Image quality and radiation doses of volumetric 320-row computed tomography angiography with prospective electrocardiogram-gating in the assessment of coronary arteries in neonates and infants.

BACKGROUND: Diagnostic imaging of coronary arteries is required in neonates and infants suspected of congenital or acquired coronary artery anomalies and in pre- and postoperative assessment of complex congenital heart diseases (CHD). AIM: Our study aimed to evaluate the image quality of volumetric 320-row computed tomography angiography (CTA) with prospective electrocardiogram (ECG)-gating for coronary arteries in neonates and infants with heart diseases, analyze factors influencing image quality and assess a radiation dose related to the procedure. METHODS: The study included 110 CTA performed in neonates and infants with CHD. RESULTS: CTA was performed in 37 girls and 73 boys at a median (interquartile range [IQR]) age of 3.0 (0.5-5.0) months, median (IQR) body weight of 5 (3.66-6.5) kg, and median heart rate (HR) of 133 (92-150) beats per minute. The orifices of the left coronary artery were visible in 100% of CTA, the orifices of the right coronary arteries were visible in 96%, whereas all coronary segments were assessable in 45% of CTA. Patients with non-diagnostic segments were significantly younger, median (IQR) age of 2.0 (0.21-5.00) months, had lower body weight of 4.6 (3.45-6.07) kg and faster HR of 136.5 (120-150) beats per minute (P <0.05) than patients with diagnostic image quality in all segments (4.0, 2-6 months, 6.0, 4.2-7 kg, and 130; 110-150 beats per minute, respectively; P <0.05). CONCLUSIONS: CTA performed with volumetric 320-row prospective ECG-gating allows for good visibility of the coronary arteries with an acceptable radiation dose. Children aged >15 days, with body weight >4.85 kg and HR <130 beats per minute are good candidates for excellent quality non-invasive CTA of all segments of coronary arteries.

Normal values of native T1 and T2 relaxation times on 3T cardiac MR in a healthy pediatric population aged 9-18 years.

BACKGROUND: Native myocardial T1 and T2 relaxation times are diagnostic tools used in clinical practice for adult and pediatric populations. Use of a mapping technique requires accurate knowledge of normal ranges in healthy patients, which is lacking in pediatric populations. PURPOSE: To establish normal values for native T1 and T2 mapping in healthy pediatric subjects of different ages and sex. STUDY TYPE: Prospective. POPULATION: Thirty-eight healthy children (9-18 years; mean age 14.0 ± 2.7). FIELD STRENGTH: Cardiac MR with a 3T scanner. T1 and T2 mapping using MyoMaps software. ASSESSMENT: T1 and T2 relaxation times were calculated from a 0.7-1.0 cm2 region of interest placed at the mid-ventricular short-axis slice in the interventricular septum by two observers. Inter- and intraobserver variability was assessed. STATISTICAL TESTS: The Student's t-test or the Mann-Whitney test for unpaired samples was applied to compare one continuous variable between two category groups. One-way analysis of variance (ANOVA) or a Kruskal-Wallis test was applied to compare one continuous variable between three category groups. Correlation between two continuous variables was assessed with a Pearson or Spearman test. RESULTS: The mean native T1 relaxation time was 1223 ± 29 msec and T2 relaxation time was 43 ± 4.5 msec. There was no correlation between T1 /T2 values and age or body surface area (for T1 P = 0.94 and 0.90 and for T2 P = 0.19 and 0.64, respectively). There was weak correlation between T1 values and body mass index (BMI) (r = 0.448, P = 0.005). T2 values were significantly higher in females compared with males (44.6 ± 4.2 vs. 40.4 ± 3.8 msec, P = 0.002). We found a significant rise of T2 relaxation time in the pubertal period (age 13-15 years) comparing to prepubertal (age 9-12 years). Inter- and intraobserver agreement of T1 (r = 0.93; r = 0.99) and T2 (r = 0.96; r = 0.95) were high. DATA CONCLUSION: We report normal values of native T1 and T2 relaxation times obtained with Myomaps software for 3T cardiac MR in a healthy pediatric population. LEVEL OF EVIDENCE: 2 Technical Efficacy: Stage 2 J. Magn. Reson. Imaging 2020;51:912-918.

Cardiovascular magnetic resonance with parametric mapping in long-term ultra-marathon runners.

