18F-fluorodeoxyglucose positron emission tomography/CT in the diagnosis of right-sided endocarditis in children and adults with infective endocarditis.

OBJECTIVE: Infectious endocarditis poses a diagnostic challenge due to its highly variable clinical presentation. To establish a definitive diagnosis, different imaging modalities are essential. In recent years, positron emission tomography/CT has gained increasing significance in diagnosing infective endocarditis; however, its application in the pediatric age group remains limited. This study encompasses patients definitively or potentially diagnosed with infectious endocarditis at our institution from 2018 to 2023. METHODS: A total of 29 patients underwent 18F-fluorodeoxyglucose positron emission tomography/CT examinations, with 19 of them presenting with right-sided infective endocarditis. RESULTS: Evidence consistent with infective endocarditis was observed in 18 (94.7%) of the patients. Pulmonary septic embolism was identified in 15 (78.9%) cases, and splenic involvement was noted in 12 (57.8%) cases. Transthoracic/transesophageal echocardiography failed to reveal vegetation or provided uncertain results in six patients, whereas fluorodeoxyglucose-positron emission tomography-CT exhibited involvement. Subsequently, the diagnosis of infective endocarditis was confirmed post-surgery based on the fluorodeoxyglucose-positron emission tomography-CT findings. CONCLUSION: Our results, along with our clinical experience, demonstrate that fluorodeoxyglucose-positron emission tomography-CT is a safe and viable method for diagnosing right-sided endocarditis, which is often challenging to visualize using echocardiography.

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.

Transcatheter palliation of aortic pseudoaneurysm in the early post-operative period: "bridge to surgery".

Aortic pseudoaneurysm is a rare, life-threatening complication that can occur after cardiac surgery, trauma, or infections. Surgical repair of aortic pseudoaneurysm is the conventional treatment, but it is associated with very high morbidity and mortality especially in early post-operative period. However, very limited reports of successful transcatheter repair of aortic pseudoaneurysm related to surgery appear in the literature. Herein, a case of a 9-year-old female who developed a pseudoaneurysm, after aortic reconstruction, that was successfully treated percutaneously using atrial septal occluder is presented.

Transcatheter Closure of Aortopulmonary Window in Infants with Amplatzer Duct Occluder-I.

Aortopulmonary window (APW) is a septation defect between the ascending aorta and main pulmonary artery, and it accounts for 0.2-0.6% of all congenital heart diseases. The diagnosis is made by detecting the defect between the ascending aorta and pulmonary artery while both semilunar valves are fully developed. Based on the anatomical characteristics, APW is classified into four types: proximal (Type 1) defect, distal (Type 2) defect, total (Type 3) defect and intermediate (Type 4) defect. APW is traditionally treated by surgery, and there are a few reports about transcatheter APW closure in infancy. Only defects with adequate superior and inferior rims can be considered for device closure. We describe two cases who underwent transcatheter APW closure with the Amplatzer duct occluder-I (ADO-I). Our experience shows that the ADO-I can achieve good results in closure of APW for selected patients.

Transcatheter pulmonary valve implantation in tetralogy of Fallot and Ebstein's anomaly with one and a half ventricular repair.

The coexistence of tetralogy of Fallot and Ebstein's anomaly is extremely rare. There are only a few case reports in the literature, and surgical options for the treatment are controversial. There is insufficient data on long-term follow-up of patients and management of complications. In this case report, we present a 20-year-old adult with operated tetralogy of Fallot, Ebstein's anomaly, and Glenn anastomosis who underwent transcatheter pulmonary valve implantation for severe pulmonary insufficiency.

Percutaneous closure of a left ventricular pseudoaneurysm after transcatheter ventricular septal defect closure.

Left ventricular pseudoaneurysm is very rare in children. Although surgery is conventional treatment, recently, percutaneous closure of pseudoaneurysms has been described. Here, we present the first case where a patient developed left ventricular pseudoaneurysm after percutaneous ventricular septal defect device closure and was treated by a second percutaneous method.

