Enteral Nutritional Support by Percutaneous Endoscopic Gastrostomy in Children with Congenital Heart Disease

One of the major problems of children with severe congenital heart disease (CHD) is their poor nutritional status. Among other consequences, it influences the surgical outcome. Retrospectively we present our experience with percutaneous endoscopic gastrostomy (PEG) in 15 children with CHD. This technique allows enteral nutritional support without the disadvantages related to long-term nasogastric tube feeding. Major complications were absent, and minor complications were rare both at PEG insertion, which was performed under deep sedation, and during feeding via PEG tube. In 4 of the 8 children who were followed for at least 6 months the age-matched body weight increased more than one standard deviation. In 2 other patients it increased more than 0.5 standard deviations. In 7 children the tube was removed after 2.5 to 42 months since enteral support was no longer necessary. Apart from initial reservations the parental acceptance of PEG was good. We conclude that the PEG is a safe and reliable technique to support enteral nutrition in children with severe CHD.     

Ventricular Arrhythmias: When to Worry

Although isolated premature ventricular contractions may be seen in as many as 15% of normal newborns, one third of normal adolescents, and two thirds of adolescents and adults with repaired heart disease, sustained ventricular arrhythmias are relatively rare in young normal hearts. Sudden cardiac health is rare in young normal hearts, although there is an increased incidence in dilated cardiomyopathies and following repair of particular congenital heart lesions. Noninvasive and invasive techniques imperfectly stratify these patients. Patients with cardiomyopathy often have ventricular arrhythmias, although the risk of mortality is more closely linked to ventricular function. There are many infants and pediatric patients with apparently normal hearts who have combinations of asymptomatic nonsustained ventricular tachycardia and potentially serious symptoms. The clinical concern is to identify diagnoses such as long QT syndrome associated with recurrent cardiac syncope and premature mortality so that appropriate choices can be made regarding drug and device therapy. Although this broad range of disease places a premium on careful evaluation, selective therapy, and continued research, serious symptoms, even in the absence of ectopy, are concerning in any patient.     

Severe Intrauterine Growth Retardation, Aged Facial Appearance, and Congenital Heart Disease in a Newborn with Johanson–Blizzard Syndrome

We report a female newborn with Johanson–Blizzard syndrome associated with extreme intrauterine growth retardation, aged facial appearance, and atrial septal defect. Other features are microcephaly, prominent veins over the scalp, alopecia over the vertex, wide-open fontanelle, high forehead, antimongoloid slant, edematous eyelids, the absence of eyebrows and eyelashes, beaked nose with alae nasi, low-set ears, thin lips, and micrognathia. Investigations revealed deafness and congenital hypothyroidism. We believe that this association of severe intrauterine growth retardation and congenital heart disease represents the components of this syndrome.     

A Cross Between Truncus Arteriosus Communis and Aortopulmonary Septal Defect: A Hitherto Undescribed Entity

Case history and necropsy findings of a 5-month-old infant with a unique heart defect with features of truncus arteriosus communis and aortopulmonary defect in combination with severe tricuspid stenosis are presented. There is a wide spectrum of remarkable heart defects between truncus arteriosus communis and aortopulmonary septal defect.     

Heparin and in-vitro experimental lung hypoplasia

 Pulmonary hypoplasia (PH) is a leading contributor to the lethality of congenital diaphragmatic hernia (CDH). Studies now suggest that PH arises prior to visceral herniation. Growth factors (GF) are pivotal to this embryonic lung growth. With striking in-vitro effects on lung morphogenesis, GF are under investigation as therapies for PH. Heparin modulates the kinetics of heparan-sulphate binding ligands that drive lung development. We hypothesised that heparin may rescue PH by favourable alteration of endogenous pulmonary GF activity. Normal and hypoplastic lung primordia were microdissected on day 13.5 of gestation and cultured for up to 78 h in plain media with and without heparin. In-vitro morphological development was studied by serial measurements of terminal bud count, lung area, and lung perimeter. Nitrofen-exposed lungs cultured with heparin showed no significant improvements in terminal bud count, lung area, and lung perimeter at 30, 54, and 78 h compared to untreated hypoplastic lungs maintained in vitro. In normal lungs heparin demonstrated no sustained significant morphological effects compared to untreated control lungs. In this study, heparin did not stimulate branching morphogenesis of normal or hypoplastic lungs in our organ culture system. Known at higher concentrations to inhibit smooth-muscle proliferation, heparin may ameliorate pulmonary vascular hypermuscularisation with the prospect of benefiting CDH infants on extracorporeal membrane oxygenation. Future studies will address the impact of exogenous GF on hypoplastic lung development in organ culture.     

