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The article reports on a case of hydrops fetalis caused by Kell antibodies, antibody formation having been triggered by pregnancy. Since the relative frequency of Kell (and rhesus sub-group) intolerance is increasing due to the successes achieved by anti-D-immunoglobulin prophylaxis, the question arises, on account of the possibly severe course of the disease, whether the Kell factor should be taken into consideration in all blood transfusions in girls during their childhood and in women of childbearing age.

Hypernephromas may give rise to extensive thrombus formation in the inferior vena cava. In a 70-year-old female patient, two-dimensional echocardiography revealed a well-defined mass (2 X 2 cm in dimension) in the right atrium with occlusion of the tricuspid valve and thrombo-embolic material spreading from the inferior vena cava to the right atrium. This finding was confirmed by computed tomography and cavography. Computed tomography of the abdomen detected a large left-sided renal tumour and thrombotic occlusion of the whole vena cava. The patient was not willing to undergo surgery and the masses of thrombo-tumorous material in the right atrium and ventricle spread rapidly. Meanwhile a spontaneous recanalization of the oval foramen occurred. The patient died from massive pulmonary embolism. Only the signs and symptoms of right heart failure with confirmed thrombus formation in the right atrium led to the diagnosis of hypernephroma.

31 patients (Pt) with Mitral Valve Repacement (MVR), 22 Pt with Aortic VR (AVR) and 10 Pt with Double VR (DVR) are investigated hemodynamically and by ergometry. Normal working capacity (100% WC) has been achieved in 59% of Pt with AVR, in 34% with MVR and in 22% with DVR. Pulmonary artery mean pressure (PAMP) in this group was 19.5 mmHg, in a second group with a WC of 80% PAMP was 22.2 mmHG. 77% OF Pt with AVR are working regularly, in MVR 58% and in DVR 50%. In the average of these Pt, 7 months after VR professional occupation is started again. PAMP and pulmonary vascular resistance (APR) is significantly lower in the working vs. the non working group: PAMP 19.8/23.7 mmHg; APR 115/145 dynes sec cm-5, respectively. In MVR, the transprosthetic pressure gradient at rest was 4.1 (working PPT) and 7.5 mmHg (other); in AVR statistically no significant difference in the gradient could be found. In general, 68% of the working Pt are employed in a preferably sedentary job. The mean duration of daily work was estimated to 6.7 hours. It is concluded that AVR yields more favourable results in terms of regaining normal working activity than MVR. Cardiovascular function and physical capability in Pt with DVR are approximately comparable to MVR.

The diagnostic value of pulsed Doppler echocardiography (PDE) had not been sufficiently assessed up until now. Invasive catheter velocitometric measurements in the central vessels give quantitative information on the blood movement across the aortic and pulmonary valves. It is particularly useful in the quantification of aortic regurgitation. We successfully investigated 52 patients by means of PDE (ATL 500 A); 20 were suffering pure aortic incompetence, 11 pure stenosis and 21 had combined stenosis and regurgitation. Fifteen patients without aortic valvular disease served as controls. Our findings were compared with the results of cardiac catheterization and angiography in each case. In addition, 14 patients with aortic regurgitation were studied invasively by catheter velocitometry. The obtained regurgitation values were compared to the PDE method. In the PDE the underlying criteria for the assessment of the recordings were as follows: formal analysis of the analog signal and of the turbulence content during systole and diastole; in the flow velocity tracings aortic incompetence showed a steep increase with high peak to peak aortic velocities and scant turbulence formation; the reverse flow during regurgitation was accompanied by a high grade turbulent velocity pattern. The area under the diastolic (regurgitant) flow velocity curve (the time-amplitude integral) corresponded significantly with the angiographic severity of aortic insufficiency (r = 0.87). In aortic stenosis, turbulence formation leads to an approximately flat velocity profile across the ascending aorta, if the region in the vicinity of the valve is omitted. The flow velocity analog signals are considerably disturbed. However, the turbulence content which can be qualitatively estimated from the recordings, correlates well with the calculated valve area. In combined aortic valve stenosis and incompetence, the prevailing turbulent pattern does not always permit one to assess sufficiently the severity of the stenotic component, whereas the grade of incompetence can be, in general, evaluated. PDE complements the existing non-invasive techniques and probably essentially enriches non-invasive diagnostics.

