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Biomedical subjects

J Z Przybojewski

Publications and source records attributed to J Z Przybojewski.

66 records · Page 4Linked to original sources

Rheumatic constrictive pericarditis. A case report and review of the literature.

A case of calcific constrictive pericarditis in a young White woman with a convincing history of previous acute rheumatic fever complicated by a possible valvular lesion is presented. Cardiac catheterization confirmed the suspicion of significant cardiac compression. Successful pericardiectomy was carried out, but microscopical examination of the excised pericardium failed to demonstrate a cause. In view of the strong past history of acute rheumatic fever and mild mitral insufficiency demonstrated at cardiac catheterization, the author proposes that the calcific constrictive pericarditis was of rheumatic origin. A review of the literature on the association between rheumatic infection and constrictive pericarditis follows.

Adult↗

A study of a family with inherited disease of cardiac and skeletal muscle. Part III. Genealogical considerations and associations with low intelligence.

A family with inherited cardiac and skeletal muscle disease was also found to have members with low intelligence. The effects of social and environmental conditions upon the behaviour of family members are described, with particular attention to the sociomedical problems created by the combination of a hereditary disease and low intelligence.

Adolescent↗

A study of a family with inherited disease of cardiac and skeletal muscle. Part II. Skeletal muscle morphology and mitochondrial oxidative phosphorylation.

Skeletal muscle morphology and mitochondrial oxidative phosphorylation capacity were examined in a family whose members showed very combinations of mental subnormality, cardiomyopathy and muscle weakness. Light and electron microscopic findings suggested a neuropathic process, while tests of mitochondrial function indicated a state of tight coupling of oxidative phosphorylation, a feature in marked contrast to those in biochemical studies so far reported.

Adenosine Triphosphate↗

A study of a family with inherited disease of cardiac and skeletal muscle. Part I. Clinical, electrocardiographic, echocardiographic, haemodynamic, electrophysiological and electron microscopic studies.

A family consisting of parents and their 6 sons were investigated to elucidate the relationship between a hypertrophic cardiomyopathy, musculoskeletal abnormalities and mental subnormality. The proband was diagnosed as having definite hypertrophic obstructive cardiomyopathy and the remaining family members were shown to have a spectrum of hypertropic non-obstructive cardiomyopathy. Mild muscle weakness was present in 3 sons. All the subjects except for 1 son showed definite signs of electromyographic abnormality, whereas sensory and motor conduction velocities were normal. All the EEGs except for that of the proband were normal. Testicular hypoplasia was present in 3 sons. The inheritance pattern appears to be polygenic autosomal recessive in type. Definite evidence of linkage between hypertrophic cardiomyopathy and HLA awaits further data.

Adolescent↗

Pseudoxanthoma elasticum with cardiac involvement. A case report and review of the literature.

A young Black man with many features of pseudoxanthoma elasticum (PXE), confirmed by skin biopsy, complained of classic angina pectoris, decreasing effort tolerance, and palpitations. Clinically he was in severe congestive cardiac failure which was confirmed by echocardiography and cardiac catheterization, investigations which indicated the presence of a 'congestive' cardiomyopathy. Selective coronary arteriography showed normal epicardial vessels. Antemortem endomyocardial biopsy in this condition is described for the first time in the literature. This showed abnormal light microscopic and electron microscopic features. It is postulated that the predominant cause of congestive cardiac failure and angina pectoris in this disease is a diffuse arteriopathy secondary to elastic fibre dysgenesis, involving the small intramural coronary vessels ('small-vessel disease'). Hitherto it has been accepted that the endocardial changes have been most important in the pathophysiology. A review of the literature as it applies to cardiac involvement in PXE is undertaken.

Adult↗

Post-infarction ventricular septal defect and aneurysm formation.

A fairly young man suddenly developed a large ventricular septal defect (VSD) after an extensive anterolateral and anteroseptal myocardial infarction. Because of congestive cardiac failure refractory to medical treatment he underwent simultaneous closure of the VSD and left ventricular aneurysmectomy 6 weeks after the myocardial infarction. Some 14 months after operation the patient is completely asymptomatic on the minimum of medical therapy and is able to live normally. The literature on these two combined lesions complicating acute myocardial infarction and their surgical correction was caused by coronary spasm of the Prinzmetal variety with underlying normal coronary arteries.

