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B O Olofsson

Publications and source records attributed to B O Olofsson.

51 records · Page 3Linked to original sources

Myeloma-associated cardiac amyloidosis. A case report.

A patient with Bence-Jones myeloma and amyloidosis was treated with cytotoxic drugs and plasmapheresis, resulting in rapid improvement of myeloma-associated symptoms and signs. However, amyloidosis-associated symptoms, especially hypotension, grew worse. Echocardiographic examination demonstrated hypertrophy and a hyperrefractile appearance of the myocardium, thought to be pathognomonic of amyloid heart disease. A permanent pacemaker was inserted for treatment of Adams-Stokes attacks caused by sick sinus syndrome and atrioventricular conduction disturbances. Postmortem examination of the heart demonstrated abundant amyloid deposits corresponding to the altered acoustic qualities of the myocardium. Echocardiographic examination is a valuable non-invasive method for demonstration of amyloid deposits in the heart in multiple myeloma.

Aged↗

Cardiopulmonary function in sarcoidosis.

Hemodynamics, angiocardiographic findings and lung function were evaluated in 10 patients with clinically, radiologically and histopathologically established sarcoidosis. All 10 patients had cardiac symptoms and/or abnormal ECG. Right and left heart catheterization, angiocardiography, spirometry and diffusion capacity were performed in all patients. Pulmonary hypertension was present in the two patients with stage III sarcoidosis and in 7 of 8 patients with stages I and II. Right ventricular end-diastolic pressure was elevated in 6 patients. Seven patients had findings compatible with a failing left ventricle with elevated end-diastolic pressure and/or elevated pulmonary capillary wedge pressure. None of the patients had entirely normal hemodynamic findings. Angiocardiography showed a low ejection fraction in 6 patients but no wall asynergy. Right ventricular angiography and coronary angiography were normal in all patients. Findings compatible with failure of the left and right ventricle of the heart was common in our sarcoid patients with cardiac symptoms and/or abnormal ECG. Impairment of the pulmonary function does not seem to be the only reason for the hemodynamic findings, thus indicating the possibility of myocardial sarcoidosis.

Angiocardiography↗

Pacemaker treatment in familial amyloidosis with polyneuropathy.

Involvement of the heart is common in familial amyloidosis with polyneuropathy (FAP) and is manifested by disturbances of atrioventricular and intraventricular conduction and dysfunction of the sinus node. Pacemaker implantation was performed in 20 patients with FAP in northern Sweden between 1968 and 1983 for the following indications: complete heart block (11), second degree heart block (1), sinus node dysfunction (5), and atrial fibrillation with a slow ventricular rate (3). There was a prompt improvement of symptoms attributable to a slow heart rate. Dislodgement of the electrode occurred in four patients; no other serious complications were observed. From our experience we conclude that at least one out of ten patients with FAP will eventually require pacemaker treatment. Cardiac pacing is an effective adjunct in the symptomatic treatment of FAP, although the ultimate prognosis depends mainly on the underlying disease.

Adult↗

Disturbances of cardiac rhythm and conduction in familial amyloidosis with polyneuropathy.

Sixteen consecutive patients with familial amyloidosis with polyneuropathy of varying duration and severity underwent 24 hour ambulatory electrocardiographic monitoring. A high incidence of sinus node dysfunction, supraventricular and ventricular arrhythmias, and disturbances of atrioventricular conduction was found. Considerably more arrhythmias and disturbances of conduction were detected by long term electrocardiographic monitoring than by conventional 12 lead electrocardiograms. During a follow up period of three to 14 months five patients needed treatment by a pacemaker, three of them because of symptomatic complete heart block, one because of second degree heart block with heart failure, and one because of symptomatic dysfunction of the sinus node. The tachyarrhythmias did not require specific treatment. Long term electrocardiographic monitoring is a useful adjunct in the evaluation of patients with familial amyloidosis with polyneuropathy as it may detect otherwise unrecognised symptomatic disturbances of heart rhythm. The results may be valid also for other forms of amyloidosis involving the heart.

Adult↗

Non-invasive assessment of the presence and severity of cardiac amyloidosis. A study in familial amyloidosis with polyneuropathy by cross sectional echocardiography and technetium-99m pyrophosphate scintigraphy.