In patients with mitral valve replacement an acute cardiac decompensation most often signalizes malfunction of the prosthesis. Echocardiography proves to be of great value in the noninvasive diagnostic to detecting valve dysfunction. The echocardiogram of two patients few weeks after cardiac surgery was strongly suggestive of perivalve leak with severe regurgitation: enlarged left atrium and left ventricle, hyperactive septal motion and hyperkinetic motion of the left ventricular posterior wall, unique "hump" in early diastole with normal excursion, opening and closing velocity of the disc. The echocardiogram of one patient exhibited the pattern of mitral stenosis: large left atrium, decreased amplitude of excursion of the opening of the disc with a slowing and "rounded" opening upstroke as well as a slowed and "rounded" downstroke with prolonged opening and closing rates of the disc. One patient with aortic and mitral valve replacement showed no echocardiographic signs of abnormal disc motion. The reason for the cardiac decompensation was an acute malfunction of the left ventricle. The echocardiographic findings were confirmed by cardiac catheterization, left ventricular cineangiography or direct inspection during open heart surgery.

67 patients (pts)--20 with intact cardiac valve function, 15 with pure mitral stenosis, 20 with both mitral valve insufficiency and stenosis, 12 pts with pure mitral incompetence - have been investigated by non-invasive pulsed Doppler-echocardiography (PDE) previous to invasive right and left heart catheterization. The following criteria for evaluation of the PDE registrations are adopted: 1) Time-related correlation of ECG and flow velocity. 2) Formal analysis of the flow-velocity tracings. 3) Turbulence formation. 4) Maximum duration of flow. In pure mitral insufficiency, systolic turbulence formation is detected within the left atrium, which is present according to the severity only locally or throughout the entire left atrium. We calculated a specificity for the diagnosis of mitral incompetence of 94% and a sensitivity of 78%. In mitral stenosis a severity-dependent ventricular inflow velocity is prevalent - with an insidious onset until a dome-like or saw-tooth-like profile. Grading for severity of mitral stenosis can be performed as follows: the time from onset to maximum flow velocity of the diastolic inflows is measured and related to the time period of the cardiac cycle. A specificity of PDE for mitral stenosis of 88% and a sensitivity of 91% have been found. PDE is also being applied for assessment of hemodynamic efficacy of prosthetic mitral-valve replacement. In the evaluation of mitral valve dysfunction, PDE as a non-invasive technique is of high specificity and sensitivity as compared with the hemodynamic results of invasive procedures.

Thirty-one patients with chronic polyarthritis under medical treatment had x-rays of their hands performed at the beginning of the illness and after seven years, on average. The findings were compared with clinical and biochemical data. Despite treatment, there was marked progression in the periarticular destructive changes, although the clinical features had improved. The extent of periarticular destruction was significantly greater amongst seropositive than amongst seronegative patients, both at the beginning and the end of the study, but there was no significant difference in the rate at which this progressed. It was not possible to demonstrate a statistically significant correlation between the radiological appearance of the joints and the clinical and laboratory findings.

The alteration of cardiac function in the presence of intracardiac tumors varies considerably according to their localization, size, and mode of insertion. Various cardiovascular diseases can be mimicked, e.g., primary valve dysfunction, cardiomyopathy, intracardiac shunts. Embolic events are frequent secondary symptoms. However, subtle noninvasive techniques such as M-mode and sector echocardiography, pulsed Doppler echocardiography, digital video subtraction angiography (DVSA), and computed tomography permit a thorough diagnosis of intracardiac tumorous masses without heart catheterization and catheter angiography. In this paper, myxomas within the left atrium (LA) and the right ventricle (RV) are described. Leading symptoms were systemic embolism and mitral stenosis (LA localization), low cardiac output and right to left shunt (RV localization). First suspicion of intracardiac tumor was raised by M-mode and sector echocardiography, whereas the diagnosis of LA myxoma was in one case missed by computed tomography and the RV myxoma was missed by right ventricular catheterization. In that case, only the four-chamber subxiphoidal sector echogram substantiated the diagnosis and explained the hemodynamic constellation of very low pulmonary pressures combined with right to left shunt. By the new technique of DVSA, all three tumors were plainly visualized. Nevertheless, echocardiography proved to be the most reliable technique of detecting intracardiac tumors.

