Validation of two echocardiographic indexes to improve the diagnosis of complex coarctations

Objectives: Coarctation of the aorta is one of the most common congenital heart defects. Its diagnosis may be difficult in the presence of a patent ductus arteriosus, of other complex defects or of a poor echocardiographic window. We sought to demonstrate that the carotid-subclavian artery index (CSA index) and the isthmus-descending aorta ratio (I/D ratio), two recently described echocardiographic indexes, are effective in detection of isolated and complex aortic coarctations in children younger and older than 3 months of age. The CSA index is the ratio of the distal aortic arch diameter to the distance between the left carotid artery and the left subclavian artery. It is highly suggestive of a coarctation when it is ≪1.5. The I/D ratio defined as the diameter of the isthmus to the diameter of the descending aorta, suggests an aortic coarctation when it is less than 0.64. Methods: This is a retrospective cohort study in a tertiary care children's hospital. Review of all echocardiograms in children aged 0-18 years with a diagnosis of coarctation seen at the author's institution between 1996 and 2006. An age- and sex-matched control group without coarctation was constituted. Offline echocardiographic measurements of the aortic arch were performed in order to calculate the CSA index and I/D ratio. Results: Sixty-eight patients were included in the coarctation group, 24 in the control group. Patients with coarctation had a significantly lower CSA index (0.84±0.39 vs 2.65±0.82, p≪0.0001) and I/D ratio (0.58±0.18 vs 0.98±0.19, p≪0.0001) than patients in the control group. Associated cardiac defects and age of the child did not significantly alter the CSA index or the I/D ratio. Conclusions: A CSA index less than 1.5 is highly suggestive of coarctation independent of age and of the presence of other cardiac defects. I/D ratio alone is less specific than CSA alone at any age and for any associated cardiac lesion. The association of both indexes improves sensitivity and permits diagnosis of coarctation in all patients based solely on a bedside echocardiographic measuremen

Brugada syndrome in childhood: a potential fatal arrhythmia not always recognised by paediatricians. A case report and review of the literature

We report on a youngster followed by his paediatrician from birth until 14years of age for premature beats, most likely of ventricular origin. The sudden death of his sister provoked a re-assessment of his electrocardiograms (ECG), resulting in the diagnosis of Brugada syndrome and the subsequent implantation of a cardioverter defibrillator. This syndrome is a well known entity in adult cardiology, first described by Brugada and Brugada in 1992. It is considered to be the second most common cause of death in young adults after road traffic accidents. In children, however, the Brugada syndrome is not well known and we believe to be certainly underdiagnose

A noninvasive diagnostic tool to differentiate myocarditis from myocardial infarction: late gadolinium enhanced cardiac magnetic resonance

Studies in adults have shown that late gadolinium enhanced cardiac magnetic resonance is a safe and noninvasive diagnostic tool which allows one to differentiate myocardial infarction from myocarditis. We believe that it may also be highly useful in the paediatric population for the same purpos

Pulmonary artery banding: long-term telemetric adjustment

Objective: Adjustment of pulmonary blood flow is difficult in pulmonary artery banding for complex congenital heart defects. A new wireless, battery free, telemetrically controlled, implantable device (FloWatch®, EndoArt, S.A., Lausanne, Switzerland) allowing for progressive occlusion/reopening of the device through a remote control at the wanted percentage of occlusion (adjustable pulmonary artery banding) underwent experimental evaluation. Methods: Eleven mini-pigs underwent FloWatch® implantation around the main pulmonary artery through left thoracotomy. The first group (n=4), mean age 18.2±0.1 weeks, mean body weight 12.0±0.1 kg, underwent FloWatch® implantation as device tolerance test. The second group (n=7), mean age 8.6±3.4 weeks, mean body weight 5.1±1.5 kg, underwent functional evaluation: at implantation, 1, 3, 5, 8 and 10 weeks after implantation, the device was progressively occluded and reopened, with Doppler evaluation of the developed pressure gradient. Results: The four mini-pigs of first group were sacrificed at mean age of 42.3±0.1 weeks, mean body weight 25.1±3.2 kg (mean interval of 24 weeks after implantation); the device was still functioning and histology revealed almost normal morphology of the pulmonary artery. In all seven mini-pigs of second group the possibility of narrowing/releasing the pulmonary artery was confirmed at implantation and during follow-up: at last control their mean age was 20.5±2.8 weeks and the body weight 12.7±3.7 kg. Conclusions: Complete adjustment of pulmonary blood flow is now possible with an implantable device allowing for pulmonary artery banding with early and late telemetric flow contro

