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At least 595 records · Page 33Linked to original sources
PubMed1982

Excessive moderator bands in the left ventricle of 21 cats.

Excessive numbers of moderator bands bridging the left ventricular septum and free wall and entangling papillary muscles were associated with heart failure and death in 21 cats. Clinical findings included dyspnea, anorexia, hypothermia, cardiomegaly, pleural effusion, plumonary edema, heart murmurs, gallop rhythm, electrocardiographic abnormalities (especially conduction disturbances), increased left ventricular end-diastolic pressure, angiocardiographic evidence of left ventricular restriction, and aortic thromboembolism. Pathologic changes included a morphologically distinct network of abnormal numbers of moderator bands in the left ventricle, left ventricular hypertrophy (younger cats--mean age, 4 years) or dilatation (older cats--mean age, 8.7 years), left atrial enlargement and hypertrophy, and pulmonary edema with heart failure cells in the alveoli. Heart weights of affected cats were significantly less than those of cats with congestive, hypertrophic, and restrictive cardiomyopathy (endocardial fibrosis), but were not significantly less than heart weights of clinically normal cats. Pathologic changes were characteristic of the syndrome grossly and histologically, but clinical findings were not clearly definable.

Animals↗
PubMed1983

[Adiastole caused by a secondary cardiac hemochromatosis. Successful treatment with an iron chelating agent].

Severe congestive cardiac failure developed in a few weeks in a 44 year old man who had undergone porto-caval anastamosis for post-hepatitis cirrhosis one year previously and then treated for anaemia by repeated blood transfusion and chronic daily oral iron therapy. Infiltrative, congestive and restrictive cardiomyopathy was diagnosed in the presence of global cardiomegaly, electrocardiographic changes (microvoltage, diffuse ST-T wave changes), echocardiographic appearances (dilatation of the left ventricle, with hypertrophic and hypokinetic walls), and hemodynamic signs of adiastole with equalisation of filling pressures at 15 mmHg and a cardiac index of 1,88 l/min/m2. Cardiac haemochromatosis was confirmed by the laboratory (serum iron: 35 mumol/l; siderophilin saturation: 100 p. 100; serum ferritin: 1854 ng/ml; induced siderouria: 51 mg/24 hours) and histological findings (endomyocardial biopsy showing pigment overload). The absence of a family history, of homozygote A3 antigen, of diabetes, of iron overload on hepatic biopsy one year previously, excluded the diagnosis of familial idiopathic haemochromatosis. A secondary form of the disease was diagnosed on a possible genetic predisposition (heterozygote A3 antigen) and on environmental factors (blood transfusions, iron therapy, cirrhosis, alcoholism and perhaps the porto-caval anastamosis. Cardiac haemochromatosis was cured in this case by iron chelating therapy comprising daily subcutaneous infusions of 2 g of desferrioxamine for 2 months. The cure was confirmed by regression of the signs of clinical cardiac failure and of cardiomegaly, the increase in QRS voltages and the near normalisation of the hemodynamic and laboratory findings.

Adult↗
PubMed1984

[Differential diagnosis between constrictive pericarditis and cardiac amyloidosis by computerized M-mode echocardiography].

A diastole is a non specific haemodynamic syndrome which may result from constrictive pericarditis or from a restrictive cardiomyopathy. The aim of this study was to differentiate these two types of condition by analysis of computerised M mode recordings of the left ventricle. Three groups of patients were studied: 5 cases of confirmed cardiac amyloidosis (Am); 5 cases of constrictive pericarditis confirmed surgically (CP) and 10 normal subjects (NL). The study was based on analysis of parameters of ventricular filling and of diastolic thinning of the LV free wall. A significant difference was observed between AM and CP but not between PC and NL. Amyloidosis was characterised by a reduction in the maximum velocity of endocavitary diameter lengthening (AM 0,84 +/- 0,56 cir/sec; PC 3,95 +/- 0,77, p less than 0,01), prolongation of the rapid filling phase (AM 0,42 +/- 0,17 sec; PC 0,16 +/- 0,06 sec, p less than 0,02) and a decrease in maximum velocity of free wall thinning (AM 0,45 +/- 0,23 th/syst/sec; PC 4,79 +/- 2,1, p less than 0,01). The diastolic thickness of the free wall was greater in the amyloidosis group (AM 1,73 +/- 0,61 cm; PC 1,05 +/- 0,21, p less than 0,05) and correlated with the reduction of maximum velocity of free wall thinning. Parameters of global diastolic filling did not distinguish the two conditions. The M mode recordings were therefore digitalised to provide graphs of chamber filling and wall thinning and their derivatives.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1980

Angiocardiographic and enzyme studies in a patient with type II glycogenosis (Pompe's disease). A case report.

