Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Sarcoidosis of the myocardium, an illness occurring predominantly in young adults, frequently becomes clinically apparent when the disease is far advanced. Since the thallium myocardial perfusion scan (TMPS) is known to be capable of detecting granulomas, it seemed to be promising as a noninvasive means of screening for this complication of sarcoidosis. We, therefore, examined 44 consecutive patients with sarcoidosis, none of whom had clinical evidence of heart disease, utilizing TMPS, echocardiography, electrocardiography, systolic time interval ratios (PEP-LVET) and 24-hour Holter monitoring. TMPS disclosed left ventricular defects in 14 patients (32 per cent) and abnormalities of the right ventricle in an additional four patients. Left ventricular abnormalities on the TMPS were associated with echocardiographic evidence of left ventricular dysfunction (widened mitral E point septal separation) but not with abnormalities on Holter monitoring or electrocardiograms. Systolic time interval ratios were insensitive but highly specific for the presence of abnormalities on the TMPS. We conclude that the TMPS frequently discloses abnormalities in sarcoidosis, which may be a reflection of its sensitivity in this setting.
 ...
PMID:Thallium-scan myocardial defects and echocardiographic abnormalities in patients with sarcoidosis without clinical cardiac dysfunction. An analysis of 44 patients. 736 31

Heart-lung transplantation is a complex, expensive, and resource-intensive procedure. It is performed more often where there is coexistence of end-stage pulmonary disease with advanced cardiac disease that is not secondary to pulmonary hypertension (e.g., some forms of congenital heart disease, some situations with Eisenmenger's syndrome, and possibly the coexistence of end-stage heart and end-stage lung disease). Available data indicate that some disease states are associated with a higher probability of successful outcome after HLT than are others. Objectively validated and reliable patient selection criteria cannot be obtained from the current medical literature. Expert opinion provided to OHTA contained varying degrees of detail and uniformity with respect to patient selection (Tables 11 and 12). While most agree that "irreversible cardiopulmonary disease" is an indication for this procedure, there are no published data that have provided details as to how patients so designated were selected as candidates for HLT. For example, for chronic obstructive pulmonary disease (emphysema), neither expert opinion nor published studies of HLT provided data indicating the stage of the disease at which a decision to transplant is reasonable and objectively justifiable. A similar lack of detail for cystic fibrosis, bronchiectasis, pulmonary fibrosis, sarcoidosis, asbestosis, and other diseases was noted. Thus, there are insufficient data regarding transplant recipients to permit the development of objective criteria for a threshold for HLT in the various disease states. The circumstances are more problematic regarding suggested contraindications for HLT. While objective survival rates obtained from registry data indicated that survival bore little relationship to recipient age, various opinions provided to OHTA specified a maximum age for HLT candidates, ranging from under 45 years to 60 years. Adverse factors and contraindications, such as "other significant disease," "recurrent pulmonary emboli," "hepatic disease," "infection," and "insulin-dependent diabetes" were nonspecific and unsupported by published, objective evidence. More ambiguous contraindications included "psychiatric illness," "poor family support structure," "history of noncompliance with medical regimens," and lack of "emotional stability." While psychiatric, psychologic, or sociologic evaluations of potential HLT recipients undeniably have relevance to patient selection, use of such criteria should be supported at least by evidence of generally consistent application across the transplant community. Of note was the recommended requirement that the recipient possess "adequate financial resources," which implies that the benefits of HLT are not expected to be equitably provided to the public.(ABSTRACT TRUNCATED AT 400 WORDS)
 ...
PMID:Institutional and patient criteria for heart-lung transplantation. 771 18

Eighty-one consecutive patients, 32 males and 49 females, with biopsy-proven systemic sarcoidosis, none of whom had clinical evidence of heart disease, underwent M-mode and two-dimensional echocardiography, ECG and chest X-ray in order to investigate the frequency of sarcoid pericardial effusion. Mild or moderate size pericardial accumulations were found by echocardiography in 17 patients (21%), four males and 13 females (mean age 49.7 +/- 9.2 years). Pericardial effusion could not be correlated with clinical symptoms or physical signs, chest X-ray and ECG findings. All 17 patients with pericardial effusion were also studied by technetium-99m pyrophosphate radionuclide myocardial imaging, targeting to reveal the coincident presence of specific heart muscle disease or 'infiltrative cardiomyopathy'. The results showed abnormal scans in 13 of 14 patients with technically satisfactory scans, indicating the coincidence of sarcoid myocardial involvement in 92% of the patients with pericardial effusion, representing 16% of the total population studied. Thus, pericardial effusion in sarcoidosis should not be considered a rare condition, while concomitant presence of positive technetium-99m pyrophosphate radionuclide myocardial imaging could suggest that sarcoid pericardial effusion is often accompanied by specific heart muscle disease. This observation has not been well established previously in the literature.
 ...
PMID:Pericardial effusion concomitant with specific heart muscle disease in systemic sarcoidosis. 797 54

