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Reactive arthritis by staphylococcus epidermidis: report of an unusual case.

In the literature many cases of Staphylococcus epidermidis (SE) complications are reported, but we have not found any reference about reactive arthritis secondary to SE. We report an unusual case of a patient with SE bacteriaemia, who developed elbow arthritis, asymmetrical sacroiliitis, keratoderma and restrictive cardiomyopathy. The clinical pictures, the instrumental and biochemical findings, in particular the positivity of HLA B27, allow us to set this case in the complex and heterogeneous chapter of reactive arthritis.

Adult↗

[Pericardium--radiologic diagnosis].

Evaluation of the pericardium using the capabilities of computed tomography (CT) and magnetic resonance imaging (MRI) remains one of the last requests to the radiologists within the spectrum of cardiac diagnostics. New technical developments in CT and MRI improve diagnostic accuracy in diagnosing pericardial disease and help to define adequate therapeutic management. The purpose of this article is to review the diagnostic possibilities of the radiologist in pericardial diseases with emphasis on CT and MRI. The anatomy of the normal pericardium including pericardial recessus and sinuses is reviewed followed by a brief discussion of congenital abnormalities. Particular attention is paid to acquired pericardial diseases including the potential characterization of pericardial effusions. Pericardial thickening and pericardial constrictions are discussed and the differentiation between pericardial constriction and restrictive cardiomyopathy is highlighted because of the therapeutic implications. Finally a brief review of primary and metastatic pericardial tumours is given.

Diagnosis, Differential↗

Immunologic profile of patients with protein-losing enteropathy complicating congenital heart disease.

The immunologic profile of patients with congenital heart disease complicated by protein-losing enteropathy (PLE) is undefined. The aim of this study was to assess the lymphocyte subpopulation and immunglobulin (Ig) pattern in patients with PLE complicating congenital heart disease. The immunologic profile of six patients with congenital heart disease complicated by PLE was compared to that of controls without PLE matched for age and cardiac interventions. Enteric protein loss was documented by Tc99m-labeled albumin scintigraphy. The lymphocyte subpopulations were enumerated using flow cytometry, whereas serum IgG, IgA, and IgM concentrations were measured by the turbidimetric technique. The cardiac diagnoses included complex cyanotic heart disease post-Fontan procedure (n = 3), and one each of tetralogy of Fallot, restrictive cardiomyopathy, and valvar pulmonary stenosis. In patients with PLE, the T lymphocyte (CD3+) count was significantly lower (300 +/- 186 vs 2070 +/- 1171/microl, p = 0.017); both the helper/inducer lymphocytes (CD4+) (127 +/- 158 vs 927+/- 377/microl, p = 0.006) and suppressor/cytotoxic lymphocytes (CD8+) (129 +/- 49 vs 850 +/- 695/microl, p = 0.057) reduced with reversal of CD4(+)/CD8(+) ratio (0.81 +/- 0.68 1.64 +/- 0.89, p = 0.027). Furthermore, IgG level was significantly reduced (5.12 +/- 2.84 vs 12.5 +/- 1.58 g/L, p = 0.005) and IgA level tended to be lower (1.36 +/- 1.37 vs 2.50 +/- 0.80 g/L, p = 0.095). In contrast, the B lymphocyte (CD19+) count (340 +/- 151 vs 618 +/- 427/microl, p = 0.25), natural killer cell count (CD16(+) 56(+) CD3(-)) (252 +/- 212 vs 276 +/- 251/microl, p = 0.85), and IgM level (0.98 +/- 0.59 vs 1.12 +/- 0.25 g/L, p = 0.67) were similar for both groups. None of the patients developed opportunistic or severe viral infections. Abnormal immunologic profile of both the cellular and humoral arms of the immune system occurs in patients with congenital heart disease complicated by PLE. Nonetheless, these abnormalities perhaps appear quantitative rather than qualitative in nature, although further functional studies of antibody production and lymphocyte proliferation assays are required to support this proposition.