PURPOSE: There is a direct reverse dose-effect relationship between the amount of physical activity and cardiovascular risk. It is unknown whether this is true for extreme, persistent endurance training. The aim of the study was to assess structural changes of the heart in long-time ultra-marathon runners with special focus on myocardial fibrosis using parametric mapping. METHOD: We studied a group of 30 healthy, male ultra-marathon runners (mean age 40.9 ±â€¯6.6 yrs, median 9 yrs of running with frequent competitions) and 10 matched controls not engaged in any regular activities. All of them underwent cardiovascular magnetic resonance (CMR) with 3 T scanner including T1-mapping, late gadolinium enhancement (LGE) and extracellular volume (ECV) quantification. RESULTS: Athletes demonstrated significantly larger heart chambers and left ventricular (LV) mass. LV systolic function was unchanged. 73.3% of athletes fulfilled volumetric criteria for dilated cardiomyopathy or arrhythmogenic right ventricular cardiomyopathy. Non-ischemic, small volume LGE was found in 8 athletes and in 1 control (27% vs. 10%, p = 0.40). It was localised at insertion points (5 athletes, 1 control) or in the septum or infero-lateral wall (3 athletes). Athletes with insertion point LGE had higher right ventricular end-diastolic volume index in comparison to athletes without LGE (p = 0.04), which suggests its relation to volume overload. There were no differences between athletes and non-athletes in terms of ECV values (26.1% vs. 25%, p = 0.29). CONCLUSIONS: Ultra-marathon runner's hearts demonstrate a high degree of structural remodelling, but there is no significant increase in focal or diffuse myocardial fibrosis.

Late diagnosis of total anomalous pulmonary venous drainage in a 5.5-month-old infant.

A 5.5-month-old girl was admitted with non-specific signs and symptoms like dyspnea at rest, tachypnea, fatigue, low body weight and cyanosis on exertion. Physical examination revealed a barrel-shaped chest; no pathological sounds over the heart or both lung areas were detected. The above mentioned symptoms might suggest a circulatory problem. Echocardiography and computed tomography angiography were performed. These examinations revealed supracardiac type total anomalous pulmonary venous drainage. Echocardiographic signs of pulmonary hypertension and severe right ventricle overload were detected. Detailed analysis of both imaging examinations revealed atypical obstruction of the pulmonary venous return: narrowing of the proximal part of superior vena cava. An urgent surgery was performed, with no complications in the postoperative period. A follow-up echocardiography showed normalization of cardiac function and pulmonary pressure as well as normalization of flow profile within the superior vena cava. The paper presents a non-invasive diagnostic process in the described case, and discusses the causes of late diagnosis.A 5.5-month-old girl was admitted with non-specific signs and symptoms like dyspnea at rest, tachypnea, fatigue, low body weight and cyanosis on exertion. Physical examination revealed a barrel-shaped chest; no pathological sounds over the heart or both lung areas were detected. The above mentioned symptoms might suggest a circulatory problem. Echocardiography and computed tomography angiography were performed. These examinations revealed supracardiac type total anomalous pulmonary venous drainage. Echocardiographic signs of pulmonary hypertension and severe right ventricle overload were detected. Detailed analysis of both imaging examinations revealed atypical obstruction of the pulmonary venous return: narrowing of the proximal part of superior vena cava. An urgent surgery was performed, with no complications in the postoperative period. A follow-up echocardiography showed normalization of cardiac function and pulmonary pressure as well as normalization of flow profile within the superior vena cava. The paper presents a non-invasive diagnostic process in the described case, and discusses the causes of late diagnosis.

Nijmegen Breakage Syndrome Complicated With Primary Pulmonary Granulomas.

Nijmegen breakage syndrome (NBS) is a rare autosomal recessive disease characterized by microcephaly, growth retardation, severe immunodeficiency, and predisposition to lymphoid malignancy. In this report, we describe a case of a 9-year-old boy, previously diagnosed with NBS and symptoms of dyspnea, dry cough, and fever. Despite initial recognition of pneumonia, there was no response to broad spectrum antimicrobial treatment, negative results from microbiological tests, and unclear changes in lung imaging were observed. Therefore, further diagnostics were focused on suspected lymphoid malignancy and involved lung biopsy. Unexpectedly, histopathological examination revealed noncaseating granulomas. The introduction of systemic steroids resulted in significant improvement of the patient's clinical condition. This is the first description of primary pulmonary noncaseating granulomas without nodular involvement in a child with NBS.

Cardiac Magnetic Resonance Assessment of the Structural and Functional Cardiac Adaptations to Soccer Training in School-Aged Male Children.

Physical training is associated with changes in cardiac morphology called the "athlete's heart", which has not been sufficiently studied in children. The aim of the study was to analyze cardiac adaptation to exercise in pre-adolescent soccer players. Thirty-six soccer players (mean age 10.1 ± 1.4 years) and 24 non-athlete male controls (10.4 ± 1.7 years) underwent cardiac magnetic resonance. Measurements of myocardial mass, end-diastolic and end-systolic volume, stroke volume and ejection fraction for left and right ventricle (LV, RV) were performed. Additionally, left and right atrial (LA, RA) areas and volumes were analysed. Relative wall thickness (RWT) was calculated to describe the pattern of cardiac remodeling. Interventricular wall thickness and LV mass were significantly higher in athletes, but remained within the reference (6.9 ± 0.8 vs. 6.2 ± 0.9 mm/√m2, p = 0.003 and 57.1 ± 7.4 vs. 50.0 ± 7.1 g/m2, p = 0.0006, respectively) with no changes in LV size and function between groups. The RWT tended to be higher among athletes (p = 0.09) indicating LV concentric remodeling geometry. Soccer players had significantly larger RV size (p < 0.04) with similar function and mass. Also, the LA volume (p = 0.01), LA area (p = 0.03) and LA diameter (p = 0.009) were significantly greater in players than in controls. Cardiac adaptations in pre-adolescent soccer players are characterized by an increased LV mass without any changes in LV size and systolic function, which is typical of resistance training with tendency to concentric remodeling. This is accompanied by increase of LA and RV size. It should be taken into account during annual pre-participation evaluation.

