Open heart interventions in premature low- and very-low-birth-weight neonates: risk profile and ethical considerations.

In premature, very-low-birth-weight (VLBW) neonates, complex cardiac malformations can be successfully repaired under conditions of cardiopulmonary bypass. However, due to the immaturity of organ systems, these patients are exposed to a specific risk resulting from noxious effects of extracorporeal circulation, especially on the central nervous system. Two premature neonates with low and very low birth weight of 1160 g and 1650 g, were operated on using cardiopulmonary bypass for severe pulmonary artery stenosis and truncus arteriosus communis type II, respectively. The neonate with pulmonary valve stenosis survived, but at 2-year-follow-up examination motoricity retardation as a result of cerebral immaturity-related changes was evident. The other neonate died suddenly on the fifth postoperative day of a massive intracranial haemorrhage. Due to the fact that the natural history of VLBW children is a priori characterized by a high incidence of major neurological handicaps, open heart surgery may by improving survival chances contribute to an increased incidence of mentally handicapped children.

Left-side pulmonary vein obstruction after arterial switch operation in infants with D-transposition of the great arteries.

We describe two cases of left-side pulmonary vein obstruction observed after the arterial switch operation (Jatene) for D-transposition of the great arteries. This appears to be related to left-sided pulmonary vein obstruction occurring coincidently with D-transposition of the great arteries, rather than a consequence of arterial switch operation.

Spiral CT angiography in an infant with severe hypoplasia of a long segment of the descending aorta.

CT angiography, or the spiral CT technique, is a promising minimally invasive method of visualising the arterial vascular system and can be applied in children in whom ultrasound, MRI and/or angiography or cardiac catheterisation cannot be performed, or where an exact diagnosis cannot be made. CT angiography should be considered in special cases as a diagnostic alternative to MRI, ultrasound and angiography. As an example of the possibilities of CT angiography, a case is described in which hypoplasia of the descending aorta was diagnosed and a postoperatively encountered perigraft reaction was demonstrated. Perforation of the blood vessel could be excluded by CT angiography.

Neurogenic lesions after posterolateral thoracotomy in young children.

In follow-up examinations of young patients we often noticed upward displacement of the left shoulder in cases where left-sided posterolateral thoracotomy had been carried out. Finding little discussion of this side-effect in the literature, we undertook the present study. The shoulder girdle of 69 patients who had undergone surgery for coarctation of the aorta between 1964 and 1984 was carefully examined to assess displacements and dysfunctions. In addition, in 24 of the patients electrodiagnostic examination was carried out of the associated muscles and nerves which were affected by the operation. We found disturbances of the muscular apparatus of the shoulder girdle in 80% of the cases: upward displacement of the shoulder (55%), downward displacement of the shoulder (8.7%), deviation of the scapula at rest (72.5%) and maximal elevated arms (40.6%), scapula alata (56.5%), and alterations of the posterior axillary line (39.1%). When considering only the cases of distinct upward or downward displacement of the shoulder, at least 25% of the operated children were affected. Children operated early (surgery during the first year of life) had more distinct alterations (47.6%) than those with later surgery (14.6%). Electromyographic examinations indicate that mainly disorders of the peripheral nerves caused by the operation led to these alterations of the shoulder girdle musculature. Although, except for one patient, no functional disorders were found, there were several cases in which the alteration caused quite severe cosmetic problems. One should pay more attention to positioning for surgery and do careful preparation to reduce these sequelae.

[Silent persistent ductus arteriosus]. / Der stumme persistierende Ductus arteriosus.

We report on 14 observations undertaken by ourselves of silent persistent ductus arteriosus (SPDA). During a three year period with the routine use of color doppler flow evaluation we found cases of SPDA in 14 children. A very little ductus arteriosus is already known to us through earlier heart catheter examinations which were undertaken because of other isolated heart defects (16 cases in 345 isolated heart defects over 10 years). We also consider the fact that SPDA is not an infrequent result after the occlusion of a patent ductus arteriosus with the Rashkind-Occluder-System and after operative ligatures of patent ductus arteriosus. Finally, we compare our results concerning the frequency of SPDA with current literature and discuss consequential therapies.

[Peri-graft reaction. A complication after implantation of a prosthesis shunt in children with congenital cyanotic heart defects]. / Perigraft-Reaktion. Eine Komplikation nach Anlage eines Prothesenshunts bei Kindern mit angeborenen zyanotischen Herzfehlern.

