Bicuspid aortic valve caused subaortic stenosis with bulging of valve calcification through subaortic area in a young patient.

Bicuspid aortic valve is the most common congenital cause for the development of aortic valve calcification and stenosis. Calcification cause valvular stenosis or valvular insufficiency due to coaptation failure. We report a unique case of calcification of bicuspid valve was extending to left ventricular outflow tract and attached to interventricular septum which caused subvalvular stenosis.

A retrospective analysis of dexmedetomidine and morphine in the fast-track and ultra-fast-track extubation protocol after congenital cardiac surgery.

BACKGROUND AND AIM OF THE STUDY: After congenital cardiac surgery, the duration of mechanical ventilation (MV) is related to the clinical status, type of operation, and the sedative-analgesic agents used postoperatively. This study aims to examine the effects of dexmedetomidine and morphine on the fast-track extubation (FTE) and ultra-fast-track extubation (UFTE) protocol after congenital cardiac surgery. METHODS: A total of 251 pediatric patients were divided into two groups: 118 patients in the morphine group (Group M) and 133 patients in the dexmedetomidine group (Group D). We retrospectively reviewed medical data including hemodynamic parameters, duration of MV and cardiovascular intensive care unit (CICU), additional sedative/analgesic requirement, adverse events, the need for reintubation, and noninvasive MV, sedation, and pain scores. RESULTS: The mean mechanical ventilation duration of Group D was significantly shorter than Group M (3.74 ± 0.83 h in Group D, 5.72 ± 1.54 h in Group M, respectively) (p = .001; p < .05). In Group D, the success rate of FTE was 92.5% (n = 123) and UFTE was 7.5% (n = 10) (p = .001). In Group M, the success rate of FTE was 72.9% (n = 86) and UFTE was 0% (n = 0) (p > .05). CONCLUSIONS: Dexmedetomidine and morphine have clinical benefits which encourage their use for FTE protocol. Dexmedetomidine has more benefits compared to morphine. It can be used in UFTE protocol, besides its use in FTE protocol with fewer side effects.

Measuring potassium level in packed red blood cells before using: Word of caution for congenital cardiac surgery.

BACKGROUND AND AIM OF THE STUDY: Transfusion-associated hyperpotassemia is a serious complication of packed red blood cell (PRBC) transfusion after congenital cardiac surgery. Our study aimed to identify risk factors and potential preventive measures of transfusion-associated hyperpotassemia in neonates and infants after congenital cardiac surgery. METHODS: Pediatric patients who underwent congenital cardiac surgery and need transfusion were enrolled in this prospective study. The potassium concentration of PRBC was checked from the sample taken from the segment. The volume of transfusion, age of PRBC, potassium concentration of unit were recorded. The estimated increment of potassium level in patients after PRBC transfusion was calculated. RESULTS: Seventy-four individual patients, 95 distinct transfusions, 112 blood products were evaluated. The mean age of the blood unit was 3.8 ± 1.4 days. The mean potassium concentration in the PRBCs was 9.9 ± 2.4 mmol/L. A weak correlation was observed between the potassium value of the PRBC and the age of PRBC (p = 0.049, r = 0.2, y = 0.24 × x + -0.68). There was a weak correlation between the potassium value of PRBCs and the age of the unit (p < 0.001, r = 0.37, y = 2.8 × x + -3.6). CONCLUSIONS: Before transfusion, even PRBC is fresh, measuring the potassium level of PRBC and the potassium that will be given to the pediatric patient with transfusion can prevent transfusion-related hyperpotassemia and related complications. Otherwise, high potassium levels, which may be overlooked despite being fresh, may cause serious complications, even cardiac arrest, especially in neonates and infants.

A prenatal diagnosis of unusual aortopulmonary communication: tubular aortopulmonary window.

We described a very rare case of aorto-pulmonary communication with right aortic arch and crossed pulmonary artery that cannot be placed in the typical anatomic classification of aortopulmonary window. At 23 weeks gestation, fetal echocardiography revealed a large tunnel-like communication connecting the great vessels proximal to the main pulmonary artery bifurcation, rather than a classic aortopulmonary window between the ascending aorta and the main pulmonary artery.

First Harvest of Pediatric and Congenital Heart Surgery Multicenter Database in Turkey: Novel Application of Real-Time Online Reporting.

