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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., (© 2018 Wiley Periodicals, Inc.)

The article presents a case study of a 15-year-old male patient with a congenital Gerbode defect and aortic valve non-coronary cusp prolapse. Topics discussed include the association between a direct Gerbode defect and aortic valve prolapse, emphasizing the rarity of this occurrence, and highlighting the anatomical factors contributing to the relationship between these conditions, providing valuable insights for clinicians.

Background: Hypertrophic cardiomyopathy (HCM) is the second most common pediatric cardiomyopathy. Although there is a large body of literature about HCM in adults, there is limited information on HCM in childhood. We evaluated various aspects of pediatric HCM patients treated at our center. Methods: We identified 152 pediatric patients with HCM between October 2011 and October 2019. Clinical history, invasive (ICD, pacemaker, electrophysiologic study, catheter ablation therapy) and non‐invasive (ECG, holter moniterization, echocardiography, cardiac MR, genetic study, medicam treatment) data were collected and evaluated. Results: The mean ± standard deviation age of patients was 8.9 ± 5.7 years (1 month–18 years) and 67.8% were male. The most frequent clinical symptoms were murmur and palpitations. Three cases (2%) had aborted sudden death as the first manifestation of HCM. Of these patients, 120 (78.9%) had non‐syndromic HCM and 32 (27.2%) had syndromic HCM. Asymmetric septal hypertrophy was common (48.3%) in the non‐syndromic group, whereas concentric hypertrophy was common (56.2%) in syndromic group. Left ventricular outflow tract obstruction (LVOTO) occurred in 39 (25.6%) patients. Nine (5.9%) patients underwent electrophysiologic study and/or ablation and 16 patients underwent surgical intervention. Implantable cardioverter defibrillator (ICD) insertion was performed in 38 patients (26 transvenous, 12 epicardial). ICDs were inserted in three (7.9%) patients for secondary prevention; in the remaining patients (92.1%) the devices were placed for primary prevention. Mean SD follow‐up time was 27.1 ± 22 months. Five (3.3%) patients died during the follow‐up. No patient had heart transplantation or a long‐term assistive device. Conclusion: The etiology of HCM is heterogeneous and present at any age. It is important to determine the timing of surgery and potential risks for sudden cardiac arrest. As most cases of HCM are familial, evaluation of family members at risk should be a routine component of clinical management. [ABSTRACT FROM AUTHOR]

Abstract: Background: The aim of this study was to evaluate the results of non‐operational sustained tachyarrhythmia in patients <1 year of age at the present center. Methods: Between November 2010 and November 2016, the demographic characteristics, type and localization of the tachyarrhythmia, echocardiographic findings, and medical and/or ablation therapy for patients <1 year of age with sustained tachyarrhythmia were evaluated. Results: Of 99 patients, 91 had sustained supraventricular tachycardia, and eight had sustained ventricular tachycardia. The median age was 30 days (range, 1–350 days), and the median weight was 4.2 kg (range, 2–13 kg). The common symptoms were palpitation and restlessness (n = 49, 49.5%), or the tachycardia was detected during routine inspection (n = 25, 25.3%) or fetal echocardiography (n = 11, 11.1%). Nineteen individuals (19%) had left ventricular (LV) dysfunction on first echocardiography. Twenty individuals had congenital heart disease. Common diagnoses were Wolff‐Parkinson‐White syndrome (n = 27), focal atrial tachycardia (n = 10), permanent junctional reciprocating tachycardia(n = 6), and atrial flutter (n = 6). Seventeen patients underwent medical therapy combined with cardioversion. The most commonly used abortive and acute therapy agents were adenosine, esmolol, and amiodarone. The most common combination therapy was propranolol and amiodarone, followed by a propranolol and propafenone combination. Nine individuals were treated with catheter ablation due to either resistance to medical therapy or LV dysfunction. Conclusions: Tachyarrhythmias in children <1 year of age are mostly caused by accessory pathways and require multidrug therapy. Catheter ablation is an effective alternative therapy in the case of resistance to medical therapy and/or LV dysfunction. [ABSTRACT FROM AUTHOR]