Two different clinical manifestations of perigraft-reactions complicating the implantation of prosthetic aortopulmonary shunts in children with cyanotic congenital heart disease are described and discussed. The incidence of this complication in the past 5 years in our own patients was 3%. The appearance of a localized mass on the chest film surrounding the prosthesis requires the exclusion of a hematoma, aneurysm or inflammation. In the case of a massive serous pleural effusion examination of the ingredients leads to diagnosis. Concerning our own patients the fluid is identical with serum; the leakage through the prosthesis can therefore be called plasmapheresis. The claimed pathogenetic causes are discussed intensively, including a chylothorax promoting this complication in the second case. Local variations in porosity and structure of the implanted prosthesis may play a critical role in the pathogenesis of perigraft-reaction, as indicated by both our reported cases. In the event of unsuccessful observation of a localized perigraft seroma or of unsuccessful chest drainage of a massive pleural serous effusion, graft replacement and placement of a different graft material are found to have the best results.

Left hemitruncus in adulthood: diagnostic role of magnetic resonance imaging.

The diagnosis of left hemitruncus and large patent ductus arteriosus was made by magnetic resonance imaging in an adult patient with recurrent haemoptysis and dyspnoea on exertion. Previous cardiac catheterization and echocardiography failed to establish the complete diagnosis. Magnetic resonance imaging using spin-echo and gradient-echo pulse sequences is a useful imaging modality to evaluate anatomical and functional abnormalities in patients with complex congenital heart disease.

[Tracheal instability in tracheo-esophageal abnormalities]. / Trachealinstabilität bei tracheo-ösophagealen Fehlbildungen.

The clinical pattern of signs and symptoms of respiratory complications due to flaccid trachea has been analysed in 83 children treated in our hospital between 1983 and 1988 for tracheo-oesophageal malformations. These signs and symptoms are classified according to endoscopic findings, and are thus arranged according to various degrees of severity. Of the surviving children who were followed up and who were suffering from oesophageal atresia Vogt III B, only 5 of 57 were without a pointer towards flaccid trachea, whereas in oesophageal atresia Vogt II there were two of three. The two children with an isolated tracheo-oesophageal fistula showed abnormal findings both clinically and via endoscopy. 16 of the 20 children with very severely pronounced flaccid trachea--defined by the occurrence of life-threating apnoeas and an endoscopically identifiable tracheal collapse of more than two-thirds of the lumen--were subjected to surgery via aortosternopexy. A marked and identifiable improvement was obtained in 15 cases. Complications caused by surgery consisted of temporary phrenicus lesions in two cases.

Balloon valvuloplasty of pulmonary stenosis in infants and children--co-operative study of the German Society of Pediatric Cardiology.

Balloon valvuloplasty (BVP) of pulmonary stenosis (PST) was performed in 305 infants and children in 20 heart centres of the Federal Republic of Germany. 273 patients suffered from isolated PST. Their age ranged from 3 days to 18 years (mean = 5.85 +/- 4.82 years), their weight from 2.9 to 60 kg. The pressure gradient was measured invasively in the sedated or anaesthetized child, partly by CW Doppler. BVP was performed by mono- or trefoil catheters, mostly oversized. As a result, mean pressure gradient fell from 71.7 +/- 32 mmHg before to 31.8 +/- 25 mmHg immediately after BVP (P less than 0.0001). The gradient was reduced by less than 30% in 13% of the patients, by 30-49% in 23%, and by greater than or equal to 50% in 64%. Among 33 patients with a gradient reduction of less than 30%, nine were aged less than 1 year, nine had dysplastic valves, and nine had a short-term infundibular reaction and good late results. Gradient reduction was not improved with oversized balloons and was independent of the initial gradient. Follow-up for a mean 11.2 +/- 8 months showed a further slight improvement, probably due to regression of infundibular hypertrophy. In the whole group we observed infundibular reaction in 12%, serious complications with one late death in 4%. From the start, BVP proved to be a secure and effective tool for elimination of pulmonary stenosis.

[Non-invasive diagnosis of atrial septal defects and anomalous pulmonary venous return using magnetic resonance tomography]. / Nichtinvasive Diagnostik von Vorhofseptumdefekten und fehlmündenden Lungenvenen mit der Kernspintomographie.

The present study was to evaluate the diagnostic information that may be obtained in patients with atrial septal defect (ASD) and/or with anomalous pulmonary venous return using spin echo and gradient echo magnetic resonance imaging (MRI). The interatrial septum and pulmonary veins were studied in 31 patients and in 16 healthy volunteers. In all patients cardiac anatomy and dilatation due to right-sided volume overload was well depicted. In 87% of the patients examined by spin echo technique and in 100% of the patients examined by gradient echo technique the defect was reliably documented. All cases of anomalous pulmonary venous return were shown. MRI diagnosis of ASD was improved by combined use of spin echo and gradient echo techniques. The new MRI sequence was particularly helpful in patients with small ASDs and anomalous pulmonary venous return.