An infant with hypotonia, gross cardiomegaly and heart failure is described. Angiocardiography revealed a hypertrophic restrictive cardiomyopathy. The diagnosis of type II glycogenosis was confirmed by the total absence of alpha-1,4-glucosidase in cultured skin fibroblasts. It is now possible to offer prenatal diagnosis by amniocentesis to women at risk of having affected children.

Clinical Enzyme Tests↗
PubMed1982

Atypical erysipelas caused by group G streptococci in a patient with cured Hodgkin's disease.

Erysipelas developed in a young woman whose condition had been in remission for ten years after treatment of stage IIIA Hodgkin's disease. The erysipelas was atypical both in its clinical manifestation and its causative organism. The patient had an erythematous, macular eruption on both buttocks and thighs. Group G streptococci, a rare cause of erysipelas, were isolated from both blood cultures and a skin biopsy specimen. The unusual clinical manifestation of the disease when the patient was initially seen may have been the result of a group G streptococcal bacteremia, coupled with impairment of the lymphatic drainage of the involved area from a partial thoracic duct obstruction and a restrictive cardiomyopathy, both secondary to previous irradiation treatment.

Adult↗
PubMed1995

[MRI in cardiology: clinical applications and perspectives].

MRI is an effective tool in the evaluation of cardiovascular diseases. With continued improvements in MR angiography, velocity mapping, myocardial tagging, imaging speed and display, it is anticipated that MRI will play an ever-increasing role in the morphological evaluation of many cardiovascular anomalies. The need for quantification in cardiology has led to the development of a variety of techniques to assess cardiac size and function. In attempting to answer these clinical questions, MRI provides major advantages several: extraordinary flexibility, powerful contrast mechanisms, sensitivity to flow and motion, and freedom from ionizing radiation, contrast agent and acoustic windows. Because of accurate initial results in quantifying cardiac chamber size, global and segmental function, it is likely that MRI methods will play an increasing role in the evaluation of cardiac structure and function. Recently, MRI has emerged as an important tool in the evaluation of great vessel disease, particularly in the evaluation of both congenital and acquired abnormalities of the aorta. Early evidence suggests that magnetic resonance may help in distinguishing constrictive pericarditis from restrictive cardiomyopathy (e.g., amyloid heart disease). Cardiac-MRI lends itself to assessment of intracardiac masses. Spin-echo imaging alone is often sufficient for diagnosis. Cine-MRI has important additive value, however, when a mass lesion shows dynamic motion, or when abnormal flow patterns in conjunction with a mass lesion require evaluation.

Echocardiography↗
PubMed1994

[Pericardium and heart transplantation].

The authors emphasise the role of the pericardium in the post-operative complications of cardiac transplantation: its role is as important as it is underestimated in its frequency, severity and the pathogenic discussions that it induces. This study, based on a retrospective analysis of 191 anatomo-clinical cases (156 autopsies and 35 retransplantations) of patients undergoing cardiac transplantation before the 1/03/1993 including macroscopic and histopathologic analysis of the pericardial lesions, histochemical analysis of the lymphocytic populations, and analysis of associated coronary and myocardial lesions. The results were classified as early or late (after the 75th postoperative day) complications. Early complications included 27 suppurating mediastino-pericarditis (13 aspergillosis and 14 bacterial), 16 autonomous non-infective pericardial complications (6 haemopericardiums, 6 organised compressive haematomas, 4 early constrictive pericarditis) and 9 lymphocytic epicarditis associated with acute myocardial rejection. Late complications included one common constrictive symphysis and 14 reactivated epicarditis associated with transplantation coronary disease (chronic rejection) with associated lymphoplasmocytic nodules and a myocardial vasculitis. These observations suggest two important conclusions: 1) the need for active diagnostic measures to detect pericardial complications in cases of cardiac transplant dysfunction in the early postoperative period with no signs of acute rejection on myocardial biopsy: the presence of epicardial lesions on biopsy should be taken into account and not just assumed to be a postoperative epiphenomenon; 2) the presence of epicarditis due to reactivated epicarditis may cause inextensible hardening of the epicardium which adds to the effects of restrictive cardiomyopathy of chronic rejection.