Adolescent↗

[Left ventricular diastolic function in pregnancy in patients with arterial hypertension. A prospective study with M-mode echocardiography and Doppler echocardiography].

INTRODUCTION: During pregnancy eminent cardiovascular changes occur. The aim of the following study was to investigate the course of hemodynamic parameters under increased volume load during pregnancy in women suffering from mild arterial hypertension. METHODS: Altogether 47 women (age: 25 +/- 4 years) with mild arterial hypertension detected during pregnancy underwent echocardiography at the 9th, 24th and 33rd week of gestation. Furthermore echocardiography was performed postpartum at weeks 1 and 8. The control group comprised 45 healthy pregnant women. In all patients left ventricular muscle mass index and systolic shortening fraction were measured. The following Doppler echocardiographic parameters were ascertained: peak early diastolic and peak late diastolic flow, VE/VA ratio, acceleration time, deceleration time and isovolumetric relaxation time. RESULTS: During pregnancy all patients had an increase of left ventricular muscle mass index and a decrease of fractional shortening. All patients developed a relevant diastolic dysfunction. While the control group developed signs of disturbed relaxation as reduction of peak early diastolic flow (0.89 +/- 0.07 versus 0.82 +/- 0.08 m/s*), VE/VA ratio and an increase of isovolumetric relaxation time (72 +/- 12 versus 123 +/- 7*) at the 33rd week of gestation (* p < 0.01), all pregnant women with mild arterial hypertension developed a diastolic dysfunction with signs of delayed relaxation already at the beginning of gestation. 26 pregnant women with arterial hypertension developed a restrictive diastolic filling pattern at 24 weeks of gestation. The other 21 pregnant women only showed restriction for a short time at the end of gestation. In healthy pregnant women, volume load results in a reversible physiologic left ventricular hypertrophia, a significant alteration of diastolic left ventricular function in terms of a disturbed relaxation pattern and a temporary decrease of systolic function. In comparison hypertensive pregnant women show a delayed relaxation at the beginning of pregnancy and 50% developed early signs of restrictive cardiomyopathy. These changes may predispose to critical complications during pregnancy.

Adult↗

Sequential (domino) transplantation of the liver in a transthyretin-50 familial amyloid polyneuropathy. Special reference to cardiological diagnosis and complications.

BACKGROUND: General shortage of cadaveric organs has led to a search for alternative methods to expand the donor pool. Sequential (domino) transplantation is yet another attempt to compensate for the declining consent to organ donation. PATIENTS AND METHODS: To qualify for a domino liver transplantation, the following preconditions must be fulfilled: (1) extrahepatic disease must exist, (2) liver must be fully functional, and (3) the genetic defect in the host should recur within a sufficient latency period. Familial amyloid polyneuropathy (FAP) is an autosomal dominant disease which involves a genetic defect for transthyretin (TTR), which is predominantly produced in the liver. RESULTS: In this report, we describe a rare case of a FAP TTR-50 variant undergoing domino liver transplantation. Since myocardial symptoms precede peripheral polyneuropathy, special emphasis should be placed on arrhythmias and the restrictive cardiomyopathy necessitating a veno-venous bypass or a cardiac pacemaker in order to improve cardiac contractility. The type of anastomosis of the suprahepatic inferior vena cava and possible alternatives are discussed. CONCLUSION: Despite ethical problems, the advantages of the domino procedure are obvious: (1) expansion of the donor pool, (2) ability to use living donors, and (3) presence of very short ischemic time and thus excellent liver function. Due to the kinetics of TTR production and deposition, donors and recipients of FAP livers should be followed up using an extensive neurological and cardiological protocol.

Adult↗

Tropical endomyocardial fibrosis (Davies' disease): case report demonstrating the role of magnetic resonance imaging.

