Gabriella Agnoletti

Melody transcatheter pulmonary valve implantation. Results from the registry of the Italian society of pediatric cardiology

Percutaneous implantation of pulmonary valve has been recently introduced in the clinical practice. Our aim was to analyze data of patients treated in Italy by using the Melody Medtronic valve.

Closure of the patent ductus arteriosus with the new duct occluder II additional sizes device

The objective of this study was to evaluate the technical feasibility, safety, and efficacy of the new device Amplatzer duct occluder II additional sizes (ADO II AS) for closure of patent ductus arteriosus (PDA). Background: Transcatheter device closure is the standard care for PDA. Currently available technology is not designed for closure of small PDA in young children.

Liver stiffness modifications shortly after total cavopulmonary connection

OBJECTIVES Hepatic damage in Fontan circulation setting is well known. Mainly represented by fibrosis, it is still poorly understood. Transient hepatic elastography (Fibro-Scan) is a useful tool to test liver stiffness and is commonly used in the evaluation of liver fibrosis. Unfortunately, the increase of inferior vena cava pressure consequent to total cavopulmonary connection can probably interfere with Fibro-Scan evaluation altering the result evaluation with the conventional scale. To verify this hypothesis and to quantify the impact of venous pressure on Fibro-Scan results, we checked perioperative liver stiffness changes in patients undergoing total cavopulmonary connection. METHODS A prospective observational study was carried out on 9 patients undergoing extracardiac total cavopulmonary connection. Mean age at operation was 4.3 ± 0.6 years, and mean weight 14 ± 2.4 kg. Hepatic stiffness was analysed with Fibro-Scan evaluation immediately before and 4 months after surgery. Results were matched with several perioperative data: pulmonary pressures, conduit size, fenestration, hepatic enzymes and coagulation setting. RESULTS Preoperative hepatic stiffness was 6.2 ± 1.5 kPa, with a significant increase to 11.2 ± 4 kPa at a mean follow-up of 4 months (P < 0.01). Mean alanine aminotransferase was 16.9 ± 8.3 mg/dl preoperatively and 16.3 ± 8.7 mg/dl at discharge. Both values showed a significant correlation with liver stiffness raise, their Pearson Correlation Indexes being 0.8 and 0.7, respectively (P < 0.01). Mean aspartate aminotransferase/alanine aminotransferase ratio at discharge was 1.7 ± 0.5 and showed a significant negative correlation with liver stiffness raise, its Pearson Correlation Index being -0.7 (P < 0.01). CONCLUSIONS Liver stiffness increases rapidly after total cavopulmonary connection and perioperative variations in some liver enzymes appear to correlate with this change. Since a true anatomical damage is known to develop gradually with Fontan circulation, early liver stiffness raise is likely due to parenchymal congestion only. Fibro-Scan can easily recognize and assess the entity of such a change. For these reasons, this diagnostic tool must be considered useful only to monitor liver stiffness changes and evolution with time, but a conventional evaluation of results, like in other acquired hepatic fibrosis, can be misleading.

Life-Threatening Tumors of the Heart in Fetal and Postnatal Age

OBJECTIVES To evaluate the role of histology in diagnosis and management of biologically benign heart tumors causing life-threatening symptoms and even death in children and fetuses. The clinical impact of a multidisciplinary approach including 2-D echocardiography, histology, genetics, and cardiac surgery has not yet been fully elucidated. STUDY DESIGN Forty-one consecutive antenatal (n = 17) or postnatal (n = 24) detected cardiac masses were evaluated by 2-D echocardiography (in alive patients) or at autopsy, and 12/41 cases with definite histologic diagnosis of primary and benign cardiac tumor were entered in this study. RESULTS Rhabdomyomas (n = 6), hemangiomas (n = 3), central fibrous body chondroma (n = 1), fibroma (n = 1), or left atrial myxoma (n = 1) were histologically diagnosed in 4 fetuses and in 8 children. Death occurred in 6 patients showing diffuse or infiltrative tumors, 2/6 experiencing intrauterine death or sudden and unexpected infant death. Seven patients underwent surgery, 4/7 are alive and well at >5 years follow-up, whereas 3 deaths followed partial tumor resection. Two fetuses with extensive tumor/s were aborted. Tuberous sclerosis complex gene mutations were seen in patients with rhabdomyomas. CONCLUSIONS Histology represents the best diagnostic approach in life-threatening pediatric cardiac tumors allowing definite diagnosis in cases other than rhabdomyoma and in sudden deaths, influencing clinical management and counselling. 2-D echocardiography remains the main tool for early clinical diagnosis and follow-up. A multidisciplinary approach is advisable because of rarity, difficult management, and possible associations with inheritable diseases.

Selective-site pacing in paediatric patients: use of the SelectSecure System and risk of vein occlusion

AIMS Few data are available about selective-site pacing in children. A 4.1 Fr lead is now available: it has to be introduced through an 8.4 Fr steerable catheter, and it raises concerns as to the long-term patency of the subclavian vein. The aim of this study was to systematically evaluate the patency of the subclavian vein in children who had selective-site pacing by the SelectSecure Lead System (SSLS) for atrioventricular block and look for possible local early and late complications. METHODS AND RESULTS From June 2006 to January 2010, we implanted 20 patients. Mean age at implantation was 9 years (range 3-16), mean weight 31 kg (range 13-57). All patients received a dual-chamber pacemaker (PM). The patency of the subclavian vein was evaluated by ultrasound 1, 3, and every 6 months after the PM implantation. We also looked for oedema, swelling, changes in temperature or colour of the skin, and visible superficial circulation. Forty leads were implanted in 20 patients. After a median follow-up of 19 months (range 6-44), no clinical signs or symptoms of vein occlusion were detected in the whole population. None of the patients had an alteration of blood flow at Doppler examination. CONCLUSION The SSLS is a promising and safe system for permanent intracardiac pacing in paediatric patients. We show that it does not increase the risk of venous thrombosis. Until a venography study with a larger population and longer follow-up regarding venous patency and overall complications using SSLS will be performed, our results although promising have to be considered preliminary data.

An unusual heart

In a female foetus of 31 weeks we diagnosed a mass of 15 × 17 mm, occupying most of the left ventricular cavity, which did not cause arrhythmia, cardiac failure, or obstruction. After birth, we confirmed the presence of an intraventricular mass and performed 3D echocardiography to highlight the spatial relationship with cardiac structures. The mass had an unusual heart shape and was visibly smaller …

Endomyocardial biopsy safety and clinical yield in pediatric myocarditis: An Italian perspective.

The objective of this investigation is to evaluate the safety, the impact of endomyocardial biopsy (EMB) results in myocarditis management and the incidence of different etiologies of myocarditis in a pediatric population.

Selective site pacing in paediatric patients: how to recreate a new loop in the right atrium to follow body growth.

In children, intracardiac loops are created to avoid a leads fracture due to rapid body growth. Selective site pacing needs the insertion of two 4.1 Fr lumenless leads that do not allow direct manoeuvrability and should be extracted when tension on the lead occurs. We describe an easy, safe, and inexpensive technique to reposition the leads and generate new intracardiac loops.