A double-blind placebo controlled trial of diltiazem in Duchenne dystrophy.

The role of calcium accumulation in the pathogenesis of Duchenne muscular dystrophy (DMD) has already been discussed. Several trials with different calcium-blocking drugs have revealed no clinical benefit. In addition, the present study includes histological investigations and computer tomography to verify therapeutic effects. In a randomized placebo-controlled double-blind study, 13 DMD patients aged from 3-10 years (mean, 7 years) were treated with 5 mg/kg diltiazem daily for 1 year. Compared with before therapy, the number of calcium-positive muscular fibres was remarkably reduced in the treated DMD patients, but not in the placebo group. The evaluation of all other biochemical and clinical parameters revealed no significant effects of the diltiazem therapy. The muscular X-ray density measured by computer tomography decreased under treatment. After the evaluation of the double-blind study, the code was broken. Therapy, however, was continued in the treated group and started in the placebo group. After 3 years of diltiazem therapy the clinical status of all 26 patients of the study and 20 additional DMD patients who were treated with diltiazem was compared with 46 untreated DMD patients of the same age and stage in our department. No obvious clinical benefit of diltiazem therapy could be observed.

[Psychologic adjustment in pediatric cardiologic interventions. Observations and recommendations]. / Psychische Bewältigung kinderkardiologischer Eingriffe. Beobachtungen und Empfehlungen.

Under the assumption that no appropriate psychological care of children exists in the area of pediatric cardiology, a pediatric-cardiologic team worked together with a child-and-adolescent-psychiatrist to define the possibilities of preventing psychic traumatisation associated with cardiac interventions on children. The emotional reactions of children between 5 and 15 years of age and of their parents in connection with a cardiac catheter examination, as well as the role of the intervening doctor in his own eyes and in the eyes of the child, will be described. Practical recommendations for patients, families and institution follow, concerning the preparation of cardiac interventions with children and a detailed preparation program for cardiac catheter examination.

The determination of pharmacokinetic parameters of ketotifen in steady state in young children.

Ketotifen kinetics at steady state in small children compared to adults show very good absorption for a dose of 2 X daily 1 mg. Relative to adult kinetics, a higher dose in relation to body weight is justified, since a more rapid kinetic pattern was proven for the substance in the volunteer collective tested.

[Hypoplastic left heart syndrome--clinical and pathologico-anatomic findings]. / Das hypoplastische Linksherzsyndrom--klinische und pathologisch-anatomische Befunde.

In twenty-two cases with hypoplastic left heart syndrome the anamnestic criterias and the clinical features were analysed. The pathologic specimens were examined in thirteen cases. The relations of the left heart to the right heart were measured. The same information is to be measured by two-dimensional echocardiography intra vitam. It should be discussed whether it is possible to perform an operative procedure in some rare cases of hypoplastic left heart syndrome.

Congenital aortic thrombosis with complete obliteration of the aortic arch and the great vessels.

Thrombosis of the great arteries rarely occurs in the neonate. We report a case of thrombosis of the aortic arch, the brachiocephalic truncus, the left carotid and subclavian arteries, seen in a one-day-old neonate. The clinical findings were at first misdiagnosed as interrupted aortic arch syndrome, though, in retrospect echocardiography was very suggestive of the correct diagnosis. On the basis of the histological examination we assume, as the most likely cause for thrombosis, an aortitis in the prenatal period. Following thrombectomy and Gore-Tex bypass from the ascending to the descending aorta, combined with ligature of a PDA, the child developed a low output state and died on the table. The postmortem examination revealed that the latter was due to multiple infarcts of the left ventricular myocardium.

[The angiocardiographic image following determination of axial-oblique projection with 2-dimensional echocardiography]. / Kardioangiographische Darstellungen nach Bestimmung der axial-schrägen Projektionsrichtung mit zweidimensionaler Echokardiographie.

A precise prediction of axial-oblique angulation in cineangiocardiography by noninvasive investigation is possible. A simple method is described using two-dimensional echocardiography to determine the cross-sectional plane. The perpendicular X-ray beam presents the angiocardiographic feature related to the echocardiographically determined plane. The described method is most helpful in diagnosis of simple morphological changes (ASD, VSD) as well as in complex congenital heart defects. To our knowledge this is the first report of the application of two-dimensional echocardiography to cardioangiographic axial-oblique angulation.

Tachyarrhythmic syncopes in children with structurally normal hearts with and without QT-prolongation in the electrocardiogram.

