Factors Affecting Mortality and Morbidity in Total Correction Surgery Applied to Patients with Tetralogy of Fallot: 26 years of our experience

doi:10.21203/rs.3.rs-1966529/v1

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Factors Affecting Mortality and Morbidity in Total Correction Surgery Applied to Patients with Tetralogy of Fallot: 26 years of our experience

https://doi.org/10.21203/rs.3.rs-1966529/v1

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Background: Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart defect, includes a broad spectrum of RVOTO and multiple variants with surgical interventions and postoperative management tailored to each patient. In our study, we compared the morbidity and mortality rates of our two different surgical approaches according to the surgical criteria that have changed in the last 26 years.

Material and Method:Total correction surgery was performed on 967 patients with tetralogy of Fallot by the same surgical team in our clinic. The patients were divided into 2 groups, 340 in Group 1 and 627 in Group 2, according to preoperative criteria. Demographic data of the patients, preoperative evaluation methods, additional pathologies of the patients in the preoperative period, palliative operations performed before total correction and early postoperative complications were evaluated.

Results: A significant difference was observed in the mortality and morbidity outcomes of the 2 groups compared. Since the number of patients < 1 year was higher in Group 2 compared to Group 1, less palliative surgery was performed before total correction. Our patients in Group 2 had a lower incidence of postoperative complications, low cardiac output, and arrhythmia.

Conclusion: Correction of the pathology at an early age in symptomatic patients with a diagnosis of Tetralogy of Fallot positively affects the prognosis of the patient. In addition, early diagnosis and treatment in all patients with or without symptoms have a significant impact on the prognosis of complications that may occur in the postoperative period. We believe that one of the biggest factors in surgical success is an experienced surgical team that has worked together for many years.

Total correction

palliative operation

arrhythmia

right ventricle dysfunction

Congenital heart diseases (CHD) are classified as acyanotic or cyanotic and account for approximately 1% of live births [1]. Tetralogy of Fallot, one of the cyanotic heart diseases, occurs in an estimated 1 in 3500 or 0.23 to 0.63 per 1000 births [2]. With these findings, it was observed that TOF accounts for approximately 7–10% of all congenital heart defects and is the most common pathology among cyanotic congenital heart diseases [3,4].

The first person to describe TOF was Niel Stenson in 1671 [5]. Niel Stenson described TOF but did not mention the signs of the disease [2,5]. Along with the developments, Etienne Fallot published the first full explanation and findings of TOF in 1888 [6]. Maude Abbott, who used the term TOF for this pathology known since 1671, introduced this term to the world literature in 1924 [2,5].

TOF consists of a tetrad or can be called a disease group consisting of 4 defects. Ventricular septal defect (VSD), pulmonary stenosis (PS), overriding aorta and right ventricular hypertrophy (RVH) are the pathologies that make up this tetrad. This quadruple pathology observed causes anterior and leftward displacement of the infundibular septum. In addition to the RVOTO degree, there is a wide range of anatomical variability within the 4 pathologies called tetralogy [7].

Ductal dependence, severity of obstruction in the subpulmonary outflow tract, frequency of hypercyanotic seizures and degree of cyanosis are the criteria that determine when the surgical operation will be performed [3]. Newborns with TOF (> 75%) can wait 3 to 6 months for elective surgical repair unless they have urgent surgical criteria. Newborns with low birth weight, requiring PDA-related PGE1 infusion, or with frequent hypercyanotic seizures require palliation or complete surgical repair without waiting 3 to 6 months. For those who have palliation in the neonatal period, a full repair can be done between 4 and 6 months. Newborns with TOF who have severe airway problems because of absent pulmonary valve require surgical intervention in the neonatal period [8]. Deciding on the timing of surgical intervention depends on the current practice and expertise of each surgical center. Based on their experience, some centers have observed low mortality associated with first palliation, and as a result, they prefer repair following first palliation rather than early primary repair [9].

In our study, we aimed to present our experiences with 967 patients who underwent total correction due to Tetralogy of Fallot between 1995–2021.

Patients who were operated on in our clinic with the diagnosis of Tetralogy of Fallot (TOF) between 1995 and 2021 were retrospectively scanned. Our patients were evaluated by the same surgical team and experienced pediatric cardiology council, and it was decided to perform total correction surgery. A total of 967 patients who underwent complete correction surgery during this period were divided into 2 groups as those operated between 1995–2003 (Group 1) and those operated between 2004–2021 (Group 2). There were 340 patients in Group 1 and 627 patients in Group 2.

