Lucio Zannini

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.

Long-term results of catheter-based treatment of pulmonary atresia and intact ventricular septum

Objective: To review the outcome of patients with pulmonary atresia with intact ventricular septum after interventional perforation of the pulmonary valve, to assess the capability of this procedure to avoid neonatal or late intervention and to obtain a long-term biventricular repair. Design: Retrospective interventional study and clinical follow-up study. Setting: Tertiary referral centre. Patient population: Between November 1994 and December 2007, 40 neonates underwent radiofrequency perforation. Median age at pulmonary valvotomy was 28 hours (range 1–147 hours) and median weight was 2925 g (range 1900–4400 g). Main outcome measures: Procedural success and complication rates; early-term and long-term follow-up results. Results: The procedure was successful in 39 patients but 16 of them needed neonatal surgery. The overall mortality was 7.5%. At a median follow-up of 82 months, four patients underwent a bidirectional Glenn procedure, whereas all the other patients achieved a biventricular circulation without any further intervention in 19 of them. Patients who died or needed additional intervention with or without biventricular circulation failure had a higher incidence of bipartite right ventricular (65% vs 15.8% of those not needing additional intervention; p = 0.004) and a lower median tricuspid Z value (−2 (range −3.5 to 1) vs −0.5 (range −2 to 1); p = 0.004)). Conclusions: The results confirm that percutaneous interventional perforation is an effective first-stage procedure in patients with pulmonary atresia with intact ventricular septum. The right heart appeared to be adequate to maintain a long-term biventricular circulation in the large majority of cases.

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 Adults Hospital and of Gaslini Childrens 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.

Giant aneurysm following coil occlusion of patent ductus arteriosus.

A case is described in which a giant aneurysm developed following successful PDA coil occlusion in an infant with Marfan syndrome. This rare and severe complication brings into question the need for careful evaluation of these children before and after transcatheter occlusion. Cathet. Cardiovasc. Intervent. 50:186–189, 2000.

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.

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.

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.