Forty children with presumed ventricular tachyarrhythmic syncopes in the absence of structural heart disease were studied. Twenty-nine patients, one of whom was deaf, had a prolonged QT-interval in the resting electrocardiogram (Group 1); eleven patients had a normal QT-interval (Group 2). The median QTc-interval vas 0.51 s in Group 1 and 0.40 s in Group 2. Familial occurrence suggesting autosomal dominant inheritance was found in 21 of 28 normally hearing patients in Group 1 and in 2 of 11 patients in Group 2. Syncopes were definitely stress-induced in 22 patients in Group 1 and in all 11 patients in Group 2. Of 23 patients in Group 1 in whom an electrocardiogram was obtained during physical exercise, only one showed severe ventricular dysrhythmia. In contrast, all eleven patients in Group 2 developed severe ventricular dysrhythmia with exercise. Treatment with beta-blocking medication prevented further syncopes in 15 of 19 patients with several previous attacks in Group 1 and in 3 of 5 patients of Group 2. Four of the 29 patients in Group 1 died suddenly and one more remained apallic after an attack. Of the 11 patients in Group 2, four died suddenly and one retains severe cerebral damage after resuscitation from ventricular fibrillation. We conclude that, besides the group of patients with the long QT-syndrome, there may be a distinct group of patients with a consistently normal QT-interval and severe ventricular dysrhythmia with exercise. Patients of both groups are threatened by sudden death and are improved by treatment with beta-blocking medication.

[The percutaneous cardiac catheterization in the diagnosis of congenital cardiac failure (author's transl)]. / Die perkutane Herzkathetereinführung in der Diagnostik kongenitaler Herzfehler.

The results of a modified percutaneous approach of 113 cardiac catheterizations will be reported. The success rate for the right heart catheterization from the unselected cases in 63% in infants, 84% in those one to five years old and 96% in those children older than five years. For the retrograde left heart catheterization, the percutaneous approach was successful in infants in 91% (except one patient with aortic coarctation) and successful in all patients older than one year.

[Haemodynamics in congenital pulmonary lymphangiectasis (author's transl)]. / Hämodynamik bei kongenitaler pulmonaler Lymphangiektasie.

Respiratory insufficiency is the clinical principal sign of cystic pulmonary lymphangiectasis. Clarification of cardiovascular morphology and haemodynamics is the basic prerequisite for the classification and therapy of the disease pattern. The prognosis is unfavourable if it is found that the pulmonary circulation does not flow through the lungs, reaching instead the systemic circulation via the persisting ductus arteriosus. The histopathological findings as well as the results of dissection of the cardiovascular system clarify the question as to the pathogenesis of this disease and allow subdivision of the disease into a primary and a secondary manifestation of cystic pulmonary lymphangiectasis.

[New angles of view in cineangiography of congenital heart disease (author's transl)]. / Neue Projektionsmethoden zur cineangiokardiographischen Darstellung angeborener Herzfehler.

Axial oblique views were performed during cineangiography of congenital heart disease on patients of a paediatric cardiological unit for a period of two years. The resulting angle-patterns are shown in a synoptical review, and indications for angiographic demonstration of morphological conditions in congenital heart disease are discussed. In comparison with the literature to date, this is the first report of experience with a biplane synchronised "C" arm cineangiographic system.

[Stereovectorcardiography. Analysis of congenital excitation-conduction anomalies using a new type of microelectronic equipment (author's transl)]. / Stereo-Vektorkardiographie. Analyse kongenitaler Reizleitungsanomalien mit einer neuen mikroelektronischen Technik.

The results of the first clinical trial of a stereo-VCG used for the quantitative and qualitative analysis of congenital anomalies of excitation-conduction are elaborated and discussed. Normal values for paediatric cardiological patients were calculated for the following parameters with the help of measurements from 78 patients: Pmax, Rmax, Tmax-stereovectors, their elevation and azimut, the stereo-angle between Rmax/Tmax, Pmax/Tmax and Pmax/Rmax as well as the periods from the beginning of the ventricular excitation until Rmax and Tmax. The patients were divided into 4 age groups. The group of normal individuals was compared with 22 patients having anomalies of conduction. The results show that this new technique allows more differentiated analyses of the vector loops registered according to Frank's orthogonal corrected registering system than the conventional measurements in cases of congenital anomalies.

[Cardiomyopathy in juvenile diabetics? (author's transl)]. / Kardiomyopathie bei juvenilen Diabetikern?

