[Sudden cardiac arrest in children and adolescents: diagnosis, clinical presentation and peculiarities]. / L'arresto cardiaco in età pediatrica: diagnosi, presentazione clinica e peculiarità.

Sudden cardiac arrest/death in pediatric patients is a rare but potentially preventable event. Cardiomyopathies and channelopathies are the most common causes which are detectable with ECG and transthoracic echocardiography in asymptomatic subjects. Coronary artery anomalies are a rare cause of sudden cardiac arrest/death, but these events suggest that ECG and echocardiography, focused on the site of origin of the coronary arteries, should be both part of the screening tool of young athletes. Finally, the rare cardiac arrest events in young patients with ventricular preexcitation without prior symptoms or markers of high risk suggest that transcatheter ablation should be considered in all pediatric patients with ventricular preexcitation because it can eliminate the small long-term risk of sudden cardiac arrest/death, but a careful consideration of the most appropriate timing is mandatory.

Performance and Failure of Right Ventricle to Pulmonary Artery Conduit in Congenital Heart Disease.

Surgical implantation of a right ventricle to pulmonary artery (RV-PA) conduit is an important component of congenital heart disease (CHD) surgery, but with limited durability, leading to re-intervention. The present single-center, retrospective, cohort study reports the results of surgically implanted RV-PA conduits in a consecutive series of children and adults with CHD. Patients with CHD referred for RV-PA conduit surgical implantation (from October 1997 to January 2022) were included. The primary outcome was conduit failure, defined as a peak gradient above 64 mm Hg, severe regurgitation, or the need for conduit-related interventions. Longitudinal echocardiographic studies were available for mixed-effects linear regression analysis. A total of 252 patients were initially included; 149 patients were eligible for follow-up data collection. After a median follow-up time of 49 months, the primary study end point occurred in 44 (29%) patients. A multivariable Cox regression model identified adult age (>18 years) at implantation and pulmonary homograft implantation as protective factors (hazard ratio 0.11, 95% confidence interval [CI] 0.02 to 0.47 and hazard ratio 0.34, 95% CI 0.16 to 0.74, respectively). Fever within 7 days of surgical conduit implantation was a risk factor for early (within 24 months) failure (odds ratio 4.29, 95% CI 1.41 to 13.01). Long-term use of oral anticoagulants was independently associated with slower progression of peak echocardiographic gradient across the conduits (mixed-effects linear regression p = 0.027). In patients with CHD, the rate of failure of surgically implanted RV-PA conduits is higher in children and after nonhomograft conduit implantation. Early fever after surgery is a strong risk factor for early failure. Long-term anticoagulation seems to exert a protective effect.

Enhancing stroke risk prediction in patients with transient ischemic attack: insights from a prospective cohort study implementing fast-track care.

Background and aims: Fast-track care have been proved to reduce the short-term risk of stroke after transient ischemic attack (TIA). We aimed to investigate stroke risk and to characterize short- and long-term stroke predictors in a large cohort of TIA patients undergoing fast-track management. Methods: Prospective study, enrolling consecutive TIA patients admitted to a Northern Italy emergency department from August 2010 to December 2017. All patients underwent fast-track care within 24 h of admission. The primary outcome was defined as the first stroke recurrence at 90 days, 12 and 60 months after TIA. Stroke incidence with 95% confidence interval (CI) at each timepoint was calculated using Poisson regression. Predictors of stroke recurrence were evaluated with Cox regression analysis. The number needed to treat (NNT) of fast-track care in preventing 90-day stroke recurrence in respect to the estimates based on baseline ABCD2 score was also calculated. Results: We enrolled 1,035 patients (54.2% males). Stroke incidence was low throughout the follow-up with rates of 2.2% [95% CI 1.4-3.3%] at 90 days, 2.9% [95% CI 1.9-4.2%] at 12 months and 7.1% [95% CI 5.4-9.0%] at 60 months. Multiple TIA, speech disturbances and presence of ischemic lesion at neuroimaging predicted stroke recurrence at each timepoint. Male sex and increasing age predicted 90-day and 60-month stroke risk, respectively. Hypertension was associated with higher 12-month and 60-month stroke risk. No specific TIA etiology predicted higher stroke risk throughout the follow-up. The NNT for fast-track care in preventing 90-day stroke was 14.5 [95% CI 11.3-20.4] in the overall cohort and 6.8 [95% CI 4.6-13.5] in patients with baseline ABCD2 of 6 to 7. Conclusion: Our findings support the effectiveness of fast-track care in preventing both short- and long-term stroke recurrence after TIA. Particular effort should be made to identify and monitor patients with baseline predictors of higher stroke risk, which may vary according to follow-up duration.

