Plural immunoglobulin synthesis in a single cell: an ultrastructural study of two cases with three M-proteins.

Electrophoresis revealed two cases of malignant lymphoma that each contained three M-proteins (IgM lambda.lgG kappa.lgG lambda and IgM lambda.IgM kappa.lgG kappa) in the sera. To determine cellular origin of each M-protein, atypical lymphoid and plasmacytoid cells of both cases were examined by electron microscopy. Atypical lymphoid and plasmacytoid cells possessed rough endoplasmic reticula (RERs) in varying degrees, as seen by conventional electron microscopy, and showed double-stainability for plural antibodies against immunoglobulins following double stainings of immunoelectron microscopy using immunogold staining. Rabbit antibodies against human IgM, lgG, free kappa-light chain and free lambda-light chain were used for the immunoelectron microscopic staining. By the double staining method, plural immunoglobulins, IgM/IgG, IgM/free kappa, IgM/free lambda, IgG/free kappa, IgG/free lambda and free kappa/free lambda, were simultaneously detected in varying degrees in the Golgi area, RERs, and dense bodies of lymphoid and plasmacytoid cells. In conclusion, this study directly exhibited, through electron microscopy, that plural immunoglobulins were synthesized at the same time in a single cell, and that the process of immunoglobulin synthesis in the lymphoid and plasmacytoid cells was different from that in a normal B-cell.

[Tricuspid and mitral regurgitation due to congenital hypoplasia of atrioventricular valves in the aged: a case report].

A 76-year-old woman with a history of repeated right-sided cardiac failure during the past 2 years presented with tricuspid and mitral regurgitation due to congenital hypoplasia of atrioventricular valves. Two-dimensional echocardiography demonstrated enlarged right atrium and right ventricle, and discoaptation between the leaflets. Color Doppler echocardiography revealed severe tricuspid regurgitation through the gap between the leaflets. Autopsy showed congenital hypoplasia of the leaflets and the chordae tendineae in the tricuspid and mitral valvular apparatus.

A case of cardiac sarcoidosis with remarkable atrophy of the left ventricular septum on two-dimensional echocardiography.

Sarcoidosis is a multisystem granulomatous disorder of unknown etiology which mainly affects the lungs, skin, the lymphoreticular system, and the heart. We report a case of cardiac sarcoidosis in which a remarkably thin ventricular septum was demonstrated on two-dimensional echocardiography.

Bilateral coronary ostial stenosis associated with aortitis syndrome.

A patient with aortitis syndrome showed severe stenosis of the bilateral coronary ostium. We discuss the coronary angiographic findings and the treatment.

[Effects of oral flecainide treatment of refractory tachyarrhythmias].

Oral flecainide treatment was given to five patients who were refractory to conventional antiarrhythmic agents. The five patients included one with atrioventricular reentrant tachycardia (AVRT), one with non-sustained ventricular tachycardia (nsVT) and three with sustained VT (sVT). Flecainide produced favorable responses in patients of AVRT, nsVT and sVT with arrhythmogenic right ventricular dysplasia (ARVD). In the case of AVRT, flecainide exhibited a preventive effect on tachycardia induced by programmed electrical stimulation (PES). In the case of nsVT, flecainide markedly reduced the number of VPC and abolished the VT on the Holter ECG. In the case of sVT with ARVD, sVT was not induced by PES after the flecainide. Long-term treatment with flecainide on these three cases produced complete prevention of tachycardias. As an adverse effect of flecainide, an aggravation of congestive heart failure was recognized in one case with cardiac sarcoidosis. PQ interval and QRS interval in all the cases were prolonged after flecainide. The results indicate that flecainide is a useful antiarrhythmic agent for tachyarrhythmias refractory to treatment with conventional drugs.

[Lassa fever associated with effusive constrictive pericarditis and bilateral atrioventricular annular constriction: a case report].

