[Circumscribed apical left ventricular hypertrophy. Dynamic development and long term progression]. / Umschriebene apikale Linksherzhypertrophie. Dynamische Entwicklung und Langzeitverlauf.

HISTORY AND CLINICAL FINDINGS: A 64-year-old obese man had for 15 years suffered from exercise-independent retrosternal pressure sensation, radiating to the neck and back. Shortly after the onset of these symptoms he had undergone coronary angiography with negative results. But at that time the resting ECG showed discrete T wave negativity in the left precordial leads. INVESTIGATIONS: At the present admission the ECG showed deeply inverted T waves in the left precordial and limb leads and a positive Sokolow-Lyon index of 4.8 mV. Left ventricular angiography demonstrated in enddiastole a circumscribed myocardial hypertrophy limited to the apex and of typical "ace of spade" shape. DIAGNOSIS, TREATMENT AND COURSE: Left-heart catheterization and angiocardiography provided the diagnosis of circumscribed apical left ventricular hypertrophy (ALVH). As the patient had only minor symptoms no treatment was given. CONCLUSION: Circumscribed ALVH can show marked dynamic development in long-term observations. If there is marked T wave negativity, even with previously normal LV angiography, circumscribed ALVH should be included in the differential diagnosis. Patients with atypical angina pectoris and increasingly suggestive ECG changes should, even if previous coronary angiography had been negative, undergo transthoracic echocardiography with a high-frequency transducer, special attention being paid to muscular changes at the LV apex.

[Circumscribed apical left ventricular hypertrophy as the cause of a pronounced T-wave negativity]. / Umschriebene apikale Linksherzhypertrophie als Ursache einer ausgeprägten T-Wellen-Negativierung.

HISTORY AND CLINICAL FINDINGS: Two patients, one aged 57 the other 44 years, complained of nonspecific symptom-palpitation, left precordial pain and dyspnoea. There were no contributory findings on physical examination in both cases. INVESTIGATIONS: Both patients had hyperlipoproteinaemia, the younger one hyperuricaemia as well. The electrocardiogram showed deeply negative symmetrical T waves in the precordial leads. Transthoracic and transoesophageal echocardiography demonstrated thickening of the apical left ventricular wall. Coronary heart disease was excluded in both patients by coronary angiography. TREATMENT AND COURSE: The findings were fully explained to both patients. As they had only minor symptoms, no specific treatment was given. CONCLUSION: Isolated apical left ventricular hypertrophy should be considered as a possible cause of marked T-wave inversion in the precordial leads; this special form of hypertrophic cardiomyopathy is rarely seen in Europeans. As autosomal dominant inheritance of this condition has been described in two families, other family members should be studied by echocardiography whenever this abnormality is found.

[Tilt-table study in vasovagal syncope. The diagnostic gain from isoprenaline administration]. / Die Kipptischuntersuchung bei vasovagaler Synkope. Diagnostischer Zugewinn durch Isoprenalin-Gabe.

OBJECTIVE: The cause of syncope remains unclear in half of the cases, even after extensive neurological and cardiological examination. A study was, therefore, undertaken to determine the number of patients with the suspected diagnosis of vasovagal syncope that were confirmed by the tilting table test and how often it required the additional administration of isoprenaline to do so. PATIENTS AND METHODS: A tilting table test was performed on 75 patients (49 men, 26 women; mean age 41 [17-80] years) with syncopes of uncertain cause, previous examinations having failed to discover any neurological or cardiological cause. The test was done with a head-up angle of 60 degrees for 30 min. ECG and arterial blood pressure by indwelling catheter were recorded continuously. If the test was negative, isoprenaline was given intravenously at a rate of 5 micrograms/min during a five-minute period in the horizontal position, followed by 10 minutes at 60 degrees head-up position. RESULTS: Vasovagal syncope or presyncope was induced in 49 of the 75 patients during the tilting table test, a sensitivity of 65%. But 45% of the tests were positive only with the administration of isoprenaline, i.e. an increase in sensitivity to 81.5%. In 96% of the patients with a positive test there was conformity of symptoms between the induced and the spontaneously occurring syncopes. CONCLUSION: The tilting table test is a valuable means of investigating cases of syncope. More than half of the cases of syncope of uncertain cause can be correctly diagnosed classified in this way. The additional use of isoprenaline infusion greatly increases the sensitivity of the method.

[Single right coronary artery with absence of the left coronary ostium]. / Singuläre rechte Kranzarterie bei fehlendem linkskoronarem Ostium.