The authors studied 75 nonselected patients (30 men and 45 women; mean age 48 years) with sarcoidosis retrospectively to assess the applicability of nuclear examinations for detecting myocardial involvement. All patients were studied with Tl-201 myocardial perfusion scan (TMPS), Ga-67 myocardial scan (GMS), and left ventricular ejection fraction (LVEF) using ECG-gated radionuclide ventriculography. The positive GMS group had a significantly higher occurrence of abnormality on TMPS (100%) than the negative GMS group (41%). Patients with TMPS abnormality and positive GMS showed significantly lower LVEF (36.3%) than those of normal perfusion or negative GMS. When LVEF decreased, occurrences of TMPS abnormality and positive GMS increased. The authors demonstrate that in patients with sarcoidosis LVEF was closely related to TMPS and GMS, and LVEF reflected the progression of sarcoid heart disease and can be considered a reliable indicator of cardiac function. Patients with sarcoidosis with TMPS abnormality and positive GMS should be thought to have myocardial sarcoidosis and their cardiac functions can be assessed by ECG-gated radionuclide ventriculography, while the remaining patients should be examined with TMPS and GMS periodically for detection of myocardial involvement.
 ...
PMID:Myocardial involvement in patients with sarcoidosis. An analysis of 75 patients. 806 73

Persistent or recurrent acute allograft rejection (AR) refractory to high-dose steroid therapy can adversely affect long-term outcomes of heart-lung (HLT), bilateral-lung (BLT), and single-lung (SLT) transplantations. The use of total lymphoid irradiation (TLI) for the management of refractory acute AR in six transplant recipients (two men, four women; mean age, 29.8 +/- 3.8 years) is detailed. There are two HLT (primary pulmonary hypertension [PPH], cystic fibrosis [CF]), 1 BLT (pulmonary hypertension postventricular septal defect repair), and 3 SLT (sarcoid, PPH, congenital heart disease with atrial septal defect) recipients. Refractory AR is defined as persistent rejection unresponsive to high-dose steroid therapy in all cases. The BLT and SLT recipients had at least two moderate and one mild AR events per patient. The HLT recipients had at least two moderate acute heart and one severe and one mild asynchronous acute lung rejection events per patient. A total of 800 cGy of total lymphoid irradiation (TLI) was administered over a 5-week period. Mild and transient leukopenia was the only observed side effect. The patient with PPH received TLI 313 days after HLT for recurrent AR at another institution and died of ARDS 4 weeks after completing TLI. The patient with CF received TLI 707 days after HLT and died 457 days after TLI of severe obliterative bronchiolitis (OB) with multiorgan failure. The patient with BLT received TLI 176 days after transplant and died 372 days after TLI of respiratory failure related to severe rejection. One patient with SLT received TLI 78 days after transplant and died 679 days after TLI of severe acute AR. The two remaining patients with SLTs have been free from acute AR for more than 4 years. The patient with sarcoidosis received TLI 37 days after SLT following a clinical rejection event and two severe acute AR events. He is alive with normal lung function 5 years later. The patient with PPH received TLI 108 days after SLT following three moderate acute AR events and is alive with stable OB 4 years later. These limited preliminary results suggest that TLI has merit for the treatment of intractable acute AR following HLT and lung transplantation.
 ...
PMID:Total lymphoid irradiation for refractory acute rejection in heart-lung and lung allografts. 862 53

A 49-year-old man with cardiac sarcoidosis is presented. He suffered from congestive heart failure, and left ventricular asynergy and reduced function was evident by echocardiogram and left ventriculogram. A light microscopic examination of the endomyocardial biopsy revealed nonspecific myocarditis without giant cells or noncaseating granulomas. Under an electron microscope, however, several epithelioid cells were found in the specimen. The serum level of lysozyme was elevated. The patient had a past history of sarcoidosis of the eyes and lungs 22 years previously. Cardiac diseases presenting epithelioid cells other than sarcoidosis were clinically ruled out. Thus, the diagnosis of cardiac sarcoidosis was made based on both clinical and ultrastructural findings, and corticosteroid therapy was initiated. In the second biopsy, performed 4 months later, a noncaseating granuloma was found. Generally, the incidence of histological diagnosis of cardiac sarcoidosis by light microscopy is relatively low in endomyocardial biopsy specimens. The present case suggests that the addition of an ultrastructural examination may improve the diagnostic usefulness of the endomyocardial biopsy in cardiac sarcoidosis, since electron microscopy can clearly identify the presence of even one epithelioid cell.
 ...
PMID:Usefulness of electron microscopy in the diagnosis of cardiac sarcoidosis. 890 3