Preoperative selection criteria were different in Group 1 and Group 2. For Group 1 patients, being older than 6 months in the preoperative period and having a McGoon index greater than 1.8 were considered sufficient to decide on total correction surgery. Echocardiographic diagnosis was considered sufficient for surgery unless there was an obvious necessity in the patients. Catheter angiography was performed in necessary patients. Pulmonary artery structure and coronary anatomy were evaluated preoperatively in patients with MAPCA, and coil embolization was applied to the collaterals if necessary.

In Group 2 patients, McGoon index above 1 was considered sufficient for surgery without age restriction. Most of the patients in this group underwent catheter-angiography to better evaluate the pulmonary artery anatomy and the presence of MAPCAs, CT angiography to evaluate pulmonary arterial structure. MAPCAs were closed with coil embolization before surgery.

Demographic data of the patients in Group 1 and Group 2, preoperative pulmonary artery structures, additional cardiac pathologies, palliative operations before total correction surgery, surgical methods applied to the pulmonary artery system, causes of complications and mortality in the early postoperative period were analyzed and their results were compared.

The independent sample t-test was used to calculate whether there was a significant difference between the means of the 2 groups. We obtained this statistic using the Independent Samples T test in SPSS 2020. Those with p > 0.005 were considered significant.

Patients who were operated on in our clinic with the diagnosis of Tetralogy of Fallot (TOF) between 1995 and 2021 were retrospectively scanned. A total of 967 patients who underwent complete correction surgery during this period were divided into 2 groups as those operated between 1995–2003 (Group 1) and those operated between 2004–2021 (Group 2). There were 340 patients in Group 1 and 627 patients in Group 2. Demographic data of these groups are shown in Table 1.

Table 1

Information of patients in the perioperative period
	Group 1 (n = 340)	Group (n = 627)
Age (year)	2.5	1.8
Infant (< 1 year)	46	251
Female	155	295
Male	185	332
Hemiplegia	2	1
Cerebral Palsy	3	3

The age range of Group 1 patients ranged from 6 months to 31 years, with an average of 2.5 years. 46 of these patients (15%) were infants (under 1 year old). Of these patients, 161 were male and 146 were female. 5 patients in this group had neurological problems before the operation. Hemiplegia was present in 2 patients and cerebral palsy in 3 patients.

The age range of Group 2 patients was between 15 days and 43 years, with a mean age of 1.8 years. 251 of these patients (40%) were infants (under 1 year old). Of these patients, 332 were male and 295 were female. 4 patients in this group had neurological problems before the operation. 3 patients had cerebral palsy and 1 patient had hemiparesis.

Preoperative selection criteria were different in Group 1 and Group 2.

For group 1 patients, being older than 6 months in the preoperative period and having a McGoon index greater than 1.8 were considered sufficient to decide on total correction surgery. Echocardiographic diagnosis was considered sufficient for surgery unless there was an obvious necessity in the patients. In group 2 patients, McGoon index above 1 was considered sufficient for surgery, without age restriction. Most of the patients in this group underwent angiography in order to better evaluate the pulmonary artery anatomy and the presence of MAPCAs and to close these MAPCAs with a coil before surgery, if necessary.

Preoperative evaluations of Group 1 and Group 2 patients are shown in Table 2.

Table 2

Preoperative evaluation methods of patients
	Group 1 (n = 340)	Group 2 (n = 627)
ECO	225	318
Anjio	115	247
MAPCA Coil	9	27
ECO + BT	-	62

While 225 (66%) of Group 1 patients were operated on with echocardiographic examination alone, only 115 (34%) patients underwent preoperative angiography. Coil embolization was applied to the MAPCA of 8 (2.4%) patients. While 318 (51%) of Group 2 patients were operated with only echocardiographic examination, preoperative angiography was performed in 247 (39%) patients. ECO + CT was applied to 62 patients (10%), and MAPCA embolization with coils was applied to 27 (4.3%) patients. The rate of presence of MAPCA was significantly higher in Group 2 than in Group 1. There was no difference between the groups in terms of other concomitant pathologies.