Tropical endomyocardial fibrosis (TEMF), a restrictive cardiomyopathy of unclear etiology, is an endemic disease in equatorial Africa, South America and India. The patients are usually young, the onset of the disease and its clinical manifestations insidious, and the prognosis poor. We currently present a 50-year-old Congolese female who was referred with symptoms of progressive right-sided heart failure due to isolated TEMF of the right ventricle. Surgical resection of regional endomyocardial fibrosis was not possible and our patient was referred for cardiac transplantation. Cardiac magnetic resonance imaging (CMR) demonstrated the primary and secondary structural and functional abnormalities. CMR seems ideally suited to diagnose this condition and monitor response to medical and/or surgical therapy.

Cardiac Output, Low↗

Recent role of imaging in the diagnosis of pericardial disease.

Noninvasive cardiac imaging techniques have made a striking impact on the evaluation and management of pericardial disorders. Two-dimensional and Doppler echocardiography are the methods of choice in the evaluation of pericardial effusion and cardiac tamponade. Magnetic resonance imaging, computed tomography, and transesophageal echocardiography are valuable in the assessment of pericardial thickness in suspected cases of constrictive pericarditis. Filling dysfunction associated with constrictive pericarditis is well demonstrated by Doppler flow velocity recordings of intracardiac flow jets, and pulmonary and hepatic venous flow streams. Tissue Doppler echocardiography, by which tissue velocity of myocardial regions and mitral annulus are analyzed, offers additional information in the differentiation of constrictive pericarditis and restrictive cardiomyopathy. Magnetic resonance imaging and computed tomography are the techniques of choice in the recognition of unusual disorders such as pericardial cysts, tumors invading the pericardium, and congenital absence of pericardium. Noninvasive imaging aids not only in the diagnosis of pericardial diseases, but also in the guidance of optimal therapy.

Blood Flow Velocity↗

Current treatment in cardiac amyloidosis.

Involvement of the heart is a common finding in amyloidosis. The heart is usually infiltrated by amyloid fibrils in primary amyloidosis and age-related forms of amyloidosis, less commonly in transthyretin familial amyloidosis, and rarely in secondary amyloidosis. The most common clinical presentation is restrictive cardiomyopathy with right-sided heart failure. The second most frequent presentation is congestive heart failure due to systolic dysfunction, followed by arrhythmias and orthostatic hypotension. The diagnosis of amyloidosis requires tissue sample confirmation; at present, Congo red staining in polarized light is the diagnostic method of choice. The characterization of protein fibril type by immunohistochemistry or biochemistry is essential for patient prognosis and treatment. The therapeutic approach consists of specific treatment of amyloidosis and supportive treatment for cardiac-related symptoms. The treatment depends on the type of amyloidosis and the stage of disease. The mainstay of supportive treatment of cardiac failure is diuretic therapy. Primary amyloidosis treatment protocol includes melphalan and prednisone chemotherapy. Heart transplantation is only a palliative treatment. Stem cell transplantation is an emerging treatment alternative. Combination therapy of melphalan and stem cell transplantation has been shown to be a promising treatment strategy. Secondary amyloidosis requires aggressive treatment of the associated inflammatory and neoplastic process. Age-related (senile) amyloidosis benefits from supportive cardiac treatment when applicable. Transthyretin amyloidosis, the most common cardiac hereditary amyloidosis, is treated by liver or combined liver-heart transplantation. New therapies based on chemical and immunologic reaction with amyloid or its precursor are under intensive development.

Journal Article↗

Constrictive pericarditis: new aspects.

The current status of constrictive pericarditis is reviewed with regard to its etiology, physical signs, electrocardiographic findings, and hemodynamic features. Angiographic aspects are also presented. The role and limitations of M-mode echocardiography in this disease are emphasized. The value of other noninvasive studies such as measurement of systolic time intervals, myocardial scanning, and high-speed echocardiography is described. Emphasis is placed on the invasive and noninvasive methods that may be useful in separating restrictive cardiomyopathy from constrictive pericarditis. Methods of treatment, indications for pericardial resection, and the current operative results are commented on briefly.