Cardiac involvement in patients with sarcoidosis is an important consideration for those who are concerned with this strange disease. Sarcoidosis is not an acute malignant disease but may be noticed at the time of sudden, expected death as fatal myocardial sarcoidosis at autopsy. Even with modern advances in our ability to diagnose heart disease, cardiac sarcoidosis is still often overlooked because of its subclinical disease progression. In view of this, an extensive review of previously published literature and of our own case analyses has been carried out because of the authors' long-term experience with performing Konno's endomyocardial biopsy, which was originally developed in 1962 at the author's institution. However, the sensitivity of endomyocardial biopsy in detecting sarcoid granuloma is low (20-30%), and, instead, various kinds of nongranulomatous pathologies are often seen. During the course of our research it was found that there might exist a racial difference in cardiac sarcoidosis. Cardiac death was much more frequent in Japanese patients. The possibility that heart disease in sarcoidosis is caused by cor pulmonale due to advanced pulmonary fibrosis should be reevaluated because only a limited amount of background data is available. The author's review clarified the fact that cardiac sarcoidosis is caused by myocardial or pericardial involvement, resulting in various kinds of bradyarrhythmias or tachyarrhythmias and/or congestive heart failure. Electrocardiographic (ECG) and Holter monitor readings provide a simple and effective method for early detection of this disease. The incidence of ECG abnormalities in a total of 963 sarcoidosis patients was 22.1%, which was more frequent than that of the sex- and age-matched healthy control subjects (17.9%; p < 0.025). Echocardiography and radionuclide studies also provide useful clinical information. Careful follow-up and early corticosteroid administration followed by small maintenance doses may prevent the progression of the disease and improve prognosis. Owing to the progress in antiarrhythmic drugs and pacemaker implantation, the primary cause of death in cardiac sarcoidosis has changed from sudden death (1976 report) to congestive heart failure (1985 report).
 ...
PMID:Cardiac sarcoidosis: diagnostic, prognostic, and therapeutic considerations. 895 63

Organ transplantation is an option for sarcoidosis patients with end-stage lung, liver or heart disease. Survival statistics vary for the organ transplanted but are not too different from survival rates for other systemic disorders. Although infection and rejection are troublesome for all organ recipients including those with sarcoidosis, there is the added problem of recurrence of sarcoidosis in the allograft. Sarcoidosis is not an absolute contraindication for organ transplantation for the majority of transplantation centers.
 ...
PMID:Role of transplantation (lung, liver, and heart) in sarcoidosis. 941 64

Sarcoidosis is a multi-systemic granulomatous disease of unknown cause. It commonly involves lymph nodes, lungs, eyes, and skin. Cardiac sarcoid may be isolated, or associated with systemic involvement. Cardiac involvement is found in 20-50% of autopsied patients with sarcoidosis. However, it only gives rise to clinical manifestations in about 5% of patients. Cardiac involvement by sarcoid has been reported to manifest as complete heart block, papillary muscle dysfunction, congestive heart failure, pericarditis and/or effusion, conduction abnormality or arrhythmia, chest pain, and sudden death. The most common site of involvement is the interventricular septum base, which when involved may lead to heart block or arrhythmia. We report a case of sudden death in a 33-year-old male with a history of surgically repaired congenital heart disease. Although his congenital heart disease was originally postulated to be important in his death, autopsy examination revealed cardiac sarcoid with prominent involvement of the conduction system.
 ...
PMID:Cardiac sarcoidosis--an occult cause of sudden death: a case report and literature review. 960 13

The fundamental indication for cardiac transplantation is advanced heart failure that is unresponsive to medical therapy in patients with coronary artery disease or dilated cardiomyopathy. Other potential indications include advanced valvular or congenital heart disease and, more rarely, hypertrophic or restrictive cardiomyopathy, sarcoidosis, myocarditis, and primary unresectable cardiac tumors. Determining which patients have symptoms that are truly refractory to medical therapy is difficult. Ejection fraction or clinical status during acute decompensation is not a sufficient criterion for candidacy.
 ...
PMID:Selecting candidates for cardiac transplantation. How to assess exclusion criteria and predict who will benefit. 1015 Apr 3


<< Previous 1 2 3 4 5 6 7 8 Next >>