pathologies in children, which are mostly caused by an infection or trauma. Surgical and perioperative antibiotic therapies are mandatory in the treatment. Surgical timing and operational strategy are also critical factors. Herein, we report the successful repair of a giant mycotic pseudoaneurysm of the ascending aorta following a previous cardiac surgery in a 7-year old girl.

Results for surgical closure of isolated ventricular septal defects in patients under one year of age.

BACKGROUND: This study evaluated the outcomes of patients undergoing surgical repair of isolated ventricular septal defect (VSD) in the first year of life with particular attention to age and severity of pulmonary hypertension (PH). METHODS: Between July 1, 2002 and May 31, 2012, 282 patients aged less than one year underwent isolated VSD closure at a median age of five months (range, 21 days to 1 year) and a median weight of 5.3 kg (range, 2.9 to 12.5 kg). Patients were divided into three groups according to the age at surgery (0-3, 3-6, and 6-12 months), and groups were compared in regard to severity of PH associated with morbidity and mortality. RESULTS: Four (1.4%) early and four (1.4%) late deaths occurred postoperatively. All mortalities were patients with severe PH, aged between 3 and 12 months. Although hemodynamic studies revealed a higher incidence of persistent postoperative PH in patients above three months of age, there was no statistically significant difference in morbidity associated with prolonged mechanical ventilation, and long intensive care unit and hospital stays between age-related groups. CONCLUSION: In this study, the incidence of mortality was higher in patients over three months of age undergoing repair of isolated VSD; the data suggest that the mortality may be decreased in patients with severe PH who were operated on earlier in life. We conclude that in infants with severe PH, early surgical repair (less than three months) of isolated VSDs is strongly advised to achieve more favorable results.