The role of exercise testing in congenital heart disease patients after coronary artery reimplantation

Introduction: The Ross procedure requires like the arterial switch operation (ASO) coronary artery reimplantation. There is no information on coronary artery stenosis at the site of the reimplantation in Ross patients as has been described for ASO patients. This study assessed therefore the prevalence of positive exercise tests (pETs) suggesting myocardial ischemia in Ross and ASO patients in comparison to tetralogy of Fallot patients (TOF), the latter not undergoing coronary artery reimplantation. The study evaluated further, whether a coronary artery complication was confirmed by a supplementary non- and/or invasive evaluation. Methods: Results of exercise tests, echocardiography, coronary computed tomography (CCT) and coronary angiography (CA) were retrospectively reviewed in 30 Ross, 14 ASO and 30 TOF patients. Results: Exercise capacity did not differ between groups. There was a trend to more frequent pETs in Ross patients (Ross vs. ASO vs. TOF, N, %): 8 (27) vs. 1 (7) vs. 2 (7) p = 0.062. CCT was performed in 30% Ross, 21% ASO, 3% TOF (p = 0.023); CA in 20% Ross, 21% ASO and 13% TOF patients (p = 0.727). None of these investigations revealed a coronary artery stenosis. Patients with pET showed a higher right ventricular outflow tract (RVOT) gradient (44 ± 30 vs. 22 ± 13 mmHg, p = 0.004) and more frequently a severe RVOT stenosis (27 vs. 0%, p < 0.006). Conclusions: pETs are rather related to RVOT stenosis than to coronary artery complications after Ross procedure, ASO and TOF repair. The role of exercise testing for detection of myocardial ischemia is limited and should be restricted to evaluation of exercise capacity

Interventions to Decrease Carotid-Intima Media Thickness in Children and Adolescents With Type 1 Diabetes: A Systematic Review and Meta-Analysis.

INTRODUCTION Hyperglycemia is associated with a higher cardiovascular risk, as evidenced by increased carotid-intima media thickness (CIMT) in youth with diabetes. We conducted a systematic review and meta-analysis to assess the effect of pharmacological or non-pharmacological interventions on CIMT in children and adolescents with prediabetes or diabetes. METHODS We conducted systematic searches of MEDLINE, EMBASE, and CENTRAL, together with supplementary searches in trial registers and other sources for studies completed up to September 2019. Interventional studies assessing ultrasound CIMT in children and adolescents with prediabetes or diabetes were considered for inclusion. Where appropriate, data were pooled across studies using random-effect meta-analysis. Quality was assessed using The Cochrane Collaboration's risk-of-bias tool and a CIMT reliability tool. RESULTS Six studies involving 644 children with type 1 diabetes mellitus were included. No study involved children with prediabetes or type 2 diabetes. Three randomized controlled trials (RCTs) evaluated the effects of metformin, quinapril, and atorvastatin. Three non-randomized studies, with a before-and-after design, evaluated the effects of physical exercise and continuous subcutaneous insulin infusion (CSII). The mean CIMT at baseline ranged from 0.40 to 0.51 mm. The pooled difference in CIMT was -0.01 mm (95% CI: -0.04 to 0.01) for metformin compared to placebo (2 studies; 135 participants; I2: 0%). The difference in CIMT was -0.01 mm (95% CI: -0.03 to 0.01) for quinapril compared to placebo (1 study; 406 participants). The mean change from baseline in CIMT was -0.03 mm (95% CI: -0.14 to 0.08) after physical exercise (1 study; 7 participants). Inconsistent results were reported for CSII or for atorvastatin. CIMT measurement was rated at a higher quality on all reliability domains in 3 (50%) studies. The confidence in results is limited by the low number of RCTs and their small sample sizes, as well as the high risk of bias in before-and-after studies. CONCLUSIONS Some pharmacological interventions may decrease CIMT in children with type 1 diabetes. However, there is great uncertainty with respect to their effects and no strong conclusions can be drawn. Further evidence from larger RCTs is required. SYSTEMATIC REVIEW REGISTRATION PROSPERO, CRD42017075169

Complicated Postoperative Course after Pulmonary Artery Sling Repair and Slide Tracheoplasty.