We report the rare congenital anomaly of a singular right coronary artery in absence of the left coronary ostium. In a 31-years-old man, coronary arterial angiography demonstrated a right coronary artery which, arising from the right Sinus Valsalvae, first described the normal right coronary arterial course but in the apical region continued to follow, in reverse direction, the normal course of the left anterior descending artery. The proximal diameter of the vessel measured 5.2 mm. Thallium scintigraphy showed no ischemia of the anterior wall. The risk of developing circumscribed atherosclerosis due to anatomical reasons seems not to be increased, as no additional bifurcation nor kinking was to be found.

New miniaturized versus conventional biplane transesophageal transducers: recent clinical experience in adults.

A subset of patients have substantial discomfort on examination with transesophageal echocardiography with the conventional probe, whereby the dimensions of the probe play a decisive role. Miniaturized biplane transducers have recently become available (2 x 32 channels, dimensions 9.5 x 8.7 mm, and circumference approximately 30% less than the conventional echoscope) and allow ultrasound examination at 3.5, 5.0 and 7.0 MHz. A prospective study was carried out in 90 patients to compare difficulties on insertion of the probe, subjective evaluation by the patient during examination, and the two-dimensional image, as well as Doppler and color-coded Doppler quality of the miniaturized biplane versus the conventional probe. In 62 patients, intubation of the esophagus proved less difficult with the smaller instrument and more difficult in nine cases. Seventy-six patients reported that they suffered less discomfort on use of the narrow instrument. Concomitant parasympatholytic medication was needed with the smaller probe in seven cases and 17 times with the conventional probe. As anticipated, quality of the two-dimensional image attained by the miniaturized probe was lower. With transmit/receive frequency of 7.0 MHz, however, image resolution was excellent in the near field of 5 cm and nearly equivalent to that of the conventional probe (5.0 MHz). Pulsed-wave and continuous wave Doppler and color-coded Doppler information from both probes was similar in quality. Whenever examination with a conventional transesophageal transducer promises to be difficult, or when sedation is contraindicated because of a severe illness or respiratory insufficiency, transesophageal echocardiography should be considered with a smaller biplane probe at higher transmit-receive frequencies.

[Right ventricular thrombi in primary antiphospholipid syndrome]. / Rechtsventrikuläre Thromben bei primärem Antiphospholipid-Syndrom.

Over a period of several months a 33-year-old man had recurrent pulmonary emboli. No thromboses could be demonstrated in the peripheral venous system. Transoesophageal echocardiography showed two spherical space-occupying structures in the right ventricle which were removed operatively under the suspected diagnosis of multilobular myxomas. However, their histological examination revealed pure thrombi that had grown by apposition. This unusual findings of right-ventricular thrombi could not be explained pre- and intraoperatively by any local thrombi-favouring changes in the right heart. Tests of clotting mechanisms demonstrated lupus anticoagulant (kaolin-clotting-time mixture test: LA index 21.7 [normal: < 15]), as well as an increased IgG cardiolipin antibody concentration of 19.3 U/l). As no underlying disease was discovered, the diagnosis was by definition primary antiphospholipid syndrome. No further thrombo-embolism has occurred during continuing oral anticoagulation with phenprocoumon.

[Comparison of conventional and miniaturized biplane echoscope: initial clinical results]. / Konventionelle und miniaturisierte biplane Echoskope im Vergleich: Erste klinische Ergebnisse.

A subset of patients experiences substantial discomfort on examination with transesophageal echocardiography using the conventional echoscope, whereby the dimensions of the probe play a decisive role. Miniaturized biplane transducers have recently become available (2 x 32 crystals; dimensions 9.5 x 8.7 mm; circumference approx. 30% less than the conventional echoscope), which allow ultrasound examination at 3.5, 5.0 and 7.0 MHz. A prospective study was carried out in 70 patients to compare difficulties on insertion of the probe, subjective evaluation by the patient during examination, and the 2D-image as well as Doppler and color-coded Doppler quality of the miniaturized biplane echoscope. In 43 patients, intubation of the esophagus proved less difficult with the smaller instrument, it was more difficult in 8 cases. 54 patients reported that the discomfort was definitely easier to bear on use of the narrow instrument. Concomitant parasympathicolytic medication was needed with the smaller probe in 4 cases, and 11 times with the conventional echoscope. The quality of the 2D-image attained by the miniaturized probe was naturally lower. Employing multi-Hertz technology at a frequency of 7 MHz, however, imaging of the anatomy was excellent in the near field of 5-6 cm, and nearly equivalent to that of the conventional probe (5 MHz). The quality of PW-and CW-Doppler as well as color-coded Doppler information was identical. Whenever examination with a conventional echoscope promises to be difficult, or when sedation is contraindicated due to a severe illness or respiratory insufficiency, transesophageal echocardiography should be considered with a smaller biplane probe at higher frequencies.