Background Syncope is a frequent complaint in children and adolescents and may be a significant sign of serious pathology. Although patient history, family history, and physical examination are sufficient to reach a diagnosis in most cases of syncope, the cause of syncope still cannot be determined after initial investigation in one-third to half of all patients. The aim of this study was to evaluate the diagnostic yield of implantable loop recorder ( ILR) in children with unexplained syncope. Methods A retrospective review was carried out of clinical data, indications, findings, and a final management strategy in patients who underwent ILR implantation. Results A total of 12 patients with a mean age of 9.4 ± 4.5 years underwent ILR (Reveal Plus; Medtronic) implantation. ILR implantation indication was syncope in all of the patients. Family history, routine cardiac assessment, including resting 12-lead electrocardiogram, transthoracic echocardiography, 24 h Holter recording, and event recorder findings, were normal with the exception of one patient with (previously corrected) tetralogy of Fallot. After an average of 20 months (range, 1-36 months), six patients developed symptoms. ILR memory showed torsades de pointes-ventricular fibrillation ( n = 3), catecholaminergic polymorphic ventricular tachycardia ( n = 1), asystole and ventricular tachycardia ( n = 1), and normal sinus rhythm ( n = 1). At the time of writing six patients were still in follow up with no symptoms after an average of 25.2 months. Conclusion Implantable loop recorder plays an important role in the diagnosis of life-threatening arrhythmias in which syncope is otherwise unexplained. ILR implantation should be remembered in children whose symptoms are strongly correlated with rhythm disturbances. [ABSTRACT FROM AUTHOR]

Background The aim of this study was to examine the causes of cardiac tamponade in children undergoing percutaneous pericardiocentesis. Method Patients who presented with other complaints but were diagnosed with cardiac tamponade based on clinical and echocardiographic findings between January 2010 and January 2013 were retrospectively investigated. Electrocardiography, telecardiography and transthoracic echocardiography were performed. Pericardiocentesis was performed percutaneously under continuous blood pressure and rhythm monitoring with echocardiography and fluoroscopy. Pericardial fluid was analyzed on hemography and biochemistry. Results Fourteen patients (six boys, eight girls; median age, 7 years) underwent pericardiocentesis for cardiac tamponade. At presentation, 78% had dyspnea, 56% chest pain, and 49% fever. All had cardiomegaly, and their cardiothoracic index was 0.56-0.72. Also, all patients had sinus tachycardia; 78%, low QRS voltage; 70%, ST- T changes; and 50% QRS alternans. On echocardiography the widest diameter of pericardial effusion was between 12 mm and 36 mm depth around the heart. The pericardial fluid was purulent in one, serohemorrhagic in seven, serofibrinous in two, and serous in four cases. Pericardiocentesis was unsuccessful in two patients, who underwent open surgical drainage, with no complications. Based on pericardial fluid characteristics and additional tests, cardiac tamponade was caused by an infection in five patients, hypothyroidism in two, familial Mediterranean fever in two, malignancy in one, acute rheumatic fever in one, collagen tissue disease (systemic lupus erythematosus) in one, catheter placement-associated damage in one, and idiopathic pulmonary arterial hypertension in one patient. Conclusion Pericardial effusion and cardiac tamponade in children have varied causes, and early treatment is life saving. [ABSTRACT FROM AUTHOR]

Aim We evaluated ambulatory patients with Duchenne muscular dystrophy from the cardiovascular standpoint and studied the correlation between the results of electrocardiographic (ECG) findings, left ventricular ejection fraction (LVEF), troponin T and N-terminal pro-brain natriuretic peptide (NT-proBNP) levels and patients' North Star Ambulatory Assessment scores. Methods: Fifty patients of ages 6-12 (8.9 ± 2.8) were enrolled in this cross-sectional study. Cardiac evaluation included electrocardiography, echocardiography and cardiac enzyme tests. Results North Star scores ranged from 6/34 to 34/34. Twenty-eight patients (56%) had ECG changes. The most frequently seen ECG abnormalities were short PR interval (14%, n= 7), right ventricular hypertrophy (16%, n= 8), prolonged QTc interval (10%, n= 5), prominent Q wave (10%, n= 5) and T wave inversion (44%, n= 22). In 10 patients (20%), LVEF was below 55%, troponin T and NT-proBNP levels were significantly elevated ( P= 0.003 and P < 0.001, respectively). When North Star scores were compared to patients' age, enzyme levels, ECG and echocardiographic results, we discovered negative correlation with age ( P < 0.001) and troponin T levels ( P= 0.02) and positive correlation with LVEF ( P= 0.02). Conclusion Patients with North Star scores of ≤16 are more at risk of developing cardiomyopathies. Troponin T is a cardiac index that can be used for evaluating myopathic patients and it seems to be correlated with the proBNP levels and LVEF values. [ABSTRACT FROM AUTHOR]