Adult↗
PubMed1994

The clinicopathological spectrum of renal amyloidosis.

BACKGROUND: Renal amyloidosis is an uncommon cause of nephrotic syndrome. The clinical conditions in Chinese people remain obscure. This is a retrospective review of the clinicopathological spectrum of 12 cases diagnosed in Taichung Veterans General Hospital from October 1982 to November 1993. METHODS: Charts and renal pathological slides were reviewed retrospectively. A scoring system was set to evaluate the degree of amyloid deposition in the renal tissue. The clinical profile, immunological data, pathological picture and final outcomes are presented and discussed, with literature review. RESULTS: There were 12 cases of primary amyloidosis including 1 case of multiple myeloma. All were confirmed by renal biopsy. The cases were all male with mean age of onset as 53.3 +/- 11.3 (range: 32 to 65 years). The mean follow-up duration was 22.9 +/- 32.8 months. The initial average creatinine clearance was 66.7 +/- 42.7 ml/min; mean daily urine protein was 7.0 +/- 4.1 grams. Nephrotic syndrome was the main clinical manifestation, present in all 12 cases. Other presenting symptoms and signs included: malaise in 7 cases; hypertension and anorexia in 4 cases; limb numbness in 3 cases; low back pain, dizziness and microhematuria in 2 cases each; anemia, headache, stroke, restrictive cardiomyopathy, hepatomegaly, syncope, body weight loss, dysphagia and skin itching in one case individually. Amyloid cardiomyopathy was present in 4 of the 8 patients who received echocardiography. The mean serum albumin level was 1.9 +/- 0.7 mg/dl, globulin level 3.1 +/- 1.1 mg/dl. Urinary Bence-Jones protein examination was performed in 8 cases; none revealed positive response. The mean immunoglobulin (Ig) level for the patients included: IgG 1018 +/- 901 mg/dl, IgA 262.0 +/- 313.8 mg/dl, IgM 104.8 +/- 84.2 mg/dl. There were at least 4 cases with high levels of one Ig but depressed levels in the others. M-component was shown by immunoelectrophoresis (IEP) in 90% of the cases. IEP impression in 10 cases revealed 2 cases of IgA lambda, 2 cases IgA kappa, 3 cases IgG lambda and 1 case IgG kappa monogammopathy, 1 case free lambda myeloma and 1 case negative. The ratio of kappa to lambda chain was 3:6. Bone marrow biopsy performed in 8 cases found only 1 case with multiple myeloma, one with amyloidosis; the other 6 cases were unremarkable. Mesangium was the site of heaviest amyloid deposition, followed by tubular basement membrane, artery and interstitium. The median survival time for those whose total score was lower than 3 points was 97.5 months; 3 to 5 points, 14 months; 6 points or more, 18.5 months. The median survival time was 21.6 months and 3-year-survival rate was 32.7%. The 2 cases with long-term survival were of 111 months and 84 months. The possible reason included: 1) Organ-limited renal amyloidosis; 2) Light amyloid deposition; 3) Younger age; 4) Other undetected favorable factors. CONCLUSIONS: 1) Renal amyloidosis is not a frequent diagnosis of nephrotic syndrome in Taiwan, but it should be suspected in every patient over 50 years old with a recent onset of proteinuria. 2) Renal amyloidosis can be diagnosed only by renal biopsy. 3) Primary renal amyloidosis is a disease of poor prognosis.

Adult↗
PubMed1994

[MRI of the heart: morphological and functional aspects].

The performances of cardiac magnetic resonance imaging have been recently improved by the possibility of obtaining functional information by means of gradient echo sequences. Cavity volumetry and wall thickness and mass measurements are now possible. Ultrarapid sequences are useful for the analysis of myocardial perfusion and methods for measuring blood flow and temporal labelling of the ventricular wall open up new prospects for functional evaluation of the heart. In clinical practice, MRI can be useful for the exploration of cardiac parietal and intracavitary masses, constrictive pericarditis, hypertrophic and restrictive cardiomyopathy and cardiac malformations. Assessment of valvular and coronary heart disease is based on new techniques and is currently under evaluation.

Heart Defects, Congenital↗
PubMed1994

Aortic root replacement with pulmonary autograft in children.