[Color Doppler echocardiographic diagnosis in generalized arteriosystemic coronary fistulae]. / Farbdopplerechokardiographische Diagnostik bei generalisierten arteriosystemischen Koronarfisteln.

A 60-year-old woman was referred because of retrosternal pain. Transthoracic color-coded Doppler echocardiography showed, in the apical region of the left ventricle, multiple connections between the epicardium and the left ventricular chamber. The finding was concordant with a generalized arteriosystemic coronary artery fistula. Coronarography afterwards with injection of dye into left and right coronary arteries resulted in an intense homogenous opacification of the ventricle through multiple small coronary artery fistulas. This case report represents the first description of color-coded Doppler echocardiography in the diagnosis of generalized arteriosystemic coronary artery fistulas.

[Morbidity and mortality in sleep apnea and nocturnal bradyarrhythmia]. / Morbidität und Letalität bei Schlafapnoe und nächtlichen Bradyarrhythmien.

The as yet unanswered question, whether patients with sleep apnoea and apnoea-associated bradyarrhythmias have a higher morbidity and mortality rate, was retrospectively investigated in 132 men with sleep apnoea (apnoea index > 10/h). Sleep-associated bradycardic arrhythmias were recorded in 71 (mean age 50.1 years; group 1). For comparison served 61 men with sleep apnoea but no bradyarrhythmias (mean age 51.4 years; group 2), matched for age and weight. There was no significant difference between the two groups as to diagnosis and initial findings on ergometry, lung function tests and blood gas analysis. The apnoea index of 48.1 +/- 23.9/h in group 1 was significantly higher than that of 31.9 +/- 20.1/h in group 2 (P < 0.001). During a follow-up period of a mean of 41.1 (19-66) months, two patients in group 1 died (of myocardial infarction), while one died (of bronchial carcinoma) in group 2 (follow-up period of 29.6 [18-54] months). The two deaths in group 1 were in a subgroup of 16 patients who had declined treatment or had used it irregularly. There was no death among those who had been treated (n = 54), by nasal continuous positive airway pressure, operation or pacemaker implantation. However, no causal relationship could be established from these data between increased mortality rate and apnoea-associated arrhythmias.

Renal artery stenosis: grading with image-directed Doppler US evaluation of renal resistive index.

PURPOSE: To determine the role of intrarenal Doppler ultrasound (US) in detection of moderate to severe (> 50%) renal artery stenosis (RAS). MATERIALS AND METHODS: In 72 patients, 142 kidneys were examined with conventional angiography and color duplex US. Renal size, mean intrarenal-arterial resistive index (RI), and difference of mean RIs between both kidneys (delta RI) were determined and compared with severity of RAS as determined with quantitative angiography. RESULTS: In 32 patients, angiography showed mild RAS < or = 50% in 13, moderate RAS in 10, and severe RAS in nine. Both renal size and mean RI values were decreased significantly (P < .001) only for severe RAS compared with values in 40 control subjects. For delta RI, no significant difference was noted between controls and patients with mild RAS; highly significant differences, however, were noted for both moderate and severe RAS (P < .001). Sensitivity and specificity of a cutoff delta RI of > 5% were 82% and 92% for RAS > 50% and 100% and 94% for moderate RAS and RAS > 60%. CONCLUSION: Color Doppler US and analysis of intrarenal Doppler spectra are recommended as a useful method for noninvasive diagnosis and grading of RAS. In bilateral RAS > 50%, however, calculation of delta RI is potentially biased by undergrading of stenosis.

[Nocturnal ventricular arrhythmia in patients with sleep apnea and suspected coronary heart disease]. / Nächtliche ventrikuläre Arrhythmien bei Patienten mit Schlafapnoe und Verdacht auf koronare Herzerkrankung.