A case of Lassa fever associated with effusive constrictive pericarditis and bilateral atrioventricular annular constriction was reported. A 49-year-old man, who had been diagnosed by indirect fluorescent antibody test as the first case of Lassa fever in Japan, was referred to the Hiroo Hospital because of syncope, progressive hepatomegaly, ascites and pericardial effusion in spite of pericardiocentesis and corticosteroid therapy. On admission, his blood pressure was 92/60 mmHg and he had a paradoxical pulse. Two-dimensional echocardiography revealed a localized pericardial effusion adjacent to the right ventricular wall and behind the left ventricular posterior wall. Bilateral atrioventricular annular constriction was also present. On pulsed Doppler echocardiography, the peak inflow velocities of the right and left ventricles increased during atrial systole. Right heart catheterization revealed a mean diastolic pressure gradient of 8 mmHg across the tricuspid valve. After pericardiectomy, a diastolic dip and plateau pattern became evident in the right ventricular pressure tracing, suggesting the presence of residual constriction. However, the atrioventricular annular constriction was no longer evident on two-dimensional echocardiography. This is considered the first reported case of subacute effusive constrictive pericarditis caused by Lassa fever.

Intracoronary urokinase in acute myocardial infarction: prevalence of total coronary occlusion during the early hours, effects on myocardial infarct size and left ventricular function, and outcome of residual coronary stenosis.

The effects of intracoronary thrombolysis (ICT) were studied in 88 acute myocardial infarction patients. Total coronary occlusion was observed in 67 of the 88 patients (76.1%) who were evaluated within 6 hours of the onset of symptoms. Among these 67 patients 42 (62.7%) were successfully recanalized by intracoronary urokinase. The recanalization rate was higher in the lesion at the left anterior descending artery, in younger patients (49 years or less) and in patients with a shorter history of pre-infarction angina. Eight of 11 patients (72.7%) with subtotal coronary occlusion and 17 of 35 patients (48.6%) with recanalization after ICT showed spontaneous regression of the residual coronary stenosis at the chronic stage angiography. There was no re-occlusion in the subtotal occlusion group and only 6 cases of re-occlusion (17.1%) in the recanalization group. The majority of re-occlusions progressed from the lesion with 99% residual stenosis and delayed filling. Accordingly the true value of additional percutaneous transluminal coronary angioplasty would be limited to the latter cases. Reduction in infarct size and improvement in left ventricular function were limited to those patients with incomplete or subtotal coronary occlusion and were not seen in cases with total obstruction which was recanalized by ICT.

A comparison of the effects of beta-blockers with and without intrinsic sympathomimetic activity on hemodynamics and left ventricular function at rest and during exercise in patients with coronary artery disease.

The effects on hemodynamics and left ventricular function of propranolol and pindolol, beta-blockers without and with intrinsic sympathomimetic activity (ISA), respectively, were compared in six men with stable exertional angina at rest and during symptom-limited bicycle exercise. The study was controlled and double blinded, and the order of drug administration was randomized. The dosage of each drug (propranolol 0.15 mg/kg i.v., pindolol 0.02 mg/kg i.v.) was chosen to reduce exercise tachycardia by approximately 18%. Exercise capacity and duration were similar after each drug, and both drugs similarly reduced ST depression during exercise. At rest, propranolol significantly reduced heart rate, systolic arterial pressure, rate-pressure product, stroke volume index, cardiac index, and left ventricular ejection fraction, and increased systemic vascular resistance. In contrast, pindolol significantly reduced only the rate-pressure product and cardiac index, and to a lesser extent than propranolol. During exercise on the other hand, both drugs reduced the heart rate, arterial pressure, rate-pressure product, and cardiac index to a similar degree. Neither drug altered pulmonary artery wedge pressure, systemic vascular resistance, stroke volume index, left ventricular ejection fraction, or left ventricular end-diastolic and end-systolic volume indices. At equipotent beta-blocking dosages, ISA in pindolol reduced the effects of beta-blockade on resting hemodynamics and left ventricular function compared to propranolol, but during exercise the influence of ISA was reduced; and both pindolol and propranolol exert similar effects on hemodynamics and left ventricular function.

