[Round liver lesions in type I glycogenosis]. / Leberrundherde bei Glykogenose Typ I.

We report a 24 year follow-up of a patient with type 1 glycogen storage disease with new development of adenoma of the liver. The diagnostic and therapeutic consequences are discussed.

Mitral valve aneurysm revealed by cross-sectional echocardiography in a patient with mitral valve prolapse.

We report a cerebral ischemia in a 23-year-old woman, in whom a mitral valve aneurysm with thrombotic masses was diagnosed by cross-sectional echocardiography. A prolapsing mitral leaflet was also visualized.

Atrioventricular dissociation detected by suprasternal M-mode echocardiography: a clue to the diagnosis of ventricular tachycardia.

Twenty-two patients were studied by suprasternal M-mode echocardiography during ventricular tachycardia (VT). Adequate echocardiograms were obtained from 19 patients. Thirteen patients showed atrioventricular (AV) dissociation and 6 patients a ventriculoatrial contraction pattern according to the left atrial (LA) contraction obtained from the suprasternal notch. In 1 of these 6 patients, a 2:1 block retrograde was found by echocardiography. In another patient, an intermittent block occurred in the retrograde direction. In 4 patients, a constant relation between the QRS complex and LA contraction soon after the beginning of the QRS complex was seen, demonstrating a 1:1 ventriculoatrial conduction. According to the LA contraction obtained from the suprasternal echocardiogram, 13 patients showed AV dissociation and 6 patients a retrograde conduction to the LA. From the analysis of the 12-lead standard electrocardiogram obtained simultaneously during VT, AV dissociation could be recognized in only 3 patients. Thus, AV dissociation during VT is more easily diagnosed with suprasternal M-mode echocardiography than with the standard electrocardiogram.

Intracoronary thrombolysis with an acylated streptokinase-plasminogen activator (BRL 26921) in patients with acute myocardial infarction.

The fibrinolytic efficacy and systemic effects on coagulation variables of intracoronary administration of an acylated streptokinase-plasminogen complex (BRL 26921) were assessed in 23 patients with an acute transmural myocardial infarction. The infarct vessel was totally occluded in 22 patients and subtotally stenosed in 1 patient. Reperfusion was achieved in a total of 17 patients (74%), in 2 patients with the use of a guide wire. Reperfusion time in those patients treated with BRL 26921 alone amounted to 42 +/- 37 minutes. Reocclusion occurred in two patients subsequently. Four patients died; in two of these, intracoronary thrombolysis was unsuccessful. Reptilase time increased from 13 +/- 3 to 49 +/- 31 seconds (p less than 0.001), fibrinogen levels decreased from 280 +/- 65 to 126 +/- 76 mg% (p less than 0.001). Factor V decreased from 96 +/- 11 to 53 +/- 26% (p less than 0.001), and factor VIII from 99 +/- 1 to 55 +/- 36% (p less than 0.001). Peripheral hyperplasminemia, defined as a reduction of fibrinogen (less than 100 mg%) with a reduction of factor V and VIII (less than 75%) simultaneously occurred in eight patients. Six (75%) of these 8 patients demonstrated reperfusion, whereas 9 (64%) of 14 patients without peripheral hyperplasminemia were also successfully reperfused. Bleeding complications occurred in two patients who demonstrated hyperplasminemia. Thus, effective intracoronary thrombolysis could be achieved with only minor effects on peripheral coagulation variables in the majority of patients.

[Successful transvenous electric ablation of the AV conduction system in therapy-refractory atrial flutter]. / Erfolgreiche transvenöse elektrische Ablation des AV-Uberleitungssystems beim therapierefraktären Vorhofflattern.

The technique of closed-chest ablation of the atrioventricular (AV) conduction system was used in 3 patients (53-62 years of age) with atrial flutter not responding to medical management. A persistent third-degree AV block was induced in all patients. Moderate increases in CK and CKMB enzymes but not in Tc-pyrophosphate uptake were observed in these patients. 5 days after the procedure a permanent pacemaker was implanted in each case. We conclude that closed-chest ablation of the AV conduction system is a safe and efficient procedure in selected patients with drug-resistant supraventricular arrhythmias.

[Diagnosis of thoracic aortic aneurysms with 2-dimensional echography]. / Diagnostik thorakaler Aortenaneurysmen mittels zweidimensionaler Echographie.