Afterload reduction is an accepted therapeutic principle in the management of acute aortic (Ai) and mitral insufficiency (Mi). The question whether acute and chronic converting-enzyme inhibition by captopril has a beneficial hemodynamic effect in chronic Ai and Mi has been investigated in 17 patients with Ai and 10 with Mi. Ejection and regurgitation fraction (RF) were measured by radionuclide ventriculography (RNV) before, after 25 mg captopril and after 3-5 months of long-term treatment. The humoral response of the renin-angiotensin system (RAS) was quantified by analysis of angiotensin I and II. Captopril lowered under acute and chronic treatment RF in Ai and Mi by 32%. Angiotensin II levels decreased by the same order of magnitude. Acute and chronic vasodilation was followed by a distinct but well tolerated fall in blood pressure, especially in patients with Mi. These favourable hemodynamic effects of captopril make this therapy an adjunct but not an alternative to valve replacement.

In 94 subjects with normally functioning heart valve prostheses (51 aortic and 43 mitral valve prostheses) and in 35 patients with intact aortic and mitral valves, blood flow velocity within the heart and the aortic root have been recorded using pulsed Doppler velocity studies in patients with diseased valves of the left heart. In addition, a further 7 patients were investigated using invasive catheter tip velocitometry, pre- and postoperatively. The preversus postoperative changes of maximum velocity and acceleration is characterized as follows: postoperative flow velocity tracings show approximately normal profiles comparable to normal valve function. Turbulence formation is diminished and the steep uptroke of the normal flow pattern is restituted. Differences in transprosthetic blood flow patterns dependent on the implanted prosthesis model can be defined. Bioprostheses, in particular the Carpentier-Edwards device, reliably approximate normal amplitude-time characteristics. This is also true for the St. Jude Medical prosthesis with central flow properties. Velocitometric signs of valve dysfunction were detected in 9 patients: sensitivity was 100%; specificity ranged from 76% in aortic to 96% in mitral prostheses. Pulsed Doppler echocardiography therefore is a useful complement in the non-invasive haemodynamic tools and can be repeatedly applied to a patient with prosthetic cardiac valve replacement.

A retrospective analysis was undertaken of 31 patients with classical or proven chronic rheumatoid arthritis (CRA) who had been on a regimen of basic medication (gold salts, D-penicillamine, chloroquine, azathioprine--alone or in combination). Disease activity was checked by clinical, biochemical, immunological and radiological criteria. The laboratory results could not be altered by the basal medication and there was no relationship with the clinical and radiological findings, except for immunological results. None the less, the clinical symptoms improved under basal medication, even though the functional index got worse. The degree of joint destruction at the beginning and end of the observation period was markedly higher for seropositive than sero-negative cases, without significant differences in the rate of progression of joint destruction. Thus, definite long-term remission was achieved regarding the inflammatory changes but not the progressing joint destruction.

A 40-year-old man suffered for 5 years from a progressive proximal myopathy mimicking an atypical limb-girdle dystrophy. A "myopathic" pattern with myotonic and pseudomyotonic discharges was determined by electromyography. Enzyme histochemical and ultrastructural investigations of muscle and liver biopsies pointed to a glycogenosis. Biochemical investigations of muscle and liver samples confirmed this diagnosis, disclosing an acid maltase deficiency. Glycogen filled lysosomes were also revealed electron optically in skin fibroblasts but not in white blood cells. The literature concerning the late onset forms of acid maltase deficiency (type II glycogenosis) has been reviewed, and the clinical course has been compared with that of the infantile form (Pompe's disease). In early infancy the disease has a short and fatal course, with involvement of many organs. primarily skeletal muscules, liver and heart. In the late infantile and juvenile forms the course of the disease is slower, the organ involvement beeing not as severe; muscular symptoms begin to prevail. In adults, type II glycogenosis mimics muscular dystrophy with its prolonged course and the almost exclusive clinical involvement of proximal muscles. Biochemical and ultrastructural investigations have nevertheless demonstrated that other organs and tissues are also involved. The reasons for the variability of organ involvements in different ages are as yet unknown.

The effect of captopril-mediated afterload reduction on regurgitation was investigated in 10 patients with aortic insufficiency. Regurgitation was quantitated by the regurgitation fraction and the relation of regurgitant volume to enddiastolic volume, which were derived from gated radionuclide ventriculography. 19 patients with coronary artery disease and no evidence of valvular heart disease served as controls. In patients with coronary artery disease no significant regurgitation was found. In patients with aortic regurgitation the blood concentration of angiotensin I increased whereas that of angiotensin II decreased significantly after captopril-medication; thus, the conversion of angiotensin I to II was reduced to about 50% of the control value. Whereas blood pressure and heart rate did not change significantly, the regurgitation fraction and the normalized regurgitant volume were significantly reduced. The ejection fraction remained essentially unchanged. These findings suggest a favorable influence of captopril-induced afterload reduction on hemodynamics in aortic regurgitation.