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Biomedical subjects

N H Goebel

Publications and source records attributed to N H Goebel.

14 recordsLinked to original sources
PubMed1989

[Normal values of left ventricular function. Biplanar angiocardiography with reference to sex-specific differences].

Normal values of left ventricular function have been determined in 24 patients by means of biplane angiocardiography. Global parameters were chamber volume, ejection fraction and muscle mass. Regional ventricular function was determined by means of an orthogonal and radial axial system. Radial axis shortening parameters showed smaller standard deviations and smaller coefficient of variations than orthogonal parameters. The smallest coefficient of variation elicited regional area reduction methods. Women showed significantly lower chamber volumes and muscle mass than men. There was no difference between the two sexes in the ejection fraction and wall thickness.

Adult↗
PubMed1979

[Goldenhar syndrome].

The basic features of Goldenhar syndrome are preauricular appendices, epibulbar dermoids, vertebral and cardiac anomalies, and hypoplasia of the lungs. The syndrome appears to be caused by early damage during the first or second month of embryonic development. Prognosis is most often determined by the underlying heart disease. In the present study a 24-year-old female student is reported who exhibited a preauricular appendix on the right side, severe thoracic scoliosis, aplasia of the middle and lower lobes of the right lung, dextropositio cordis and an atrial septal defect with severe pulmonary hypertension. Cineangiocardiography revealed a mitral valve prolapse with slight mitral regurgitation and a dilated right ventricle with severe tricuspid regurgitation. Left and right ventricular function was slightly to moderately reduced. Selective coronary arteriography revealed a coronary fistula from an atrial branch of the left coronary artery to the right atrium. Symptomatic therapy with digitalis, anticoagulation and repeated venesection was initiated because of the severe hemodynamic findings. However, the patient developed syncope at increasingly frequent intervals and died 3 months after the initial examination.

Adult↗
PubMed1979

Left ventricular function in patients with hypertrophic nonobstructive cardiomyopathy.

From 1970 to 1977 the diagnosis of hypertrophic, nonobstructive cardiomyopathy was made by clinical, echocardiographic and hemodynamic findings in 13 patients (2 females and 11 males). Echocardiography showed asymmetric septal hypertrophy in 10 of the 12 evaluated patients (83%) and symmetric hypertrophy of the septum and the posterior wall in 2 patients (17%). Systolic anterior motion of the anterior mitral leaflet was found in 5 patients (42%) although there was no obstruction of the outflow tract. Significantly higher angiographic ejection parameters were present in the 13 patients with cardiomyopathy compared to 13 control patients and the angiographic lateral wall thickness was significantly larger in patients with cardiomyopathy than in controls. The clinical course was followed during 36 mth; a stable course was found in 8 (group A) and a deterioration in 5 patients (group B). Physical working capacity and left ventricular systolic ejection fraction were significantly higher in group A than in group B. Ejection fraction in group B was similar to that of the controls whereas physical working capacity was depressed. Thus, in nonobstructive cardiomyopathy a higher than normal ejection fraction is needed to maintain a normal physical working capacity. Th increased ejection performance seems, however, not to be related to an increase in muscle fiber shortening, but can be explained by geometric reasons.

Adult↗
PubMed1978

[Familial mesomelial dwarfism (Nievergelt syndrome)].

Familial mesomelic dwarfism was first described in 1944 by K. NIEVERGELT, who reported on a father and 3 sons by 3 different mothers who had shortening of the middle segment limbs. in the present study the family described by NIEVERGELT in 1944 is reevaluated and the mode of inheritance investigated over a period of 3 generations. Six patients with mesomelic dwarfism were found out of 43 family members. Two patients, a son of the first patient with mesomelic dwarfism and his son, were seen at our institution. Both presented a rare deformity-combination of the upper and lower extremities. In the upper extremities radio-ulnar synostosis, asymmetrically shaped elbow joints, subluxations of the radial head and a deficient supination capacity of the forearm were diagnosed. The deformities were nearly symmetrical, but a slight predilection for the left forearm was noted. In the lower extremities atypical club-feet with supination of the forefeet, shortening of tibia and fibula and marked synostosis of tarsal and metatarsal bones were seen. The legs were rhombic and supination and pronation of the forefeet were severely reduced. Synostosis of the tibia and fibula and deformities of the toes were found in both patients. The mode of inheritance was considered to be autosomal dominant with high penetrance. It is concluded that familial mesomelic dwarfism is an autosomal dominant disease of the upper and lower limbs with atypical club-feet, marked radio-ulnar, tibia-fibular and tarsal synostosis and deformities of the elbow joints.

Chromosome Aberrations↗
PubMed1977

[Clinical aspects and course of endomyocardial fibrosis].

In 6 women with the hemodynamic, angiographic and histological findings of endomyocardial fibrosis the clinical course was followed for 30 months. All six cases showed the typical partial obliteration of one (4 patients) or both (2 patients) heart chambers. Left ventricular pressure-volume relations elicited decreased distensibility of the left ventricle. The functional state in 2 patients with severe mitral insufficiency was improved following successful mitral valve replacement. One patient with left ventricular, and 2 patients with biventricular, obliteration were given drug therapy and in one case recompensation lasting 2 years was obtained. One patient with partial obliteration of the left ventricle and severe biventricular failure died after an observation period of 4 years. In summary it can be said that (1) endomyocardial fibrosis in Switzerland is observed mainly in females; (2) the clinical picture is dominated by severe congestive heart failure in which the heart size is only slightly enlarged; (3) systolic performance is normal or only moderately depressed despite severe restriction to filling and/or av-valve insufficiency; (4) partial obliteration of the right or left ventricle may be detected by echocardiography; (5) rheumatic fever may occasionally be the cause of endomyocardial fibrosis; (6) mitral valve replacement is accompanied by appreciable improvement in the patient's condition.

