Treatment of a patient with congenitally corrected transposition of great arteries associated with ascending aortic hypoplasia and type A interrupted aortic arch without aortic cross clamping leading to myocardial ischemia

İnterrupted aortik ark tedavisi karmaşık bir süreçtir. Cerrahi rekonstrüksiyon genellikle myokard iskemisine neden olan aortik kros klempleme, kalbin durdurulması ve hatta kansız operasyon sahası için derin hipotermi gerektirir.Bu yazıda, normotermik şartlarda, kalbi durdurmadan çıkan aort, aortik ark ve proksimal desendan aort replasmanını selektif kanülasyon ve tüm vücut perfüzyonunu sağlayan cerrahi teknik ile tedavi ettiğimiz konjenital düzeltilmiş büyük arter transpozisyonu, tip A kesintili aortik ark ve hipoplazik asendan aortu olan hastamızı sunuyoruz.Treatment of interrupted aortic arch is complex. Surgical reconstruction consists of procedures that cause myocardial ischemia, such as aortic cross clamping, cardioplegia, and hypothermia. In this article, we present a patient with congenitally corrected transposition of great arteries together with Type A interruption as well as hypoplastic ascending aorta and the aortic arch. The replacement of the aorta, aortic arch, and the proximal descending aorta was performed under normothermic conditions without cardioplegia, which was achieved with a technique that preserves the systemic blood supply by using selective cannulation

New variation of scimitar syndrome with tracheal bronchus, upper lobe agenesis, and ventricular septal defect in a child

Scimitar syndrome (SS) is a rare and complex disease charac-terized by cardiovascular and bronchopulmonary malforma-tions. In typical SS, anomalous pulmonary drainage of part orall of the right lung into the inferior vena cava, anomaloussystemic arterial supply of the right lung from either the tho-racic or abdominal aorta, and hypoplasia of the right lungoccur.1Abnormal bronchial anatomy, abnormal diaphragm,hemivertebrae, and genitourinary tract anomalies can alsomanifest in SS.2,3Generally, the symptoms of this syndromeappear 7 months after birth.4According to the literature, therehave been numerous cases where additional features of thetypical syndrome have been reported with Scimitar variants

Co-occurrence of interrupted aortic arch and Apert syndrome: A case report

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Evaluation of executive functions in children with rheumatic heart diseases

Background Acute rheumatic fever (ARF) is a multisystemic inflammatory disease in children and young adults. The most notable complications of ARF are rheumatic heart disease (RHD) and Sydenham's chorea (SC). There have been many reports about executive dysfunctions with children who have SC. "Executive function" is an umbrella term that is used to describe higher level cognitive functions. The aim of this study is to determine the executive functions of children with RHD. We evaluated executive functions in healthy children with the same sociodemographic characteristics as children with RHD. Methods Our study was designed as a cross-sectional randomized study, including children with RHD aged between 12 and 18, and healthy controls. The difference between the patient and control group participants in terms of age, gender, education level, education level of the parents, family income level, and executive functions were investigated. Executive functions composed of Digit Sequence Test, Verbal Fluency Test, Trail-Making Test, Stroop Test, Wisconsin Card Sorting Test. Results In our study, a total of 30 children with RHD were followed up at the pediatric cardiology outpatient clinic of Bezmialem Vakif University Hospital composed the patient group. The control group was made up of 30 healthy children of the same sex and age group as the patient group. The mean age of the case group was 14.73 +/- 1.84 years. The Digit Span Test, Verbal Fluency Test, Trail-Making Test, Wisconsin Card Sorting Test, and Stroop Test produced no statistically significant differences between the RHD patients and the controls. Conclusions No statistically significant difference was found between the RHD patients and control patients in any executive function test. It was suggested that executive dysfunction might not develop in RHD patients before developing SC

Balanced double aortic arch causing persistent respiratory symptoms mimicking asthma in an infant

Double aortic arch (DAA) is a common form of complete vascular ring. The condition leads to airway obstruction and compression of the esophagus. A balanced type of DAA is an extremely rare anomaly. The anatomical aberration cannot be easily distinguished by transthoracic echocardiography when an infant has chronic respiratory distress. Herein we presented a case of an 11-month-old infant who had chronic respiratory distress and a balanced type of DAA

Development of myocardial infarction in a 12-year-old female after the use of inhaled salbutamol

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) accounts for 0.023% of all cases reported in pediatric patients. According to literature, only a handful of ALCAPA patients are able to reach adulthood. Clinical manifestations of ALCAPA range from fatigue during exercise to sudden death in adulthood. Herein, we described a 12-year-old symptomatic patient with ALCAPA who had severe chest pain after using salbutamol treatment for presumed asthma. ALCAPA is one of the curable versions of myocardial ischemia and infarction in childhood. Due to clinical findings in conjunction with electrocardiogram and echocardiography, a computed tomography scan with coronary angiography was performed and the diagnosis of ALCAPA was confirmed. We presented this case because ALCAPA-related myocardial ischemia and infarction in children are rare with only sporadic cases reported. This case illustrated the need for close monitoring and surgery as the best treatment for ALCAPA associated with myocardial infarction

