From cyanosis to diagnosis: A case report on right atrial fibroma in a pediatric patient.

A 3-year-old female patient presented with symptoms of cyanosis and intermittent eyelid edema, leading to the discovery of a lobulated mass in the right atrium, obstructing the superior vena cava. Despite the inability to entirely remove the mass due to its origins in the right atrium myocardium and its extension towards the sinoatrial node, successful surgical intervention and subsequent histopathological evaluation identified the mass as a fibroma, and postoperative symptoms were significantly alleviated.

Effects of Systolic Dysfunction on Clinical and Diagnostic Parameters in Pediatric Patients with Isolated Left Ventricular Non-compaction.

OBJECTIVE: Left ventricular non-compaction is a rare cardiomyopathy following an early arrest in endomyocardial morphogenesis. This study aimed to present the clinical and electrocardiographic characteristics, diagnostic features, treatment strategies, effects of systolic dysfunction on clinical and diagnostic parameters, and follow-up of pediatric patients diagnosed with left ventricular non-compaction. METHODS: We retrospectively reviewed children with isolated left ventricular non-compaction at Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training and Research Hospital from January 2010 to June 2020. RESULTS: Fifty-five children were diagnosed with left ventricular non-compaction. Thirty-two patients (58.2%) were male, and the median age of presentation was 8.5 years (1 month-17.9 years). The median follow-up of the study was 19 months (1-121 months). Fourteen (25.5%) presented with systolic dysfunction (ejection fraction < 45%), and 2 presented with resuscitated/aborted cardiac arrest. Electrocardiographic abnormalities were present in 78.2%. Fragmented QRS was observed in 6 patients, and QTc duration was 450 milliseconds and above in 17 patients (30.9%). Electrocardiographic abnormalities, low QRS voltage, fragmented QRS, and thrombus were common in patients with ejection fraction < 45% group. Atrial and ventricular arrhythmias (including ventricular fibrillation-VF) were found with similar frequency in both ejection fraction < 45% and ≥45% groups. One patient with a complete atrioventricular block and 1 with long QT syndrome and severe bradycardia underwent permanent pacemaker implantation. Five (9.1%) patients died. CONCLUSIONS: Left ventricular non-compaction has heterogeneous clinical findings in childhood. It is essential to follow-up with the patients closely for the development of ventricular dysfunction or arrhythmias due to the progressive course of the disease. Further studies are needed since life-threatening ventricular arrhythmias can be seen, even in patients with preserved ejection fraction.

A rare prenatal diagnosis: congenital absence of aortic valve.

Congenital absence of the aortic valve is characterised by the absence of aortic valve and severe regurgitation. The rest of the reported cases were mostly diagnosed either on postnatal echocardiography or autopsy. Here, we present a foetal case with the absence of the aortic valve and "inverse circulatory shunt".

Comparison of echocardiography and 320-row multidetector computed tomography for the diagnosis of congenital heart disease in children.

INTRODUCTION: Echocardiography (echo) is the primary non-invasive imaging modality for the assessment of congenital heart disease (CHD). Computed tomography angiography (CTA) also has potential to examine the anatomy of complex heart anomalies as well as extracardiac involvement. OBJECTIVES: The aim of this study is to determine the impact of new CTA technology in the diagnosis of CHD and to compare echo and CTA in terms of diagnostic accuracy. METHODS: Forty-five patients who underwent preoperative echo and CTA assessment in the intensive care unit were included in this study. The results were assessed for three main types of CHD (cardiac malformations, cardiac-major vessel connections and major vessels). The main groups were also divided into subgroups according to surgical features in order to assess them more objectively. Imaging methods were compared for diagnostic accuracy, sensitivity and specificity, while surgical findings were accepted as the gold standard. RESULTS: Patients' median age and weight were two months (three days-eight years) and 12 kg (2.5-60 kg), respectively. In 45 operated cases, 205 subgroup malformations were assessed. Diagnostic accuracy was significantly greater in echo (echo vs. CTA: 98.4% and 96.2% [chi-square=6.4, p=0.011]). During surgery, 84 cardiac malformations (echo vs. CTA: 97.4% and 95.1% [chi-square=4.9, p=0.03]), 47 cardiac-major vessel connections (echo vs. CTA: 98.3% and 95.4% [chi-square=7.5, p=0.03]), and 74 major vessel malformations (echo vs. CTA: 96% and 98% [chi-square=1.8, p=0.48]) were confirmed. CONCLUSION: Echocardiography and CTA are imaging methods with high diagnostic accuracy in children with CHD. The use of echocardiography together with CTA, especially for the visualization of extracardiac anatomy, provides additional information for clinicians.