[Transcatheter coil occlusion of patent ductus arteriosus and follow-up results]. / Coil ile transkateter patent duktus arteriyozus kapatilmasi ve izlem sonuçlari.

OBJECTIVE: Although many articles have been published about transcatheter coil occlusion, few studies have been conducted on the long-term outcomes of patent ductus arteriosus closure with a coil. This study is an examination of the follow-up data of patients who underwent transcatheter patent ductus arteriosus closure with a coil. METHODS: Between May 1996 and May 2018, 243 patients underwent transcatheter patent ductus arteriosus closure with a coil. The patients were divided into 2 groups based on the timing of the use of ductal occluders at the facility. Until the end of 2005, every ductus was closed with a coil. Since initiating the use of ductal occluders in 2006, coils are mostly used to close small, elongated ducts, and occasionally, some complex ducts. RESULTS: The median age of the patient was 4.33 years, the median weight was 15 kg, and the median duct diameter was 2 mm. In this study group, 98 (40%) patients were male and 145 were female (60%). The angiographic features of the ductus were classified as type A in 126 patients, type B in 15, type C in 6, type D in 9, type E in 74, and 13 were recorded as other types. In 238 of the patients, the patent ductus arteriosus was successfully closed with a coil. In 5 (2.2%) cases, the procedure was unsuccessful. Echocardiography follow-up continued for an average of 4.1+-4.1 years (1 day-19 years). A residual shunt was observed in 42 patients, and spontaneous occlusion was seen in 29 of the 42 patients. CONCLUSION: The results of this study indicated that closure of the patent ductus arteriosus with a coil was a safe and effective method.

Aortic arch anomalies detected in foetal life by echocardiography.

Aortic arch anomalies refer to congenital malformations of position or branching pattern of the aortic arch. To-date, only a few small studies have documented prenatal detection of aortic arch anomalies. In this article, we share our experience in detecting aortic arch anomalies. Foetal echocardiograms, clinic and genetic histories of 33 patients who had been diagnosed with aortic arch anomaly from 2007 to 2015 were reviewed. In 15 patients, right aortic arch with mirror image branching; in 13 patients, right aortic arch with left ductus arteriosus and aberrant left subclavian artery; in three patients, left aortic arch with aberrant right subclavian artery; in one patient bilateral ductus and right aortic arch with aberrant left subclavian artery and in one patient double aortic arch were detected. In a patient with isolated right aortic arch, 22q11 microdeletion had been revealed. Given this data, we strongly suggest foetal karyotype analysis when aortic arch anomalies are identified. Impact Statement What is already known on this subject: The data about the prenatal diagnosis of aortic arch anomalies are limited. What the results of this study add: In our study, 653 patients were examined by foetal echocardiography during the study period. Thirty three patients who had been diagnosed with aortic arch anomaly prenatally and confirmed after delivery were enrolled in the study. In 15 patients, right aortic arch with mirror image branching; in 13 patients, right aortic arch with left ductus arteriosus and aberrant left subclavian artery; in three patients, left aortic arch with aberrant right subclavian artery; in one patient bilateral ductus and right aortic arch with aberrant left subclavian artery and in one patient double aortic arch were detected. Trisomy 18 was detected in the patient with bilateral ductus arteriosus and Di George syndrome (22q11 microdeletion) was determined in two patients with right aortic arch. While in the first patient, there were no other intracardiac anomalies; in the second patient with 22q11 microdeletion, Fallot tetralogy accompanied the right aortic arch. What the implications are of these findings for clinical practice and/or further research: This results showed that aortic arch anomalies can be associated with genetic anomalies even when they are found without other congenital heart disease. Given these data, we strongly suggest foetal karyotype analysis and genetic testing when aortic arch anomalies are identified.