The reproducibility of nongeometric analysis of cardiac output and left ventricular volume by radionuclide angiography.

This study examines the reproducibility of individual radionuclide attenuation factors used in the calculation of cardiac output and left ventricular volume by the nongeometric radionuclide method. Twenty male patients were studied at rest with thermodilution measurements of cardiac output on two separate days. Simultaneous equilibrium radionuclide angiograms were performed and left ventricular stroke volume and cardiac output were determined by the nongeometric method. Individual patient attenuation factors were calculated as the ratio of thermodilution and radionuclide cardiac output measurements at each study. There was a close linear relationship between radionuclide and thermodilution measurements of cardiac output in each study (r = 0.88 study 1, r = 0.97 study 2). A similar relationship was found for measurements of left ventricular stroke volume (r = 0.86, study 1, r = 0.97 study 2). Individual radionuclide attenuation factors ranged from 2.49 to 3.46 in study 1 and from 2.77 to 3.29 in study 2. The individual attenuation factors were reproducible to within 10% in 13 patients and to within 15% in 19 patients. When cardiac output was calculated from the radionuclide data of study 2, by means of individual attenuation factors previously determined in study 1, there was a good correlation with the simultaneous thermodilution measurements of cardiac output (r = 0.92, SEE = 0.38 L/min). Individual radionuclide attenuation factors show little variation in serial studies. Thus the nongeometric radionuclide technique can be used to make accurate serial measurements of cardiac output and left ventricular volume.

Improvement in exercise capacity and associated changes in hemodynamics and left ventricular function after the addition of metoprolol to nifedipine in patients with stable exertional angina.

In 10 men with stable exertional angina, the changes in exercise capacity, hemodynamics, and left ventricular (LV) function were measured after 20 mg sublingual nifedipine (N) and again after adding 100 mg oral metoprolol (M). Nifedipine alone did not significantly improve exercise workloads (+18%) and duration (+21%), but the addition of metoprolol increased both parameters by a further 37 and 32%, respectively (both p less than 0.005 vs. N). After nifedipine the onset of angina was slightly delayed (5.14 +/- 2.41 min placebo (P), 6.00 +/- 2.31 min N, p less than 0.1) and occurred at higher workloads (36 +/- 17 W P, 43 +/- 8 W N, p less than 0.1). After the addition of metoprolol, the onset of angina was delayed substantially more (9.57 +/- 2.22 min, p less than 0.001 vs. P and N) and occurred at much higher workloads (62 +/- 20 W, p less than 0.001 vs. P and N). At rest (R) and during exercise (E), nifedipine decreased systemic vascular resistance (-36% R, -27% E, both p less than 0.001) and mean arterial pressure (-18% R, -21% E, both p less than 0.001), and increased heart rate (+15% R, +11% E, both p less than 0.001), Pulmonary artery wedge pressure on exercise increased less (22 +/- 7 mmHg P, 13 +/- 5 mmHg N, p less than 0.001). After adding metoprolol, the major change was a reduced heart rate (-25% vs. N at R and E, both p less than 0.001), and arterial pressure was unaltered. Pulmonary artery wedge pressure on exercise increased to 18 +/- 5 mmHg (p less than 0.05 vs. N). Exercise LV ejection fraction and volume did not change significantly after adding metoprolol despite marked improvement in angina. In this acute exercise study in patients with stable exertional angina, metoprolol added to nifedipine markedly improved exercise capacity by preventing the increase in heart rate seen with nifedipine. In our patients with relatively normal LV function at rest, the combination was safe and produced no deleterious effects on LV function.

[Reliability and limitation of various diagnostic methods including nuclear medicine in myocardial disease (author's transl)].