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.

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.

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.

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.

The assessment of patients with situs inversus and corrected transposition of the great arteries.

INTRODUCTION: Congenitally corrected transposition of the great arteries (ccTGA) is a rare congenital heart defect. There are different subgroups according to the location of the heart in the thorax, apical position and situs. OBJECTIVES: The purpose of this study was to assess pediatric patients with situs inversus (SI) ccTGA (SI-ccTGA), a rare subgroup of this condition, in detail. METHODS: The records of patients with SI-ccTGA followed between January 1, 2010 and January 1, 2019 in our clinic were analyzed retrospectively. Demographic features, associated cardiac defects, arrhythmias and follow-up data were recorded. RESULTS: Twenty-one out of 120 ccTGA patients had SI. The median age was 30 months (4 days-18 years). There were hemodynamically significant associated lesions in 85.7% (n=18) of the patients. A large ventricular septal defect was found in 16 patients (76.2%), severe pulmonary stenosis in 11 (52.4%), pulmonary atresia in six (28.5%), and severe tricuspid regurgitation in two (9.5%). Eleven out of 21 patients had biventricular physiology and eight had single-ventricle physiology. Bidirectional cavopulmonary anastomosis followed by a hemi-Mustard-Rastelli operation were planned for the remaining two patients. Twelve out of 18 patients with associated defects (66.6%) were operated and surgery was planned for three more patients (16.6%). The remaining three patients were scheduled for clinical follow-up. Arrhythmias developed in two (9.5%) patients on follow-up; ablation was performed in one of them and pacemaker implantation followed by cardiac resynchronization therapy was performed in the other. Two patients died during follow-up, one after a central shunt operation and the other preoperatively due to pneumonia and sepsis. CONCLUSION: SI-ccTGA is not a mirror image of situs solitus ccTGA (SS-ccTGA) due to important anatomic and physiologic differences between them. SI-ccTGA patients have a lower risk of tricuspid valve regurgitation than SS-ccTGA patients. The timing of clinical presentation of these patients mainly depends on the type and severity of the associated lesions, as in all subtypes of ccTGA.

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.

Risk Factors for Major Adverse Events after Surgical Closure of Ventricular Septal Defect in Patients Less than 1 Year of Age: A Single-Center Retrospective.

OBJECTIVE: To reveal the risk factors that can lead to a complicated course and an increased morbidity in patients < 1 year old after surgical ventricular septal defect (VSD) closure. METHODS: We reviewed a consecutive series of patients who were admitted to our institution for surgical VSD closure who were under one year of age, between 2015 and 2018. Mechanical ventilation (MV) time > 24 hours, intensive care unit (ICU) stay longer than three days, and hospital stay longer than seven days were defined as "prolonged". Unplanned reoperation, complete heart block requiring a permanent pacemaker implantation, sudden circulatory arrest, and death were considered as significant major adverse events (MAE). RESULTS: VSD closure was performed in 185 patients. The median age was five (1-12) months. There was prolonged MV time in 54 (29.2%) patients. Four patients (2.2%) required permanent pacemaker implantation. Hemodynamically significant residual VSD was observed in six (3.2%) patients. Extracorporeal membrane oxygenation-cardiopulmonary resuscitation was performed in one (0.5%) patient. Small age (< 4 months) (P-value<0.001) and prolonged cardiopulmonary bypass time (P=0.03) were found to delay extubation and to prolong MV time. Low birth weight at the operation was associated with MAE (P=0.03). CONCLUSION: Higher body weight during operation had a reducing effect on the MAE frequency and shortened the MV duration, ICU stay, and hospital stay. As a conclusion, for patients who are scheduled to undergo VSD closure, body weight should be taken into consideration.

