Twenty-five years' experience with the arterial switch operation

OBJECTIVE: The arterial switch operation was introduced in 1976 to correct transposition of the great arteries and has since replaced atrial palliation. This study examines the long-term outcomes of the arterial switch operation. METHODS: Included in the study were 195 patients who underwent the arterial switch operation from 1977 through June 2000. Patients were evaluated for functional class, pulmonary stenosis, left ventricular function, arrhythmias, aortic sequelae, and coronary disease. RESULTS: Overall perioperative mortality was 15%, but rates dropped in the last 5 years to 4% for complex transposition and 0% for simple transposition. Of 151 survivors, 2 died late; 1 died of persisting pulmonary hypertension and 1 died of ventricular fibrillation after coronary pathologic changes. At last follow-up, a total of 145 patients were in New York Heart Association functional class I and 4 patients were in class II. The most frequent complication was pulmonary stenosis, necessitating 45 reinterventions in 26 patients. Left ventricular dysfunction was noted in 5 patients. Arrhythmias were seen in 5 patients: 2 patients had ventricular fibrillation (1 died and 1 required a defibrillator implantation), 1 had sick sinus syndrome, 1 had atrial flutter, and 1 had a single attack of supraventricular tachycardia. Aortic valve incompetence was absent or trivial in 146 patients, 3 had mild incompetence, 1 had moderate incompetence, and 1 had severe incompetence. Coronary sequelae were found in 5 of the 61 patients who underwent angiography. CONCLUSION: Long-term clinical outcome of the arterial switch operation is good, and perioperative mortality is now low. Morbidity is dominated by pulmonary stenosis and coronary artery disease, with the potential for lethal arrhythmias.     

Estrogen receptor-mediated down-regulation of corticotropin-releasing hormone gene expression is dependent on a cyclic adenosine 3＇ ,5＇-monophosphate regulatory element in human placental syncytiotrophoblast cells

Placental CRH plays a major role in the mechanisms controlling human pregnancy and parturition. Understanding how placental CRH production is regulated is therefore of importance. Previously we have shown that placental expression of CRH peptide and mRNA are inhibited by estrogens, in contrast to the stimulatory effects of estrogen on hypothalamic CRH production. Our current study found that in placental cells cotransfected with a CRH promoter construct and an estrogen receptor-alpha expression vector results in a differential regulation whereby 17beta estradiol (E2) decreased and the putative pure estrogen antagonist, ICI 182780,     

Polyarteritis nodosa in children: radiological aspects and diagnostic correlation

The radiological findings in 14 children with polyarteritis nodosa (PN) were evaluated in relation to clinical, laboratory, and histological findings. In this series, there were two major groups of radiological findings, one associated with renal insufficiency and the other with intestinal vascular insufficiency. No radiographic finding was pathognomonic. Two patients were suspected of having small bowel intussusception (SBI), and SBI was documented at laparotomy in a third patient. The diagnosis and management of PN in children is dependent upon the correlation of clinical and laboratory findings. Associated pertinent radiological features which may be useful in the management of children with PN are reviewed.     

Networks for integrated care provision: an economic approach based on opportunism and trust

In this paper, we provide the economic rationale for an important issue in the health care sector, namely the network formation, e.g., in The Netherlands. The presence of such cooperation structures is hard to explain using the basic concept of the economic organization (EO) theory, i.e., the dichotomy of hierarchy versus market. However, acknowledging the aspect of trust renders the clan concept to be a powerful tool in understanding the viability of intra- and inter-organizational cooperation in the health sector. The main reason for this is the manner in which the professionals involved perform, as well as the importance of the tacit knowledge of the actors employed in the various health institutions. First, we address the conversion from supply towards demand orientation and the resulting pressure on multi-professional cooperation between health care providers. Then, relevant EO concepts will be reviewed, while introducing theory on knowledge, learning, and trust. Moreover, we offer conclusions for the health care sector on a concept-by-concept basis. Finally, we propose the notion of interclan, a clan-inspired notion for inter-organizational cooperation, and analyse the observed network formation.     

Surgical treatment of hypoplastic left heart syndrome: limited success

OBJECTIVE: To inventory the treatment of hypoplastic left heart syndrome (HLHS) in the Netherlands and its results. DESIGN: Retrospective. METHOD: Data were collected from all patients (n = 117) diagnosed with HLHS in the Wilhelmina Children's Hospital-University Medical Center Utrecht and the University Hospital Rotterdam-Sophia Children's Hospital and born in the period 1 March 1988-31 May 2000. Type and time of intervention, and mortality were recorded and cumulative survival was analysed by Kaplan-Meier analysis. Cumulative survival was compared between early and late series and between the two hospitals. RESULTS: The study group comprised 68 boys and 49 girls, all neonates. At the time of the investigation, the mean duration of follow-up was 185 days (range: 0-3855). Fifty-eight children had received no treatment; all of these had died. Fifty-nine children were scheduled for the Norwood procedure; six of them died before operation. The 53 patients who underwent the first stage of the Norwood procedure had 1-month, 1-year, 2-year, and 5-year survival chances of 55%, 30%, 27%, and 24% respectively. Survival chances between the two time periods and the two hospitals showed no significant differences. CONCLUSION: The Norwood procedure was performed in almost half of the children with HLHS. It is only moderately successful; however, it seems the only realistic choice in the management.     

Less severe retinopathy of prematurity induced by surfactant replacement therapy

To assess the effect of surfactant replacement therapy (SRT) on the prevalence and severity of retinopathy of prematurity (ROP), we compared data from 160 SRT-treated preterm infants with data from 230 historic controls. The prevalence of ROP was 30.6% in the treatment group and 23.4% in the control group. Severe ROP (stages 3-4) was seen in 6.1% of the infants with ROP in the treatment group and 20.3% of the ROP patients in the control group. Surfactant therapy had no influence on the prevalence of ROP (odds ratio 1.4, 95% confidence interval 0.797-2.459, p = 0.242). However, SRT was associated with a decreased risk for severe ROP, compared to mild ROP (odds ratio 0.226, 95% confidence interval 0.056-0.905, p = 0.036). These data suggest that SRT is associated with a decreased risk for severe ROP.