Roux-en-Y jejunostomy button in infants

Establishing enteral feeding in high-risk infants with significant gastroesophageal reflux is a difficult challenge. Some patients are considered at very high risk for fundoplication and gastrostomy due to unstable medical conditions, dense upper-abdominal adhesions due to previous surgical procedures, or unfavorable anatomy. We describe a less invasive operation that provides jejunal feeding in a way that is safe, reliable, and convenient for the family. Accepted: 4 March 1999     

Expanded use of surfactant replacement therapy

There are a number of respiratory diseases affecting infants in which there is surfactant dysfunction or deficiency. Surfactant is inactivated by cholesterol, free fatty acids and bilirubin in meconium aspiration syndrome, by haemoglobin and red blood cell lipids in pulmonary haemorrhage and plasma proteins are the culprit in conditions associated with increased alveolar capillary permeability. Surfactant production can be adversely affected by damage to the type 2 pneumocytes by viruses and neutrophil derived reactive oxygen metabolites. Not surprisingly, therefore, the efficacy of exogenous surfactant has been tested, usually in animal models and anecdotal series in “non respiratory distress syndrome” respiratory disorders. Improvements in oxygenation have usually been described. Relatively few randomized trials, however, have been performed, but those undertaken have demonstrated longer term benefits. In term infants with severe respiratory failure, surfactant administration significantly shortened the duration of extracorporeal membrane oygenation and, in those in the early phase of severe respiratory failure or with meconium aspiration syndrome, it significantly reduced the need for extracorporeal membrane oygenation. In meconium aspiration syndrome, a smaller number of surfactant treated patients compared to controls developed airleaks. Surfactant administration was also associated with a reduction in the duration of ventilation and intensive care unit stay in patients with meconium aspiration syndrome or bronchiolitis. Those data are very promising and should encourage studies to identify the optimum type of surfactant, dosage regimen and administration method. Conclusion Further randomized trials are required to fully assess the efficacy and cost benefit ratio of surfactant treatment in “non respiratory distress syndrome” respiratory diseases. Received: 24 January 2000 / Accepted: 6 April 2000     

Insulin-like growth factor binding protein-3 proteolysis and growth of athyreotic infants in the first weeks of life

To gain a better understanding of the growth of athyreotic newborns in the first weeks of life, we evaluated auxological parameters and determined the serum levels of growth hormone (GH), insulin-like growth factor I (IGF-I) and free IGF-I, and IGF-binding protein-3 (IGFBP-3) in 15 hypothyroid infants (10 females) at a mean age of 25 d of life, immediately before the beginning of L-thyroxine therapy, and at 3 and 6 mo of life. Fourteen normal infants (9 females) of the same age were studied as controls. IGFBP-3 proteolytic activity was evaluated in 8 patients and in 8 controls at 25 d and 6 mo of life. There was no significant difference concerning weight and length between the patients and controls at birth, 25 d, 3 and 6 mo of life. The blood GH, IGF-I and IGFBP-3 levels were significantly lower in patients at diagnosis than in controls of the same age (p < 0.01 for all parameters), as well as IGFBP-3 studied by Western blotting. At diagnosis, the patients' free IGF-I level was within the control range, but the free IGF-I percentage of total IGF-I was higher than in the controls (p < 0.01). IGFBP-3 proteolytic activity was found to be greater in the patients (p < 0.01). At 6 mo of life, after therapy, none of these parameters was different from those of the controls. CONCLUSION: Increased IGFBP-3 proteolytic activity in our patients at diagnosis, favouring IGF-I bioavailability, could account for normal free IGF-I levels and in turn for their normal growth pattern during the first weeks of life and before the start of treatment.     

Centralization of Pediatric Heart Surgery in Sweden

In Sweden, which has a population of 8.9 million people, pediatric heart surgery was previously performed in four cities. After a long, difficult process, centralization of pediatric heart surgery to two centers was achieved in 1993. The overall 30-day mortality for open-heart surgery on infants and children of 9.5% before the centralization (1988–1991) was reduced to 1.9% in 1995–1997. A causal relationship between the mortality rates before and after the centralization is impossible to prove. Heart surgery was concentrated to the two centers with the lowest surgical mortality, and the reduction in surgical mortality was observed over a short period of time which makes it likely that the centralization of the surgical activity promoted the improved results. During the later time period the amount of more complex surgery was clearly increased compared to that performed previously.     

经导管介入治疗室间隔缺损的现状及进展

室间隔缺损(VSD)是新生儿期最常见的先天性心脏病。后天性VSD少见,主要见于外伤引起的室间隔破裂和急性心肌梗死导致的室间隔穿孔。传统的治疗方法是外科手术,随着介入器材及技术的不断发展,介入治疗VSD已成为可行的治疗方法。现就近年来VSD介入治疗的现状及进展予以综述。