Pulmonary artery anomaly was detected in 40 (11.79%) patients in Group 1 and in 68 (10.8%) patients in Group 2 in the imaging studies of the patients. The pulmonary artery anomalies detected in the groups are shown in Table 3. In Group 1, stenosis close to pulmonary atresia was found in 21 patients, significant bifurcation stenosis in 14 patients, absence of left pulmonary artery in 3 patients, and absence of right pulmonary artery in 2 patients. In Group-2, stenosis close to pulmonary atresia was found in 37 patients, significant bifurcation stenosis in 26 patients, absence of left pulmonary artery in 4 patients, and absence of right pulmonary artery in 1 patient.

Table 3

Pulmonary artery anomalies in patients
	Group 1 (n = 40)	Group 2 (n = 68)
Absence of Left Pulmonary Artery	3	4
Absence of Right Pulmonary Artery	2	1
Severe Bifurcation Stenosis	14	26
Stenosis Close to Pulmonary Atresia	21	37

The pathologies accompanying our 967 patients with tetralogy of Fallot are shown in Table 4. Broad ASD in 71 patients, MAPCA in 48 patients, coronary anomaly in 37 patients, tricuspid anomaly in 12 patients, complete AVCD in 8 patients, dextrocardial situs inversus in 4 patients, permanent AV block in 4 patients, mitral regurgitation in 3 patients, subaortic stenosis in 2 patients, aortic stenosis in 2 patients detected.

Table 4

Additional pathologies of the patients in the preoperative period (n=967)
Broad ASD	71
Coronary Anomaly	37
CAVCD	8
Mitral Failure	3
Subaortic Stenosis	2
Dextrocardi - Situs Inversus	4
Aortic Coarctation	3
Double Aortic Arch	2
Permanent A-V Block	4
MAPCA	48
Tricuspid Valve Pathology	12

Palliative surgeries performed in our patients before total correction are shown in Table-5. Palliative surgery was performed before complete correction in 74 patients (21.7%) in Group 1. BT shunt was performed in 61 patients, RVOT reconstruction was performed in 10 patients, and central shunt was performed in 3 patients. Palliative surgery was performed on 48 patients (7.6%) in Group 2. BT shunt was performed in 32 patients, central shunt was performed in 12 patients, and RVOT reconstruction was performed in 4 patients.

Table 5. Previous operations of patients (n=967)

	Group 1 (n = 340)	Group 2 (n = 627)
Reoperation	74	48
BT Shunt	61	32
Central Shunt	3	12
ROVT Reconstruction	10	4

Table 6 shows the methods applied for the pulmonary arterial system in total correction surgery. Transanular patch was applied to 265 (78%) of 340 patients in Group 1, transatrial repair was applied to 43 (12.6%) patients, infundibular patch was applied to 21 (6.2%) patients, and RV-PA condiut was applied to 11 (3.2%) patients. Transanular patch was applied to 477 (76.1%) of 627 patients in Group 2, transatrial repair was applied to 81 (12.9%) patients, infundibular patch was applied to 54 (8.6%) patients, and RV-PA condiut was applied to 15 (2.4%) patients. While unifocalization was applied to 2 of 11 patients in Group 1 with MAPCA, and ligation was performed to 9 of them intraoperatively, 21 of 32 patients in Group 2 were angiographically coiled preoperatively. 7 of the other patients in this group underwent unifocalization, and the other 6 underwent intraoperative ligation. Total correction was performed in two patients in each group with Complete Atrioventricular Canal Defect. Coronary anomaly, another pathology frequently associated with this pathology, was observed in 14 patients (4.5%) in Group 1, while it was observed in 13 patients (3.9%) in Group 2. In this respect, there was no statistical difference between the groups. In group 1, limited ventriculotomy was performed in 8 patients with coronary anomaly, conduit replacement in 3, and limited ventriculotomy and conduit replacement in the other 3 patients.

Table 6

Methods applied for the pulmonary arterial system in surgery
	Group 1 (n = 340)	Group 2 (n = 627)
Transanular Patch	265	477
Infundibular Patch	21	54
Transatrial Repair	43	81
RV-PA Conduit	11	15

In Group 2, limited ventriculotomy was performed in 7 patients with coronary anomaly, conduit replacement in 3, and limited ventriculotomy and conduit replacement in the other 3 patients.

Preoperative angiography and coil embolization were performed in 36 of our 48 patients with MAPCA. Intraoperative unifocalization was applied to 6 patients and MAPCA ligation was applied to 6 patients intraoperatively.

