Role of linezolid therapeutic drug monitoring in the treatment of MRSA tracheo-pulmonary infection in a 10-month-old infant.

The paper reports on the use of therapeutic drug monitoring for linezolid in the decision-making process to continue or not its administration in an infant. Linezolid is effective against methicillin-resistant Staphylococcus aureus but is off-label in pediatrics. The use of therapeutic drug monitoring, as in our case, allows an informed decision on administration of the drug (in this case on withdrawal) increasing patients safety.

Lithium battery lodged in the oesophagus: A report of three paediatric cases.

BACKGROUND: Over the last years the ingestion of disk batteries has become frequent in children with serious consequences. The severity of injuries is related to the growing use of new lithium batteries that may cause catastrophic damages when lodged in the oesophagus. METHODS: The notes of three consecutive children with lithium batteries lodged in the oesophagus, admitted to our Institute from 2010 to 2014, were reviewed. Clinical presentation, management, and outcome were considered. RESULTS: The first child, a 22-month-old girl, died of a sudden exsanguination due to an aorto-oesophageal fistula. The second case, a 5-year-old boy, had an exploratory thoracotomy to exclude aortic lesion before battery removal, and a spontaneous oesophageal perforation. The third child, a 18-month-old boy, fully recovered after suffering ulcerative oesophageal burns. CONCLUSION: Lithium battery lodged in the oesophagus is a paediatric emergency requiring a multidisciplinary approach that can be implemented in a tertiary children's hospital.

Two decades of experience with the Ross operation in neonates, infants and children from the Italian Paediatric Ross Registry.

OBJECTIVE: Children undergoing Ross operation were expected to have longer autograft, but shorter homograft durability compared with adults. In order to define the outcome in the second decade after Ross operation in children, a nationwide review of 23 years of experience was undertaken. METHODS: 305 children underwent Ross operation in 11 paediatric units between 1990 and 2012. Age at surgery was 9.4±5.7 years, indication aortic stenosis in 103 patients, regurgitation in 109 and mixed lesion in 93. 116 (38%) patients had prior procedures. Root replacement was performed in 201 patients, inclusion cylinder in 14, subcoronary grafting in 17 and Ross-Konno in 73. RESULTS: There were 10 (3.3%) hospital and 12 late deaths (median follow-up 8.7 years). Survival was 93±2% and 89±3% and freedom from any reoperation was 76±3% and 67±6% at 10 and 15 years. 34 children had autograft 37 reoperations (25 replacement, 12 repair): three required transplantation after reoperation. Freedom from autograft reoperation was 86±3% and 75±6% at 10 and 15 years. 32 children had right heart redo procedures, and only 25 (78%) conduit replacements (15-year freedom from replacement, 89±4%). Prior operation (p=0.031), subcoronary implant (p=0.025) and concomitant surgical procedure (p=0.004) were risk factors for left heart reoperation, while infant age (p=0.015) was for right heart. The majority (87%) of late survivors were in NYHA class I, 68% free from medication and six women had pregnancies. CONCLUSIONS: Despite low hospital risk and satisfactory late survival, paediatric Ross operation bears substantial valve-related morbidity in the first two decades. Contrary to expectation, autograft reoperation is more common than homograft.

"Baby Heart Project": the Italian project for accreditation and quality management in pediatric cardiology and cardiac surgery.

Optimization of the relationship between the supply and the demand for medical services should ideally be taken into consideration for the planning within each national Health System. Although government national health organizations embrace this policy specifically, the contribution of expert committees (under the scientific societies' guarantee in any specific medical field) should be advocated for their capability to collect and analyze the data reported by the various national institutions. In addition, these committees have the competence to analyze the need for the resources necessary to the operation of these centers. The field of pediatric cardiology and cardiac surgery may represent a model of clinical governance of particular interest with regard to programming and to a definition of the quality standards that may be extended to highly specialized institutions and ideally to the entire Health System. The "Baby Heart Project," which represents a model of governance and clinical quality in the field of pediatric cardiology and cardiac surgery, was born from the spontaneous aggregation of a committee of experts, supported by duly appointed Italian Scientific Societies and guided by a national agency for accreditation. The ultimate aim is to standardize both procedures and results for future planning within the national Health System.

