[The heart-hand syndrome. A new variant of disorders of heart conduction and syndactylia including osseous changes in hands and feet]. / Herz-Hand-Syndrom. Eine neue Variante mit Erregungsleitungsstörungen am Herzen und Syndaktylien einschliesslich ossärer Veränderungen an Händen und Füssen.

A male infant was born at the 36th week of gestation by cesarean section because of an intrauterine bradycardy. There was a 2nd degree a. v.-bloc postnatal. The heart rate was normal after the 5th day of life with a QT-interval of 150 percent resp. 120 percent after the 15th day. Additionally there were syndactylies of hands and feet. The infant suddenly died at the age of 5 months. Examination of the parents revealed a QT-interval of 115 percent as well as synostoses of the carpus in the father. These microsymptoms are very important for genetic counseling of the family. There is a recurrence risk of 50 percent for further children because a dominant trait is to be suggested.

[Quantitative Doppler echocardiography diagnosis of congenital aortic and pulmonary artery stenoses]. / Quantitative Doppler-echokardiographische Diagnostik von angeborenen Aorten- und Pulmonalstenosen.

The aim of the present study was to check the value of cw-Doppler echocardiography for the assessment of severity of congenital aortic and pulmonary stenoses in children and adolescents. Over a period of 3 years 217 children and adolescents with aortic and 98 with pulmonary stenosis, resp., underwent echocardiography. In 21 patients with aortic and in 23 with pulmonary stenosis pressure gradients were determined by cw-Doppler as well as intracardiac pressure measurement. The comparison showed excellent accordance between both sorts of data for aortic stenoses (r = 0.939) and pulmonary stenosis (r = 0.969). These results show that Doppler echocardiography is a reliable diagnostic method for the determination of pressure gradients. Thus, it allows correct selection of children and adolescents with aortic stenosis for surgery. So far, 12 patients have been operated on without preoperative cardiac catheterization. Indication for balloon valvuloplasty in patients with valvular pulmonary stenosis can be based safely on 2 D- and Doppler echocardiography.

[Bacterial heart diseases in childhood. An overview of diseases 1971-1987]. / Bakterielle Herzerkrankungen im Kindesalter. Eine Ubersicht der Erkrankungen von 1971-1987.

The authors give a review of patients with bacterial cardiac disease treated in the Paediatric Clinic of Karl-Marx-University Leipzig between 1971 and 1987. During this period 32 patients were observed, which amounts to about 2 to 3 patients per year. The majority of patients had congenital heart disease. Despite antibiotic therapy the prognosis of this infection is still serious: One third of the patients died, and diagnosis is made with considerable delay in some cases. Problems and diagnostic and therapeutic approaches are discussed.

[Echocardiography diagnosis of persistent ductus arteriosus]. / Echokardiographische Diagnostik des persistierenden Ductus arteriosus (PDA).

Over a period of 2 years PDA was diagnosed in 151 children by means of 2-D-echo combined with Doppler technique. In 47 patients the lesion was also visualized by colour coded Doppler echocardiography. Sixty-seven children had isolated ductus arteriosus, whereas the others had additional cardiac malformations. In 90 patients PDA was confirmed by invasive diagnosis and/or operation or by autopsy. The diagnosis was made on the first echocardiographic examination in all but 2 infants with subaortic ventricular septal defect and pulmonary hypertension. The practical procedure is described in detail. The authors conclude that combined two-dimensional and Doppler echo allow a safe diagnosis of PDA.

[Oxacillin-induced granulocytopenia]. / Uber Oxacillin-induzierte Granulozytopenie.

A girl at the age of nearly 13 years with a haemodynamic insignificant defect of the ventricular septum fell ill with a staphylococcal endocarditis of the aortic valve. After a high dosage therapy with oxacillin for some weeks occurred a leucocytopenia which was reversible after discontinuation of this therapy. Because we could find only few reports about this problem in the german literature, we will report about our patient and give a short survey of literature.

[Staphylococcal endocarditis in the mitral valve prolapse syndrome]. / Uber Staphylokokken-Endokarditis beim Mitralklappenprolaps-Syndrom.

A girl, aged 13 8/12 years, in whom has been known a syndrome of the mitral valve prolapse since the 10th year of age, fell ill with an acute endocarditis by staphylococcus aureus. As the therapy with antibiotics remained without any effects and due to an early embolic dispersion including the brain an operative approach was no more possible death occurred. The pathologic-anatomical investigations resulted in a distinct ulceropolypous endocarditis of the mitral valve. At the instance of this case report the most important facts nowadays known about the relation between the syndrome of mitral valve prolapse and endocarditis are summed up.

[Subclavian steal syndrome in childhood (author's transl)]. / Subclavian-Steal-Syndrom im Kindesalter.

The authors report on four children with subclavian steal associated with coarctation or interrupted aortic arch. All children showed a stenotic origin of the left subclavian; in addition three of them had an extreme hypoplasia of the proximal segment of this artery. Two children had typical symptoms of insufficiency of the vertebral and basilar circulation (headache, dizziness, syncopal attacks, vision and hearing disorders) since the age of 7 and 8 years, respectively. The clinical findings, such as reduced pulses and blood pressure of the respective arm, may suggest the provisional diagnosis. The principles of congenital subclavian steal are discussed.

[Post-perfusion syndrome in childhood (author's transl)]. / Das Postperfusionssyndrom im Kindesalter

Following cardiac surgery with extracorporeal circulation three children became ill with a postperfusion syndrome. Besides typical clinical and haematological findings (fever, hepatosplenomegaly, lymphomonocytosis with "atypical" lymphocytes) demonstration of characteristic antibody sequential titres (IgG and IgM antibodies against cytomegalovirus) was possible. The importance of this syndrome rests in the problems of differential diagnosis which occur at the beginning of clinical symptoms (differentiation from endocarditis, septicaemia, hepatitis).

[Results following surgery of fibrous subvalvular aortic stenosis in childhood and adolescence]. / Ergebnisse nach Operation von subvalvulären fibrösen Aortenstenosen im Kindes- und Jugendalter

From 1963 up to the end of June 1974 32 patients were operated on for fibrous subvalvular aortic stenosis in Leipzig (age at operation 6-17 years). 18 patients had concomitant cardiovascular malformations, 4 patients died. The results are discussed in detail. Contrary to other publications the authors adopt the attitude that the operation in subvalvular stenosis is to be recommended even with gradients as low as 50 mm Hg, particularly when haemodynamically significant concomitant malformations are present. Therefore, the measuring of the left ventricular pressure and the localisation of the stenosis by intracardiac diagnostics should be performed not too late. It may be possible that the measuring of the left ventricular pressure must be repeated some years later. Also for the exact evaluation of the post-operative results recatheterisations are necessary.