Between September 1988 and February 1993, 14 patients whose ages ranged from 3 months to 16 years (mean 11.1 +/- 4.3 years) underwent replacement of the aortic root with the autologous pulmonary root for aortic valve disease. The follow-up was 4 years (cumulative total of 25.2 patient-years). There was no early mortality. Late mortality (one patient) was 7.1% (95% confidence limits 0% to 21%). This patient had juvenile rheumatoid arthritis and died of consequent congestive heart failure with autograft failure 6 months after operation. Event-free survival after 4 years was 78.6% (95% confidence limits 50% to 95%). One patient was reoperated on because of autograft failure caused by a relapse of rheumatic fever. One patient operated on for critical neonatal aortic stenosis has subnormal exercise tolerance because of restrictive cardiomyopathy and pulmonary homograft regurgitation. The other 12 patients were in New York Heart Association functional class I at the end of follow-up. There was no prevalence of bacterial endocarditis. There were no signs of primary structural degeneration of the pulmonary autograft. During follow-up, in eight patients, increased anulus diameter of the pulmonary autograft could be demonstrated by precordial two-dimensional echocardiography, suggesting growth of the autograft. Our experience shows that aortic root replacement with the pulmonary autograft can be done with low mortality and morbidity in children with aortic valve disease. The operation seems to be contraindicated in children with juvenile rheumatoid arthritis because of the risk of recurrence of rheumatic disease in the autograft. The pulmonary autograft has also been shown to be susceptible to recurrence of rheumatic inflammation in children with a history of acute rheumatic fever. Despite pulmonary autograft replacement of the aortic valve in infants with critical valvular aortic stenosis and endocardial fibroelastosis, clinical results may be poor. Growth of the autograft is suggested by echocardiographic follow-up. We consider aortic root replacement with the pulmonary autograft the procedure of choice in children who require aortic valve replacement.

Adolescent↗
PubMed1993

[Imaging of the pericardium using magnetic resonance].

Evaluation of the pericardium has been rediscovered with the advent of sectional imaging modalities such as magnetic resonance. Owing to the improved image quality, faster imaging and cine display, MRI has proved to have a useful role in assessment of the pericardium. This article illustrates the authors' wide experience with MRI of normal and abnormal pericardium in numerous patients. The anatomy of the normal pericardium is reviewed, as are the different MR imaging techniques used Advantages of MRI in specific situations are discussed. Congenital anomalies are covered but particular attention is paid to acquired diseases, such as pericardial effusions, with emphasis on the characterization of the different pericardiac fluid collections. Pericardial thickening and constrictive pericarditis, which are easily differentiated from restrictive cardiomyopathy, are also discussed. Finally, primary and metastatic tumors are described.

Heart Neoplasms↗
PubMed1993

[Contractility and left ventricular filling in heart failure: an echocardiographic evaluation].

In this paper we analyze the possibility of investigating the various modalities of pump function alteration by the echocardiographic technique through examination of the different parts of the cardiac cycle. While M-Mode and 2D echocardiography make a precise assessment of the systolic function parameters possible, with the Echo-Doppler technique we can study diastolic function and obtain information about many hemodynamic parameters. "Strain dependent" and "strain independent" diastolic function are studied almost exclusively with the Echo-Doppler technique: the diagnosis of restrictive cardiomyopathy can be established without invasive procedures such as cardiac catheterization. We suggest that, in most heart diseases with cardiac failure, diastolic and systolic dysfunction coexist: in mitral and aortic regurgitation, echocardiography can give precise information on the presence of ventricular dysfunction, even in the presence of very few or no symptoms, which helps the cardiologist make an early surgical or non surgical decision when dealing with such valve pathologies.

Cardiomyopathies↗
PubMed1995

MR evaluation of the pericardium.

The pericardium is well visualized on magnetic resonance imaging (MRI) because of the superb contrast resolution and multiplanar capability of the technique. This article highlights some of the clinical uses of MRI in evaluating the pericardium and provides comparison with echocardiography and computed tomography (CT) scan. Specific pericardial diseases that are described include pericardial effusion, constrictive pericarditis, neoplastic pericardial disease, congenital abnormalities of the pericardium, and paracardiac masses. The value of MRI in differentiating constrictive pericarditis from restrictive cardiomyopathy is emphasized. The article discusses the impact of newer MRI techniques such as cine MRI on the assessment of pericardial disease.

Heart Diseases↗
PubMed1995

[The importance of measuring pulmonary venous flow using doppler echocardiography].