Coronary anomaly, another pathology frequently associated with this pathology, was observed in 19 patients (5.5%) in Group 1, while it was observed in 18 patients (2.9%) in Group 2. LAD in 32 of 37 patients with coronary anomalies and RCA in 5 patients narrowed the right ventricular outflow tract. In our 11 patients, resection through the PA and through the atrium was possible with limited ventriculotomy, and total correction was completed. Conduit replacement was performed in 26 patients. Dacron conduit was performed under the coronary artery with limited ventriculotomy in 15 patients, and only contegra conduit was performed in 11 patients.

Appropriate operations were performed for the patients who were found to have additional anomalies during the operation. Tricuspid repair was performed in 8 of 12 patients with tricuspid valve pathology. Glenn procedure was applied to 4 patients with hypoplastic tricuspid valve. Total correction surgery with double patch was performed in 8 patients with complete AV canal defect. 6 of these patients had previously undergone BT shunt surgery. Mitral valve repair (cleft repair and annuloplasty) was successfully performed in 3 patients with mitral regurgitation. Aortic coarctation was repaired with 3 pericardial patches. Two posterior arches with double aortic arches were repaired with divergence. Permanent pacemaker was inserted in 3 patients with permanent AV block. Patients with large ASDs were either closure with a pericardial patch or had their ASD reduced to the patent foramen ovale.

Early complications in the postoperative period in Group 1 and Group 2 are shown in Table-7. When the groups were compared, no significant difference was found between the postoperative complications. The most common complications developing in the early postoperative period are; low cardiac output, nodal tachycardia, transient AV block.

Table 7

Pathologies observed in our patient group in the early postoperative period
	Group 1	Group 2
Low cardiac output	44 (%12.9)	63 (%10)
Nodal tachycardia (JET)	13 (%3.8)	15 (2.4)
Temporary A-V block	22 (%6.5)	57 (%9)
Revision (Bleeding, Tamponad)	6 (%1.7)	7 ( %1.1)
Neurological complication	4 (%1.2)	5 (0.7)
Infection-Sepsis	5 (%1.5)	9 (%1.2)
Reversible diaphragmatic paralysis	4 (%1.2)	3 (%0.4)
Permanent A-V block	2 (%0.6)	1 (%0.2)
Acute renal failure	13 (%3.8)	11 (%1.8)
Sudden cardiac arrest	3 (%0.9)	4 (%0.6)

In Group 1, 2 (4.3%) of 46 patients in the infant group (< 1 year old) died, while 9 (3%) of 294 patients in the > 1 age group died. The causes of death of the patients in Group 1 are shown in Table-8. Low cardiac output due to restrictive RV dysfunction was found in 6 patients, low cardiac output in the left ventricle due to JET in 2 patients, sudden cardiac arrest in 2 patients and death due to neurological complications in 1 patient. 7 (1.1%) of 627 patients in Group 2 died. 4 (1.6%) patients in the infant group (< 1 year old) and 3 (0.8%) patients out of 376 patients in the > 1 age group died. As seen in Table-8, in Group 2, 3 patients died due to low cardiac output due to restrictive RV dysfunction, 2 patients died due to sudden cardiac arrest, 1 patient died due to aspiration pneumonia and 1 patient died due to neurological complications. Of the 340 patients in Group 1, 11 (3.2%) died. In Group 1, 2 (4.3%) of 46 patients in the infant group (< 1 year old) died, while 9 (3%) of 294 patients in the > 1 age group died. The causes of death of the patients in Group 1 are shown in Table-8. Low cardiac output due to restrictive RV dysfunction was found in 6 patients, low cardiac output in the left ventricle due to JET in 2 patients, sudden cardiac arrest in 2 patients and death due to neurological complications in 1 patient.

Table 8. Causes of mortality observed in our patient group

	Group 1	Group 2
Restrictive right ventricle	6	3
JET and associated observed LCO	2	2
Aspiration pneumonia	2	1
Neurological complication	1	1

TOF is the most common cyanotic congenital heart defect. With this knowledge, TOF repair constitutes a significant portion of the CHD population requiring postoperative care. TOF includes a wide variety of RVOTO and anatomical variants. And because of these multiple variants, the process is completed with surgical intervention and post-operative management that is tailored to each patient. Early recognition and treatment of postoperative complications is essential for a better prognosis of our patient population.