Twelve patients with familial amyloidosis with polyneuropathy were examined both by cross sectional echocardiography and by technetium-99m pyrophosphate scintigraphy to assess involvement of the heart non-invasively. All 12 patients had echocardiographic abnormalities. The most prominent findings were highly refractile myocardial echoes, thickened heart valves, and increased thickness of the heart walls. Four patients had abnormal myocardial uptake of technetium-99m pyrophosphate. The remaining eight had equivocal or no myocardial uptake and were considered to have normal scintigrams. A certain amount of amyloid is probably required to produce an abnormal scintigram, although lesions with less amyloid can evidently be identified by echocardiography. Neither the duration of polyneuropathy nor its severity showed any relation to the echocardiographic or scintigraphic findings. It is concluded that cross sectional echocardiography is superior to technetium-99m pyrophosphate scintigraphy in detecting cardiac involvement in familial amyloidosis with polyneuropathy and that these results may also be applicable to other forms of amyloidosis.

Adult↗

Echocardiographic features in familial amyloidosis with polyneuropathy.

Twenty-two patients with the Swedish variant of familial amyloidosis with polyneuropathy were studied by M-mode and two-dimensional echocardiography. These patients had few symptoms consistent with cardiac disease but, nevertheless, echocardiograms of only two of them, both with a short history of the disease, were considered normal in all aspects. The most common abnormality was increased thickness of the interventricular septum found in 20 (91%) of the patients. This septal hypertrophy was asymmetric in 12 (55%) of them. Two-dimensional echocardiography revealed a characteristic hyperrefractile appearance of the myocardium in 15 (68%) of the patients. Thus, echocardiography showed a characteristic pattern in these patients. This study also indicates that cardiac amyloidosis can be diagnosed in the preclinical, asymptomatic state by M-mode and two-dimensional echocardiography.

Adult↗

The cardiac atrioventricular conduction system in familial amyloidosis with polyneuropathy. A clinico-pathologic study of six cases from Northern Sweden.

Cardiac conduction disturbances are frequent in amyloidosis with cardiac involvement, but the pathogenesis of these electrocardiographic abnormalities is not clear. In the present study, the conduction systems in six patients with the Swedish variety of familial amyloidosis with polyneuropathy (FAP), have been analyzed. The results suggest that in FAP, direct amyloid infiltration of the atrioventricular conduction system accounts for the majority of the electrocardiographic disturbances.

Adams-Stokes Syndrome↗

Cor triatriatum.

Explore the source record for details and available documents.

Adult↗

Hemodynamic and angiocardiographic observations in familial amyloidosis with polyneuropahy.

Eleven patients with familial amyloidosis with polyneuropathy were studied by heart catheterization; eight of them also by right and left ventriculography and selective coronary angiography. Signs of congestive heart failure, often reported as typical of cardiac amyloidosis, were found in only one patient. The systolic function was relatively unimpaired, but in several patients hemodynamic data suggested restrictive cardiomyopathy. Six patients showed signs of right or left ventricular outflow tract obstruction. Thus, no uniform hemodynamic pattern could be identified, but the changes found indicate impaired diastolic function.

Aged↗

Pulmonary function in familial amyloidosis with polyneuropathy.

Pulmonary function tests were performed in 14 patients with familial amyloidosis with polyneuropathy. Low maximum expiratory and inspiratory pressures were found in all patients with advanced polyneuropathy, indicating that the neuropathy in this disease involves not only the extremities but also the respiratory muscles. The spirometric findings were consistent with a slight restrictive pattern in several patients, but in two of them the expiratory and inspiratory flows were also low. The intrapulmonary gas distribution was uniform in all. The diffusing capacity was reduced in patients who had had the disease for a long time, which suggests alveolo-capillary block caused by amyloid deposits. The arterial oxygen tension at rest was essentially normal in these patients.

Adult↗

Atrioventricular and intraventricular conduction in familial amyloidosis with polyneuropathy.

Atrioventricular and/or intraventricular conduction disturbances were found in 48 (67%) out of 71 patients with familial amyloidosis with polyneuropathy. Low voltage, often described in various forms of heart amyloidosis, was seen in one patient only. Myocardial infarction patterns, also often reported in heart amyloidosis, were not present in our patients. The prevalence of conduction disturbances increased with the duration of the disease. In 28 out of 47 patients from whom more than one ECG recording was available, a progression of conduction disturbances could be seen. Ten patients developed high-degree conduction disturbances and required permanent pacemaker treatment.

Adult↗