Perfusion Study Helps in the Management of the Intraseptal Course of an Anomalous Coronary Artery.

Anomalous origin of a coronary artery from the opposite aortic sinus of Valsalva can present in various ways, ranging from a benign and incidental finding to sudden cardiac death. The variant with an intraseptal subpulmonary course (sometimes referred to as intraconal), is widely perceived to carry a low risk of ischemia and has been considered to be a benign variant, not requiring surgical treatment. In one of our recent patients, however, nuclear scintigraphy highlighted a myocardial perfusion deficit in the territory supplied by the allegedly benign anomalous coronary artery, prompting the need for a more aggressive surgical approach.

Atrial flow regulator for failing Fontan circulation: an initial European experience.

Transcatheter creation or enlargement of an atrial septal defect has been used to promote adequate blood flow and mixing in some forms of congenital heart defects or as a relief valve in right or left atrial hypertension, resulting in better cardiac output and/or systemic saturation. We report a case of a 4-year-old male affected by complex congenital heart disease who was admitted for management of severe cyanosis following a staged pericardial fenestrated Fontan procedure. Transoesophageal echocardiogram showed a wide fenestration of 9 mm in size with a severely dilated pericardial Fontan system. To avoid a new surgical procedure and as part of a compassionate use programme, we decided to implant an atrial flow regulator device (4 mm in diameter) with percutaneous approach with the goal of reducing the right-to-left shunt and increasing the pulmonary flow. Preprocedural oxygen saturation was 75%, whereas after 2 months of follow-up, we observed a progressive increase of up to 95% with significant reduction in the pericardial Fontan system dimensions at echocardiography.

Atrial septal defect morphology and stenting in hypoplastic left heart syndrome after hybrid palliation.

Aims The aim of this study was to describe atrial septal defect morphology in hypoplastic left heart syndrome, to report the incidence of restrictiveness and its relationship with defect morphology, to correlate restriction with midterm outcome, and to describe our interventional approach to restrictive defect. Methods and results From 2011 to 2015, 31 neonates with hypoplastic left heart syndrome underwent hybrid procedure with pulmonary artery banding and ductal stenting at our Institution. Restrictive physiology of the atrial septal defect was based on Doppler gradient >6 mmHg through the defect and on clinical signs of pulmonary hypertension. The mean gradient was then measured invasively. Restrictive defect occurred in 11/27 patients (40%). The restrictive group showed three ostium secundum defects (27%) and eight complex morphologies (73%). Conversely, in the non-restrictive group, we observed 11 ostium secundum defects (69%) and five complex morphologies (31%). Early balloon atrioseptostomy was required in three cases. Late restriction occurred in eight patients and was dealt with balloon dilation, stenting, or atrioseptectomy. There was no significant difference between restrictive and non-restrictive groups in terms of early or 12-month survival. CONCLUSIONS: Complex morphologies were more frequently related to restrictiveness. Stenting technique has a crucial role, as the procedure carries a significant risk for stent migration. Effective treatment of restrictive atrial septal defect is related to a better outcome, as it leads to equalisation of survival between patients with and those without restrictive atrial septal defect.

Telemedicine as an effective intervention to improve antibiotic appropriateness prescription and to reduce costs in pediatrics.

Implementation of antimicrobial stewardship program is a pivotal practice element for healthcare institution. We developed a remote infectious disease consultancy program via telemedicine in a high-specialized pediatric cardiac hospital. A consultation for antibiotic strategy for each patient was available via telemedicine in addition to biweekly discussion of all clinical cases. Aim of this study was to evaluate the impact of the remote stewardship program in terms of a) appropriateness of antibiotic prescription; b) incidence of multi-resistant infection; and c) cost. A 'before - after' study was performed comparing the period immediately before starting the program and one year after. There was a trend in the reduction of nosocomial infectious disease rate (9.5 vs 6.5 per 1000 person days), with a reduction in the overall antibiotic cost (25,000 vs 15,000 EUR) and in the average antibiotics packages used per admission (9 vs 6.7 packages). A significant reduction in the multi-drug resistant isolation rate was observed (104 vs 79 per 1000 person days, p = 0.01). In conclusion, the infectious disease meeting via telemedicine has been an effective tool for economic and professional development and multidisciplinary management of complex patients. The appropriate use of antibiotics reduced the multi-drug resistant bacteria selection, thus improving patient safety.

Early hybrid approach and enteral feeding algorithm could reduce the incidence of necrotising enterocolitis in neonates with ductus-dependent systemic circulation.

BACKGROUND: The reported incidence of necrotising enterocolitis in neonates with complex CHD with ductus-dependent systemic circulation ranges from 6.8 to 13% despite surgical treatment; the overall mortality is between 25 and 97%. The incidence of gastrointestinal complications after hybrid palliation for neonates with ductus-dependent systemic circulation still has to be defined, but seems comparable with that following the Norwood procedure. METHODS: We reviewed the incidence of gastrointestinal complications in a series of 42 consecutive neonates with ductus-dependent systemic circulation, who received early hybrid palliation associated with a standardised feeding protocol. RESULTS: The median age and birth weight at the time of surgery were 3 days (with a range from 1 to 10 days) and 3.07 kg (with a range from 1.5 to 4.5 kg), respectively. The median ICU length of stay was 7 days (1-70 days), and the median hospital length of stay was 16 days (6-70 days). The median duration of mechanical ventilation was 3 days. Hospital mortality was 16% (7/42). In the postoperative period, 26% of patients were subjected to early extubation, and all of them received treatment with systemic vasodilatory agents. Feeding was started 6 hours after extubation according to a dedicated feeding protocol. After treatment, none of our patients experienced any grade of necrotising enterocolitis or major gastrointestinal adverse events. CONCLUSIONS: Our experience indicates that the combination of an "early hybrid approach", systemic vasodilator therapy, and dedicated feeding protocol adherence could reduce the incidence of gastrointestinal complications in this group of neonates. Fast weaning from ventilatory support, which represents a part of our treatment strategy, could be associated with low incidence of necrotising enterocolitis.

Carotid artery intima-media thickness and stiffness index ß changes in normal children: role of age, height and sex.

AIMS: The need for early markers of atherosclerosis in paediatric ages has been emphasized recently. Few data are available on the behaviour of carotid intima-media thickness (cIMT) and arterial stiffness in normal children. METHODS: We studied prospectively 131 healthy children (78 boys), aged 3-16 years, by high-definition ultrasonography and echo-tracking technique in order to evaluate cIMT and stiffness index ß. RESULTS: Stiffness index ß underwent a significant age-related increase (P < 0.001), and a positive relationship between cIMT and height was found in boys. In addition, stiffness index ß and cIMT were not related to each other (P = 0.97). CONCLUSION: This study provides information about two markers of subclinical atherosclerosis, cIMT and carotid stiffness index ß, in normal children aged 3-16 years. We found a significant age-related and height-related increase of stiffness index ß in both sexes, whereas cIMT was positively related to height only in boys.

Erythrocytosis and severe asphyxia: two different causes of neonatal myocardial infarction.

Neonatal acute myocardial infarction is a rare event that carries a high mortality rate. We describe the cases of two newborns who survived acute myocardial infarction and discuss the management. The first neonate was born with severe asphyxia and left ventricular myocardial infarction with ventricular tachycardia. In this patient, systemic flow was maintained by right-to-left shunting through the patent ductus arteriosus. The second neonate presented with a haematocrit of 80% and an inferolateral myocardial infarction. Intensive treatment of low cardiac output syndrome led to survival of both high-risk neonates. In the follow-up, at 48 and 4 months, respectively, ventricular function recovered in both patients.