Neurological abnormalities in individuals with Marfan syndrome: results from a genetically confirmed Italian cohort.

BACKGROUND AND AIMS: Neurological abnormalities have been frequently reported in individuals with Marfan Syndrome (MFS). However, available data relies solely on retrospective studies predating current diagnostic criteria. METHODS: Cross-sectional study comprehensively investigating neurological abnormalities within a prospective cohort of adults (≥ 18 years) with genetically confirmed MFS referred to an Italian hub center for heritable connective tissue diseases (Jan. 1st - Nov. 15th, 2021). RESULTS: We included a total of 38 individuals (53% female). The commonest neurological symptom was migraine (58%), usually without aura (73%). Neuropsychological testing was generally unremarkable, whilst anxiety and depression were highly prevalent within our cohort (42% and 34%, respectively). The most frequent brain parenchymal abnormality was the presence of cortico-subcortical hypointense spots on brain MRI T2* Gradient-Echo sequences (39%), which were found only in patients with a prior history of aortic surgery. Migraineurs had a higher frequency of brain vessels tortuosity vs. individuals without migraine (73% vs. 31%; p = 0.027) and showed higher average and maximum tortuosity indexes in both anterior and posterior circulation brain vessels (all p < 0.05). At univariate regression analysis, the presence of brain vessels tortuosity was significantly associated with a higher risk of migraine (OR 5.87, CI 95% 1.42-24.11; p = 0.014). CONCLUSIONS: Our study confirms that neurological abnormalities are frequent in individuals with MFS. While migraine appears to be associated with brain vessels tortuosity, brain parenchymal abnormalities are typical of individuals with a prior history of aortic surgery. Larger prospective studies are needed to understand the relationship between parenchymal abnormalities and long-term cognitive outcomes.

Quantitative measurement of dural ectasia: associations with clinical and genetic characteristics in Marfan syndrome.

PURPOSE: Dural ectasia (DE) may significantly impact Marfan syndrome (MFS) patients' quality of life due to chronic lower back pain, postural headache and urinary disorders. We aimed to evaluate the association of quantitative measurements of DE, and their evolution over time, with demographic, clinical and genetic characteristics in a cohort of MFS patients. METHODS: We retrospectively included 88 consecutive patients (39% females, mean age 37.1 ± 14.2 years) with genetically confirmed MFS who underwent at least one MRI or CT examination of the lumbosacral spine. Vertebral scalloping (VS) and dural sac ratio (DSR) were calculated from L3 to S3. Likely pathogenic or pathogenic FBN1 variants were categorized as either protein-truncating or in-frame. The latter were further classified according to their impact on the cysteine content of fibrillin-1. RESULTS: Higher values of the systemic score (revised Ghent criteria) were associated with greater DSR at lumbar (p < 0.001) and sacral (p = 0.021) levels. Patients with protein-truncating variants exhibited a greater annual increase in lumbar (p = 0.039) and sacral (p = 0.048) DSR. Mutations affecting fibrillin-1 cysteine content were linked to higher VS (p = 0.009) and DSR (p = 0.038) at S1, along with a faster increase in VS (p = 0.032) and DSR (p = 0.001) in the lumbar region. CONCLUSION: Our study shed further light on the relationship between genotype, dural pathology, and the overall clinical spectrum of MFS. The identification of protein-truncating variants and those impacting cysteine content may therefore suggest closer patient monitoring, in order to address potential complications associated with DE.