BACKGROUND: Patients with sleep apnea and nocturnal brady- and tachyarrhythmia are considered to be patients at especially high risk within the group of all apnea patients. PATIENTS AND METHODS: 13 patients with sleep apnea (apnea-index > 10 events/h), suspected coronary heart disease and known increased frequency of nocturnal premature ventricular contractions (PVC) were studied. Polysomnography, long-term ECG and six-lead ECG were performed. RESULTS: Within the period studied (1.00 to 6.00 o'clock), an average of 47 PVC per hour was recorded (range 4 to 337/h). In two patients 24 episodes of nocturnal myocardial ischemia were observed, but were not accompanied by PVC. Interestingly only 387 of 1371 premature ventricular contractions (28.2%) were associated to apnea/hyperventilation episodes. Arrhythmia occurred mainly during sleep stages I/II and REM (n.s.). There was a tendency towards more frequent PVC with more pronounced oxygen desaturations. CONCLUSION: Patients with coronary heart disease, obstructive sleep apnea and severe hyoxemia are at higher risk of developing nocturnal PVC because reduced hypoxic tolerance of the heart may lead to electrical instability.

[Biplane transesophageal echocardiography. Diagnostic improvement over the mono-plane technique]. / Biplane transösophageale Echokardiographie. Diagnostischer Zugewinn zur monoplanen Technik.

Monoplane transesophageal echocardiography (TEE) is a well established diagnostic tool of examination of great value in determining pathological changes in both atria, atrioventricular valves, the left-ventricular outflow tract, and in the thoracic aorta (Table 1). With the monoplane technique, however, it is never possible to obtain more than parallel, or oblique transverse views of the heart and surrounding vessels. The only means with which to examine anatomic structures in their cranio-caudal dimension by way of this method is to make a composite of a number of transverse sections. This makes three-dimensional interpretation of monoplane images difficult. The biplane transesophageal technique provides images of orthogonal sections to the transverse plane, allowing three-dimensional reconstruction and thus greatly improved insight into the cardial anatomy. By ante- or retroflection and lateral angulation of the probe, it becomes possible to see structures as a whole, the greatest dimension of which may not lie in the strictly sagittal section, but on a craniocaudal diagonal plane, e.g. the ascending aorta, or the aortic valve plane. The diagnostic gain of additional data through biplane TEE stems from its images of cardial structures, which remain either unsatisfactory or not attainable on monoplane examination (Table 2). Above all this pertains to the superior vena cava in its longitudinal extension (Figure 6), the right-ventricular outflow tract with pulmonary valve, the longitudinal two-chamber view (Figure 3), and the CW-Doppler analysis in presence of tricuspid valve regurgitation (Figure 13). Transversal visualization of the aortic arch is only feasible by using biplane imaging technique (Figure 12). Compared to the monoplane technique, it shows clearly more distinct views of the apex of the left ventricle (Figure 1), the atrial anatomy (Figures 5 and 6), and here in particular the pathology of interatrial septum (Figure 7), as well as the aortic valve and the ascending aorta (Figures 8, 10 and 11). By using the longitudinal imaging plane left atrial appendage can be seen without additional anteflection of the probe, thus, reducing stress to the patient during examination. The loss of an infinite range of planes available to the multiplane technique is a disadvantage, but this can usually be compensated by appropriate flecting of the probe and adequate simultaneous lateral angulation. The range of rotation of the probe in the multiplane method allows better three-dimensional imaging of anatomic structures and regurgitant jets than do the mono- and biplane techniques, and comparable data are often only attained under much longer examination with the biplane instrument.(ABSTRACT TRUNCATED AT 400 WORDS)

[The coincidence of acute intermittent porphyria and Crohn's disease]. / Koinzidenz von akuter intermittierender Porphyrie und Morbus Crohn.

In the course of four weeks, tetraparesis developed in a 28-year-old man with Crohn's disease for the last six years, treated with glucocorticoids and sulphasalazine. The cause was acute intermittent porphyria which had been previously overlooked because of the similar abdominal symptoms of Crohn's disease. Treatment with glucose, propranolol and haemarginate failed to bring about any improvement, but there was a remission of the porphyria. This case demonstrates that diagnosis of acute intermittent porphyria in the presence of Crohn's disease is difficult, and if delayed therapeutic measures in the face of persisting neurological complications are of little benefit.

Influence of computer-modulated profile haemodialysis on cardiac arrhythmias.

