The role of posterior aortopexy in the treatment of left mainstem bronchus compression

OBJECTIVES: We reviewed the role of posterior aortopexy for left mainstem bronchus compression in infants and children. METHODS: Eighteen children with respiratory symptoms were enrolled between 2005 and 2015 for surgical decompression of the left mainstem bronchus. The children were managed from diagnosis to follow-up by a dedicated tracheal team. Primary outcomes were the complete relief of symptoms or improvement with respect to preoperative clinical status. RESULTS: The median age was 4 years (0.3-15.4) and the median weight was 13.2 kg (3, 1-40). Symptoms or indications for bronchoscopy included difficult weaning from mechanical ventilation (n = 3, 17%), difficult weaning from tracheotomy (n = 4, 22%), recurrent pneumonia (n = 4, 22%), wheezing (n = 3, 17%), atelectasis (n = 1, 5.5%), bitonal cough (n = 1, 5.5%) and stridor (n = 2, 11%). Associated malformations were present in 88.7%. The diagnosis was made by bronchoscopy and computed tomography. Indication for surgery was the presence of pulsations and reduction in the diameter of the left mainstem bronchus compression of more than 70%. Surgery was performed by left posterolateral thoracotomy. Aortopexy was done under bronchoscopic control. No early or late deaths were observed, nor were reoperations necessary. Residual malacia was observed in 8 children (44%). Median follow-up was 4.1 years (0.1-7.1). At last follow-up, 17/18 (94.4%) children showed adequate airway patency. CONCLUSIONS: The intrathoracic location of the left mainstem bronchus predisposes it to compression. Vascular anomalies represent the most frequent causes. Aortopexy has been advocated as a safe and useful method to relieve the compression, and our results confirmed these findings. Management of these patients is challenging and requires a multidisciplinary team

Echocardiographic Screening of Anomalous Origin of Coronary Arteries in Athletes with a Focus on High Take-Off

Anomalous aortic origin of coronary arteries (AAOCA) represents a rare congenital heart disease. However, this disease is the second most common cause of sudden cardiac death in apparently healthy athletes. The aim of this systematic review is to analyze the feasibility and the detection rate of AAOCA by echocardiography in children and adults. A literature search was performed within the National Library of Medicine using the following keywords: coronary artery origin anomalies and echocardiography; then, the search was redefined by adding the keywords: athletes, children, and high take-off. Nine echocardiographic studies investigating AAOCA and a total of 33,592 children and adults (age range: 12-49 years) were included in this review. Of these, 6599 were athletes (12-49 years). All studies demonstrated a high feasibility and accuracy of echocardiography for the evaluation of coronary arteries origin as well as their proximal tracts. However, some limitations exist: the incidence of AAOCA varied from 0.09% to 0.39% (up to 0.76%) and was lower than described in computed tomography series (0.3-1.8%). Furthermore, echocardiographic views for the evaluation of AAOCA and the definition of minor defects (e.g., high take-off coronary arteries) have not been standardized. An echocardiographic protocol to diagnose the high take-off of coronary arteries is proposed in this article. In conclusion, the screening of AAOCA by echocardiography is feasible and accurate when appropriate examinations are performed; however, specific acoustic windows and definitions of defects other than AAOCA need to be standardized to improve sensitivity and specificity

Tele-echocardiography between Italy and Balkan area

A project (PIS-SRCE) has been started for promoting international medical cooperation in the Balkan area according to the Stabilization and Association Process, the European Union\u27s policy framework for the Western Balkan countries. Information and communication technology is presently mature to set up a telemedicine network breaking down geographical barriers and providing specialized medical care virtually anywhere in the world. Videoconferencing equipment is commercially available to transmit securely over Internet echocardiography or other modality images in addition to standard audio/video signals. Real-time transmission capability is crucial for allowing specialists to drive remotely proper echo scanning of cardiac structures in patient or foetus with suspected congenital heart disease

Echocardiographic nomograms for chamber diameters and areas in Caucasian children