OBJECTIVES: A new congenital heart surgery database (CKCV) with real-time online reporting function was recently developed in Turkey. All standard international parameters were used, but Aristotle Comprehensive Complexity score was modified. In this study, the first analysis of the CKCV Database is reported. METHODS: The CKCV Database included 2307 procedures from 12 centers between January 2018 and March 2020. All parameters, including 10 real-time online reports, which represent the number of centers, number and mortality rates of all procedures, number of extracorporeal membrane oxygenation (ECMO) and results, details of postoperative complications, age-group statistics, analysis for priority status, mean intensive care and hospital stay durations of the procedures, results of Aristotle Basic, Modified Aristotle Comprehensive (MACC) and Society of Thoracic Surgeons-European Association (STAT) Score Categories, comparison of centers were analyzed. RESULTS: Most common 10 procedures were ventricular septal defect (VSD) repair (n = 273), tetralogy of Fallot (TOF) repair (n = 243), atrial septal defect (ASD) repair (n = 181), complete AVSD repair (n = 95), cavopulmonary anastomosis (n = 81), systemic to pulmonary shunt (n = 79), modified Fontan (n = 71), subaortic resection, (n = 66) PA banding (n = 66), and arterial switch operation (n = 66). Cardiopulmonary bypass was used in 84.6% of the procedures. Overall mortality rate was 6.0%. A total of 618 major and 570 minor complications were observed in 333 and 412 patients, respectively. According to six MACC categories, number of the patients and mortality rates were I (293; 0.3%); II (713; 1.4%); III (601; 3.3%); IV (607; 12%); V (84; 35.7%); and VI (9; 55.6%), respectively. Analysis of five STAT Categories showed 0.7, 3.8, 5.4, 14.9, and 54.7% mortality rates. CONCLUSIONS: CKCV Database has a great potential for nationwide quality improvement studies. Users could instantly analyze and compare their results to national and international aggregate data using a real-time online reporting function. This is the first multicenter congenital database study in Turkey.

Surgical procedures for coronary arteries in pediatric cardiac surgery: Risk factors and outcomes.

BACKGROUND: Limited data exist regarding the coronary revascularization procedures needed during the repair of several congenital and pediatric cardiac malformations. We aimed to determine risk factors for in-hospital mortality and long-term outcomes of various pediatric coronary revascularization procedures. METHODS: We retrospectively reviewed the records of 32 consecutive pediatric patients who underwent coronary revascularization procedures at our institution between May 1995 and June 2020. In-hospital mortality, risk factors, surgical indications, revascularization patency, and mid- and long-term follow-up data were investigated. Patients were categorized into the coronary artery bypass grafting (n = 11) and other coronary artery procedure (n = 21) groups. RESULTS: The median age and weight of patients at the time of surgery were 9 months and 4.8 kg, respectively. There were five in-hospital deaths (5/32, 15.6%). The mortality rates were 27.2% (3/11) in the coronary artery bypass grafting group and 9.5% (2/21) in the other coronary artery procedure group (p = .206; 95% confidence interval: 0.496-25.563). The mortality rates for planned and rescue procedures were 8.3% (2/24) and 37.5% (3/8) (p = .06), respectively. The median follow-up time was 12.5 years. Control imaging studies for coronary patency were performed in 70.3% (19/27) of surviving patients. The overall coronary patency rate was 94.7% (18/19). CONCLUSIONS: Pediatric coronary revascularization procedures with elective-planned indications can be performed with good outcomes. Young age and rescue and emergency procedures may carry an increased risk of in-hospital mortality, although not found to be statistically significant. Surviving patients require lifelong follow-up regarding the patency of reperfused coronary arteries.

Using eye speculum as a sternal retractor for delayed sternal closure after pediatric heart surgery.

Sternal closure can lead to hemodynamic and respiratory instability after some complex pediatric cardiac surgeries. In such cases, delayed sternal closure is applied to facilitate postoperative recovery. During open sternum several instruments have been defined to prevent the sternum from compressing the heart; however, most of them have limitations. We, therefore, have attempted to use a new instrument to keep the sternum open in neonates and infants. This instrument has been used as an adult eye speculum which is made of a flexible metal wire. It is delicate, ready to use, easily placed, and good fitted to the pediatric sternal edges.

Successful treatment of thoracic aortic graft infection by omental flap following vacuum-assisted closure therapy.