Eight patients with thoracic aortic aneurysms were investigated by angiography, computed tomography and two-dimensional echography. According to results of computed tomography or angiography six patients had a dissecting aortic aneurysm (five patients type 1 and one patient type 3 according to DeBakey) and two patients had a thoracic aortic aneurysm without dissection. In addition, thrombi in the descending aorta were shown in three patients by computed tomography or by angiography. Type and extent of the aneurysms could be evaluated by two-dimensional echography in all patients. Only in one patient could the origin of dissection in the ascending aorta not be localised precisely by echography. In three patients thrombi in the descending aorta could be demonstrated. For assessment of type and extent of the aneurysm the suprasternal approach was superior to precordial, subcostal and apical imaging. Parasternal investigation resulted in echographic demonstration of dissection in only two cases. Two-dimensional echography is a technically less demanding and useful method in the diagnosis of thoracic aortic aneurysms.

[Sonographic diagnosis of Budd-Chiari syndrome]. / Sonographische Diagnose eines Budd-Chiari-Syndroms.

In a 28-year-old female patient a diagnosis of Budd-Chiari syndrome was established post partum on the basis of characteristic ultrasonographic findings. The results permit establishment of the syndrome in the early phase of the disease. Early diagnosis of hepatic vein occlusion is particularly important because directed therapeutic regimes may lead to revascularisation of hepatic veins and may thus prevent liver cell necrosis and subsequent hepatic failure.

[Closed intracardiac cardioversion in therapy resistant atrial flutter]. / Geschlossene intrakardiale Kardioversion bei therapierefraktärem Vorhofflattern.

Cardiac frequency could not be lowered in a 62-year-old patient with atrial flutter and 2:1 conduction. Using an electrode catheter positioned in the His bundle area an electric current of 80 Ws was effected. A third-degree atrioventricular block developed which regressed after 6 hours. The electrophysiologic assessment after one week showed a marked diminution of AV node conduction capacity.

[Arrhythmias in subjects with a healthy heart]. / Herzrhythmusstörungen bei Herzgesunden.

Of 350 patients who had extensive non-invasive and invasive cardiological diagnostic tests, 56 had completely normal results. 24-hour ECG monitoring of the latter revealed the following ventricular arrhythmias-ventricular extrasystoles (VES): 36% without, 23% with rare ones (less than 30/h), 5.4% with more than 30/h, 25% with polytopic VES, 7.1% with paired VES and 3.6% with ventricular tachycardia. There was no preferential VES pattern. Mean duration of VES was 0.15 s. There were no VES with a prematurity index of less than 1. Extending ECG monitoring to 96 hours (10 persons without heart disease) did not reveal any more significant VES, but registering for less than 24 hours definitely underestimated the frequency of VES in persons without heart disease. In those without angiographic evidence of heart disease 36% had complex and 5.4% frequent VES. Complex arrhythmias, however, are rare in the individual subject and generally not accompanied by frequent VES. Frequent complex arrhythmias or both frequent and complex arrhythmias, as well as VES with a prematurity index less than 1, are suggestive of organic heart disease.

[Separation of the left atrium from the right pulmonary artery in the suprasternal echocardiogram: a parameter of left atrial pressure]. / Die Separation des linken Vorhofs von der rechten Pulmonalarterie im suprasternalen Echokardiogramm: ein Parameter des linksatrialen Druckes.

A separation of the left atrium from the right pulmonary artery during atrial contraction may be observed in the suprasternal echocardiogram. In 280 catheterized patients with sinus rhythm, we investigated whether left atrial separation is a parameter from which an estimate of the left atrial pressure can be obtained. In 239 of the 280 patients, the suprasternal echograms were of a quality such that it could be seen whether there was, a left atrial separation. In 182 patients, a normal left atrial pressure (greater than or equal to 12 mm Hg) was found; in 57 patients, the left atrial pressure was elevated. An atrial separation was observed in 183 patients, and in 56 patients it was lacking. Lack of left atrial separation indicates a left atrial pressure elevation with a sensitivity of 73.7% and a specificity of 92.3%. If a left atrial pressure above 18 mm Hg was considered elevated, the sensitivity of this echoparameter amounted to 90.3%, and the specificity was 86.5%. The study shows that the left atrial separation from the right pulmonary artery separation from the right pulmonary artery in the suprasternal echocardiogram is a parameter valuable in providing a rough estimate of the left atrial pressure.