Closure of the VSD, resection of the muscle bundles in the RVOT with or without augmentation, strengthening of the pulmonary artery and pulmonary artery branches, and removal of severe occlusion in the pulmonary valve ring, if indicated, are the surgical procedures that should be performed to achieve complete surgical repair of TOF. In addition to complete repair surgery, only VSD closure and minimal muscle resection of the infundibulum are sufficient in infants with well developed pulmonary artery and mild RVOTO. Infants with moderate to severe RVOTO require ventriculotomy and resection of the hypertrophied muscle in addition to closure of the VSD during the course of the surgical procedure. Closure of the VSD is performed via a transatrial approach. This approach allows the length of the surgeon's incision in the right ventricle to be minimal enough to be just the length necessary to relieve RVOTO. The VSD is closed by suturing from the right side of the septum with a patch cut to size.

The surgeon takes special care when suturing to avoid damaging the bundle of His and Purkinje fibers. The annulus of the pulmonary valve is measured during surgery and examined for adequate size. The size of the PV ring is considered insufficient when the z-score is less than 2 standard deviations from normal. If the PV annulus size is insufficient, the ventriculotomy incision is extended to the PA through the PV, and one such procedure is called the transannular patch technique [6]. Transanular patch repair can result in moderate to severe pulmonary failure and volume overload in the right ventricle. Transannular patch repair also increases the long-term risk of RV dysfunction and ventricular arrhythmias. Because of these risks, current surgical techniques focus on maintaining PV competency and tolerating mild RVOTO. If the PV annulus is adequate in size, a transatrial-transpulmonary valve-sparing technique is preferred. This technique spares the PV at the expense of tolerating mild RVOTO. If the PA is hypoplastic, a patch can be used supravalvar and/or subvalvar into the RV if necessary. Current techniques also encourage avoidance of excessive right ventricular muscle resection to avoid associated RV diastolic dysfunction.

Infants with TOF/PA or diffusely small PAs may require an RV-PA conduit. The type of conduit varies depending on surgeon expertise and preference. An RV-PA conduit commits an infant to multiple surgical repairs over a lifetime [10].

After 2005, we changed our surgical criteria for tetralogy of Fallot in our clinic. In our clinic, we preferred the strategy of applying total correction surgery in every case without palliative surgery. If the patient was symptomatic, we operated on each patient regardless of age. We did not use parameters that were previously in our criteria, such as the patient's age or the Mc-Goon index. We made sure that the Mc-Goon index was greater than 1. In the presence of severe hypoplastic annulus and pulmonary artery, we performed central shunt or limited RVOT reconstruction.

The most important reasons affecting morbidity and mortality in the postoperative intensive care unit are related to low cardiac output. In our study, low cardiac output was found in 12.9% and 10% of patients in Group 1 and Group 2, respectively.

After TOF repair, almost 50% of patients experience restrictive RV physiology and RV diastolic dysfunction [8,11]. LCOS is a common postoperative complication seen in approximately 25% of children. In addition, LCOS is a common postoperative complication seen in approximately 25% of children and is often associated with JET.

To prevent the development of low cardiac output and RV diastolic dysfunction, we have some recommendations that you can follow during and after surgery. Cardiopulmonary bypass, ventriculotomy, myocardial edema, and dysfunctional patches on the ventricular septum and RVOT are among the most common causes of RV diastolic dysfunction. Because of this information, our surgical recommendations are not to make the ventriculotomy too long, to avoid excessive infundibular resection, to divide the moderator band if it is short and hypertrophic, and to leave a small shunt at the atrial level.

In the postoperative period, our recommendations are to minimize positive pressure ventilation with early extubation. Because this situation significantly increases the occurrence of early postoperative low cardiac output syndrome (LCOS) due to insufficient RV filling and decreased effective compliance of the LV. The reduction in effective compliance of the LV is due to leftward cambering of the septum. To reduce this risk, we recommend leaving an atrial septal defect (ASD) to allow a small right-to-left shunt to continue at the atrial level. Right-to-left shunting is allowed from the small ASD left open by performing total correction surgery at an early age. Thus, low cardiac output is prevented by increasing left ventricular filling. The residual ASD is left uncorrected to maintain cardiac output even if it lowers the patient's systemic oxygen saturation.

The echo finding consistent with severely restrictive RV physiology is the presence of antegrade flow into the PAs during diastole [12–14]. Due to the inability of the RV to fill during diastole, the RV acts as a passive channel, which can be easily detected by the presence of antegrade flow into the PAs during late diastole at echo.