Electrocardiography (ECG), echocardiography, nuclear method, cardiac catheterization, left ventriculography (LVG) and endomyocardial biopsy (biopsy) were performed in 40 cases of cardiomyopathy (CM), 9 of endocardial fibroelastosis (EFE) and 19 of specific heart muscle disease (SHMD), and the usefulness and limitation of each method was comparatively estimated. In CM, various methods including biopsy were performed. The 40 patients were classified into 3 groups, i.e., 1) hypertrophic (17), 2) dilated (20) and 3) non-hypertrophic . non-dilated (3) on the basis of left ventricular ejection fraction (LVEF) and hypertrophy of the ventricular wall assessed by LVG and/or echocardiography. The hypertrophic group was divided into 4 subgroups: 9 septal, 4 apical, 2 posterior and 2 anterior. M-mode scan was useful for detecting hypertrophy at the site of the ventricular septum and posterior wall, but not at the site of the anterior wall and apex. The hypertrophy was detected in 18 out of 20 cases using nuclear method. The posterior wall was hypertrophic but the septum was normal in 2 cases. In 2 of 3 non-hypertrophic . non-dilated cases, the left ventricle was oval in shape by LVG, echocardiography was normal, but significant pathological changes were seen in the biopsy, and there were abnormal ECG findings. There was no correlation between the ECG findings and the site of ventricular hypertrophy. Left ventricular ejection fraction measured by LVG (Kasser) had a closer correlation to LVEF obtained by nuclear method (multigated blood pool scan; r = 0.97) than LVEF by echocardiography (Teichholz; r = 0.79), although LVEF obtained by nuclear method was slightly higher than LVEF obtained by LVG. Myocardial perfusion defect was found in 10 of 20 cases of dilated cardiomyopathy (DCM) and the site of relative hypokinesis coincided with the site of the defect in 6 of 7 cases. A semi-quantitative myocardial perfusion defect index (PDI) and histo pathological contractility failure index (HCFI) obtained by the biopsy were devised. HCFI is the score of myocardial degeneration, fragmentation and fibrosis. The PDI plus HCFI had a close correlation with LVEF (r = -0.898). In 9 cases of DCM, LVEF was more reduced than right ventricular ejection fraction. The perfusion defect was also found in 4 cases of EFE and 4 cases of SHMD, i.e., sarcoidosis, postmyocarditis, Kugelberg-Welander disease and cardiac tumor. We conclude that the nuclear study is useful in assessing the site of the abnormal ventricular thickening, perfusion defect and ventricular function. Echocardiography is most useful in detecting ASH. The biopsy gives the sole diagnostic clue, especially in non-hypertrophic . non-dilated cardiomyopathy. ECG is useful in all cases but correlation with the site of disproportional hypertrophy was not obtained.

[Two-dimensional echocardiographic and RI angiographic features of aneurysm of the ascending aorta in patients with annuloaortic ectasia (author's transl)].

The purpose of this study was to compare the diagnostic value of two-dimensional echocardiography with that of other methods in the detection and localization of aneurysm involving the ascending aorta in patients with annuloaortic ectasia. Two-dimensional echocardiography, RI angiography, CT scan and aortography were performed in 19 patients (12 patients with Marfan's syndrome, 4 with aortitis syndrome and 3 with postoperative perivalvular aneurysm). Eight of 12 patients with Marfan's syndrome had dissection in the ascending aorta which was confirmed at surgery or autopsy. The following observations were obtained. 1) Dissection of the ascending aorta was clearly demonstrated on the two-dimensional echocardiogram in 7 patients by recording the intimal tear and flap, and in these cases the short axis two-dimensional echocardiogram of the ascending aorta was more useful in identifying the site and extent of dissection. 2) In patients with postoperative perivalvular aneurysms, RI angiography proved to be a more useful and sensitive technique in differentiating a leakage into the aneurysm from clots in the aneurysm. 3) CT scanning proved to be an insensitive technique to detect dissection of the ascending aneurysm and to differentiate a leakage from clots in the perivalvular aneurysm. From these observations, we concluded that two-dimensional echocardiography and RI angiography proved to be sensitive techniques in detecting dissection of the ascending aneurysm and evaluating a postoperative aneurysm in patients with annuloaortic ectasia.