Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018801 (heart failure)
 72,216 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

If well managed, pregnancy in women with sarcoidosis is usually carried to term with no problem, excepting rare contraindications. There is no specific risk for the embryo-fetus. Fertility is unchanged. Pregnancy can be contraindicated in case of respiratory failure due to heart failure or central nervous system disorders. A vital capacity less than 100 ml or pulmonary hypertension contraindicate pregnancy. Pregnancy should be discouraged during a period of active disease progression., but pregnancy in itself does not aggravate sarcoidosis. A flare up 3 to 6 months after delivery is not unusual. Therapeutic indications are the same as outside pregnancy, corticosteroid therapy being the only proven treatment during this period. Methotrexate and antimalaria drugs are contraindicated. Methotrexate, even with low weekly doses has a teratogenic effect, particularly between 6 and 8 weeks gestation. Likewise, congenital anomalies have been described with antimalaria drugs although the risk is less than with methotrexate.
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PMID:[Sarcoidosis and pregnancy]. 1063 2

Takayasu arteritis (TA) is a rare manifestation of systemic large vessel vasculitis which affects predominantly the aorta and its main branches, but often remains unrecognised owing to delayed diagnosis and non-characteristic clinical features. Sarcoidosis, too, is a systemic inflammatory disease which can affect virtually any organ system. Reports about the coincidence of both diseases have appeared. The case presented here is characterised by a significant time lag between detection of TA and appearance of clinical signs of sarcoidosis. The woman, now 39 years old, had erythema nodosum, circumscript alopecia, and recurrent uveitis, which dated back to 1980 and was attributed to sarcoidosis. At least 12 years later aortic valve insufficiency with progressive cardiac failure developed. Histology performed at the time of aortic valve prosthesis in 1997 disclosed a diagnosis of TA, which was confined to the aortic root. Incidentally, sarcoidosis was diagnosed in adjacent lymph nodes. A thorough check up failed to detect further manifestations of TA; thus, possibly, the patients had aortitis similar to, but not identical with, TA. Several related cases previously reported are discussed, suggesting that both diseases may be inherently related as they are characterised by certain non-specific, immunoinflammatory abnormalities. This case report suggests that the prevalence of TA, or related forms of arteritis, may be higher than expected and should be considered, especially in younger patients with non-characteristic cardiovascular symptoms and suspected systemic inflammatory disease. Moreover, the association with sarcoidosis in this and other previously described cases suggests that the two diseases may be related and that TA or TA-like vasculitis may even be a complication of sarcoidosis.
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PMID:Concurrence of sarcoidosis and aortitis: case report and review of the literature. 1105 59

Secondary and infiltrative cardiomyopathies are the least common forms of cardiomyopathy and often are the most difficult to treat. In all cases, efforts should be made to establish a specific diagnosis because the removal or avoidance of the causative agent (eg, alcohol, cocaine, persistent tachycardia) holds the best promise for reversal of ventricular dysfunction. Patients who present with a dilated cardiomyopathy (DCM) should be treated with standard heart failure therapy. However, the "standard" is changing and clinicians need to take heed of results of recent trials establishing the role of beta-blockers, aldosterone, and angiotensin receptor antagonists in addition to the regimen of digoxin, diuretics and angiotensin-converting enzyme (ACE) inhibitors. In contrast, patients who present with a more infiltrative clinical picture often manifest more diastolic dysfunction and need strict volume control to maintain euvolemia. For patients with biopsy-proven myocarditis, immunosuppressive therapy generally should be considered in an effort to maintain and potentially improve ventricular function. Patients with sarcoid heart disease have shown the greatest response to high-dose corticosteroids. Patients with hemochromatosis related cardiomyopathy should be treated with iron chelation therapy and phlebotomy. The role of cardiac transplantation is limited, as most of the secondary and infiltrative causes of cardiomyopathy are associated with an adverse posttransplant outcome. Other surgical options, such as left ventricular assist devices, may offer hope to patients who would otherwise be ineligible for cardiac transplantation. On the horizon, biventricular pacing and treatments targeted at cytokines and hormonal receptors hold the promise of improving symptoms and prolonging survival by counteracting the deleterious effects of these secondary mediators.
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PMID:Secondary and Infiltrative Cardiomyopathies. 1109 42

