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.

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.