Use of a telescopic system for transcatheter radiofrequency perforation and balloon valvotomy in infants with pulmonary atresia and intact ventricular septum.

BACKGROUND: Pulmonary atresia and intact ventricular septum is a complex congenital heart disease with great morphological variability. Approximately two-thirds of patients may be suitable for transcatheter pulmonary valvotomy. We reviewed our experience in the use of two different percutaneous approaches to evaluate the impact on fluoroscopy time and morbidity of a new technique to perform transcatheter radiofrequency perforation and valvotomy in newborns with pulmonary atresia and intact ventricular septum. METHODS AND RESULTS: In all, 31 patients underwent radiofrequency perforation of the pulmonary valve. The first 14 infants were treated using a 5 French Judkins right coronary catheter, which was manoeuvred directly underneath the atretic pulmonary valve (Group A). The others were treated using a telescopic system consisting of Northstar Lumax Flex and White Lumax Guiding Catheters (Cook; Group B). In both groups, after radiofrequency perforation of the pulmonary valve, a 0.014-inch superfloppy guidewire was advanced into the descending aorta and balloon dilations were performed. Required fluoroscopy time was significantly lower in Group B (48.5 ± 28.1 versus 24.9 ± 14.4 minutes, respectively; p < 0.01). A higher incidence of unfavourable events including the need for early surgery was found in Group A. CONCLUSION: In our experience, telescopic catheter proved to be a valid option able to decrease the fluoroscopy time of percutaneous radiofrequency perforation of pulmonary valve and consequently patients' exposure to procedure-related risks.

Incidence and clinical relevance of primary congenital anomalies of the coronary arteries in children and adults.

OBJECTIVES: To describe our experience in the management of coronary artery anomalies both in an adult and in a paediatric population and to compare the two groups for finding out differences in terms of angiographic incidence and treatment. PATIENTS AND METHODS: Databases at the Department of Cardiology of San Martino Adult's Hospital and of Gaslini Children's Hospital were searched for all patients with a diagnosis of coronary artery anomaly who underwent coronary angiography between 1994 and 2006. RESULTS: Coronary anomalies were diagnosed in 76 (1%) adult patients. Anomalous left circumflex artery was the commonest coronary anomaly (25%). Anomalous left coronary artery from pulmonary artery and myocardial bridges were the only anomalies responsible for angina-like symptoms. No patients except the one with anomalous left coronary artery from pulmonary artery needed surgical intervention. In the paediatric population, we found 28 (0.9%) patients with coronary anomalies. Anomalous left coronary artery from pulmonary artery was the most common anomaly (48%) and always required emergency surgical treatment; in addition there were two patients with stenosis of the left main coronary artery. CONCLUSION: Coronary artery anomalies may be associated with very acute, even life-threatening symptoms in children, whereas they are usually clinically silent and detected by accident on coronary angiography in adults. Recognition of coronary artery anomalies enables early treatment or close follow-up in children, whereas it could be useful in case of cardiac surgery in adults.

Impact of prenatal diagnosis on outcome of pulmonary atresia and intact ventricular septum.

OBJECTIVES: To determine the impact of fetal echocardiography on the management of pregnancy and of newborns affected by pulmonary atresia and intact ventricular septum (PAIVS) and to evaluate the outcome of infants with and without prenatal diagnosis of PAIVS. METHODS: We searched our database for cases of PAIVS prenatally and postnatally diagnosed during the period January 1993-December 2009. Postnatal follow-up was available in all cases included in the study. Karyotyping and fluorescent in situ hybridization analysis for the DiGeorge critical region (22q11.2) were performed in all but one case. RESULTS: The study comprised 60 cases of PAIVS: 36 with (Group A) and 24 without (Group B) prenatal diagnosis. In Group A, there were two intrauterine deaths, six postnatal deaths (five early after birth) and one termination of pregnancy. In this group, radiofrequency (RF) perforation was successfully performed in 25 cases; 20/25 infants had a biventricular (BV) repair, without further operation in 13 of them. No patient of Group B died. In this group, RF perforation was successfully performed in 22 cases; 20/22 had a BV repair without further procedure in 15 of them. CONCLUSIONS: Prenatal diagnosis of PAIVS allows a reliable prognosis of severity and planning of proper surgical repair strategies. Fetuses that are prenatally diagnosed present a more severe spectrum of the disease; for the cases capable of getting through the neonatal period, the mortality rate and the need for further intervention were not significantly different when compared with babies with only postnatal diagnosis.