Risk factors for major adverse events after surgical closure of ventricular septal defect in patients less than 1 year of age: a single-center retrospective

Abstract Objective: To reveal the risk factors that can lead to a complicated course and an increased morbidity in patients < 1 year old after surgical ventricular septal defect (VSD) closure. Methods: We reviewed a consecutive series of patients who were admitted to our institution for surgical VSD closure who were under one year of age, between 2015 and 2018. Mechanical ventilation (MV) time > 24 hours, intensive care unit (ICU) stay longer than three days, and hospital stay longer than seven days were defined as "prolonged". Unplanned reoperation, complete heart block requiring a permanent pacemaker implantation, sudden circulatory arrest, and death were considered as significant major adverse events (MAE). Results: VSD closure was performed in 185 patients. The median age was five (1-12) months. There was prolonged MV time in 54 (29.2%) patients. Four patients (2.2%) required permanent pacemaker implantation. Hemodynamically significant residual VSD was observed in six (3.2%) patients. Extracorporeal membrane oxygenation-cardiopulmonary resuscitation was performed in one (0.5%) patient. Small age (< 4 months) (P-value<0.001) and prolonged cardiopulmonary bypass time (P=0.03) were found to delay extubation and to prolong MV time. Low birth weight at the operation was associated with MAE (P=0.03). Conclusion: Higher body weight during operation had a reducing effect on the MAE frequency and shortened the MV duration, ICU stay, and hospital stay. As a conclusion, for patients who are scheduled to undergo VSD closure, body weight should be taken into consideration.

The role of intraoperative epicardial echocardiography in pediatric cardiac surgery.

AIM: Our aim was to evaluate the findings and the role of intraoperative epicardial echocardiography (IEE) in the management of pediatric cardiac surgery patients. METHODS: Patients evaluated with IEE between December 2015 and December 2017 were analyzed retrospectively. Demographic data, preoperative transthoracic echocardiography (TTE), and IEE reports were evaluated. RESULTS: A total of 410 patients evaluated by IEE were included in the study. Of these, 52% were women, and 48% were men. The median age was 8.5 months (range: 1 month-7 years), and median body weight was 7.1 kg (range: 3.3-61 kg). The most common diagnoses were tetralogy of Fallot (TOF; n = 148), ventricular septal defect (VSD; n = 117), atrial septal defect (ASD; n = 57), and complete atrioventricular septal defect (AVSD; n = 48). There were minor residual lesions not requiring reestablishment of cardiopulmonary bypass (CPB) in 16.6% (n = 68), while major residual lesions requiring return to CPB were determined in 5.1% (n = 21). Major residual lesions were detected in 7 patients with TOF (4 severe right ventricular outflow tract obstructions, 2 pulmonary artery stenosis, 1 residual VSD shunt), 6 patients with VSD (hemodynamically significant residual shunts), and 5 patients with complete AVSD (3 left atrioventricular valve regurgitations, 1 right atrioventricular valve regurgitation, 1 left ventricular outflow tract obstruction). Transient bradycardia was observed in 5 patients. CONCLUSION: Intraoperative epicardial echocardiography provides good guidance during congenital heart surgery. IEE helps to clarify the surgical planning and decreases morbidity and mortality due to unnecessary invasive procedures, especially for pathologies involving the pulmonary artery and its branches, as well as for apical ventricular septal defects.

Rhythm disturbances and treatment strategies in children with congenitally corrected transposition of the great arteries.

BACKGROUND: We aimed to evaluate rhythm abnormalities in cases of congenitally corrected transposition of the great arteries (ccTGA) and associated treatment strategies. PATIENTS AND METHODS: This retrospective cohort study included 65 pediatric patients with ccTGA who were admitted to the clinic between 2009 and 2017. The patients were divided into two groups, and surgical data, Holter electrocardiographic (ECG) recordings, ECG recordings, electrophysiological data, and device implantation data on the two groups were compared. RESULTS: Group I (n = 53, 82%) consisted of patients with significant associated lesions, and Group II (n = 12, 18%) consisted of those with minor or no associated lesions (isolated ccTGA). Rhythm abnormalities were diagnosed in 22 (34%) of the patients based on initial ECG findings and Holter ECG recordings. Eleven (17%) of these patients had atrioventricular (AV) block of different degrees, and the other 11 (17%) had supraventricular arrhythmia (SVA). The median follow-up was 49 months (range, 9-89 months), and the rhythm remained normal in 26 (42%) of the patients. Three patients died on follow-up. Of 40 patients with normal initial findings, nine required pacemaker implantation due to complete heart block, and SVA developed in seven patients on follow-up. No ventricular tachycardia was seen initially or on follow-up. Ablation was performed in four patients. During the follow-up period, pacemakers were implanted in 12 (23%) of patients in Group I and 4 (33%) of patients in Group II due to complete heart block. Cardiac resynchronization therapy (CRT) was performed in four patients due to systemic ventricular dysfunction. Notably, all four of these patients had a pacemaker implanted postoperatively.