The treatment of sarcoid cardiomyopathy can be considered in part the treatment of the systemic disorder, and in part cardiac involvement, the manifestations of which may differ greatly. Therapy for the systemic disease is corticosteroid. Therapy for cardiac involvement includes prednisone, but because treatment must ameliorate or abolish many differing manifestations, therapy differs among patients. Asymptomatic patients (the majority) who are free from serious manifestations of the disease do not require pharmacologic or interventional treatment. Patients with dilated cardiomyopathy require treatment for congestive heart failure. High-grade atrioventricular conduction delay usually necessitates a permanent electronic pacemaker. Life-threatening arrhythmia usually requires implantation of an automatic implantable cardiac defibrillator (AICD). Antiarrhythmic drugs may also be needed. Cardiac tamponade should be treated by drainage of pericardial fluid. Pericardiectomy is usually the appropriate treatment for patients who develop significant constrictive pericarditis. Calcium channel blockers may be helpful for severe diastolic dysfunction that occurs in those with restrictive cardiomyopathy. Therapy should be given to those few patients who manifest hypertrophic cardiomyopathy to relieve left ventricular outflow obstruction. Cardiac transplantation for intractable heart failure or arrhythmia may be needed.
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PMID:Sarcoidosis and the Heart. 1109 43

A hundred and six clinically diagnosed cases of epidemic dropsy, admitted in June to August 1998 to the P-III unit of RML Hospital and the Department of Pediatrics, Safdarjang Hospital, were studied. All of them consumed mustard oil contaminated with Argemona mexicana, confirmed by ferric chloride and nitric acid tests. No specific sex predilection was seen. No child was affected below the age of 3 years. Pedal edema and reddish hyperpigmentation were the most consistent findings (100 per cent). Frank cardiac failure was seen in only 24 (22.64 per cent), yet persistent tachycardia was alarmingly high (104/106, i.e. 98.4 per cent). Notably ECG showed prolonged Q-T interval in 24 children (22.64 per cent), unrelated to serum Ca2+ level in patients with congestive cardiac failure (CCF). Color Doppler echocardiography showed biventricular dilatation in all the 24 patients with CCF. Wide pulse pressure was recorded in two patients only. Mortality occurred in only two patients (1.89 per cent). Eye involvement was a late finding. All those who survived (i.e. 104/106) recovered completely, except two patients who were left with sarcoid-like changes of skin telangiectasia.
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PMID:A two-centre collaborative study on clinico-epidemiological profile of a recent outbreak of epidemic dropsy in New Delhi (India) with special emphasis on its cardiac manifestations in pediatric patients. 1169 29

Cardiac involvement is an important prognostic factor in sarcoidosis, but reliable indicators of mortality risk in cardiac sarcoidosis are unstudied in a large number of patients. To determine the significant predictors of mortality and to assess the efficacy of corticosteroids, we analyzed clinical findings, treatment, and prognosis in 95 Japanese patients with cardiac sarcoidosis. Twenty of these 95 patients had cardiac sarcoidosis proven by autopsy; none of these patients had received corticosteroids. We assessed 12 clinical variables as possible predictors of mortality by Cox proportional hazards model in 75 steroid-treated patients. During the mean follow-up of 68 months, 29 patients (73%) died of congestive heart failure and 11 (27%) experienced sudden death. Kaplan-Meier survival curves showed 5-year survival rates of 75% in the steroid-treated patients and of 89% in patients with a left ventricular ejection fraction > or = 50%, whereas there was only 10% 5-year survival rate in autopsy subjects. There was no significant difference in survival curves of patients treated with a high initial dose (> 30 mg) and a low initial dose (> or = 30 mg) of prednisone. Multivariate analysis identified New York Heart Association functional class (hazard ratio 7.72 per class I increase, p = 0.0008), left ventricular end-diastolic diameter (hazard ratio 2.60/10 mm increase, p = 0.02), and sustained ventricular tachycardia (hazard ratio 7.20, p = 0.03) as independent predictors of mortality. In conclusion, the severity of heart failure was one of the most significant independent predictors of mortality for cardiac sarcoidosis. Starting corticosteroids before the occurrence of systolic dysfunction resulted in an excellent clinical outcome. A high initial dose of prednisone may not be essential for treatment of cardiac sarcoidosis.
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PMID:Prognostic determinants of long-term survival in Japanese patients with cardiac sarcoidosis treated with prednisone. 1170 97

Cardiac sarcoidosis induces heart failure death or sudden death in many cases and is thus often associated with a poor prognosis. In Japan 47-78% of sarcoidosis patients die of cardiac lesions. Early diagnosis is important in such cases, and a comprehensive judgment based on the endomyocardial biopsy, echocardiography and nuclear medicine examination findings should be made according to the 'Handbook of the Diagnosis of Cardiac Sarcoidosis'. Once a diagnosis is made the introduction of steroid therapy should be considered. Steroid administration should be conducted referring to the 'Guidelines to the Treatment of Cardiac Sarcoidosis'.
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PMID:[Cardiac sarcoidosis]. 1223 77