Gaslini's tracheal team: preliminary experience after one year of paediatric airway reconstructive surgery.

BACKGROUND: Congenital and acquired airway anomalies represent a relatively common albeit challenging problem in a national tertiary care hospital. In the past, most of these patients were sent to foreign Centres because of the lack of local experience in reconstructive surgery of the paediatric airway. In 2009, a dedicated team was established at our Institute. Gaslini's Tracheal Team includes different professionals, namely anaesthetists, intensive care specialists, neonatologists, pulmonologists, radiologists, and ENT, paediatric, and cardiovascular surgeons. The aim of this project was to provide these multidisciplinary patients, at any time, with intensive care, radiological investigations, diagnostic and operative endoscopy, reconstructive surgery, ECMO or cardiopulmonary bypass. Aim of this study is to present the results of the first year of airway reconstructive surgery activity of the Tracheal Team. METHODS: Between September 2009 and December 2010, 97 patients were evaluated or treated by our Gaslini Tracheal Team. Most of them were evaluated by both rigid and flexible endoscopy. In this study we included 8 patients who underwent reconstructive surgery of the airways. Four of them were referred to our centre or previously treated surgically or endoscopically without success in other Centres. RESULTS: Eight patients required 9 surgical procedures on the airway: 4 cricotracheal resections, 2 laryngotracheoplasties, 1 tracheal resection, 1 repair of laryngeal cleft and 1 foreign body removal with cardiopulmonary bypass through anterior tracheal opening. Moreover, in 1 case secondary aortopexy was performed. All patients achieved finally good results, but two of them required two surgeries and most required endoscopic manoeuvres after surgery. The most complex cases were the ones who had already been previously treated. CONCLUSIONS: The treatment of paediatric airway anomalies requires a dedicated multidisciplinary approach and a single tertiary care Centre providing rapid access to endoscopic and surgical manoeuvres on upper and lower airways and the possibility to start immediately cardiopulmonary bypass or ECMO.The preliminary experience of the Tracheal Team shows that good results can be obtained with this multidisciplinary approach in the treatment of complicated cases. The centralization of all the cases in one or few national Centres should be considered.

The scimitar syndrome: an Italian multicenter study.

BACKGROUND: Scimitar syndrome is a rare congenital heart disease. To evaluate the results of managing this malformation surgically, we have embarked on a multicenter Italian study involving seven different centers and reporting the largest published series in the medical literature. METHODS: From January 1997 to December 2007, 26 patients with scimitar syndrome who underwent surgical correction were included. Primary outcomes include hospital mortality and the efficacy of repair at the follow-up. RESULTS: Median age was 11 years (interquartile range, 1.8 to 19.9 years). Nineteen patients (73%) presented with symptoms including upper respiratory tract infections (n = 13), recurrent pneumonia (n = 10), cardiac failure (n = 4), and cyanosis (n = 2). Associated cardiac anomalies were present in 16 patients (63%). Surgical repair included intraatrial baffle repair in 18 patients (69%; group 1), and reimplantation of the "scimitar vein" onto the left atrium in 8 patients (31%; group 2). One patient died in hospital (3.8%; group 1). Postoperative complications were less frequently reported in group 1 (4 of 18 patients, 22%) compared with group 2 (5 of 8 patients, 62%). Median follow-up time was 4 years (interquartile range, 1.8 to 9.7 years). There was 1 late death (1/25 patients, 4%; group 2). Four patients (16%) showed a complete occlusion of the scimitar drainage (2 in group 1, 12%; 2 in group 2, 25%) and 3 patients (12%) required balloon dilation or stenting for scimitar vein stenosis (1 in group 1, 6%; 2 in group 2, 25%). CONCLUSIONS: The intraatrial baffle repair seems to have a lower incidence of postoperative complications and a better patency rate, at last follow-up, than the reimplantation of the scimitar vein onto the left atrium.

Bidirectional Glenn and antegrade pulmonary blood flow: temporary or definitive palliation?