Cardiomegaly↗
PubMed1977

Left ventricular contractile function in aortic stenosis evaluated by isovolumic and ejection phase indexes.

This study is to reappraise the usefulness of isovolumic as compared to ejection phase indexes for detecting abnormal left ventricular contractile function patients with a common hemodynamic abnormality, namely, chronic left ventricular pressure overload. In 41 subjects with pure or predominant aortic stenosis left ventricular pressure measurements were performed by micromanometry. Single-plane left ventricular cineangiograms were carried out in the right anterior oblique (RAO) and the A-P position. The isovolumic contractile indexes we used in this study were peak measured velocity of shortening of the contractile elements (Vpm) and Vmax obtained from linear extrapolation of total pressure-velocity curves. The end-diastolic and end-systolic RAO cineventriculograms served for the calculation of the ejection phase parameters mean velocity of circumferential fiber shortening (VCF) and mean normalized systolic ejection rate (MNSER). Of the 41 patients, Vpm was depressed in 16 (39 per cent), Vmax in 17 (42 per cent), VCF in 12 (29 per cent), and MNSER in 14 (34 per cent). When the isovolumic and the ejection phase parameters were combined, 24 patients (59 per cent) were found to have at least one of the four contractile indexes below normal. In 26 of the 41 patients the isovolumic and the ejection phase indexes provided the same conclusions as to normality of left ventricular function. In contrast, 15 patients showed discordant isovolumic and ejection phase indexes. An increased left ventricular end-diastolic pressure was only inconsistently related to an abnormal left ventricular function because in 7 of 28 patients with an end-diastolic pressure above 14 mm. Hg all contractile indexes were normal. Furthermore, a normal end-diastolic pressure was present in three of 24 patients with depressed myocardial function. It is concluded that in chronic left ventricular pressure overload from aortic stenosis neither the isovolumic nor the ejection phase indexes are superior in sensitivity for assessing contractile function. In this clinical setting the combination of both types of indexes appears to be the most reliable way for identifying patients with depressed contractile function of theleft ventricle in the basal state.

Adolescent↗
PubMed1976

[Clinical course of congestive cardiomyopathy].

In 30 patients with the clinical, hemodynamic and angiographic findings of congestive cardiomyopathy, physical working capacity (PWC) and left ventricular (LV) ejection phase indexes at rest (EI), assessed by LV ejection fraction, enddiastolic volume, and mean rate of circumferential fiber shortening were determined. Based on PWC and EI, the following 3 functional groups were obtained: group 1 (n = 6) with normal PWC and borderline EI (subclinical congestive cardiomyopathy), group 2 (n = 10) with normal PWC and decreased EI, and group 3 (n = 14) with reduced PWC and severely depressed EI. The clinical course was followed for 24 months. In group 1 the functional state remained unchanged in all 6 patients. In group 2, 3 patients, changed to functional group 3 and 2 died during the observation period. In group 3, 3 patients improved and changed to functional group 2, 5 remained unchanged and 6 died after an observation period of 16 months. It is concluded (1) that PWC in congestive cardiomyopathy may be preserved despite depressed LV function, and (2) that the functional classification has important prognostic implications, in view of the fact that annual mortality in group 1 was 0%, in group 2 7% and in group 3 32%.

Adult↗
PubMed1975

[Left ventricular cineangiography and echocardiography in patients with the mesosystolic click-telesystolic murmur syndrome].

Twenty-three patients with the midsystolic click - late systolic murmur syndrome were investigated by right and left heart catheterization, cineangiography and echocardiography. Most had symptoms such as atypical chest pain and arrhythmias. Except in one patient, slight to moderate mitral incompetence was present at rest (20 patients) or during stress testing by the infusion of aramine or rapid atrial pacing (2 patients). In all cases, an abnormal systolic mitral valve motion was found by left ventricular cineangiography. In 14 of 15 technically satisfactory echocardiograms a systolic prolapse of one or both mitral leaflets was observed. A minority of the patients had localized abnormal wall motion in the posterobasal area or moderate generalized hypokinesis.

Cineangiography↗
PubMed1975

[Proceedings: Left ventricular cineangiography and echocardiography in patients with the mesosystolic click-telesystolic sound syndrome].

Twenty-three patients with the syndrome of midsystolic click--late systolic murmur were investigated by right and left heart catheterization, cineangiography and echocardiography. Most of the patients had symptoms such as atypical chest pain and arrhythmias. Except in one patient, slight to moderate mitral incompetence was found at rest or during stress testing by infusion of aramine or rapid atrial pacing (2 patients). In all cases, an abnormal systolic mitral valve motion was found in left ventricular cineangiography. In 14 of 15 technically satisfactory echocardiograms a systolic prolapse of one or both mitral leaflets was observed. A minority of the patients had localized abnormal wall motion in the postero-basal area or moderate generalized hypokinesis.

Cineangiography↗