Postnatal Evaluation of Congenital Chest Pathologies Using a Low-Dose Computed Tomography (CT) Protocol - a Pictorial Review.

Computed tomography with its excellent spatial and temporal resolution remains a valuable diagnostic modality in pediatrics. On the other hand an increasing attention has been placed on the radiation risks associated with CT imaging, especially in children. In recent years, many advances in CT hardware and software, for example, automatic exposure control tools and iterative reconstruction techniques, have allowed for a reduction of applied radiation doses while maintaining image quality. The purpose of this paper is to present our protocol for chest CT imaging in the youngest age group, together with a pictorial review of congenital pathologies of the chest, and to emphasize factors that optimize postnatal CT imaging in infants. In our opinion, modern CT imaging with the use of dose reduction techniques and iterative reconstructions allows for a proper visualization of chest pathologies in small children, which has no influence on observer performance. The presented review of low-dose diagnostic images of a wide spectrum of congenital pathologies may serve as an example of routine utilization of the current concepts in pediatric CT optimization.

Laparoscopic splenectomy for hereditary spherocytosis-preliminary report.

Splenectomy is considered standard surgical therapy in hereditary spherocytosis. The procedure is indicated in patients with severe anemia, recurrent hemolytic, and aplastic crises. The aim of the study was to assess treatment outcomes in patients with hereditary spherocytosis who underwent total or partial laparoscopic splenectomy. Fifteen patients aged 4-17 yr underwent laparoscopic splenectomy from 2009 to 2012. Partial and total splenectomies were performed (five and 10 children, respectively). Hematologic parameters, liver function tests, and splenic volume before and after the surgery were analyzed retrospectively. Total follow-up was 1-30 months. Hospitalization and operating time were similar in both groups. In partial splenectomy group, branches of splenic arteries gave better blood supply than short gastric vessels. In both groups, hematologic parameters were improved. Postoperative markedly elevated platelet count was maintained up to 6 months, and after that, platelet count gradually decreased to normal values. Bilirubin level was decreased in early postoperative period; however, it increased later to achieve levels lower than in preoperative period. No severe general infections were observed in both groups. Laboratory parameters (hemoglobin and bilirubin concentrations and RBC) after the surgery improved in all patients, and the effect was maintained during 12 months of follow-up. Platelet count increased significantly after the surgery and was maintained at high levels during the next 6 months. However, it returned to preoperative levels within a year after the surgery. Our study showed that partial splenectomy was not inferior to total splenectomy. However, full assessment requires longer follow-up and larger group of patients.

Atypical imaging features of adrenal gland lesions in children - report of three cases and review of literature.

BACKGROUND: The differential diagnosis of adrenal pathology depends on the child's age and imaging findings. CASE REPORT: Three children without clinical symptoms of neoplasm, with an adrenal lesion discovered on diagnostic ultrasound imaging. Laboratory tests for neoplasm were negative. The final diagnosis was based on histopathological examinations after surgical resection. CONCLUSIONS: 1. The value of diagnostic imaging and laboratory tests in differential diagnosis of adrenal gland lesions is limited. 2. Malignant tumors of adrenal glands should be taken into account in children. 3. Surgical resection should be considered in diagnostic algorithm of adrenal gland masses. 4. The final diagnosis is always based on histopathological examination.

Abscess mimicking lung metastasis in a 10-year-old boy - case report.

BACKGROUND: Malignant pulmonary tumours in children are very rare; the majority are metastases. Nonspecific radiographic findings of these abnormalities are challenging and may delay the final diagnosis and treatment. CASE REPORT: A 10-year-old boy was admitted to our hospital because of the clinical and radiographic symptoms and signs of pneumonia with abscess formation in the left lower lobe. After initial improvement on antibiotic therapy, a significant deterioration of the patient's condition was observed, together with progression in radiographic examinations. The patient was treated surgically and transferred to the Haematology and Oncology Department with a final diagnosis of pulmonary metastasis of clear cell sarcoma. CONCLUSIONS: Radiographic findings of metastatic diseases may mimic non-neoplastic pulmonary conditions. A lack of specific clinical symptoms and a confusing radiographic pattern in our patient with clear cell sarcoma lung metastasis caused serious diagnostic difficulties.

An unusual oesophageal foreign body in neonate - case report.

BACKGROUND: Foreign bodies in the esophagus in children are usually swallowed accidentally during play. An oesophageal foreign body in neonate is rare and thus the diagnostic work-up may be difficult. CASE REPORT: We present a case of an 8-day-old baby with symptoms of oesophageal obstruction. An unusual foreign body was found. CONCLUSIONS: Foreign bodies in neonate's esophagus are rare; non-accidental action should be suspected. Atypical clinical and radiological symptoms may cause diagnostic difficulties.