BACKGROUND: We sought to investigate the role of the bidirectional Glenn with antegrade pulmonary blood flow in the surgical history of children with univentricular hearts. METHODS: A series of 246 patients, from three joint institutions, having univentricular heart with restricted but not critical pulmonary blood flow received a bidirectional cavopulmonary shunt with additional forward pulmonary blood flow. All patients have been studied according to their progression, or not, to Fontan operation. Two hundred and eight (84.5%) patients underwent bidirectional cavopulmonary anastomosis as primary palliation. Twenty patients (8.1%) with previous pulmonary artery banding were also enrolled in the study. Patients who had received additional pulmonary blood flow through a previous systemic to pulmonary artery shunt for the critical pulmonary blood flow were excluded. RESULTS: No in-hospital death occurred. Follow-up was complete at 100%. Mean follow-up was 4.2 +/- 2.8 years (range, 6 months to 7 years). During the observational period 73 (29.7%) patients, considered optimal candidates, underwent Fontan completion for increasing cyanosis and (or) hematocrit and (or) fatigue with exertion. Three patients expired after total cavopulmonary connection (3 of 73; 4.1% mortality rate). The remaining 173 (70.3%) patients are alive with initial palliation. All patients were still well palliated with an arterial oxygen saturation at rest about 90%. CONCLUSIONS: According to our experience and results, bidirectional Glenn with antegrade pulmonary blood flow may be an excellent temporary palliation prior to a Fontan operation, which can be performed at the onset of symptoms. Bidirectional Glenn may also be the best possible palliation for a suboptimal candidate for Fontan.

State of the art of cardiac surgery in patients with congenital heart disease.

During the last 20 years, pediatric cardiac surgery has been characterized by important changes, with reductions in surgical mortality and the achievement of complete repair at an earlier age, thus avoiding multiple procedures and strongly ameliorating the global outcome of these patients. In this review, we describe the actual trends in the surgical treatment of cardiac malformations. We analyze two groups of patients: in the first group (septal defects, tetralogy of Fallot, transposition of the great arteries, aortic stenosis and coarctation) the indications are well established and the goal is represented by a lessening of the surgical trauma and post-operative morbidity, with stable results in the follow-up. In the second group (univentricular heart, pulmonary atresia and intact ventricular septum, double discordance, conduit, hypoplastic left heart syndrome), the lesions are still considered complex and submitted to ongoing experimental and clinical research, in order to improve the post-surgical history of these diseases.

Discrete subaortic stenosis: incidence, morphology and surgical impact of associated subaortic anomalies.

BACKGROUND: The association between discrete subaortic stenosis and other subaortic anomalies is a well known but rarely reported occurrence. The aim of this study is to define the incidence, morphology, and surgical impact of associated anomalies of the left ventricular outflow tract in children operated on for discrete subaortic stenosis. METHODS: Between 1994 and 2000, 45 consecutive children were operated on for discrete subaortic stenosis. Patients were divided in two groups according to the obstructive lesion detected by echocardiography. RESULTS: A localized shelf was found as an isolated lesion in 31 patients (group A), whereas additional subaortic anomalies were found in 14 cases (31%) and were multiple in 5 cases (group B). The anomalies included anomalous septal insertion of mitral valve (7 cases); accessory mitral valve tissue (2 cases); anomalous papillary muscle (2 cases); anomalous muscular band (8 cases); and muscularization of the anterior mitral valve leaflet (1 case). Cardiopulmonary bypass and aortic cross-clamping times were significantly shorter in group A. There were no operative deaths nor major complications or deaths during follow-up. A gradient of 15 mm Hg or more was found at follow-up in 5 cases whereas aortic regurgitation was estimated to be not clinically significant in all but 1 patient. Six cases of recurrent subaortic stenosis were found in our series, 3 of them with other subaortic anomalies. CONCLUSIONS: This study shows that discrete subaortic stenosis can often be associated with other subaortic abnormalities. Surgical treatment of these anomalies produces excellent early and mid-term relief of obstruction without any increase in mortality and morbidity.

Acquired pulmonary vein obstruction after open-heart surgery.

Acquired pulmonary vein obstruction is an extremely rare complication after open-heart surgery not including pulmonary vein procedures. We report on three cases of this unusual complication presenting peculiar angiographic findings in order to understand its possible etiology.