A 49-year-old female was admitted to our hospital because of worsening of congestive heart failure on November 2000 in a state after insertion of permanent pacemaker for complete atrioventricular block in 1986, followed by a clinical history of chronic heart failure due to dilated cardiomyopathy. After admission, her general condition had been improved, but, she had massive hemoptysis suddenly and died on February 2001. At autopsy, noncaseating granulomas were observed scattering in lungs, liver and spleen, not associated with any infectious lesions, therefore indicating systemic sarcoidosis. In lungs, granulomatous arteritis in small- and medium-sized muscular arteries associated with disputation of the media and elastic laminae were observed, suggesting the direct cause of hemoptysis. This is the extremely rare case of pulmonary arteritis with systemic sarcoidosis resulting the death from massive hemoptysis.
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PMID:[An autopsy case of massive hemoptysis in systemic sarcoidosis with pulmonary granulomatous arteritis]. 1246 21

Chronic myocardial ischemia is the leading cause of impaired myocardial contractility and heart failure. To identify differentially expressed genes in human ischemic cardiomyopathy (ICM), we constructed a subtracted cDNA library using specimens of ICM compared to normal human heart. Among 100 randomly sequenced clones, seven sequences represented recently identified candidate genes for differential expression in cardiac hypertrophy. A further clone without a known hypertrophy-association coded for the adhesion molecule NCAM(CD56). RNase protection assay, immunohistochemistry, and Western blotting revealed strong overexpression of NCAM(CD56) in all hearts with ICM (n = 14) compared to normal hearts (n = 8), whereas in congestive cardiomyopathy (CCM) (n = 8), hypertrophic obstructive cardiomyopathy (n = 2), myocarditis (n = 4), and sarcoidosis (n = 2), at most slight overexpression of NCAM(CD56) was observed. NCAM(CD56) overexpression abnormally involved the whole cell membrane and the cytoplasma of cardiomyocytes only inside and adjacent to ischemia-induced cardiac scars. Normal or hypertrophic fibers at a distance from ischemic scars were devoid of NCAM overexpression. Identical alterations were observed in an experimental rat ICM model, but not in normal nor in spontaneously hypertensive rat hearts. In search of NCAM(CD56)-related transcription factors we found RUNX1(AML1) up-regulation in ICM and detected RUNX1(AML1) binding within the NCAM(CD56) promoter by electromobility shift assay. We concluded that strong overexpression of NCAM(CD56) and RUNX1(AML1) is a constant and characteristic feature of cardiomyocytes within or adjacent to scars in ICM.
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PMID:NCAM(CD56) and RUNX1(AML1) are up-regulated in human ischemic cardiomyopathy and a rat model of chronic cardiac ischemia. 1293 48

Questionnaires were sent to 46 hospitals of all over Japan in order to obtain the clinical data on sarcoidosis patients who were treated with oral corticosteroids. The number of female patients was greater than that of male patients (1.5:1), and the average age was 44.9 +/- 16.5 with peaks at 20 and at 50 to 60. The markers of disease activity were high in serum or bronchoalveolar lavage fluids (BALF): specifically, the serum angiotensin-converting enzyme (sACE) was 27.9 +/- 31.9 IU/ml (n.v. < 21.4), and the CD4/CD8 lymphocyte ratio was 6.5 +/- 5.7. Eye involvement was the most common reason for systemic steroid therapy, followed in order by lung and heart involvement. The main reasons for steroid therapy were the exacerbation of ocular symptoms, visual disturbance, respiratory symptoms, such as cough or exertional dyspnea, progression of chest radiographic findings, heart failure and severe arrhythmia, such as AV block. The initial corticosteroid dose was usually 30 mg of predinisolone per day, but for some refractory cases, a 40-60 mg per day was used. Immunosuppressive drugs, such as methotrexate, were also used in the small number of patients who responded poorly to the steroid. Overall, a good clinical response to the drug was found in 70-80% of the steroid treated patients, but in those with cardiac disease, the response rate was only 48%.
Sarcoidosis Vasc Diffuse Lung Dis 2003 Oct
PMID:Clinical characteristics of 195 Japanese sarcoidosis patients treated with oral corticosteroids. 1462 Jan 65


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