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.

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.

Combination of fecal calprotectin and initial coronary dimensions to predict coronary artery lesions persistence in Kawasaki disease.

Kawasaki Disease (KD) is systemic vasculitis involving medium-sized vessels in children. The aim of our study is to determine if fecal calprotectin (FC) could be useful in predicting the development or persistence of coronary artery lesions (CALs) in KD. We conducted a prospective monocentric study including all consecutive diagnoses of. Clinical, laboratory, echocardiographic data were recorded during the acute and subacute phase, including FC. Correlations among laboratory values, FC, clinical manifestations, IVIG-responsiveness and CALs development were investigated. We enrolled 26 children (76.9% boys; median age 34.5 months). The combination of FC > 250 microg/g and z-score > 2 during the acute phase was associated with the persistence of CALs (p = 0.022). A z-score > 2 alone during the acute phase was not related to CALs during the subacute stage (p > 0.05). A neutrophil percentage > 70% and WBC > 15,000/mmc during the acute phase significantly correlated with the presence of CALs during the subacute phase (p = 0.008). C-reactive protein (CRP) > 13 mg/dL at KD onset was significantly associated with the presence of CALs during the acute (p = 0.017) and subacute phase (p = 0.001). The combination of FC > 250 microg/g and a z-score > 2 during the acute phase of KD may be used as a predictor of CALs persistence. It can be useful especially in children with an initial CRP < 13 mg/dl.

Multisystem Inflammatory Syndrome Following SARS-CoV-2 Infection in Children: One Year after the Onset of the Pandemic in a High-Incidence Area.

SARS-CoV-2 infection in children can trigger cardiovascular manifestations potentially requiring an intensive treatment and defining a new entity named Multisystem Inflammatory Syndrome in Children (MIS-C), whose features partially overlap with Kawasaki Disease (KD). A cross-sectional study including all diagnoses of MIS-C and KD from April 2020 to May 2021 in our metropolitan area was conducted evaluating clinical, laboratory (including immunological response, cytokines, and markers of myocardial damage), and cardiac (coronary and non-coronary) features at onset of the diseases. Evolution of ventricular dysfunction, valve regurgitations, and coronary lesions was documented. The severity of the disease was also considered based on the need for inotropic support and ICU admission. Twenty-four MIS-C were diagnosed (14 boys, median age 82 months): 13/24 cases (54.17%) presented left ventricular dysfunction, 12/24 (50%) required inotropic support, and 10/24 (41.67%) developed coronary anomalies (CALs). All patients received steroids and IVIG at a median time of 5 days (IQR1:4, IQR3:6.5) from onset of fever and heart function normalized 6 days (IQR1: 5, IQR3: 7) after therapy, while CALs persisted in one. One patient (12.5%) required infliximab because of refractory disease and still presented CALs 18 days after therapy. During the same study period, 15 KD were diagnosed: none had ventricular dysfunction, while 7/15 (46.67%) developed CALs. Three out of 15 patients (20%) still presented CALs 46 days from onset. Compared to KD, MIS-C pts have significantly higher IL8 and similar lymphocytes subpopulations. Despite a more severe presentation and initial cardiac findings compared to KD, the myocardial injury in MIS-C has a rapid response to immunomodulatory treatment (median time 6 days), in terms of ventricular function, valve regurgitations, and troponin. Incidence of CALs is similar at onset, but it tends to regress in most of the cases of MIS-C differently than in KD where CALs persist in up to 40% in the subacute stage after treatment.

Pediatric rheumatic carditis in Italy and Rwanda: The same disease, different socio-economic settings.

BACKGROUND: Acute Rheumatic Fever and Rheumatic Heart Disease are the leading cause of acquired heart disease in Low-Income Countries, and a common cause in High-Income Countries. We compared rheumatic carditis, its echocardiographic presentation at diagnosis and its progression in Italy and Rwanda. METHODS: Retrospective study including all consecutive patients diagnosed with rheumatic carditis in an Italian (IT) and two Rwandan Hospitals (RW). Echocardiography was performed at diagnosis and three follow-up visits. Baseline characteristics, history of primary and secondary prophylaxis and cardiovascular complications data were collected. RESULTS: Seventy-nine and 135 patients were enrolled in IT and RW, respectively. Mitral regurgitation was the most common lesion (IT: 70%, RW: 96%) in both cohorts; mixed valve lesions and severe lesions were more prevalent in RW. Age at diagnosis (IT: 8.4 ± 2.9 yrs.; RW: 11.1 ± 2.7 yrs.; P < 0.001), adherence to secondary prophylaxis (IT: 99%; RW: 48%; P < 0.001) and history of primary prophylaxis (IT: 65%; RW: 6%; P < 0.001) were different. During the follow-up, native valve lesions completely resolved in 38% of IT and in 2% of RW patients (P < 0.001). By contrast, cardiac surgery was performed in 31% of RW and 5% of IT patients (P < 0.001). Cardiovascular complications and death were only observed in RW. CONCLUSIONS: The more severe cardiac involvement, the higher rate of valve surgery, CV complications and deaths in RW, could be due to delayed diagnosis and treatment, scarce adherence to secondary prophylaxis and differences in social determinants of health.

Prenatal sonography of the foramen ovale predicts urgent balloon atrial septostomy in neonates with complete transposition of the great arteries.

BACKGROUND: Hypoxia caused by inadequate intracardiac mixing owing to a restrictive foramen ovale is a potentially life-threatening complication in neonates with dextro-transposition of the great arteries. An urgent balloon atrial septostomy is a procedure of choice in such cases, but dependent on the availability of a 24-hour interventional cardiology facility. The prenatal identification of predictors for an urgent balloon atrial septostomy at birth would help in optimizing the management of these neonates, minimizing the risk of hypoxic damage. OBJECTIVE: This study aimed to predict with prenatal echocardiography the need of urgent balloon atrial septostomy in neonates with dextro-transposition of the great arteries. STUDY DESIGN: This was a retrospective cohort study of patients with a prenatal diagnosis of transposition of the great arteries that were delivered in our center between 2010 and 2019, for whom fetal ultrasound echocardiograms obtained at less than 3 weeks before delivery were available. The following parameters were systematically obtained at fetal echocardiography: size and appearance of the foramen ovale, septum primum excursion (foramen ovale flap angle at the maximal excursion), diameters of the atria, and size of the ductus arteriosus. Balloon atrial septostomy was defined as urgent if performed within 12 hours from birth in neonates with restrictive foramen ovale. Neonatal follow-up was obtained through medical records analysis. RESULTS: From November 2007 to April 2019, 160 fetuses with complete transposition of the great arteries were referred to our echocardiography laboratory and 60 of these were included in the analysis; 27 underwent urgent balloon atrial septostomy, 11 elective balloon atrial septostomy, and 22 no balloon atrial septostomy. The size of the foramen ovale was the best predictor of an urgent balloon atrial septostomy. A measurement of >6.5 mm had a sensitivity of 100% and a false positive rate of 45%. CONCLUSION: Fetal echocardiography predicts the need of an urgent balloon atrial septostomy in fetuses with dextro-transposition of the great arteries although with a limited precision. In our experience, a measurement of the foramen ovale within 3 weeks of delivery had the greatest accuracy.