Pulmonary artery sling (PAS) is a rare congenital condition in which the left pulmonary artery (LPA) arises from the right pulmonary artery, and then passes between the trachea and the esophagus to reach the left lung, thereby forming a sling around the airway. It is often associated with intrinsic tracheal stenosis due to complete cartilaginous rings. Therapeutic management nowadays consists of one-stage reimplantation of the LPA and tracheoplasty with cardiopulmonary bypass support. Here, we present a 7-week-old boy with PAS and long-segment tracheal stenosis (LSTS) who underwent surgical intervention consisting of reimplantation of the LPA and slide tracheoplasty. Multiple respiratory and cardiovascular complications marked the postoperative course. They consisted of recurrent failed attempts in weaning off mechanical ventilation due to bronchomalacia, left vocal cord paralysis, development of granulation tissue at the anastomosis and restenosis of the trachea, and the main stem bronchi requiring balloon dilatation. The patient also developed bilateral pulmonary artery thrombosis and stenosis of the LPA. After a prolonged hospitalization, the patient is doing well without any respiratory symptoms and has a good result on follow-up bronchoscopy 1 year after the initial surgery. The stenosis of the LPA responded well to percutaneous balloon dilatation 12 months after the primary surgery. The case illustrates that even though surgical techniques are improving and are in general associated with a low morbidity and mortality, management of PAS and tracheal stenosis can still be challenging. However, good long-term outcome can be achieved if the initial postoperative phase is overcome

Consequences of gestational diabetes mellitus on neonatal cardiovascular health: MySweetHeart Cohort study.

BACKGROUND Hyperglycaemic disorders of pregnancy are associated with offspring cardiovascular alterations. METHODS MySweetHeart cohort study aimed to assess the effect of maternal gestational diabetes (GDM) on offsprings' cardiovascular health. Newborns underwent clinical and echocardiographic examinations between 2016 and 2020. RESULTS Compared to mothers without GDM (n = 141), mothers with GDM (n = 123) were more likely to have had GDM in previous pregnancies and had higher weight, BMI, blood glucose, and HbA1c. Newborns of both groups showed similar clinical characteristics. Echocardiography was performed on the 3rd (interquartile range, IQR, 2nd-4th) day of life in 101 offsprings of mothers without and 116 offsprings of mothers with GDM. Left ventricular (LV) mass was similar. Children born to mothers with GDM had a thicker posterior LV wall (z-score +0.15, IQR -0.38/0.62, versus +0.47, IQR -0.11/+1.1, p = 0.004), a smaller end-systolic (1.3 mL, IQR 1.0-1.5 mL, versus 1.4 mL, IQR 1.2-1.8 mL, p = 0.044) but a similar end-diastolic LV volume. They also had shorter tricuspid valve flow duration and aortic valve ejection time, lower tricuspid E-wave and pulmonary valve velocities. CONCLUSIONS Newborns of mothers with or without GDM had similar clinical characteristics and LV mass. However, some echocardiographic differences were detected, suggesting an altered myocardial physiology among infants of mothers with GDM. REGISTRATION ClinicalTrials.gov (NCT02872974). IMPACT Hyperglycaemic disorders of pregnancy are known to be associated with offspring cardiovascular alterations. Clinical characteristics and estimated left ventricular (LV) mass were similar in children issued from mothers with and without gestational diabetes (GDM). Children born to mothers with GDM had a thicker posterior LV wall and a smaller end-systolic LV volume. Although LV mass is not different, myocardial physiology may be altered in these infants. Further studies should investigate the endothelial function of this population and the cardiovascular evolution of these children over time

MR and CT imaging of pulmonary valved conduits in children and adolescents: normal appearance and complications

Background: The Contegra® is a conduit made from the bovine jugular vein and then interposed between the right ventricle and the pulmonary artery. It is used for cardiac malformations in the reconstruction of right ventricular outflow tract. Objective: To describe both normal and pathological appearances of the Contegra® in radiological imaging, to describe imaging of complications and to define the role of CT and MRI in postoperative follow-up. Materials and methods: Forty-three examinations of 24 patients (17 boys and 7 girls; mean age: 10.8years old) with Contegra® conduits were reviewed. Anatomical description and measurements of the conduits were performed. Pathological items examined included stenosis, dilatation, plicature or twist, thrombus or vegetations, calcifications and valvular regurgitation. Findings were correlated to the echographic gradient through the conduit when available. Results: CT and MR work-up showed Contegra® stenosis (n = 12), dilatation (n = 9) and plicature or twist (n = 7). CT displayed thrombus or vegetations in the Contegra® in three clinically infected patients. Calcifications of the conduit were present at CT in 12 patients and valvular regurgitation in three patients. The comparison between CT and/or MR results showed a good correlation between the echographic gradient and the presence of stenosis in the Contegra®. Conclusion: CT and MR bring additional information about permeability and postoperative anatomy especially when echocardiography is inconclusive. Both techniques depict the normal appearance of the conduit, and allow comparison and precise evaluation of changes in the postoperative follow-up