Hypovolemia is an often overlooked problem. Ideal volume replacement in the patient will prevent low cardiac output. Hypovolemia should not be evaluated by monitoring central venous pressure (CVP) alone. Because CVP may be high without hypovolemia due to an RV compliance problem. These patients should be followed up with ECO.

The presence of MAPCA is a risky problem in the postoperative period. The presence of an overlooked MAPCA in intensive care period causes an increased volume load in the lungs. Preoperative detection and treatment of MAPCA is very important. In our study, MAPCA embolization was applied to 9 and 27 patients in Group 1 and Group 2, respectively, and it played an important role in our morbidity and mortality.

Hemodynamically significant arrhythmias occur in up to 12% of patients, usually within the first 24 hours following TOF repair [14]. The most common hemodynamically significant postoperative arrhythmias are JET, supraventricular tachycardia, and heart block. JET is a common postoperative arrhythmia experienced after TOF repair. After congenital heart surgery, JET is estimated to occur at a rate as high as 15% and more commonly occurs after TOF repair because of the close proximity of the repair to the atrioventricular (AV) node [15–18].

In our study, arrhythmia developed in 10.4% and 11.6% of our Group 1 and Group 2 patients, respectively. Atrial arrhythmias, heart block (HB), right bundle branch block (RBBB), junctional ectopic tachycardia (JET), and ventricular arrhythmias are common arrhythmias in the postoperative period. JET developed in 3.8% of our patients in Group 1 and 2.4% in Group 2. Transient AV block developed in 6.5% of our Group 1 patients and 9% of our Group 2 patients. Permanent AV block developed in 0.6% of our patients in Group 1, and permanent AV block was detected in 0.2% of patients in Group 2. Younger age, lower body weight, longer cardiopulmonary bypass time, longer aortic cross-clamp time, deep hypothermia and cardiac arrest are risk factors for the development of arrhythmia in the postoperative period. Typically, temporary atrial and ventricular pacing wires are placed for the diagnosis and management of postoperative arrhythmias.

Additional risk factors for JET include younger age, longer cardiopulmonary bypass and aortic cross-clamp times, hypomagnesemia, and use of vasoactive agents postoperatively [19,20]. JET occurs when the AV node has increased automaticity and spontaneously depolarizes, resulting in a mismatch between the atria and ventricles. Clinically, it is manifested by junctional ectopic tachycardia, absence of P waves, tachycardia, and a narrow QRS complex on the EKG [19]. JET is associated with increased postoperative morbidity and mortality.

In our study, although the mean age in Group 2 was smaller than in Group 1, no significant difference was found in terms of JET development. The incidence of JET is typically limited to the immediate postoperative period. The medical management of JET is designed to reduce heart rate through thermoregulation and restore synchrony between the atria and ventricles; correction of electrolyte imbalances; reduce catecholamine levels; transient atrial overrate pacing; and administration of pharmacotherapies such as dexmedetomidine, amiodarone, procainamide and propranolol [21,22].

Heart blocks develops as a result of damage to the cardiac conduction system following surgical repair of CHD [23]. Moderate to severe hemodynamic deterioration after surgery, mechanical ventilation longer than 5 days, and the need for cardiac reintervention within the first 14 days after surgery are additional risk factors for HB. HB is a potential but uncommon risk, with occurrence ranging between 1% and 3% [24]. Repairs near or adjacent to the AV node have increased risk for HB. Studies have revealed that a longer surgery time is a risk factor for complete HB. Early postoperative HB can be transient or permanent. If HB persists after 2 weeks of temporary pacing, a permanent pacemaker is recommended. This condition is typically caused by damage to the bundle of His and Purkinje fibers or by trifascicular damage associated with an increased risk of bradycardia and asystole [25].

Our patients were admitted to the intensive care unit (ICU) with medium-dose inotropic support and atrial pacing when necessary. Follow-up with echo is very important in the ICU. Residual defects or stenoses, volume status, ventricular contractility, and valve function should be evaluated, particularly for RV diastolic dysfunction, and fluid replacement performed if necessary. It is important to monitor the heart rate on the patient's monitor. Nodal rhythm is an important problem as it affects cardiac output. A rhythm of 140–150/min should be maintained with atrial pacing. Saturation is important in the post-operative period. It is expected to drop to 85% in the early period due to the PFO being left open. Low saturations should be carefully monitored.