Vertebral artery dissection caused by atlantoaxial dislocation in a patient with Marfan syndrome.

A small number of case reports have documented a link between atlantoaxial dislocation (AAD) and vertebral artery dissection (VAD), but this association has never been described in patients with hereditary connective tissue disorders. We present a case of an 18-year-old female patient, diagnosed with Marfan syndrome since the age of one, who underwent brain MRA for intracranial aneurysm screening revealing tortuosity of the internal carotid and vertebral arteries as well as atlantoaxial dislocation. Since the patient was asymptomatic, a wait-and-see approach was chosen, but a follow-up MRA after 18 months showed the appearance of a dissecting pseudoaneurysm of the V3 segment of the left vertebral artery. Despite the patient being still asymptomatic, it was decided to proceed with C1-C2 stabilization to prevent further vascular complications. Follow-up imaging showed realignment of the atlantoaxial joint and reduction of the dissecting pseudoaneurysm of the left vertebral artery. In our patient, screening MRA has led to the discovery of asymptomatic arterial and skeletal abnormalities which, if left untreated, might have led to severe cerebrovascular complications. Therefore, AAD correction or close monitoring with MRA should be provided to MFS patients with this craniovertebral junction anomaly, even if asymptomatic.

Apixaban for Prevention of Thromboembolism in Pediatric Heart Disease.

BACKGROUND: Children with heart disease frequently require anticoagulation for thromboprophylaxis. Current standard of care (SOC), vitamin K antagonists or low-molecular-weight heparin, has significant disadvantages. OBJECTIVES: The authors sought to describe safety, pharmacokinetics (PK), pharmacodynamics, and efficacy of apixaban, an oral, direct factor Xa inhibitor, for prevention of thromboembolism in children with congenital or acquired heart disease. METHODS: Phase 2, open-label trial in children (ages, 28 days to <18 years) with heart disease requiring thromboprophylaxis. Randomization 2:1 apixaban or SOC for 1 year with intention-to-treat analysis. PRIMARY ENDPOINT: a composite of adjudicated major or clinically relevant nonmajor bleeding. Secondary endpoints: PK, pharmacodynamics, quality of life, and exploration of efficacy. RESULTS: From 2017 to 2021, 192 participants were randomized, 129 apixaban and 63 SOC. Diagnoses included single ventricle (74%), Kawasaki disease (14%), and other heart disease (12%). One apixaban participant (0.8%) and 3 with SOC (4.8%) had major or clinically relevant nonmajor bleeding (% difference -4.0 [95% CI: -12.8 to 0.8]). Apixaban incidence rate for all bleeding events was nearly twice the rate of SOC (100.0 vs 58.2 per 100 person-years), driven by 12 participants with ≥4 minor bleeding events. No thromboembolic events or deaths occurred in either arm. Apixaban pediatric PK steady-state exposures were consistent with adult levels. CONCLUSIONS: In this pediatric multinational, randomized trial, bleeding and thromboembolism were infrequent on apixaban and SOC. Apixaban PK data correlated well with adult trials that demonstrated efficacy. These results support the use of apixaban as an alternative to SOC for thromboprophylaxis in pediatric heart disease. (A Study of the Safety and Pharmacokinetics of Apixaban Versus Vitamin K Antagonist [VKA] or Low Molecular Weight Heparin [LMWH] in Pediatric Subjects With Congenital or Acquired Heart Disease Requiring Anticoagulation; NCT02981472).

Complete Isolation of Left Innominate Artery in a Patient With CHARGE Syndrome: Case Presentation and Review of Reported Cases.

We report a rare case of complete isolation of the left innominate artery in a child with CHARGE (coloboma, heart defects, atresia choanae, growth retardation, genital abnormalities, and ear abnormalities) syndrome. This anatomical cluster had been undetected for a relatively large period of time and the patient was referred to us with an incomplete diagnosis even after multiple medical evaluations and a thoracic surgery during the neonatal period. In conclusion, to the best of our knowledge, this is the first case of a complete isolation of left innominate artery treated with a transcatheter approach.

