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.

[Magnetic resonance tomography in pediatric cardiology]. / Die magnetische Resonanztomographie in der pädiatrischen Kardiologie.

Magnetic resonance imaging (MR) is a relatively new non-invasive imaging method that has not been used as a routine procedure in pediatric cardiology. To evaluate MR in pediatric cardiology we performed 110 examinations in 91 children during the period from December, 1984, to November, 1987. The children were divided into the following groups: 1) Diseases of the great vessels (54 examinations in 39 children); 2) Cardiac diseases (42 examinations in 39 children); 3) Diseases of the pericardium (four examinations in three children); 4) Ten children without cardiovascular diseases (imaging of the heart in conjunction with examination of other organs). The ages of the children ranged from five weeks to 19 years in the following distribution: 13% infants, 20% children 2-5 years, 30% children 6-14 years, 37% adolescents from 15 to 19 years. On the basis of our experiences we conclude that MR can be used as an additional diagnostic tool in pediatric cardiology, especially for imaging of areas that are not well visualized by echocardiography, i.e., the great vessels and their branches and in addition, the right ventricle, in older children.

[Noninvasive determination of oxygen saturation using pulse oximetry in pediatric cardiology]. / Die nicht-invasive Bestimmung der Sauerstoffsättigung mittels Pulsoxymetrie in der pädiatrischen Kardiologie.

Pulse oximetry is a relatively new noninvasive method to measure arterial oxygen saturation (SO2). We evaluated the reliability of a pulse oximeter (N-100, Nellcor/Drger, Lübeck) in single and continuous measurements of SO2. During cardiac catheterisation we compared pulse oximeter (puls.SO2) measurements to those in simultaneously taken arterial samples (art.SO2), and obtained 203 pair measurements in 85 patients, mostly newborns and infants; in 20 of them before and during the breathing of oxygen through a mask. We also have 67 pair measurements of capillary blood samples (kap.SO2) and puls.SO2. Continuous measurements with the pulse oximeter were carried out in 17 patients for up to 116 h. Our main results are: 1. In the puls.SO2 range of 66% to 100% the measurements with the pulse oximeter were accurate (r = 0.97, Syx = 2.1). Below 66% the puls.SO2 values were on average much higher than the art.SO2 values, with an increased variance. Intraindividual oxygenation changes before and during the breathing of oxygen were accurately measured. 2. Pulse oximetry is much more informative than kap.SO2 measurements, because its information about the SO2 before blood sampling (= resting value) and following SO2 changes during the painful procedure (= stress values). 3. Continuous measurements with the pulse oximeter are very simple and very reliable and are not associated with skin injury. Because the pulse oximeters responses rapid to oxygenation changes and does not need calibration it is very valuable in the assessment of therapeutic procedures in patients with cyanotic heart disease. We conclude that the pulse oximeter is a very important new diagnostic tool in pediatric cardiology.

[Diagnosis and treatment of cardiac rhabdomyomas]. / Diagnostik und Behandlung der kardialen Rhabdomyome.

A diagnosis of primary cardiac tumors was achieved by two-dimensional echocardiography in 3 patients. Two of them were female neonates, born of the 29th and 40th gestational week who had shown intrauterine arrhythmias. Subsequently, we were able to diagnose tuberous sclerosis in both. The third child, a 121/2 year old boy, with already diagnosed tuberous sclerosis, presented an electrocardiographical preexcitation syndrome. Because of the multiplicity of the tumors and the association with tuberous sclerosis we presumed that the tumors were rhabdomyomas. As it is known that rhabdomyomas show no, or only a minimal postnatal growth, we decided neither to perform a heart catheterization nor to treat them surgically unless the children show hemodynamical deterioration, although the tumors were huge in both neonates. The subsequent 14 months showed a clear regression in the size of the tumors. One of the neonates was successfully treated with amiodarone because of ventricular tachycardia. The second neonate didn't require any treatment. We conclude that: Surgical management is only necessary in patients presenting with cardiac insufficiency and/or arrhythmias that are resistant to conventional medical treatment. Primary cardiac tumors should be looked for in patients presenting with arrhythmias, even when occurring already during intrauterine life. Diagnostic measures in order to diagnose tuberous sclerosis in patients with cardiac tumors are necessary and vice versa.