Pulmonary venous flow can be evaluated by means of pulsed Doppler echocardiography during transthoracic or even better during transoesophageal examination. In the majority a three-peak spectral curve is recorded with the highest positive wave during ventricular systole, a lower positive wave during ventricular diastole and a negative wave during contraction of the left atrium. The author discusses some factors which have an impact on the pulmonary venous flow and which must be taken into account for its correct evaluation. With regard to clinical importance the author emphasizes in particular the change of venous pulmonary flow when the pulmonary pressure in the left ventricle rises. The latter may be used for its estimation. The pulmonary venous flow is a useful parameter also for quantification of significant mitral regurgitation and the differential diagnosis of restrictive cardiomyopathy and constrictive pericarditis. With regard to the simple assessment, rapidity of assessment and its predictive value it is suited for routine use in the majority of echocardiographic laboratories.

Blood Flow Velocity↗
PubMed1996

[Flow in pulmonary veins].

Pulmonary venous flow can be evaluated by means of pulsed Doppler echocardiography during a transthoracic or transesophageal echocardiography examination. In most cases a three-peak spectral curve with highest positive wave during ventricular systole, a lower positive wave during ventricular diastole and a small negative wave during the left atrial contraction are recorded. Multiple factors affect the systolic and diastolic components of pulmonary venous flow. Pulmonary venous flow measurement may prove to be an important tool in routine judgment of diastolic left ventricular and atrial function. With regard to clinical importance, the pulmonary venous flow is a useful parameter for left atrial pressure quantification and for differential diagnosis of restrictive cardiomyopathy and constrictive pericarditis.

Blood Flow Velocity↗
PubMed1996

[Endomyocardial fibrosis (its clinico-echocardiographic characteristics)].

To compare clinical and echocardiographic features in patients with restrictive cardiomyopathy (RCMP), 15 patients (9 males and 6 females, mean age 34.93 +/- 1.03 years, duration of the disease 9 +/- 4.2 months) were examined using complete echocardiographic and doppler echocardiographic investigation in impulse regimen. Endomyocardial fibrosis was not obvious clinically, but should be suspected in dyspnea upon a weak exercise, undue fatiguability, tachycardia in normal arterial pressure and size of the heart. Echocardiographic indications, on the contrary, were rather specific. Endomyocardial fibrosis is characterized by diminished ventricular cavities, thickening of the endocardium and subvalvular structures, changed shape of ventricular cavity, echo-CG signs of passive pulmonary hypertension, diastolic dysfunction of the left and right ventricles.

Adult↗
PubMed1997

Transthyretin Ile 122 and cardiac amyloidosis in African-Americans. 2 case reports.

Two cases of cardiac amyloidosis resulting from deposition of the Ile 122 variant of transthyretin in African-Americans are presented. These cases illustrate several typical features of this disorder, including electrocardiographic abnormalities and digoxin toxicity. Transthyretin Ile 122 is a common amyloidogenic variant in African-Americans (present as a heterozygous variant in 4% of this population); therefore, the diagnosis of transthyretin Ile 122 cardiac amyloidosis should be considered in African-Americans with unexplained restrictive cardiomyopathy or arrhythmias.

Aged↗
PubMed1996

[Chronic constrictive pericarditis apropos of 3 cases disclosed by refractory cardiac failure].

Chronic constrictive pericarditis is a difficult diagnosis and may present atypically. The authors report three clinical cases and review the diagnostic strategy of constrictive pericarditis. In these three patients, the diagnosis was finally made after one or more years of symptomatic disease and after several diagnostic work ups and ineffective treatments. In cardiac failure, pericardial calcification is often not observed on chest X-ray and Doppler echocardiography is usually the diagnostic investigation. Adiastole presents with dilatation of the vena cava and atria, contrasting with normal ventricles without major valvular disease. Doppler echocardiography enables distinction of constrictive pericarditis from restrictive cardiomyopathy: normal myocardium, thickened pericardium, specific septal motion, inspiratory increase in right ventricular dimensions, premature opening of the pulmonary valve, important variations in ventricular filling with respiration, expiratory diastolic reflux in the hepatic veins. Catheterisation confirms adiastole and may suggest a pericardial aetiology in characteristic cases, associated with only mild increases in pulmonary artery pressure. If need be, the pericardial thickening > 4 mm may be observed with magnetic nuclear resonance imaging and, when a doubt remains with respect to the diagnosis of cardiomyopathy, the absence of fibrosis on endomyocardial biopsy provides the diagnosis and indication for curative surgery: pericardectomy.

Chronic Disease↗