[Tachyarrhythmias in congenital heart disease patients: diagnosis, clinical presentation and treatment]. / Tachiaritmie nel paziente con cardiopatia congenita: diagnosi, presentazione clinica e trattamento.

Tachyarrhythmias are a major cause of morbidity and mortality in adult congenital heart disease patients. There is a strong association between tachyarrhythmias and the specific congenital heart disease and the patient's surgical history. Health care for adults with congenital heart disease and arrhythmias should be coordinated by adult congenital heart disease centers of excellence that serve as a resource for consultation and referral. A complete and multidisciplinary evaluation of patients with tachyarrhythmias, including a detailed hemodynamic and electrophysiological evaluation, is mandatory to define the best treatment strategy.

Long Term Follow-Up of Patients with Systemic Right Ventricle and Biventricular Physiology: A Single Centre Experience.

BACKGROUND: A progressively increasing prevalence of congenital heart disease (CHD) in adulthood has been noticed in recent decades; CHD cases with a systemic right ventricle have a poorer outcome. METHODS: Seventy-three patients with SRV evaluated in an outpatient clinic between 2014 and 2020 were enrolled in this study. Thirty-four patients had a transposition of the great arteries treated with an atrial switch operation; 39 patients had a congenitally corrected transposition of the great arteries (ccTGA). RESULTS: Mean age at the first evaluation was 29.6 ± 14.2 years; 48% of the patients were female. The NYHA class at the visit was III or IV in 14% of the cases. Thirteen patients had at least one previous pregnancy. In 25% of the cases, complications occurred during pregnancy. Survival free from adverse events was 98.6% at one year and 90% at 6-year follow-up without any difference between the two groups. Two patients died and one received heart transplantation during follow-up. The most common adverse event during follow-up was the presence of arrhythmia requiring hospitalization (27.1%), followed by heart failure (12.3%). The presence of LGE together with lower exercise capacity, higher NYHA class and more dilated and/or hypokinetic RV predicted a poorer outcome. Quality of life was similar to the QoL of the Italian population. CONCLUSIONS: Long-term follow-up of patients with a systemic right ventricle is characterized by a high incidence of clinical events, prevalently arrhythmias and heart failure, which cause most of the unscheduled hospitalizations.

Prevalence of unruptured intracranial aneurysms in patients with Marfan syndrome: A cross-sectional study and meta-analysis.

INTRODUCTION: Marfan syndrome (MFS) is the most common inherited connective tissue disorder and its association with intracranial aneurysms (ICAs) has been debated for more than two decades. Here, we report the prevalence of ICAs at screening neuroimaging in a population of genetically confirmed MFS patients and present the results of a meta-analysis including our cohort of patients and those of previous studies. PATIENTS AND METHODS: We enrolled 100 consecutive MFS patients, who underwent screening with brain magnetic resonance angiography at our tertiary center between August 2018 and May 2022. We did a PubMed and Web of Science search to retrieve all studies on the prevalence of ICAs in patients with MFS published before November, 2022. RESULTS: Of the 100 patients included in this study (94% Caucasians, 40% females, mean age 38.6 ± 14.6 years), three had an ICA. We pooled the current study with five previously published studies, including a total of 465 patients, 43 of which harbored at least one unruptured ICA, leading to an overall ICA prevalence of 8.9% (95% CI 5.8%-13.3%). DISCUSSION AND CONCLUSION: In our cohort of genetically confirmed MFS patients, the prevalence of ICAs was 3%, which is substantially lower compared to previous studies based on neuroimaging. The high frequency of ICA found in previous studies could be explained by selection bias and lack of genetic testing, which may have led to the inclusion of patients with different connective tissue disorders. Further studies, including several centers and a large number of patients with genetically confirmed MFS, are needed to confirm our results.

[Heart involvement in multisystem inflammatory syndrome in children correlated with SARS-CoV-2 infection: a review by ANMCO/SICP]. / Le manifestazioni cardiache nella sindrome multisistemica infiammatoria del bambino correlate all'infezione da SARS-CoV-2: una rassegna a cura di ANMCO/SICP.