BACKGROUND: Although a quantitative evaluation of cardiac chamber dimensions in pediatric echocardiography is often important, nomograms for these structures are limited. The aim of this study was to establish reliable echocardiographic nomograms of cardiac chamber diameters and areas in a wide population of children. METHODS: A total of 1,091 Caucasian Italian healthy children (age range, 0 days to 17 years; 44.8% female) with body surface areas (BSAs) ranging from 0.12 to 1.8 m(2) were prospectively enrolled. Twenty-two two-dimensional and M-mode measurements of atrial and ventricular chamber diameters and areas were performed. Models using linear, logarithmic, exponential, and square-root relationships were tested. Heteroscedasticity was tested by the White test and the Breusch-Pagan test. Age, weight, height, and BSA, calculated by the Haycock formula, were used as the independent variables in different analyses to predict the mean value of each echocardiographic measurement. The influence of various confounders, including gender, type of delivery, prematurity, and interobserver variability, was also evaluated. Structured Z scores were then computed. RESULTS: The Haycock formula provided the best fit and was used when presenting data as predicted values (mean ? 2 SDs) for a given BSA and within equations relating echocardiographic measurements to BSA. Confounders were not included in the final models, because they did not show significant effects for most of the measurements. CONCLUSIONS: Echocardiographic reference values are presented for chamber area and diameters, derived from a large population of healthy children. These data partly cover a gap in actual pediatric echocardiographic nomograms. Further studies are required to reinforce these data, as well as to evaluate other parameters and ethnicities

Psychopathological Impact in Patients with History of Rheumatic Fever with or without Sydenham's Chorea: A Multicenter Prospective Study

Sydenham's chorea (SC) is a post-streptococcal autoimmune disorder of the central nervous system, and it is a major criterium for the diagnosis of acute rheumatic fever (ARF). SC typically improves in 12-15 weeks, but patients can be affected for years by persistence and recurrencies of both neurological and neuropsychiatric symptoms. We enrolled 48 patients with a previous diagnosis of ARF, with or without SC, in a national multicenter prospective study, to evaluate the presence of neuropsychiatric symptoms several years after SC's onset. Our population was divided in a SC group (n = 21), consisting of patients who had SC, and a nSC group (n = 27), consisting of patients who had ARF without SC. Both groups were evaluated by the administration of 8 different neuropsychiatric tests. The Work and Social Adjustment Scale (WSAS) showed significantly (p = 0.021) higher alterations in the SC group than in the nSC group. Furthermore, 60.4% (n = 29) of the overall population experienced neuropsychiatric symptoms other than choreic movements at diagnosis and this finding was significantly more common (p = 0.00) in SC patients (95.2%) than in nSC patients (33.3%). The other neuropsychiatric tests also produced significant results, indicating that SC can exert a strong psychopathological impact on patients even years after its onset

Right Ventricular Outflow Tract Stenting as Palliation of Critical Tetralogy of Fallot: Techniques and Results

Background. Despite current trends toward early primary repair, the surgical systemic-to-pulmonary shunt is still considered the first-choice palliation in patients with critical tetralogy of Fallot (TOF) and duct-dependent pulmonary circulation unsuitable for primary repair. However, stenting of the right ventricular outflow tract (RVOT) is nowadays emerging as an effective alternative to surgical palliation in selected patients. Methods and results. RVOT stenting is usually performed from a venous route, either femoral or, in selected cases, the right internal jugular vein. Less frequently, mostly in pulmonary infundibular/valvar atresia, this procedure can be performed using a hybrid surgical/interventional approach by surgical exposure of the RVOT, puncture of the atretic valve, and stent deployment under direct vision. The size and type of the most appropriate stent may be chosen, based on ultrasound measurements of the RVOT, to cover the right ventricular infundibulum completely and, at the same time, sparing the pulmonary valve, unless significant pulmonary valve annulus hypoplasia and/or supra-valvular stenosis is a significant component of the obstruction. In the large series so far published, early mortality of RVOT stenting is less than 2%, comparing favourably with either Blalock-Thomas-Taussig shunt or early primary repair. In addition, morbidity and clinical sequelae of this approach do not significantly differ from surgical palliation, even if RVOT stenting shows lesser durability and a higher rate of trans-catheter re-interventions over a mid-term follow-up. Finally, similar but more balanced pulmonary artery growth than surgical palliation following RVOT stenting is reported over a mid-term follow-up. Conclusions. RVOT stenting is a technically feasible, well-tolerated, and effective palliation in critical TOF. This approach is cost-effective with respect to surgical palliation either in high-risk neonates or whenever a short-term pulmonary blood flow source is anticipated due to the early surgical repair. It effectively increases pulmonary blood flow, improves arterial saturation, and promotes balanced pulmonary artery growth over a mid-term follow-up

