The preoperative and postoperative findings in 627 patients with tetralogy of Fallot.

The preoperative and postoperative findings in 627 patients operated for correction of tetralogy of Fallot (TOF) in the period 1960 to 1984 were analyzed in order to evaluate the clinical and hemodynamic late results. The average age was 7.2 years (range 9 months to 30 years). The patients were divided into 4 groups in order to determine the long-term prognosis in relation to the severity of the underlying anatomy; group I = without outflow tract patch; group II = with a patch up to the valve base; group III = with a transannular patch (TAP); group IV = TAP or valved conduit in patients with pulmonary atresia. Preceding shunt operations had been performed in 350 patients (55.8%); a primary correction was carried out in 277 cases (44.1%). Hospital mortality for all patients operated from 1960 to 1984 was 14.0%, late mortality, by contrast, was only 1.1%. In the last 4 years (1980 to 1983), the overall mortality considerably decreased to 3.4% (n = 29) for patients without TAP and to 8.0% for all operated patients including those with pulmonary atresia (n = 50). A comparison of the actuarial survival curves, moreover, demonstrates that the prognostic survival rate is unequivocally dependent on the severity of the anatomy of a TOF. The hemodynamic results obtained from 271 recatheterized patients from all 4 groups were designated excellent in 106 patients (39.1%), good in 100 (39.9%) and poor in 65 (23.9%). Proximal residual gradients across the right ventricular outflow tract (RVOT) were found in 30.2% of the corrected patients, and distal gradients in 24.3%. Fifty-two patients out of the 627 (8.2%) had to be reoperated for residual VSD, RVOT aneurysm, valvular pulmonary stenosis or peripheral stenosis. Intracardial electrophysiological tests carried out in 166 patients postoperatively showed an antegrade effective refractory period of the AV-node lasting over 400 ms in 47 patients (28.3%), as well as additional ventricular action potentials in 117 patients (70.4%), which could be correlated to extrasystoles or ventricular tachycardia in 37.9% of the cases. Additional AV-pathways were disclosed in 3 patients.(ABSTRACT TRUNCATED AT 400 WORDS)

[Emergency treatment of arrhythmias in neonates and infants]. / Notfallbehandlung von Herzrhythmusstörungen im Neugeborenen- und Säuglingsalter.

Emergency treatment of cardiac arrhythmias was required in 41 newborn and infants aged two days to 9 months (mean 77 days) from July 1977 until September 1981. Heart defects were present in 27 (65.8%). Invasive electrophysiological studies were performed in all patients. The different types of arrhythmias were: bradyarrhythmias in 9 (21.9%): bradycardia to cardiac arrest (5), congenital complete AV-block (3), postoperative complete AV-block (1). Tachyarrhythmias in 32 patients (78.1%): reentry through accessory connections (21), congenital atrial flutter (6), ventricular flutter/fibrillation (3), and AV-nodal tachycardia (2). Overdrive atrial or ventricular stimulation with a consecutive series of 15-20 impulses of 5-10 Volts abolished arrhythmic attacks in 22 patients including 4 in whom prior digitalization had no effect. In two other patients overdrive pacing achieved sinus rhythm only after i.v. Propafenon. In 4 further patients 36.2 to 63.8 mg/m2 i.v. Propafenon and in 4 other patients DC synchronized cardioversion with 1 to 3 Wsec/kg restored a normal heart rate. The 3 patients with congenital complete heart block died, one despite permanent pacing. Oral Propafenon therapy with 300 mg/m2 die in three divided doses following emergency therapy of tachyarrhythmias was discontinued in patients without arrhythmias after 1 year on drug therapy. There was no relapse after a mean follow-up period of 1.9 years. Only patients with congestive heart failure due to cardiac defects needed additional digitalisation. Thus, in our experience antiarrhythmic drug therapy with Propafenon was more effective in this age group than digitalization.

[Propafenon therapy of arrhythmias in infancy and childhood (author's transl)]. / Propafenon in der Behandlung von Herzrhythmusstörungen im Säuglings- und Kindesalter.