Acute clinical manifestations of COVID-19 are generally less severe in childhood, however a proportion of them can develop a severe systemic hyperinflammatory syndrome after SARS-CoV-2 infection, known as the multisystem inflammatory syndrome (multisystem inflammatory syndrome in children, MIS-C). Cardiovascular manifestations in MIS-C are frequent (34-82%), including myocardial dysfunction, coronary artery dilation or aneurysms, arrhythmias, conduction abnormalities, pericarditis and valvulitis. The most affected cases can develop cardiogenic shock needing intensive care unit admission, inotropic support and sometimes even mechanical circulatory support. The elevation of myocardial necrosis markers, the frequently transient left ventricular systolic dysfunction and the presence of changes on magnetic resonance imaging, support the hypothesis of an immune-mediated post-viral pathogenesis similar to myocarditis. Although MIS-C shows excellent short-term survival, further studies are needed to demonstrate complete reversibility of residual subclinical heart damage.

A case of massive fetal cardiac rhabdomyoma: ultrasound features and management.

We report the case of a massive fetal cardiac rhabdomyoma recently occurred at our clinic. A woman at 23 weeks of gestational age was referred to our center for a fetal cardiac echogenic mass of 26 mm detected at the second-trimester screening ultrasound. During pregnancy, though, the mass progressively increased its dimensions until reaching 48 mm in diameter at 37 weeks of gestation. Fetal echoencephalography and brain magnetic resonance did not show any further fetal anomalies, but molecular genetic testing at amniocentesis revealed a heterozygotic missense variant of gene TSC2 associated with Tuberous Sclerosis. The mass was therefore most likely preferable to a single large rhabdomyoma of gradually increasing dimensions. The baby was delivered at term with a cesarean section. Because of the rhabdomyoma remarkable size and newborn ECG electrical alterations, postnatal therapies with Flecainide and Everolimus were started. Everolimus treatment led to a significant and progressive reduction in the cardiac mass volume. This case, therefore, shows the efficacy of what seems to be a promising treatment in pediatric patients with large rhabdomyomas.Learning points:Rhabdomyomas may present with different features: most often they appear as multiple masses along the interventricular sept, but they may also appear as a single large thoracic mass.When a rhabdomyoma is suspected, genetic counseling is recommended.Both before and after birth, a multidisciplinary approach is useful to choose the appropriate therapy for the newborn.mTOR inhibitors therapies look like promising therapeutic approaches to stimulate the involution of rhabdomyomas.

Echocardiographic score to predict neonatal surgery for aortic coarctation in newborns with prenatal suspicion and patent ductus arteriosus.

INTRODUCTION: The evaluation of upcoming Aortic Coarctation (CoA) in new-borns with prenatal suspicion entails a close echocardiographic monitor until Arterial Duct (AD) closure, in a department with pediatric cardiological and surgical expertise. The significant number of false-positive prenatal diagnoses causes parental stress and healthcare costs. AIM: The aim of this study was to elaborate an echocardiographic prediction model to be employed at birth when PDA is still present, in patients suspected of CoA during fetal life in order to foretell CoA requiring neonatal surgical intervention. METHODS: This retrospective monocentric study included consecutive full-term and late preterm neonates with prenatal suspicion of CoA born from 01 January 2007 to 31 December 2020. Patients were divided into two groups according to the need for aortic surgery (CoA - NoCoA). All patients underwent a comprehensive transthoracic echocardiographic exam in presence of PDA. Multivariable logistic regression was used to create a coarctation probability model (CoMOD) including isthmal (D4), transverse arch (D3) diameters, the distance between a left common carotid artery (LCA) and left subclavian artery (LSA), presence/absence of ventricular septal defect (VSD) and bicuspid aortic valve (BAV). RESULTS: We enrolled 87 neonates (49 male, 56%). 44 patients developed CoA in need of surgical repair. Our index CoMOD showed an AUC = 0.9382, high sensitivity (91%) and specificity (86%) in the prediction of CoA in neonates with prenatal suspicion. We classified neonates with CoMOD > 0 to be at high risk for surgical correction of CoA, with good PPV (86.9%) and NPV (90.9%). CONCLUSIONS: CoMOD > 0 is highly suggestive of the need for CoA corrective surgery in newborns with prenatal suspicion.