In our study, when the causes of lower morbidity and mortality in Group 2 were evaluated, 5 main conditions were observed. It is listed as: The increase in experience in anesthesia and intensive care unit; the routine use of echocardiography in the operating room and intensive care unit has enabled early recognition and treatment of complications; prevention of arrhythmias such as RV diastolic dysfunction and JET; a good preoperative evaluation and imaging is very important, especially for the presence of MAPCA. MAPCA treatment significantly reduced postoperative problems. And finally, increased use of ECMO in the ICU has reduced mortality. ECMO was applied to 6 of our 7 patients who developed sudden cardiac arrest, and 3 of our patients were treated and discharged.

Nowadays, total correction surgery used in the treatment of TOF pathology is performed with low morbidity and mortality rates. Correction of the pathology at an early age in symptomatic patients positively affects the prognosis of the patient. In addition, early diagnosis and treatment in all patients with or without symptoms it has an important effect on the prognosis of complications that can be seen in the postoperative period. And we believe that one of the biggest factors on surgical success is an experienced surgical team that has worked together for many years.

TOF

Tetralogy of Fallot

MAPCA

Major Aortopulmonary Collateral Arteries

CHD

Congenital Heart Diseases

VSD

Ventricular Septal Defect

ASD

Atrial Septal Defect

PDA

Patent Ductus Arteriosus

PFO

Patent Foramen Ovale

AVCD

Atrioventricular Canal Defect

Right Ventricle

Left Ventricle

RVH

Right Ventricle Hypertrophy

RVOTO

Right Ventricular Outflow Tract Obstruction

Pulmonary Stenosis

Pulmonary artery

JET

Junctional Ectopic Tachycardia

Heart Block

CVP

Central Venous Pressure

ICU

Intensive Care Unit

LAD

Left Anterior Descending Artery

RCA

Right Coronary Artery

Ethical Approval: All methods were performed in accordance with the Declaration of Helsinki. The consent was obtained from study participants and was written, the study was submitted and approved by Istanbul S.B.Ü Kanuni Sultan Süleyman Training and Research Hospital ethics committee.

The reference number of the committee is 202208189

Compenting Interest: This declaration is not applicable.

Funding: This declaration is not applicable.

Availability of data and materials: The datasets used and analyzed during the current study are available from the corresponding author on reasonable request.

Authors and Affiliations:

1)Department of Cardiovascular Surgery, Florence Nightingale Hospital, T.C. Demiroglu Bilim University, Sisli, Istanbul, 34394, Turkey

Murat Ertürk

2)Department of Cardiovascular Surgery, Florence Nightingale Hospital, T.C. Demiroglu Bilim University, Sisli, Istanbul, 34394, Turkey

Ahmet Bülent Polat

3)Student, Florence Nightingale Hospital, T.C. Demiroglu Bilim University, Sisli, Istanbul, 34394, Turkey

Nur Karademir

4)Department of Pediatric Cardiology, Florence Nightingale Hospital, T.C. Demiroglu Bilim University, Sisli, Istanbul, 34394, Turkey

Kazım Öztarhan

Authors' contributions: ABP and ME, performed the surgical operations and edited the method part of the article; KÖ, saw patients in pediatric councils and performed preoperative examinations and postoperative care; NK, edited the statistical information along with the tables of the article; KÖ and NK, searched the literature, systematically reviewed, evaluated quality, sorted and analyzed data; KÖ, prepared a draft; KÖ and NK significantly revised the article. All authors approved the submitted version and have agreed both to be personally accountable for the author’s own contributions and to ensure that questions related to the accuracy or integrity of any part of the work. Even ones in which the author was not personally involved are appropriately investigated, resolved, and the resolution documented in the literature.