BACKGROUND: Postoperative thoracic aortic graft infection (TAGI) is a serious and potentially fatal complication. The classical approach is to replace the infected graft. However, this approach has a high mortality rate. Alternatively, treatment of TAGI without graft replacement can be performed METHOD: Herein, we present a 72-year-old case with mediastinitis and graft infection after type A aortic dissection operation and successful treatment using omental flap coverage following vacuum-assisted wound closure therapy without graft replacement. CONCLUSION: The patient had an uneventful postoperative course and remains infection-free to date.

Noninvasive monitoring of central venous oxygen saturation by jugular transcutaneous near-infrared spectroscopy in pediatric patients undergoing congenital cardiac surgery

Background and aim: In patients undergoing congenital cardiac surgery, it is crucial to maintain oxygen demand-consumption balance. Central venous oxygen saturation (ScvO2) is a useful indicator of oxygen demand and consumption balance which is an invasive method. Near-infrared spectroscopy (NIRS) is a noninvasive, continuous monitoring technique that measures regional tissue oxygenation. NIRS that is placed over the internal jugular vein cutaneous area (NIRSijv) has the potential to show ScvO2 indirectly. In this study, we aimed to determine the correlation between ScvO2 with NIRSijv in pediatric patients undergoing congenital cardiac surgery. Materials and methods: Fifty children participated in the study. Four patients were excluded for the inability of internal jugular vein (IJV) catheterization due to technical difficulties. After anesthesia induction, NIRS probes were placed on the IJV site with ultrasound guidance for the measurement of continuous transcutaneous oxygen saturation. The catheter insertion was also done through the IJV from the other side using ultrasound guidance. Cerebral oxygenation monitoring was done using NIRS with a single pediatric probe placed on the right forehead. Values of NIRSijv, cerebral NIRS (NIRSc) and ScvO2, were recorded at certain times until postoperative 24th hour. Results: Data were collected at 8 different time points. There was a significant correlation between ScvO2 and NIRSijv in all measurement time points (r = 0.91), (P = 0.001). The mean bias between ScvO2 and NIRSijv was 2.92% and the limits of agreement were from 11% to ­5.2%. There was a moderate correlation between ScvO2 and NIRSc (r = 0.45), (P = 0.001). The mean bias between ScvO2 and NIRSc was 2.7% and the limits of agreement were from +26% to ­20%. Conclusions: In this study, we found a strong correlation between ScvO2 and NIRS measurements taken from the internal jugular vein site. Accordingly, continuous noninvasive monitoring with transcutaneous NIRSijv can be an alternative method as a trend monitor for the central venous oxygen saturation in pediatric cardiac patients undergoing congenital cardiac surgery.

Valve-sparing repair with skeletonization of the pulmonary annulus for tetralogy of Fallot.

Pulmonary valve preservation in tetralogy of Fallot (TOF) repair is one of the most challenging issues. Herein, we describe a novel valve-sparing technique for TOF repair that primarily consists of skeletonization of the anterior part of the pulmonary annulus and gentle dilatation by preserving the pulmonary valve and annulus integrity. With encouraging early results, this technique is suggested to prevent severe pulmonary regurgitation and provide acceptable relief of pulmonary stenosis in patients with TOF.

Atrial septal defect closure via mini-thoracotomy in pediatric patients: Postoperative analgesic effect of intercostal nerve block.

BACKGROUND: In this study, we evaluated the efficacy of intercostal nerve block for postoperative pain management in pediatric patients undergoing atrial septal defect closure through a right lateral mini-thoracotomy. METHODS: Between January 2016 and January 2019, a total of 63 pediatric patients (37 males, 26 females; mean age 34.8±26.8 months; range, 2 to 96 months) who underwent corrective congenital heart surgery for atrial septal defect closure through a right lateral mini-thoracotomy were retrospectively reviewed. The patients were divided into two groups as those (Group 1, n=33) receiving intercostal nerve block and general anesthesia and those (Group 2, n=30) receiving general anesthesia alone. Intravenous morphine at a dose of 0.03 mg/kg was applied as rescue analgesia to the patients with a Ramsay Sedation Scale score of >4 and Children"s Hospital of Eastern Ontario Pain Scale score of >7. The total analgesic requirement, adverse effects, duration of mechanical ventilation and length of stay in the intensive care unit were recorded. RESULTS: The mean duration of mechanical ventilation and intensive care unit stay was shorter in Group 1 compared to Group 2 (3.6±1.3 vs. 9.4±2.1 h; 23±2.6 vs. 30±7.2 h, respectively) (p<0.0001). The need for postoperative rescue analgesia was statistically significantly lower in Group 1 compared to Group 2 (0.3±0.5 mg vs. 1.1±0.9 mg, respectively) (p=0.003). The mean total morphine consumption was also lower in Group 1 compared to Group 2 (4.0±2.2 mg vs. 9.0±3.4 mg, respectively) (p<0.0001). CONCLUSION: Intercostal nerve block before thoracotomy closure in pediatric patients undergoing atrial septal defect repair under mini-thoracotomy provides early extubation, shorter mechanical ventilation duration and intensive care unit stay, and reduced analgesic requirements.