Moderate and Severe Congenital Heart Diseases Adversely Affect the Growth of Children in Italy: A Retrospective Monocentric Study.

Children with congenital heart disease (CHD) are at increased risk for undernutrition. The aim of our study was to describe the growth parameters of Italian children with CHD compared to healthy children. We performed a cross-sectional study collecting the anthropometric data of pediatric patients with CHD and healthy controls. WHO and Italian z-scores for weight for age (WZ), length/height for age (HZ), weight for height (WHZ) and body mass index (BMIZ) were collected. A total of 657 patients (566 with CHD and 91 healthy controls) were enrolled: 255 had mild CHD, 223 had moderate CHD and 88 had severe CHD. Compared to CHD patients, healthy children were younger (age: 7.5 ± 5.4 vs. 5.6 ± 4.3 years, p = 0.0009), taller/longer (HZ: 0.14 ± 1.41 vs. 0.62 ± 1.20, p < 0.002) and heavier (WZ: -0,07 ± 1.32 vs. 0.31 ± 1.13, p = 0.009) with no significant differences in BMIZ (-0,14 ± 1.24 vs. -0.07 ± 1.13, p = 0.64) and WHZ (0.05 ± 1.47 vs. 0.43 ± 1.07, p = 0.1187). Moderate and severe CHD patients presented lower z-scores at any age, with a more remarkable difference in children younger than 2 years (WZ) and older than 5 years (HZ, WZ and BMIZ). Stunting and underweight were significantly more present in children affected by CHD (p < 0.01). In conclusion, CHD negatively affects the growth of children based on the severity of the disease, even in a high-income country, resulting in a significant percentage of undernutrition in this population.

Hypertrophic Cardiomyopathy with Biventricular Involvement and Coronary Anomaly: A Case Report.

Although hypertrophic cardiomyopathy (HCM) is classically considered a disease of the left ventricle, right ventricular (RV) involvement has also been reported, though still not extensively characterized. We present a case of biventricular HCM with significant RV involvement in the absence of a left intraventricular gradient: RV outflow tract gradient due to hypertrophy and near obliteration of the RV cavity. Significant RV hypertrophy may cause reduced RV diastolic filling and/or RV outflow obstruction, with potentially increased incidence of symptoms of heart failure, arrhythmias, and pulmonary thromboembolism. The optimal treatment for these patients is unclear. Our patient underwent complete treatment and elimination of right ventricular obstruction, resulting in improved symptoms and a significant reduction in postoperative gradients. Direct relief of outflow tract obstruction can be achieved with low morbidity and good intermediate- to long-term results. Conventional surgery may provide significant symptomatic improvement and should thus be considered in the setting of HCM with outflow obstruction.

[Proposal of a common model for informed consent for interventional procedures in congenital heart disease patients]. / Proposta di un modello comune al consenso informato per le procedure di emodinamica nel cardiopatico congenito.

BACKGROUND: A multidisciplinary study group involving physicians and jurists was established to review and approve an informed consent about the most frequent interventional procedures for congenital heart diseases. METHODS: The authors worked together with representatives of the Italian Society of Pediatric Cardiology and Congenital Heart Disease (SICP) Council and Jurist's expert in the field of health case-law. The final draft was shared with the major Italian centers involved in congenital interventional procedures and with AICCA, the Italian Patients Association of Congenital Heart Diseases - Adults and Children. RESULTS: At the end of this review process, a final informed consent form was developed for the most frequent procedures performed in our catheterization laboratories. All of them consist of two parts: a general statement and a procedure-related one. CONCLUSIONS: The work performed by this multidisciplinary study group, under the supervision of the SICP, resulted in a new dedicated informed consent about interventional procedures in the field of congenital cardiology, taking into account the new legal requests. This informed consent is intended to be both a document that can be used as such and a document from which to derive a specific document for each center. We believe that using similar informed consents in all Congenital Heart Disease Centers or at least have informed consents all inspired by the same setting, could be a further improvement in taking care of the patients and their families.