Adult↗

Post-infarction ventricular septal rupture combined with acute right ventricular infarction. A case report.

An elderly White woman suffering from an acute transmural inferior myocardial infarction, with possible true posterior extension, is presented. Her holosystolic cardiac murmur and hypotension are attributed to rupture of the interventricular septum which occurred between the 2nd and 3rd days after infarction. Strong evidence for concomitant right ventricular infarction is put forward and therapy is discussed. As far as the authors can determine, this combination of cardiac lesions has not been documented ante mortem previously. The apparent beneficial use of intravenous hydrallazine, for the first time in right ventricular infarction, is discussed.

Aged↗

Primary cardiac amyloidosis: A review of the literature.

A review of the literature on primary cardiac amyloidosis is presented. Its differentiation from constrictive pericarditis, both on clinical and haemodynamic grounds, is outlined. The importance of antemortem endomyocardial biopsy in establishing a definitive diagnosis of this relatively rare condition is stressed. Finally, an updated overview of the classification and immunological pathogenesis of this disease is given.

Amyloidosis↗

Primary cardiac amyloidosis: A case presentation.

A young man with primary cardiac amyloidosis presented clinically as a case of 'restrictive' cardiomyopathy with an initial diagnosis of constrictive pericarditis, probably of tuberculous origin. Cardiac catheterization showed features compatible with restrictive cardiomyopathy or constrictive pericarditis but pericardial biopsy was negative. Percutaneous right ventricular endomyocardial biopsies established amyloid infiltration of the myocardium, but rectal, tongue and liver biopsies were all negative for amyloidosis. Bence-Jones protein was absent from the urine. Serum electrophoresis, immunophoresis, and bone marrow examination all failed to detect possible multiple myelomatosis as a cause of secondary amyloidosis. Respiratory function tests showed a restrictive picture, probably secondary to chronic congestive cardiac failure. Thus, this case is the second example of primary cardiac amyloidosis in the world literature which has been dianosed by right ventricular endomyocardial biopsy. Phonocardiographic features of early aortic valve closure are reported for the first time in amyloid restrictive cardiomyopathy. Since tuberculous constrictive pericarditis is an important cause of cardiac disease in this country, with effective therapy, the authors stress more frequent use of endomyocardial biopsy as a safe diagnostic tool to exclude other similar disease states. The patient was offered the possibility of a cardiac transplantation in view of the localized nature of his disease, but refused. He now continues to respond unsatisfactorily to antifailure therapy with, presumably, a poor prognosis.

Adult↗

Two simple inexpensive photographic methods for viewing ECG-gated radionuclide blood pool images.

Although the ECG-gated radionuclide blood pool scan (GBPS) has become an established method for studying regional myocardial wall motion, it is usually performed with the aid of an expensive computer system. A simple, inexpensive method was developed to view gated radionuclide blood images by a film loop and a photographic motion detection (PHOMOT) technique. These techniques were compared with left ventricular cine angiography in 15 patients. Segmental wall movement (78 segments) showed identical results in 92% of cases. In all patients the same diagnosis was arrived at by GBPS and cine angiography. The photographic techniques developed offer a simple screening procedure to reduce cardiac catheterization in patients with suspected abnormalities of left ventricular wall contraction.

Cineangiography↗

Holter monitoring at Tygerberg Hospital, 1979-1983--an appraisal.

Over a period of 4 years Holter monitoring was performed on 607 patients in the Division of Cardiology at Tygerberg Hospital. Indications for monitoring were broadly grouped into four categories: (i) evaluation of symptoms suggestive of disorders of cardiac rhythm (210 patients); (ii) evaluation of arrhythmias associated with a specific underlying cardiac condition (139 patients); (iii) evaluation of a previously documented or suspected arrhythmia (233 patients); and (iv) miscellaneous reasons (25 patients). Findings are presented and aspects of Holter monitoring are discussed. Finally, some recommendations for improving the clinical value of our Holter analyses are made.

Arrhythmias, Cardiac↗