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Neurological and radiologic research results show an abnormal cerebral microstructure as well as abnormal neurodevelopment in patients treated for hypoplastic left heart syndrome. The aim of this study was to assess the varying cognitive performance these children have developed in dependence upon prenatal diagnosis, surgical techniques, surgical learning effects, anatomy, perfusion techniques, gender, pedagogic, and sociodemographic parameters in comparison to age-adjusted normative values. School-age children (6.3-16.9 years) with hypoplastic left heart syndrome, who were treated at the Children's heart Center Linz between 1997 and 2009, ( n = 74), were surveyed in reference to cognitive achievements. 43 patients were examined prospectively by psychologists using the Wechsler intelligence scale for children IV in order to determine the respective total intelligence quotient index for each child's developmental stage. The mean index was 84.5 (percentile rank 26.4). The statistical spread and standard deviation ranged from a minimum of 40 to a maximum of 134 ± 20.8. The results for verbal comprehension, perceptual reasoning, and processing speed corresponded with total index results and were thus lower than the mean value of the normative values. The assessment of working memory showed results in the average. Prenatal diagnosis, type of lung perfusion, anatomy, and various cerebral perfusion techniques did not significantly affect the cognitive results of the patients. The results show that hypoplastic left heart syndrome patients can be successfully tutored formally as well as personally in cognitive areas, although when compared to healthy children, they showed lower results for intellectual area parameters. [ABSTRACT FROM AUTHOR]

Unlabelled: Successful therapy of vasodilatory shock in adults and children with arginine-vasopressin (AVP) has been reported previously. Data on the use of vasopressin in neonates is limited. This retrospective study reports the effects of AVP-treatment in neonates with catecholamine-resistant systemic vasodilatation after cardiopulmonary bypass. From March 2003 through December 2005, 172 neonates underwent open-heart surgery, 17 developed vasopressor-resistant hypotension and were treated with AVP. Thirteen patients had a stage I palliation of single ventricle, two had a Ross-operation and two had an arterial switch operation. All patients received multiple traditional inotropes and vasopressors prior to administration of AVP. AVP was started at median 0.0001 U x kg(-1) x min(-1) (range 0.00005-0.0002) and titrated up to a maximum of median 0.0003 U x kg(-1) x min(-1) (range 0.0001-0.001). AVP led to a significant increase in blood pressure (from 49+/-8 mmHg to 69+/-7 mmHg) and the requirement of traditional vasopressors decreased significantly. No peripheral vasoconstriction or ischemia was observed. Four of 13 patients, all with single ventricle palliation, died. In two patients death occurred due to additional complications 6 days after AVP was discontinued. One patient, who was still on AVP, died 42 hours postoperatively after prolonged hypoxemia not responding to inhaled nitric oxide. One patient arrested on the third postoperative day when AVP was almost weaned.Conclusion: In neonates with vasodilatory shock after cardiopulmonary bypass AVP is a potent agent to increase blood pressure when traditional vasopressors are failing. [ABSTRACT FROM AUTHOR]

The arterial switch operation (ASO) has become the treatment of choice in patients with simple or complex transposition of the great arteries (TGA). The purpose of this study was to assess early and mid-term outcome after ASO in a single centre. Between 1995 and December 2005, 114 consecutive patients underwent an ASO at our institution, performed by one single surgeon. Patients charts, surgical reports and echocardiograms were retrospectively reviewed. Patients were analyzed in 3 different groups: Group I consisted of 77 neonates with TGA and intact ventricular septum, group II of 13 patients with TGA and ventricular septal defect which had to be closed surgically and group III of 24 patients with various forms of TGA in a complex setting. The patient’s median weight was 3.23 kg (1.65–8.30). Twenty-five neonates were born preterm, 18 were diagnosed prenatally. Median follow-up time was 20.7 months (0.3–128.6). The thirty day mortality was 1.75% (2/114), late mortality 0.88% (1/112) accounting for an overall mortality of 2.63%. There was only one early coronary event and so far no late events. Ten of 111 survivors required reoperation, all but 1 from group III. Prevalence of supravalvular pulmonary stenosis was 4.7%. Freedom from reoperation at 5 years of follow-up time was 87.5%. One patient required permanent pacing, no other late arrhythmias occurred. In our series the only risk factor for increased mortality and morbidity was a body weight of less than 2500 g at the time of operation. No better outcome could be demonstrated in the prenatally diagnosed patients. The ASO can be performed safely and with low mortality and morbidity even in patients with complex TGA. Follow-up of these patients is required to detect residual problems like supravalvular pulmonary stenosis, coronary problems, arrhythmias and aortic valve dysfunction. [ABSTRACT FROM AUTHOR]