Increased intracranial arterial tortuosity is associated with worse cardiovascular outcome in patients with Loeys-Dietz syndrome.

The aim of our study was to evaluate the association between intracranial arterial tortuosity and cardiovascular outcome in patients with Loeys-Dietz syndrome (LDS). We performed a retrospective analysis of all patients with genetically confirmed LDS who underwent at least one brain MRA at our institution (n = 32); demographic and clinical features were evaluated in relation to the tortuosity of intracranial arteries as measured by tortuosity index (TI), which was calculated using the formula: [(centerline length) / (straight-line length)-1] × 100. Receiver operating characteristic curve analysis for intracranial TI and the binary end point of aortic surgery showed vertebrobasilar TI (VBTI) to be the best classifier among the examined arterial segments (AUC = 0.822). Patients with higher VBTI showed a greater incidence of aortic surgery (p < 0.001) and underwent more surgical and endovascular procedures (p = 0.006), with a higher rate of operations (p = 0.002). Kaplan-Meier analysis showed a significantly longer surgery-free survival in patients with lower arterial tortuosity (p < 0.001). At multivariate analysis, higher VBTI was associated with an increased risk of surgery (p < 0.001), which was independent of gene mutation and patient age. Increased VBTI is a marker of adverse cardiovascular outcome in patients with LDS, which can be easily measured on brain MRA, and may be useful in the management of this heterogeneous patient population.

Staged approach for correction of anomalous venous return to the azygos vein.

Partial anomalous pulmonary venous return into the azygous vein is a rare pathological finding. We describe the case of a 28-year-old girl who had a successful staged approach to treat this rare congenital heart disease. To avoid potential connection of a systemic venous return to the left atrium, the proximal part of the azygous vein was occluded with a percutaneous approach, then the azygous vein flow was redirected into the left atrium with a surgical procedure.

Fetal speckle-tracking echocardiography: a comparison between two-dimensional and electronic spatio-temporal image correlation (e-STIC) technique.

BACKGROUND: Speckle tracking technology has been applied to assess ventricular deformation throughout the cardiac cycle. An electronic four dimensional probe that allows rapid acquisition of electronic spatio-temporal image correlation volumes (eSTIC) has been recently introduced. OBJECTIVES: The aim of our study was to investigate whether e-STIC acquisition improves deformation analyses reproducibility. STUDY DESIGN: We recruited fetuses between 20 and 40 weeks of gestation. We obtained a 2D video clip and an e-STIC volume of a four-chamber view. We focused on left ventricular global strain (LV-GS) and left ventricular ejection fraction (LV-FE). Intraobserver, interobserver and intermethod agreement were assessed by means of intraclass correlation coefficient (ICC) and illustrated by Bland-Altman plots. Systematic differences between measurements were assessed using a paired t-test. RESULTS: The mean difference between LV-GS values obtained with e-STIC and 2D analysis was -0.10 (95% CI -2.28, 2.08). No systematic differences were found between the two techniques for LV-GS values (p-value = .927). The mean difference between LV-FE values obtained with e-STIC and 2D analysis was 7.55 (95% CI 4.16, 10.95; p-value <.001). The inter-rater reliability of LV-GS was moderate-to-substantial for both e-STIC and 2D. The inter-rater reliability of LV-FE obtained via e-STIC was superior to that obtained via 2D analysis. The intra-rater reliability of LV-GS obtained with e-STIC was superior to that obtained with 2D analysis (ICC 0.857; 95% IC 0.761-0.917). The intra-rater reliability of LV-FE obtained via e-STIC was superior to that obtained via 2D analysis (ICC 0.647; IC 0.51-0.783). CONCLUSIONS: e-STIC seems to be a better technique than 2D analysis for intra-rater reliability of LV-GS. 4D acquisition might improve intrinsic limitations of speckle tracking echocardiography.