[Diastolic micropotentials in high-resolution surface ECG in QT syndrome]. / Diastolische Mikropotentiale im hochverstärkten Oberflächen-EKG bei QT-Syndrom.

Patients suffering from long QT syndrome are threatened by torsade de pointes tachycardias and sudden arrhythmic cardiac death. An inhomogenic sympathetic innervation of the heart with dominance of the left cervicothoracic sympathetic nerves has been considered to be a major cause of life threatening cardiac arrhythmias. This study presents the electrocardiographic and electrophysiologic results of 7 patients with long QT syndrome. In agreement with data published earlier our results of Holter monitoring, exercise testing and programmed electrical right ventricular stimulation were of no diagnostic or prognostic significance in predicting syncopal attacks or sudden arrhythmic cardiac death. Thus, the high resolution ECG methods played an important role in this study. During noninvasive recordings of signal averaged ECGs and high resolution surface ECGs with beat to beat registration, diastolic microvolt potentials could be detected in 6/7 patients within the ST segment and in 5/7 patients after the T wave. Our results evidently show that the signal averaged ECG and the high resolution surface ECG could be of diagnostic significance in patients with long QT syndrome.

Abnormal communication between the left atrium and the coronary sinus. Presentation of 2 cases and review of the literature.

A large communication between coronary sinus and left atrium is a rare cardiac anomaly. Twenty-four cases from the literature and 2 of our own observations are presented. In most cases a considerable left-to-right shunt may exist on the atrial level that can be misinterpreted as an atrial septal defect. The anomaly may be isolated or in combination with an atrial septal defect or complex cardiac malformations. It is difficult to diagnose this abnormality by cardiac catheterization and even during surgery. Continuous oximetry during a fiberoptic catheter pull-back in the coronary sinus ensures the diagnosis in the case of atrial left-to-right shunt. The anatomy, hemodynamics, diagnostic procedures, and the therapy of the cases reported are discussed.

Idiopathic mediastinal fibrosis. A case report on its surgical treatment.

A case is presented in which mediastinal fibrosis had extended to involve the right lung, the diaphragm, the lower vena cava near the heart, and both atria including the interatrial septum. There is no previous report of this disease in such an extensive form. The relatively unusual clinical picture is discussed with regard to the pathophysiologic, clinical, diagnostic, and therapeutic problems it presents. The diagnosis could only be established postoperatively in our patient. In the course of a thoracotomy carried out to resect a neoplasm, an extensive, infiltrative tumorous process was discovered which, on the one hand, could not be classified histologically by immediate section under surgery and, on the other, showed no signs of malignancy. A radical operation was, therefore, performed in the form of a right-side pneumonectomy with partial resection of the diaphragm, lower vena cava, right and left atria and interatrial septum with plastic reconstruction of these structures. The patient survived surgery, and 3 years later, is in good health without any indication of residivation.

[Cortical hyperostoses after long-term prostaglandin E2 therapy]. / Kortikale Hyperostosen unter längerfristiger Prostaglandin E2-Therapie.

Cortical hyperostoses are found to be side effects after therapy in early infancy with prostaglandin E1 and E2. Correlation seems to exist between dosage and duration of therapy. Radiologically the lesions cannot be differentiated from Caffey's disease. Pathogenetic relationship of the two diseases is discussed; 62 cases of newborn children with ductal related vitium cordis, who were treated for a short time or for longer with prostaglandin E2 are demonstrated.

[Antegrade catheterization of the aorta with a flow-directed balloon catheter following transseptal left atrial puncture: a new technique]. / Antegrade Sondierung der Aorta mit dem Flow-directed-Ballonkatheter nach transseptaler Punktion des Vorhofseptums.

The described heart catheterization technique allows the antegrade catheterization of the aorta from the left ventricle with a Swan-Ganz-flow-directed catheter via the saphenous vein and subsequent puncture of the intact atrial septum. The transseptal Brockenbrough-technique is modified by the use of a long thin polythene sheath which is fitted over the transseptal left heart catheter and can be advanced over the catheter after atrial septal puncture. A Swan-Ganz-flow-directed catheter can be passed directly to the left ventricle toward the outflow tract before it enters the aorta. This technique was used successfully on 18 children. By avoiding the retrograde arterial catheter technique additional information will be obtained.