Hallazgo casual de origen anómalo de la arteria coronaria derecha desde la arteria pulmonar en un lactante / An incidentally detected anomalous origin of the right coronary artery from the pulmonary artery in an infant

El origen anómalo aislado de la arteria coronaria derecha desde la arteria pulmonar principal es una anomalía congénita rara y se han notificado pocos casos en la población pediátrica. En este artículo informamos el caso asintomático de un lactante varón de dos meses de edad al que se le diagnosticó origen anómalo de la arteria coronaria derecha desde la arteria pulmonar principal durante la evaluación realizada para detectar anomalías cardíacas. Debido a la sospecha durante una ecocardiografía, se realizaron un cateterismo cardíaco y una angiografía coronaria para verificar el diagnóstico del origen anómalo de la arteria coronaria derecha desde la arteria pulmonar principal. El paciente se sometió a la cirugía y estaba en buen estado en el seguimiento a los dos meses. El diagnóstico temprano podría evitar que los pacientes tengan complicaciones cardiovasculares.

Isolated anomalous origin of the right coronary artery from the main pulmonary artery is a rare congenital anomaly, and few cases have been reported in the pediatric age group. Here in, we report an asymptomatic case of a 2-month-old male infant who has been diagnosed as anomalous origin of the right coronary artery from the main pulmonary artery during the evaluation for cardiac abnormalities. For a suspicion on echocardiography, cardiac catheterization and coronary angiography performed to verify the diagnosis of anomalous origin of the right coronary artery from the main pulmonary artery. The patient underwent surgery and did well after two months follow up. Early diagnosis may prevent patients from cardiovascular complications.

An incidentally detected anomalous origin of the right coronary artery from the pulmonary artery in an infant. / Hallazgo casual de origen anómalo de la arteria coronaria derecha desde la arteria pulmonar en un lactante.

Isolated anomalous origin of the right coronary artery from the main pulmonary artery is a rare congenital anomaly, and few cases have been reported in the pediatric age group. Here in, we report an asymptomatic case of a 2-month-old male infant who has been diagnosed as anomalous origin of the right coronary artery from the main pulmonary artery during the evaluation for cardiac abnormalities. For a suspicion on echocardiography, cardiac catheterization and coronary angiography performed to verify the diagnosis of anomalous origin of the right coronary artery from the main pulmonary artery. The patient underwent surgery and did well after two months follow up. Early diagnosis may prevent patients from cardiovascular complications.

El origen anómalo aislado de la arteria coronaria derecha desde la arteria pulmonar principal es una anomalía congénita rara y se han notificado pocos casos en la población pediátrica. En este artículo informamos el caso asintomático de un lactante varón de dos meses de edad al que se le diagnosticó origen anómalo de la arteria coronaria derecha desde la arteria pulmonar principal durante la evaluación realizada para detectar anomalías cardíacas. Debido a la sospecha durante una ecocardiografía, se realizaron un cateterismo cardíaco y una angiografía coronaria para verificar el diagnóstico del origen anómalo de la arteria coronaria derecha desde la arteria pulmonar principal. El paciente se sometió a la cirugía y estaba en buen estado en el seguimiento a los dos meses. El diagnóstico temprano podría evitar que los pacientes tengan complicaciones cardiovasculares.

Transcatheter correction of Scimitar syndrome: occlusion of abnormal pulmonary venous drainage and vascular supply in an infant.

Treatment of Scimitar syndrome is usually surgical; however, if there is "dual drainage" - that is, one to the inferior caval vein and the other to the left atrium - it is possible to successfully treat this anomaly via a less-invasive transcatheter approach. We report a case of Scimitar syndrome in a 21-month-old, male infant successfully treated with transcatheter embolisation.

[Our experience in the diagnosis and treatment of postural orthostatic tachycardia syndrome, vasovagal syncope, and inappropriate sinus tachycardia in children]. / Çocuklarda postüral ortostatik tasikardi sendromu, uygunsuz sinüs tasikardisi ve vazovagal senkop tani ve tedavisindeki deneyimlerimiz.