Long-term outcomes in patients who underwent surgical correction for atrioventricular septal defect.

OBJECTIVE: The follow-up results of patients operated for atrioventricular septal defect (AVSD) during 1996-2016 at Baskent University are presented. METHODS: Data obtained from hospital records consists of preoperative echocardiographic and angiographic details, age and weight at surgery, operative details, Down syndrome presence, postoperative care details, early postoperative and latest echocardiographic findings and hospitalization for reintervention. RESULTS: A total of 496 patient-files were reviewed including 314 patients (63.4%) with complete and 181 (36.6%) with partial AVSD (48.4% of all patients had Down syndrome). Atrioventricular (AV) valve morphology was Rastelli type A in 92.2%, B in 6.5%, and C in 1.3% of patients. The operative technique used was single-patch in 21.6% (108), double-patch in 25.8% (128), and modified single-patch (Wilcox) in 52.5% (260) of patients. The follow-up time was 37.79±46.70 (range, 0-198) months. A total of 64 patients (12.9%) had arrhythmias while in the intensive care unit; pacemaker was implanted in 12 patients. A total of 78 patients (15.7%) were treated for pulmonary hypertensive crisis. The early morbidity and mortality in the postoperative first month were calculated as 38% and 10%, and the late morbidity and mortality (>1 month) were calculated as 13.1% and 1.9%, respectively. The rate of reoperation in our cohort was 8.9%. CONCLUSION: Although the early morbidity and mortality are low in AVSD operations, the rate of reoperations for left AV valve insufficiency are still high. Although Down syndrome is not a risk factor for early mortality, the co-morbid factors, such as longer postoperative mechanical ventilator or inotropic support, lead to higher risk for morbidity. The frequency of pulmonary hypertension and consequent complications are also high.

In vitro validation of a self-driving aortic-turbine venous-assist device for Fontan patients.

BACKGROUND: Palliative repair of single ventricle defects involve a series of open-heart surgeries where a single-ventricle (Fontan) circulation is established. As the patient ages, this paradoxical circulation gradually fails, because of its high venous pressure levels. Reversal of the Fontan paradox requires an extra subpulmonic energy that can be provided through mechanical assist devices. The objective of this study was to evaluate the hemodynamic performance of a totally implantable integrated aortic-turbine venous-assist (iATVA) system, which does not need an external drive power and maintains low venous pressure chronically, for the Fontan circulation. METHODS: Blade designs of the co-rotating turbine and pump impellers were developed and 3 prototypes were manufactured. After verifying the single-ventricle physiology at a pulsatile in vitro circuit, the hemodynamic performance of the iATVA system was measured for pediatric and adult physiology, varying the aortic steal percentage and circuit configurations. The iATVA system was also tested at clinical off-design scenarios. RESULTS: The prototype iATVA devices operate at approximately 800 revolutions per minute and extract up to 10% systemic blood from the aorta to use this hydrodynamic energy to drive a blood turbine, which in turn drives a mixed-flow venous pump passively. By transferring part of the available energy from the single-ventricle outlet to the venous side, the iATVA system is able to generate up to approximately 5 mm Hg venous recovery while supplying the entire caval flow. CONCLUSIONS: Our experiments show that a totally implantable iATVA system is feasible, which will eliminate the need for external power for Fontan mechanical venous assist and combat gradual postoperative venous remodeling and Fontan failure.

Coronary artery bypass grafting in children.