The therapeutic dose range of propafenon was studied in 28 infants and children aged 5 days to 15.2 years (mean 8.8 years) with the following cardiac arrhythmias: paroxysmal tachycardia (20) atrial flutter (5) ventricular extrasystoles (2) and junctional tachycardia (1). With the intravenous administration of the drug in a dosage of 1.03-3.2 mg/kg/d (mean 2.28 mg/kg/d) in patients less than 15 kg and of 0.71-2.06 mg/kg/d (mean 1.26 mg/kg/d) in patients more than 15 kg body weight arrhythmias abolished. In the same patients the oral dosage was 12.2-22.6 mg/kg/d (mean 16.03 mg/kg/d) and 7.2-16.6 (mean 11.12 mg/kg/d). After 10 to 14 days a therapeutic serum level above 150 ng/ml could be maintained by a reduced oral dosage of 5.7-13.3 mg/kg/d) mean 8.5 mg/kg/d) and 3.6-11.1 mg/kg/d (mean 6.33 mg/kg/d) respectively. There were no side effects except for transitory by elevated gamma-GT in 8 patients. During a follow-up period of 1 month to 2.2 years (mean 1.6 years) 20 patients had no more arrhythmias, in 7 the number of arrhythmic attacks was reduced, and in 1 therapy failed.

[An adaptation function for describing time-activity curves in nuclear medicine (author's transl)]. / Eine Anpassungsfunktion zur Beschreibung nuklearmedizinischer Zeit-Aktivitäts-Kurven.

Radioisotopic time-activity curves can be described by a function known from the physics of heat conduction. This function, called in this paper "tracer function", is a special solution of one-dimensional differential equation of heat conduction. The function is especially useful in the quantitation of left-to-right cardiac shunts by analysis of the pulmonary time-activity curve. The first transit curve is determined from the amplitudes on the ascending slope of the curve only including the maximum. The shunt curve analysis uses two parameters derived from the first transit curve and then the position and amplitude of the shunt curve maximum only.

[Diagnosis and treatment of arrhythmias after Mustard-Brom operation for transposition of the great arteries (author's transl)]. / Diagnose und Behandlung von Arrhythmien nach Mustard-Brom-Operation bei Transposition der grossen Arterien.

Arrhythmias after Mustard's operation for transposition of the great arteries were studied in 96 patients operated upon between June 1965 and July 1979. All patients had sinus rhythm consistently before the operation. Surgical modifications aimed at preserving the sinoatrial (S-A) node have reduced but not avoided arrhythmias. During the follow-up period in 54 patients (56.25%) different arrhythmias were observed. Sick-sinus syndrome was detected as the most frequent rhythm disturbance. The highest incidence of arrhythmias was in the first 6 weeks after the operation. However, life-threatening arrhythmias and sudden death occurred even years after Mustard's operation. Overall, there were 31 deaths (32.3%) in which severe arrhythmias were involved. In 6 patients sudden death occurred 4 weeks to 3 years postoperatively, attributable to rhythm disturbances. In one patient who died from sick-sinus syndrome, the histologic features of the S-A nodal area were examined. The S-A node was replaced by dense connective tissue. In another patient who had recently undergone Mustard's operation, S-A nodal tissue was identified, but fresh hemorrhage was seen in and about the node. Different antiarrhythmic drugs were employed in the treatment of arrhythmias observed after Mustard's operation. In 34 patients (36.4%) temporary pacing was necessary. In case of failure of antiarrhythmic drugs, permanent pacing is the therapy of choice.

[The genetic aspects of Williams-Beuren syndrome and the isolated form of the supravalvular aortic stenosis. Investigation of 128 families (author's transl)]. / Zur Genetik des Williams-Beuren-Syndroms und der isolierten Form der supravalvulären Aortenstenose. Untersuchungen von 128 Familien.

