The first experience with the high-resolution multi-slice CT-angiography in pediatric cardiology in Slovakia.

OBJECTIVES: The high-resolution multi-slice computed tomography angiography (HRMS-CTA) is a new imaging method characterized by a precise isotropic imaging of any cardiovascular system structure. The purpose of this study was to review the first experience with the high-resolution multi-slice HRMS-CTA in pediatric patients with congenital heart defects as well as with acquired cardiac diseases in the Slovak Republic. METHODS: We retrospectively reviewed benefits of HRMS-CTA and its influence on the subsequent choice of the most appropriate management in 20 patients with pulmonary atresia with ventricular septal defect and multiple aortopulmonary collaterals (PA, VSD, MAPCAs) and in 15 patients with different diagnoses with expected benefit from HRMS-CTA in terms of an exact delineation of extra-cardiac vascular structures. RESULTS: The delineation of the examined extra-cardiac structures was very detailed and clear in all 35 patients. All findings definitely contributed to the choice of the most appropriate management not only in PA, VSD, MAPCAs patients (20), but also in 15 patients with different diagnoses. CONCLUSIONS: HRMS-CTA is a new complementary imaging method with the potential to replace invasive catheterization procedures in large number of patients who need extra-cardiac vascular structure imaging (Fig. 1, Ref. 18). Full Text (Free, PDF) www.bmj.sk.

Neuropsychological outcome in children with hypoplastic left heart syndrome.

OBJECTIVES: The purpose of this study is to review a single-institutional experience gained over one decade, to assess late neuropsychological outcome in patients as well as to determine Norwood operation risk factors that are related to patient and procedure and influence these results. BACKGROUND: The reports of first successful staged operation of hypoplastic left heart syndrome (HLHS) and its numerous modifications have improved the early survival also in patients with complex forms. Specifically, the identification of other specific risk factors related to patient or procedure in the first stage of palliation for HLHS may play a role in cognitive deficit. METHODS: Single-institutional, cross-sectional study of neurodevelopmental outcome. The mean age at testing was 6.9 +/- 0.3 years. There were 19 patients enrolled with completed psychological testing Standford-Binet test. RESULTS: The mean neurocognitive results were significantly below the population normative values. The mean full-scale IQ for the entire cohort was 84.1 +/- 8.2. The predictive factors are strongly associated with severe metabolic acidosis, low birth weight, hospital stay over 35 days, prolonged duration of DHA and aortic valve diameter playing an important role in the subsequent psychological outcome, especially in differential subsections of IQ scale (Tab. 2, Ref. 15). Full Text (Free, PDF) www.bmj.sk.

Experiences with fenestration closure in patients after the Fontan operation.

BACKGROUND AND OBJECTIVES: [corrected] In this study, decision and timing of fenestration closure are discussed. We review our experiences with transcatheter occlusion of fenestration or residual baffle leak using Amplatzer occluder. PATIENTS AND METHODS: We have retrospectively reviewed the cath lab records of 26 patients who have undergone closure of fenestration (or another baffle leak) in period of 2002 to July 2006. Patients with the previous fenestrated Fontan procedure (intracardiac lateral tunnel, extracardiac conduit) with the presence of clinically important cyanosis, arterial desaturation less than 88 %, previously described leak by echocardiography, impaired exercise capability, absence of effusions and appropriate hemodynamic parameters were included in the study. RESULTS: The most frequent group were patients with hypoplastic left heart syndrome (HLHS) (27 %), second most common was L-transposition of great vessels (TGA), tricuspidal valve atresia (TA) and atresia of pulmonary artery (PA) (all of them 15.4 %). CONCLUSIONS: Our results confirm that after adjusting the hemodynamical risk, transcatheter fenestration occlusion in high risk patients might be a safe and effective method to significantly increase arterial O2 saturation. The long term benefits of closure especially at exertion are yet to be shown (Tab. 2, Ref 21). Full Text (Free, PDF) www.bmj.sk.

Arrhythmias in congenital heart defects.

AIM OF THE STUDY: Evaluation of the incidence and severity of late arrhythmias in patients with predisposing congenital heart defects--either due to the anatomy of the defect itself or as a result of a particular type of surgical intervention. PATIENTS AND METHODS: In a retrospective long-term study authors analyzed 158 patients (divided into 5 groups) with congenital heart defects after surgical correction. Evaluated were: the incidence of rhythm disturbances, the type of arrhythmia and the need for medication or intervention. RESULTS: The most rhythm disturbances occurred in patients after physiological correction of D-transposition of the great arteries (68.5%) and these patients also mostly needed medication or pacemaker implantation; followed were by patients with hypoplastic left heart syndrome after Fontan procedure (40%), then were patients after long-term correction of tetralogy of Fallot (31.1%), atrial septal defect sinus venosus type with partial anomalous pulmonary venous return after Warden correction (25.7%) and congenitally corrected L-transposition of the great arteries (25 %). Most of these arrhythmias were asymptomatic and there was no need to treat them. There was an increased incidence of arrhythmias with time (p < 0.05). DISCUSSION: During childhood in patients after surgical correction late arrhythmias mostly do not represent a severe problem, but with time, when reaching adulthood, this may be an issue. It is therefore very important to understand the anatomy, physiology and the arrhythmogenic substrate of every high risk congenital heart defect (Tab. 2, Fig. 6, Ref. 10).