Overview of Lung Ultrasound in Pediatric Cardiology

Lung ultrasound (LUS) is increasing in its popularity for the diagnosis of pulmonary complications in acute pediatric care settings. Despite the high incidence of pulmonary complications for patients with pediatric cardiovascular and congenital heart disease, especially in children undergoing cardiac surgery, the use of LUS remains quite limited in these patients. The aim of this review is to provide a comprehensive overview and list of current potential applications for LUS in children with congenital heart disease, post-surgery. We herein describe protocols for LUS examinations in children, discuss diagnostic criteria, and introduce methods for the diagnosis and classification of pulmonary disease commonly encountered in pediatric cardiology (e.g., pleural effusion, atelectasis, interstitial edema, pneumothorax, pneumonia, and diaphragmatic motion analysis). Furthermore, applications of chest ultrasounds for the evaluation of the retrosternal area, and in particular, systematic search criteria for retrosternal clots, are illustrated. We also discussed the potential applications of LUS, including the guidance of interventional procedures, namely lung recruitment and drainage insertion. Lastly, we analyzed current gaps in knowledge, including the difficulty of the quantification of pleural effusion and atelectasis, and the need to differentiate different etiologies of B-lines. We concluded with future applications of LUS, including strain analysis and advanced analysis of diaphragmatic mechanics. In summary, US is an easy, accurate, fast, cheap, and radiation-free tool for the diagnosis and follow-up of major pulmonary complications in pediatric cardiac surgery, and we strongly encourage its use in routine practice

Echocardiographic Screening of Anomalous Origin of Coronary Arteries in Athletes with a Focus on High Take-Off

Anomalous aortic origin of coronary arteries (AAOCA) represents a rare congenital heart disease. However, this disease is the second most common cause of sudden cardiac death in apparently healthy athletes. The aim of this systematic review is to analyze the feasibility and the detection rate of AAOCA by echocardiography in children and adults. A literature search was performed within the National Library of Medicine using the following keywords: coronary artery origin anomalies and echocardiography; then, the search was redefined by adding the keywords: athletes, children, and high take-off. Nine echocardiographic studies investigating AAOCA and a total of 33,592 children and adults (age range: 12–49 years) were included in this review. Of these, 6599 were athletes (12–49 years). All studies demonstrated a high feasibility and accuracy of echocardiography for the evaluation of coronary arteries origin as well as their proximal tracts. However, some limitations exist: the incidence of AAOCA varied from 0.09% to 0.39% (up to 0.76%) and was lower than described in computed tomography series (0.3–1.8%). Furthermore, echocardiographic views for the evaluation of AAOCA and the definition of “minor” defects (e.g., high take-off coronary arteries) have not been standardized. An echocardiographic protocol to diagnose the high take-off of coronary arteries is proposed in this article. In conclusion, the screening of AAOCA by echocardiography is feasible and accurate when appropriate examinations are performed; however, specific acoustic windows and definitions of defects other than AAOCA need to be standardized to improve sensitivity and specificity

Quality of Life of Families with Children Presenting Congenital Heart Disease:Longitudinal Study Protocol

BACKGROUND: Advances in medicine have caused a notable increase in the survival rates of children born with congenital heart disease, even in the most complicated cases, almost mitigating the disease’s pathology from lethal to chronic. The quality of life perceived by such children is influenced by the perceptions of their parents. However, the international literature has rarely considered the entire family nucleus. AIMS: This study aims to study the temporal trend of quality of life of families with children with congenital heart disease, particularly with respect to parents following a child’s hospitalization for an invasive procedure. DESIGN: A longitudinal study. METHOD: A sample of families (that is, those including a child with congenital heart disease and their parents) will be enrolled following the patient’s discharge from the hospital and examined every 3 months for 1 year. The study’s adopted hypothesis is that there is an interdependence between the subjects of the study that is capable of influencing individual perceptions of quality of life. RESULTS: This study will attempt to identify variables (and their temporal trend) that can be attributed to the family unit and—together with physical and clinical variables—that may influence the quality of life of children with congenital heart disease. CONCLUSION: Examining family quality of life with the longitudinal method will allow us to identify the predictors and interdependence of this factor with respect to children and their parents. This will help to correct and elaborate upon care guidelines, providing better assistance to patients and their caregivers