BACKGROUND: We present our clinical experience with coronary artery bypass grafting (CABG) in children. METHODS: Ten children who underwent CABG between July 1995 and August 2017 were retrospectively analyzed. Data including congenital cardiac malformations, previous surgical procedures, age and sex, type of coronary complications, ischemic events preceding surgery, and ventricular function before and after CABG were recorded. RESULTS: The study population consisted of five males and five females with a median age of 2.5 years (range, 88 days to 15 years). Eight internal mammary arteries (IMAs) and two saphenous veins were used for grafting. Indications for bypass grafting were coronary artery (CA) complications related to the post-arterial switch operation in six, CA complications during the Ross procedure in two, and an iatrogenic CA injury during complete repair of tetralogy of Fallot with abnormal CA, crossing the right ventricular outflow tract in two patients. Six of the grafts were performed as rescue procedures. Three patients died during hospitalization. The mean follow-up time was 6.8 years (range, 3 months to 18 years). Anastomoses were evaluated by coronary angiography in four patients, and were all patent. Echocardiography revealed normal myocardial function in all patients. CONCLUSION: Our study suggests that the IMA should be the graft of choice in children due to its growth potential and long-term patency.

Huge Left Atrial Pseudoaneurysm in a 5-Month-Old Baby Presented with Supraventricular Tachycardia.

UNLABELLED: Cardiac pseudoaneurysm is an extremely rare condition in children. It can be congenital or acquired and has been primarily described in the left side of the heart. The congenital form of pseudoaneurysm is thought to be related to muscular dysplasia of the muscular layer of the heart. The acquired form is related to myocardial infarction, infective endocarditis or chest trauma. In this report we described a 5-month-old baby with left atrial pseudoaneurysm who was referred to our hospital due to tachycardia and hemodynamic instability. After diagnosis of left atrial pseudoaneurysm, aneurysmectomy was performed and the left atrial wall was successfully repaired by surgery three days after admission. In conclusion, due to life threatening complications such as dissection, arrhythmias, compression of aneurysm and tromboembolic complications, pseudoaneurysm should be treated by surgery. KEY WORDS: Chest trauma; Left atrial pseudoaneurysm; Supraventricular arrhythmias.

Easy Fenestration Technique for Extracardiac Fontan Operation.

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

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

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

The effect of modified ultrafiltration duration on pulmonary functions and hemodynamics in newborns and infants following arterial switch operation*.

OBJECTIVES: Modified ultrafiltration is used to ameliorate the deleterious effects of cardiopulmonary bypass in pediatric cardiac surgery patients. The ideal duration of modified ultrafiltration has not been established yet. We investigated the effects of extended duration of modified ultrafiltration on pulmonary functions and hemodynamics in the early postoperative period in newborns and infants who had transposition of great arteries operations. DESIGN: Single-center prospective randomized study. SETTING: Pediatric cardiac surgery operating room and ICU. PATIENTS: Sixty newborns and infants who had been scheduled to undergo transposition of great arteries operation. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Modified ultrafiltration was applied to all patients following the termination of cardiopulmonary bypass (for 10, 15, and 20 min in groups 1, 2, and 3, respectively). Pulmonary compliance, gas exchange capacity, hemodynamic measurements, inotropic support, blood loss, transfusion requirements, hematocrit level, and duration of ventilatory support were measured after intubation, at termination of cardiopulmonary bypass, at the end of modified ultrafiltration, and in the 1st, 6th, 12th, and 24th hours after admission to ICU. The amount of fluid removed by modified ultrafiltration in groups 2 and 3 was larger than that of group 1 (p < 0.01). Systolic blood pressure was significantly increased at the end of modified ultrafiltration in group 3 compared to groups 1 and 2 (p < 0.05). Hematocrit levels were significantly increased at the end of modified ultrafiltration in groups 2 and 3 compared to group 1 (p < 0.01). Therefore, RBCs were transfused less after modified ultrafiltration in groups 2 and 3 compared to group 1 (p < 0.05). Static and dynamic compliance, oxygen index, and ventilation index had improved similarly in all three groups at the end of modified ultrafiltration (p > 0.05) CONCLUSIONS:: Modified ultrafiltration acutely improved pulmonary compliance and gas exchange in all groups. Increased hematocrit and blood pressure levels were also observed in the longer modified ultrafiltration group. However, extended duration of modified ultrafiltration did not have a significant impact on duration of intubation or the stay in ICU.