To reduce hamodialysis-induced ventricular arrhythmias, each of 15 patients (age 66.9 +/- 6.2 years) with end-stage renal disease and cardiac irregularities was treated subsequently with four different computer-modulated bicarbonate haemodialysis profiles (A-D) for 2 weeks respectively: (A) constant UF, dialysate Na (138 mmol/l) and K (2 mmol/l); (B) decreasing UF, otherwise as (A); (C) decreasing UF and Na (starting with 10% higher than serum Na), otherwise as (A); (D) decreasing UF and Na, adapted K to achieve a maximal reduction of serum K of only 15%/h. Cardiac monitoring was done by 11 h ECG. Only in haemodialysis profile D a distinct reduction of ventricular extrasystoles during and after haemodialysis was obtained. It was accompanied by an improvement in the Lown classification. In addition, a weak but highly predictive correlation between the number of ventricular extrasystoles in the last hour of dialysis and the difference between pre- and post-dialysis potassium concentration in the serum could be established (r = 0.37; P less than 0.004). Computer-modulated potassium profile haemodialysis is a useful tool to reduce the number and severity of ventricular extrasystoles.

[Results of left heart catheterization study in 64 patients with nocturnal disorders of respiratory control (sleep apnea)]. / Ergebnisse der Linksherzkatheteruntersuchung bei 64 Patienten mit nächtlicher Atemregulationsstörung (Schlafapnoe).

Sleep apnea (SA) is associated with increased morbidity of the cardiovascular system, the interaction between the disordering of respiratory coordination and cardiovascular regulation being largely unknown. In 64 patients (age: mean = 54.1; range: 35-67 years) with an increased apnea index (AI greater than 10), a cardiac catheterisation investigation was performed to exclude coronary heart disease (CHD) or cardiomyopathy. CHD was excluded in 39 patients, 6 patients had coronary single-vessel disease, 9 patients coronary two-vessel, and 10 three vessel disease. In 10 patients, cardiomyopathy was detected, while high-grade impairment of the left ventricular ejection fraction (greater than 30%) was observed in five patients. With the exception of a single patient, CHD was observed only in patients in the over-fifty age group. Arterial hypertension was seen in 84% of the patients with, and in 69% of the patients without, CHD. The patient groups with and without coronary heart disease did not differ with respect to apnea index, ten minute index, or the average duration of the 30 longest apneic episodes. Anginal complaints, observed in a total of 72% of the patients, were one of the major indications for coronary angiography. These results do not support the assumption that SA is primarily a consequence of underlying cardiac disease, but do indicate that SA must be considered a cardiac risk factor, especially in view of the fact that pronounced nocturnal changes in blood gases and haemodynamics, together with malignant arrhythmias, are found in conjunction with this disturbance of breathing.

[Hyperfibrinogenemia and pathological plasma viscosity. Pathogenetic factors in unstable angina pectoris?]. / Hyperfibrinogenämie und pathologische Plasmaviskosität. Pathogenetische Faktoren bei der instabilen Angina pectoris?

Plasma viscosity and erythrocyte aggregation, as the most important rheological factors in the microcirculation, and fibrinogen were measured in the blood of groups of patients in various stages of coronary-heart disease. Patients with unstable angina had viscosity and fibrinogen levels, even before any manifest infarction, that were higher than those of patients with stable angina. Plasma viscosity and hyperfibrinogenaemia (1.39 +/- 0.08 mPa.s in 48 patients and 394.4 +/- 82.7 mg/dl, respectively, in 33) were comparable to the values in patients with acute myocardial infarction (1.37 +/- 0.09 mPa.s [n = 45] and 390.2 +/- 126.9 mg/dl [n = 27], but significantly higher (P less than 0.02) than in those with stable angina (1.33 +/- 0.08 mPa.s [n = 78] and 295.3 +/- 68.6 mg/dl [n = 44], respectively). This abnormal viscosity in unstable angina plays a part in increasing myocardial ischaemia because oxygen delivery is already diminished and capillary flow slowed down. It thus contributes to progression of the angina and must be taken into account as an additional pathogenetic factor in the clinical instability.

The structure of manumycin. III. Absolute configuration and conformational studies.

Chromic acid oxidation of manumycin (1), an antibiotic produced by Streptomyces parvulus (strain Tü 64), led to the isolation of 2-(2-methyl-4-oxo-2-pentenoylamino)-5,6-epoxy-1,4-benzoquinone (3) and (-)-(R)-2-methylhexanoic acid (4). From the absolute configuration of 4, determined by comparing its optical rotation with published data, follows the absolute configuration at the center of chirality in the diene side chain of manumycin (1) to be (6'R). Based on the direct comparison of the CD spectra of the two chromic acid oxidation products 2 and 3 with those of the antibiotic G7063-2 (5) and (-)-terreic acid (6) the stereochemistry at C-5 and C-6 of 1 was determined as (5R, 6S). From the negative CD-couplet of manumycin (1) its stereochemistry at C-4 was assigned as (4R).