Report on a cardiologic study conducted in 26 juvenile diabetics with insulin deficiency, in whom the disease had lasted for more than ten years. Electrocardiograms, vectorcardiograms and echocardiograms were recorded. The investigations did not indicate the presence of a subclinical cardiomyopathy as a sign of an early diabetic microangiopathy of the myocardium. Hence the findings by Riff and Riff (1974) as well as those by Sanderson et al (1978) have not been confirmed.

[Newborn cardiotoxicity after tocolysis with fenoterolhydrobromide]. / Neugeborenen-Kardiotoxizität nach Tokolyse mit Fenoterolhydrobromid?

In the clinical treatment of imminent premature birth, sympathomimetic amines have greatly increased in importance as an interruptor of labor pains. The purpose of this report is to discuss the clinical findings on the basis of 30 newborns admitted to our hospital (between April 1977 and June 1978) after long-term treatment of the mother and Fenoterol (Partusisten). The predominant clinical findings were: tachycardia, paroxysmal dyspnea, cyanosis, metabolic acidosis, congestive heart failure and, in the ECG, cardiac arrhythmias and T-wave inversions in the left precordial chest leads. A high correlation was found between the clinical degree of alteration and changes in both the ECG and the VCG. In all patients these changes have normalized within 8 weeks. The histological findings of 3 deceased newborns revealed typical although not specific features such as: polyploid cells especially in the subendocardial region and a streamlined fatty degeneration of the myocardium. These findings, however, cannot prove with certainty the cardiotoxic effect of Fenoterol on the myocardium, but it appears possible that an especially high dosage of Fenoterol given i. v. can cause myocardial complications. Our findings suggest that catecholamin derivates should be applicated only with extreme caution.

[Cardiomyopathy in Duchenne muscular dystrophy. Part 2: serum enzymes, vector-cardiography, and echo-cardiography in 143 patients (author's transl)]. / Kardiomyopathie bei Duchennescher Muskeldystrophie.

To diagnose a possible latent or manifested cardiomyopathy, 143 male patients between 2 and 21 years of age with confirmed Duchenne muscular dystrophy were examined for serum enzymes, by electrocardiography, vector-cardiography, and echo-cardiography. The results contain information on 1. the quantitative cellular myocardial degeneration process, 2. the disturbed cellular depolarization and nerve-conduction processes in the area of the right and left ventricular myocard, and 3. the disturbed left ventricular function which, in the initial state, is only documented by a discrete decrease of contractility caused by a manifested decreased ejection output. The pathological contraction and relaxation process of the heart muscle cell and its dependency on calcium ion transport as pathogenic background is discussed.

[Rupture of sinus of valsalva aneurysm. Differentiation from cardiac contusion (author's transl)]. / Ruptur eines Aneurysmas des Sinus von Valsalva. Differentialdiagnostischer Aspekt einer Contusio cordis.

The diagnostic aspects of rupture sinus of Valsalva aneurysm are discussed. The indication for corrective surgery can only be derived by angiocardiography. Important is the demonstration of pathophysiological hemodynamics, due to the cardiovascular anomaly. The importance of echocardiographic findings in an early phase of the clinical examination is stressed.

[Angiocardiographic and vectorcardiographic findings in Noonan's syndrome (author's transl)]. / Angiokardiographische und vektorkardiographische Befunde beim Noonan Syndrom.

The angiocardiographic and vectorcardiographic findings in 18 patients with Noonan's syndrome are presented. The excentric hypertrophy of the left ventricle affecting the superior portion of the anterior wall, the posteroinferior portion and the septum was primarily investigated. The predominant lesion was left ventricular deformity. In one case only a hemodynamic burden was caused by the excentric hypertrophy. It could be demonstrated that angiocardiographically and vectorcardiographically differentiation between idiopathic hypertrophic subaortic stenosis and excentric hypertrophy in Noonan's syndrome is impossible. A good correlation however, existed between the morphological structures and the angiocardiographic aspect as well as the vectorcardiographic findings. If hemodynamic burden of the left ventricle is present, identical therapy is proposed for Noonan's syndrome as for idiopathic hypertrophic subaortic stenosis. Left heart catheterization is therefore indicated to prove excentric hypertrophy.

[Hypoplasia of the pulmonary artery in Rieger's Syndrome (author's transl)]. / Hypoplasie der Arteria pulmonalis beim Rieger-Syndrom

A 7-year-old child with Rieger's syndrome was reported in whom hypoplasia of the peripheral branch of the pulmonary artery was diagnosed via cineangiocardiography.