OBJECTIVES: The aim of this study was to share our experience in the diagnosis and treatment of patients who presented at our clinic with syncope, pre-syncope, dizziness, and palpitations. STUDY DESIGN: Patients who were treated at pediatric cardiology clinic for complaints of syncope, dizziness, and palpitations between 2014 and 2016 were enrolled in the study. Detailed history of the patients, physical examination findings, laboratory and electrocardiogram results were recorded. Tilt table test, 24-hour Holter rhythm monitoring, and exercise test were performed, as required. Patients were diagnosed as vasovagal syncope, postural orthostatic tachycardia syndrome (POTS), or inappropriate sinus tachycardia based on these findings. Treatment of the patients was evaluated. RESULTS: Thirty patients were diagnosed as vasovagal syncope, 7 patients as POTS, and 2 as inappropriate sinus tachycardia. POTS accompanied Raynaud's phenomenon in 1 patient, hypertrophic cardiomyopathy in 1 patient, and homocystinuria in another patient. Complaints of patients with vasovagal syncope improved with non-medical therapy. Medical treatment was administered to the patients with diagnosis of POTS and inappropriate sinus tachycardia. CONCLUSION: In patients with complaints of syncope, pre-syncope, dizziness, and palpitations without structural heart disease or non-rhythm problems, cardiovascular autonomic disorders, such as POTS and inappropriate sinus tachycardia should be kept in mind, as well as vasovagal syncope.

Aortic Valve Prolapse and Aortic Regurgitation During Long-Term Follow Up in Children with Ventricular Septal Defect.

BACKGROUND: Aortic valve prolapse (AVP) and aortic regurgitation (AR) can develop in a subset of patients with ventricular septal defect (VSD). The incidence and progression of AVP and AR with VSD at long-term follow up was evaluated. METHODS: The records of 2,275 patients with isolated VSD who had been diagnosed using echocardiography at the present authors' institution between 1988 and 2014 were reviewed. RESULTS: AVP was detected in 178 patients (7.8%), using echocardiography. Of 178 patients with AVP, AR was detected in 124 (AR incidence 5.4%). A total of 142 patients was followed medically during a median of 10 years after AVP had appeared. Initially, no AR was noted in 66 of these patients, trivial AR in 41, mild AR in 30, moderate in three, and severe in two. Trivial AR developed in 20 and mild in eight of 66 patients who had no AR. In 18 of 61 patients (29.5%), trivial AR progressed to mild during a median of 3.6 years, and in five of 18 patients (27.7%) mild AR progressed to moderate during a median of 2.3 years. Postoperatively, AR improved in 17 patients, remained unchanged in 12, and worsened in four at between two months and 16.5 years of follow up (median 6.6 years). CONCLUSIONS: Frequent (six-month) echocardiographic evaluation in patients with perimembraneous or muscular outlet VSD after AVP and AR development may be useful. In addition, surgical intervention in patients with perimembraneous or muscular outlet VSD, AVP and mild AR may prevent the worsening of AR.

Cooccurrence of Postural Orthostatic Tachycardia Syndrome with Two Different Clinical Entities.

Postural orthostatic tachycardia syndrome (POTS) is an abnormal heart rate response to a positional change. Several potential mechanisms for pathophysiology of POTS are defined. This syndrome can coexist with different clinical situations. In our report, the first case was a 13-year-old female who has been followed up for diagnosis of homocystinuria. She was admitted to our outpatient clinic with complaints of dizziness after suddenly moving from supine to upright position and chest pain after exercise. Tilt table test was performed to evaluate dizziness. According to the tilt table test the patient was diagnosed with POTS. The second case was a 17-year-old female who had been evaluated in different centers with the complaints of fainting, bruising, redness, and swelling on the hands and feet after moving from supine position to upright position during the last 4 years. Postural orthostatic tachycardia syndrome was diagnosed by tilt table test and ivabradine was started. Herein, we aimed to point out the cooccurrence of different clinical entities and POTS.