A descriptive clinical study in healthy adolescents was done to evaluate the clinical shoulder balance and analyze the correlation between clinical and radiological parameters which are currently used to evaluate shoulder balance. In addition to trunk shift and rib hump, shoulder balance is one of the criteria that are used to evaluate the outcomes in spinal deformity surgery. Several methods have been proposed to evaluate the shoulder balance in scoliotic patients; however, there is no uniformity to these methods in the current literature. Patients who applied to pediatric clinic without musculoskeletal pathology formed the patient population. Volunteers were asked to fill out a questionnaire assessing shoulder balance perception and had their clinical photograph taken simultaneously with a P-A chest X-ray. The clinical shoulder balance was evaluated through analysis of the clinical photograph. The X-rays were used to evaluate the radiological shoulder balance. The evaluated parameters included coracoid height difference (CHD), clavicular angle (CA), the clavicle-rib cage intersection difference (CRID), clavicular tilt angle difference (CTAD), and T1-tilt. The study group was composed of 48 male and 43 female patients with an average age of 13.6 +/- 2.1 (10-18) years. In the questionnaire, all patients stated that their shoulders were level. The digital photographs revealed that only 17(18.7%) adolescents had absolutely level shoulders. The average height difference between shoulders was 7.5 +/- 5.8 mm. The average CHD was 6.9 +/- 5.8 mm, average CA was 2.2 +/- 1.7 degrees , average CRID was 4.8 +/- 3.6 mm, average CTAD was 4 +/- 3.2 degrees , and average T1-tilt was 1.3 +/- 1.4 degrees . CHD, CA, and CRID demonstrated high correlation with clinical pictures, whereas CTAD demonstrated moderate and T1-tilt demonstrated only mild correlation. The radiological parameters used to evaluate the shoulder balance correlate with the clinical appearance. Contrary to popular belief, shoulder balance in healthy adolescents often does not exist.

Catecholaminergic polymorphic ventricular tachycardia is a rare entity that can occur in children without cardiac disease and with a normal QT interval. It may cause syncope, convulsions, and sudden death during physical activity or emotional distress. We report the clinical features, treatment, and follow-up of 16 children with this diagnosis, emphasizing the potentially fatal nature of the disease.The mean age of patients at the onset of symptoms and at the time of diagnosis was 7.8 plus or minus 2.5 years, and 10.6 plus or minus 3.5 years, respectively. Syncope was the main complaint in 11, and 7 were treated as erroneously as having epilepsy. Diagnosis was confirmed by exercise and/or infusion of isoproterenol. Once the diagnosis was made, we started propranolol in all patients, and added verapamil if ventricular tachycardia was still inducible on a treadmill exercise test. An intracardiac defibrillator was implanted in 4 patients. Of the 16 patients, 4 died suddenly, giving a rate of mortality of 25%. In 2 of those dying suddenly, there was evidence of poor compliance to the recommended treatment. Another 2 patients had been resuscitated because of sudden cardiac arrest.Catecholaminergic polymorphic ventricular tachycardia must be considered in the differential diagnosis of syncope in children without heart disease but with a normal QT interval. Medical treatment with propranolol and verapamil may decrease the incidence of arrhythmia. Implantation of intracardiac defibrillators should be considered in those resistant to drug therapy. Delay in diagnosis, and inadequate treatment, can result in sudden cardiac death.

This study demonstrated that back-up guidewires can be used effectively and safely for pacing during BAV procedures. This technique omits probable complications related to a second vascular access and may shorten the procedure time and decreases costs by eliminating the use of an additional sheath and a temporary pacemaker electrode.

Radiofrequency ablation is a safe and effective method to manage children with supraventricular tachycardia, but patients must be observed for recurrence and new arrhythmias. Sustained tachycardia may also be induced in asymptomatic patients. A transesophageal electrophysiologic study is effective and safe for follow-up of radiofrequency ablation for assessment, diagnosis, and treatment of patients.

Tokel K, Gümüş A, Ayabakan C, Varan B, Erdoğan İ. Complications of cardiac catheterization in children with congenital heart disease. Turk J Pediatr 2018; 60: 675-683.Catheterization procedures for congenital heart disease include a broad range of procedures with a large spectrum of potential adverse outcomes. We aimed to determine the incidence of various complications during pediatric cardiac catheterizations and to designate the relative risk factors for such complications.All pediatric patients undergoing cardiac catheterizations between January 2005-December 2010 were included. Data are collected prospectively by filling out computerized catheterization reports. Patient records were scanned for potential risk factors retrospectively. Groups were divided based on cardiac diagnosis; type of procedure. Adverse events were categorized into major or minor events. A total of 2662 cardiac catheterizations were performed during this period. The mean age of patients was 53.2±64.3 months. Diagnostic catheterizations were done for 1797 (67.5%) patients, and interventional procedures were done in 865 (32.5%) cases. Adverse events were observed in 688 patients (26%) during 941 procedures. Minor and major events were seen in 21.4% and 7.1% of the procedures respectively. Most frequent major complications were anesthesia related (6%), most frequent minor complications were vascular complications (45.2%). Complications were more frequent in younger patients (p=0.0001), during interventional procedures (p=0.0001). Thirteen patients died after a cardiac catheterization; they were younger and had longer procedures compared to those who survived (p=0.0001). Vascular complications were frequent among younger patients, with prolonged procedure time and vessel access (p<0.0001). Cyanotic patients had more complications (p<0.05; OR for major and minor complications: 3.5 and 2 respectively). Minor complications were 2.7 times more likely in ventricular outflow obstructions (p<0.05).The complication rates of cardiac catheterization in children are low, but not negligible. Defining risk factors will help anticipate adverse events, which will guide in preparation for rescue procedures and improvement of patient safety systems in catheterization laboratories.