MRI evaluation of right heart functions in children with mild cystic fibrosis

Background: This study aimed to assess the ventricular anatomy, function of the right ventricle, and the haemodynamic findings of pulmonary artery in children with cystic fibrosis using cardiac MRI. Patients: This prospective study consisted of 32 children with mild cystic fibrosis and 30 age-matched healthy control participants. Methods: Cardiac MRI was used to assess right ventricular volumes, anatomy, and function and to assessment of haemodynamic findings of pulmonary artery in the control and study groups. Haemodynamic findings of pulmonary arteries were determined using pulmonary arteries peak velocity (cm/s), and pulmonary arteries time-to-peak velocity (ms) and pulmonary artery systolic pressure. All data of children with mild cystic fibrosis were compared with those of 30 age-matched healthy control group participants. Results: Our patients and their age-matched controls were aged from 6 to 17 years and from 7 to 15 years, respectively. We found that ejection fraction (%), cardiac output (L/ml), cardiac output (L/ml/m2), and systolic volume (ml/m2) were significantly lower in children with cystic fibrosis (p < 0.01). Right ventricular anterior wall thickness (mm) was significantly higher in children with cystic fibrosis (p = 0.01). No significant difference was observed between the haemodynamic parameters of pulmonary artery in the patient group. Conclusion: In our study, cardiac MRI was used to investigate whether the right ventricle was affected functionally and anatomically in children with mild cystic fibrosis. We detected a significant decrease in right ventricular systolic functions and notable alterations in the right ventricular geometry of children with mild cystic fibrosis. These alterations usually manifest themselves as hypertrophy of the right ventricle. Our study's results demonstrate no relationship between the development of pulmonary hypertension in mild cystic fibrosis children

Short-term efficacy of ORS formulation and propranolol regimen in children with POTS

Background: To evaluate the short-term effectiveness of reduced-osmolarity oral rehydration salt formulation (ORS) and propranolol in children diagnosed with postural orthostatic tachycardia syndrome (POTS) in head-up tilt testing (HUTT).Methods: Children were admitted with symptoms of orthostatic intolerance (OI) occurring in a standing position and disappearing in the supine position. Patients with heart rate increments of >= 40 bpm and symptoms of OI constituted the pediatric POTS group in HUTT. A total of 70 pediatric patients with POTS were included in the study. POTS patients were divided into two groups based on whether they were prescribed reduced-osmolarity ORS and propranolol or not. The study group comprised patients on a regimen of reduced-osmolarity ORS and propranolol (n = 34), while the control group comprised patients who were not prescribed any medication (n = 36). The frequency of symptoms and standardized symptom scores were analyzed before and after 3 months of treatment in both groups.Results: The post-treatment frequency of syncopal attacks was significantly reduced in both groups (P < 0.01 for both groups), but the post-treatment standardized symptom scores were significantly reduced in the pediatric study group compared with the control group (P < 0.01).Conclusion: The frequency of syncopal attacks was significantly reduced and the symptom scores for OI were improved in the study group. The improvement in OI symptom scores was better in the treatment group than in the control group. The control group symptoms persisted and caused extreme difficulty in their daily activities. In view of its clinical efficacy, we strongly advocate the use of combined treatment of reduced-osmolarity ORS and low-dose propranolol in pediatric patients with POTS. (C) 2020 French Society of Pediatrics. Published by Elsevier Masson SAS. All rights reserved

Diagnostic role of gray-scale and shear-wave elastography in pediatric patients with undescended testes: a prospective controlled study

Ultrasound elastography is a simple non-invasive method for measuring tissue elasticity in relation to tissue fibrosis. The aim of this study was to compare echogenicity, volume and shear wave velocities of undescended vs normally descended testes

Evaluation of cardiac arrhythmias by electrocardiographic markers in pediatric patients who have tuberous sclerosis without cardiac rhabdomyoma

Background: Tuberous sclerosis (TS) is an autosomal dominant and hereditary disorder. Cardiac rhabdomyoma and arrhythmias are the most deleterious risk factors linked to TS. Although arrhythmias in pediatric patients with TS who have cardiac rhabdomyoma have been frequently reported, arrhythmia in patients who have TS without rhabdomyoma is rarely reported in the literature. The study aimed to assess the susceptibility of pediatric patients who have TS without cardiac rhabdomyoma to cardiac arrhythmia using electrocardiographic (ECG) markers. Methods: This prospective study included 10 patients who had TS without cardiac rhabdomyoma. The control group was made up of 30 healthy children of the same age and sex as the patient group. P wave, P wave dispersion, QT dispersion, QTc dispersion, TP-e interval, and TP-e interval dispersion were calculated on 12-lead surface ECGs for each patient in both groups and compared. Results: P wave, P wave dispersion, QT dispersion, and QTc dispersion were found to be significantly higher in the patient group (P<0.001). Furthermore, patients had a greater Tp-e interval and Tp-e interval dispersion than healthy children (P<0.001). Conclusion: Pediatric patients with TS without cardiac rhabdomyoma might be prone to atrial and ventricular arrhythmias according to their prolonged ECG markers. Our findings suggest that patients with TS without cardiac rhabdomyoma need close monitoring for atrial and ventricular arrhythmias