Triedman JK, Newburger JW. Trends in congenital heart disease: the next decade. Circulation 2016;133(25):2716–33.
Apitz C, Webb GD, Redington AN. Tetralogy of Fallot. Lancet 2009;374:1462–71.
Steiner MB, Tang X, Gossett JM, et al. Timing of complete repair of non-ductal-dependent tetralogy of Fallot and short-term postoperative outcomes, a multicenter analysis. J Thorac Cardiovasc Surg 2013;16(2):1–7.
Villafane J, Feinstein JA, Jenkins KJ, et al. Hot topics in tetralogy of Fallot. J Am Coll Cardiol 2013;62(23):2155–66.
Sharkey AM, Sharma A. Tetralogy of Fallot: anatomic variants and their impact on surgical management. Semin Cardiothorac Vasc Anesth 2012;16(2):88–96.
Bove EL, Hirsch JC. Tetralogy of Fallot. In: Stark J, deLeval M, Tsang VT, editors. Surgery for congenital heart defects. 3rd edition. West Sussex, United Kingdom: John Wiley and Sons, Ltd; 2006. p. 399–410.
Davis S. Tetralogy of Fallot with and without pulmonary atresia. In: Critical heart disease in infants and children. 2nd edition. Philadelphia: Elsevier Mosby; 2006. p. 755–66.
Karl TF, Stocker C. Tetralogy of Fallot and its variants. Pediatr Crit Care Med 2016; 17(8):S330–6.
Kanter KR, Kogon BE, Kirshbom PM, et al. Symptomatic neonatal tetralogy of Fallot: repair or shunt? Ann Thorac Surg 2010;89:858–63.
Tirilomis T, Friedrich M, Zenker D, et al. Indications for reoperation late after correction of tetralogy of Fallot. Cardiol Young 2010;20(4):396–401.
Cullen S, Shore D, Redington A. Characterization of right ventricular diastolic per- formance after complete repair of tetralogy of Fallot. Restrictive physiology pre- dicts slow postoperative recovery. Circulation 1995;91(6):1782–9.
Peng E, Spooner R, Young D, et al. Acute b-type natriuretic peptide response and early postoperative right ventricular physiology following tetralogy of Fallot’s repair. Interact Cardiovasc Thorac Surg 2012;15(3):335–9.
Redington A. Low cardiac output due to acute right ventricular dysfunction and cardiopulmonary interactions in congenital heart disease (2013 Grover Confer- ence series). Pulm Circ 2014;4(2):191–9.
Sachdev MS, Bhagyavathy A, Varghese R, et al. Right ventricular diastolic function after repair of tetralogy of Fallot. Pediatr Cardiol 2006;27(2):250–5.
Rekawek J, Kansy A, Miszczak-Knecht M, et al. Risk factors for cardiac arrhythmias in children with congenital heart disease after surgical intervention in the early postoperative period. J Thorac Cardiovasc Surg 2007;133(4):900–4.
Kuzevska-Maneva K, Kacarska R, Gurkova B. Arrhythmias and conduction ab- normalities in children after repair of tetralogy of Fallot. Vojnosanit Pregl 2005; 62(2):97–102.
Hazan E, Bical O, Bex JP, et al. Is right bundle branch block avoidable in surgical correction of tetralogy of Fallot? Circulation 1980;62(4):852–4.
Horowitz LN, Simson MB, Spear JF, et al. The mechanism of apparent right bundle branch block after transatrial repair of tetralogy of Fallot. Circulation 1979;59(6):1241–52.
Smith AH. Arrhythmias in cardiac critical care. Pediatr Crit Care Med 2016;17(8 suppl 1):S146–54.
Abdelaziz O, Deraz S. Anticipation and management of junctional ectopic tachycardia in postoperative cardiac surgery: single center experience with high inci- dence. Ann Pediatr Cardiol 2014;7(1):19–24.
El-Shmaa N, El Amrousy D, El Feky W. The efficacy of pre-emptive dexmedetomi- dine versus amiodarone in preventing postoperative junctional ectopic tachy- cardia in pediatric cardiac surgery. Ann Card Anaesth 2016;19(4):614–20.
Entenmann A, Michel M, Herberg U, et al. Management of postoperative junc- tional ectopic tachycardia in pediatric patients: a survey of 30 centers in Ger- many, Austria, and Switzerland. Eur J Pediatr 2017;176(9):1217–26.
Nasser BA, Mesned AR, Mohamad T, et al. Late-presenting complete heart block after pediatric cardiac surgery. J Saudi Heart Assoc 2015;28(1):59–62.
Corcia M, Salgado G, Moreno G, et al. Clinical predictors of permanent injury to the atrioventricular conduction system after congenital heart disease surgery. A new perspective on an old problem. Prog Pediatr Cardiol 2017;46:35–8.
Bonatti V, Agnetti A, Squarcia U. Early and late postoperative complete heart block in pediatric patients submitted to open-heart surgery for congenital heart disease. Pediatr Med Chir 2013;20(3):181–6.

No competing interests reported.

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Factors Affecting Mortality and Morbidity in Total Correction Surgery Applied to Patients with Tetralogy of Fallot: 26 years of our experience

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