Objective:Double-chambered right ventricle is characterised by division of the outlet portion of the right ventricle by hypertrophy of the septoparietal trabeculations into two parts. We aim to report our experiences regarding the presenting symptoms of double-chambered right ventricle, long-term prognosis, including the recurrence rate and incidence of arrhythmias after surgery.Methods:We retrospectively investigated 89 consecutive patients who were diagnosed to have double-chambered right ventricle and underwent a surgical intervention from 1995 to 2016. The data obtained by echocardiography, cardiac catheterisation, and surgical findings as well as post-operative follow-up, surgical approaches, post-operative morbidity, mortality, and cardiac events were evaluated.Results:Median age at the time of diagnosis was 2 months and mean age at the time of operation was 5.3 years. Concomitant cardiac anomalies were as follows: perimembranous ventricular septal defect (78 patients), atrial septal defect (9 patients), discrete subaortic membrane (32 patients), right aortic arch (3 patients), aortic valve prolapse and/or mild aortic regurgitation (14 patients), and left superior caval vein (2 patients). The mean follow-up period was 4.86 ± 4.6 years. In these patients, mean systolic pressure gradient in the right ventricle by echocardiography before, immediately, and long-term after surgical intervention was 66.3, 11.8, and 10.4 mmHg, respectively. There were no deaths during the long-term follow-up period. Surgical reinterventions were performed for residual ventricular septal defect (2), residual pulmonary stenosis (1), and severe tricuspid insufficiency (1).Conclusion:The surgical outcomes and prognosis of double-chambered right ventricle are favourable, recurrence and fatal arrhythmias are unlikely in long-term follow-up.

Background and Objectives. Aortic balloon valvuloplasty (ABV) has become the first-line treatment for critical aortic valve stenosis in infants. We aimed to evaluate the short- and mid-term results of patients who underwent ABV during neonatal period, the factors affecting the success and complications of the procedure. Methods. We retrospectively examined 65 patients who underwent ABV during the neonatal period between 1998 and 2017. All hospital records including cardiac catheterization reports, echocardiographic information, and angiographic views were reviewed. Results. Forty five (69.2%) of the patients were male and mean follow-up was 6.2 &#xb1; 4.9 years (range: 6 months - 19 years). The mean age of the patients at the first ABV was 14.5 &#xb1; 10.6 days (range: 1-30 days) and body weight was 3.25 &#xb1; 0.6 kg (range: 1.5-4.8 kg). The peak systolic gradient measured during pre-valvuloplasty cardiac catheterization was 73.3 &#xb1; 22.7 mmHg (range: 30-142 mmHg), and it decreased to 29.2 &#xb1; 12.2 mmHg (range: 5-55 mm Hg) after the procedure. Valvuloplasty was successful in 59 (90.7%) patients. There was no more than mild aortic regurgitation in any patient before valvuloplasty. There was mild aortic regurgitation in 21 patients before the valvuloplasty. In the acute phase after valvuloplasty, 30 patients had mild, 15 had moderate and two had severe aortic regurgitation. There was a significant increase in the degree of aortic regurgitation related to valvuloplasty (p &lt; 0.05). The most important complication of ABV was increased aortic regurgitation (26.2%). Another important complication was femoral artery occlusion; and was detected early after valvuloplasty (61.6%). There was no serious complication or death in the acute phase. Conclusions. In newborns with valvular aortic stenosis, balloon valvuloplasty has become the first choice in many centers due to its high success rate, low mortality and morbidity, and increased clinical experience. Aortic regurgitation and femoral artery occlusion were the most important complications. Although reintervention for residual or recurrent aortic valve stenosis is common during the first year after valvuloplasty, these patients are able to reach advanced ages without the need for surgical intervention. Surgical valvotomy is a good alternative treatment for a small number of patients in whom valvuloplasty fails.

Middle aortic syndrome (MAS) follows a course with distal thoracic and abdominal aorta stenosis. It is a rare disease that is usually diagnosed after the first decade of life. Clinical reflection of MAS is often in the form of hypertension and claudication in the lower extremities. Its etiology is unclear, but is known to be associated with congenital or acquired diseases. This pathology, which is accompanied by malignant hypertension, often does not respond to medical treatment. In patients with MAS, surgical treatment is first line recommendation to prevent complications such as hypertension, heart failure, intracranial bleeding, or aortic rupture. In order to draw attention to this disease, presently described is case of high blood pressure detected during routine examination of a child who had no complaint, and discovery of long-segment stenosis in the abdominal aorta identified with echocardiography and conventional angiography.