Supravalvular aortic stenosis (SVAS) was seen in 128 families. In 23 families several members had SVAS. In 4 families the Williams-Beuren Syndrome (WBS) was present whereas members of 8 families had some features of the syndrome in addition to their cardiac lesion. In conclusion, no distinct separation can be made between WBS and SVAS. The genetic pattern is autosomal dominant with variable expressivity. The gene frequency is estimated at 10(-4) and the mutation rate at 2.5 . 10(-5).

[The spectrum of supravalvular aortic stenosis: clinical findings of 150 patients with Williams-Beuren syndrome and the isolated lesion (author's transl)]. / Spektrum der supravalvulären Aortenstenose: Untersuchungsergebnisse bei 150 Patienten mit Williams-Beuren-Syndrom und der isolierten Form der supravalvulären Aortenstenose.

The clinical findings of 150 patients with supravalvular aortic stenosis are presented. No correlation was found between the clinical findings and the hemodynamic degree of severity, with the exception that an ECG of left ventricular hypertrophy and strain was more common in patients with a LV pressure above 200 mm Hg. In this latter group of patients there is a significantly higher number of patients without the typical appearance of the Williams-Beuren syndrom. The analysis of the additional anomalies and mental retardation in the Williams-Beuren syndrom and the isolated cardiac lesion shows clearly that no distinct separation between these 2 groups is possible. This points towards the same pathogenesis.

[Typical presentation of sequential scintigraphy images in the diagnosis of cyanotic heart disease in the newborn, using a computer-assisted imaging technique (author's transl)]. / Typische Darstellung szintigraphischer Bildsequenzen zur Diagnose von zyanotischen Herzfehlern Neugeborener mit einer computergesteuerten Abbildungstechnik.

The technique of sequential scintigraphy in investigating newborn infants in order to exclude or detect cyanotic congenital heart disease has been improved. The primary scintigraphic data are recorded digitally (list mode, 1 ms, 128 by 128 pixels) and evaluated automatically as a sequence of 1/2 s frames in 1/8 s increments. A 35 mm film is exposed to a scanning pulsed light spot on a CRT-screen with quantitative correspondence between local count density and the local number of light pulses. A series of highly resolved images of the rapidly changing scintigraphic pattern is achieved in 30 min. The results of 3 investigations are discussed. They prove the importance of adequate spatial resolution and definition in time in nuclear angiocardiography in infancy. Three case results are discussed in detail. In one case cardiac catheterisation and angiocardiography had been omitted, since the radioisotopic findings excluded cyanotic congenital heart disease. Two further investigations are discussed with reference to the findings at cardiac catheterisation.

[Hazards of heart catheterization and angiocardiography in the first year of life. A review of 1366 examinations]. / Das Risiko der Herzkatheterisierung und Angiokardiographie im ersten Lebensjahr. Ein Uberblick über 1366 Untersuchungen

1366 infants in the first year of life had cardiac catheterisation with or without angiocardiography in our Department of Cardiology between 1963 and 1973. The overally mortality in the first 24 hrs after the cardiac catheterisation for the first year of life was 1.83%. The subdivision of the first month of life reveals the high mortality rate of the first two weeks of life with the highest percentage of 18.9% in the first week of life and with 10.7% in the second week of life. After that time the mortality is significantly decreasing, but still amounts to 7.6% in the first month of life. There had been no death after the seventh month of life. 14 of the 24 infants died without a specific event in increasing heartfailure and/or acidosis. In the other 10 cases death was due either to the manipulation of the catheter (partial-perforation or perforation), to the angiocardiogram (subsequent asystole) or to the position of the catheter with subsequent intramural injection of contrast material.The best correlation was between the mortality and the severity of the underlying cardiac malformation. Further complications which did lead to death 24 hrs following cardiac catheterisation are described. Our results are compared with those of other authors. The reasons of various complications are discussed. Cardiac catheterisation and angiocardiography as early as possible is necessary because palliative procedures and corrective surgery can be initiated immediately. But it is important in the very sick infant to shorten the procedure as much as possible and to keep angiographic injections at a minimum.