Angiocardiography↗

Superior vena cava Doppler flow velocity patterns in pericardial disease.

Doppler superior vena cava (SVC) flow patterns were studied in 34 patients with pericardial disease and in 8 normal adults; the pulse transducer on the echocardiographic instrument was used for respiratory monitoring, rather than a separate nasal thermistor-based device. First expiratory SVC diastolic flow velocities (Dfe) did not differ in normal subjects and patients with hemodynamically insignificant pericardial effusions (23 +/- 3 cm/s and 29 +/- 13 cm/s, difference not significant). Dfe were less than 15 cm/s only in the 14 patients with cardiac tamponade (9 +/- 3 cm/s, p less than 0.01). A ratio of systolic to diastolic flow velocity less than or equal to 1.1 in the first expiratory beat distinguished constrictive pericarditis from all other groups (p less than 0.01), although not from restrictive cardiomyopathy. Expiratory ablation of diastolic SVC flow mimicking cardiac tamponade was not observed in constrictive pericarditis. Respiratory variation in SVC flow velocities was slight in normal subjects and patients with constrictive pericarditis, increased in patients with hemodynamically insignificant pericardial effusions (p less than 0.01) and greatest in patients with cardiac tamponade (p less than 0.01). Quantitative analysis of SVC flow velocity patterns is a useful addition to the echocardiographic evaluation of pericardial disease.

Adult↗

Long-term outcome in patients with congestive heart failure and intact systolic left ventricular performance.

Congestive heart failure (CHF) is typically associated with impaired left ventricular (LV) systolic performance. Few reports exist describing the long-term outcome in patients with CHF and normal LV systolic function. Fifty-two patients initially hospitalized with CHF and intact LV function (ejection fraction greater than or equal to 45%) were followed for 7 years. Mean age when initially identified was 71 +/- 11 years (range 36 to 96), and average LV ejection fraction was 61 +/- 11%. CHF was graded by a clinicoradiographic index, with a mean of 7.0 +/- 2.3 (range 3 to 12, 13 indicates worst CHF). A third heart sound was present in 19 patients (37%), and 17 (33%) had presented with acute pulmonary edema. Principal cardiovascular diagnoses were coronary artery disease in 27 (52%), hypertensive heart disease in 16 (31%) and restrictive cardiomyopathy in 7 (13%). At 7 years, cardiovascular mortality was 46% (24 of 52), and noncardiovascular mortality was 10% (5 of 52). Survival was not correlated with age, principal diagnosis, third heart sound, pulmonary edema at presentation, LV ejection fraction, or presence or degree of LV diastolic dysfunction. Cardiovascular morbidity, consisting of nonfatal recurrent CHF, myocardial infarction, unstable angina or other cardiovascular events occurred in 29% (15 of 52). Combined cardiovascular mortality and morbidity was 75% (39 of 52). In patients with CHF, intact LV systolic function does not confer the same favorable prognosis it defines in other clinical situations. For such patients, the risk of future cardiovascular events is high, a finding that should be considered when designing therapeutic strategies in this group.

Adult↗

Mitral valve replacement in idiopathic hypereosinophilic syndrome.

Idiopathic hypereosinophilic syndrome is an eosinophil-mediated tissue injury causing multiple organ failure. Cardiac manifestations include subendocardial fibrosis, thrombus leading to peripheral emboli, restrictive cardiomyopathy, and valvar dysfunction. This article describes a patient with the syndrome and reviews surgical experience with valve replacement and long-term prognosis.

Adult↗

Ischemic cardiomyopathy.

The term "ischemic cardiomyopathy" was used initially to describe a clinical syndrome that was indistinguishable from primary congestive cardiomyopathy but due to severe, diffuse coronary artery disease. The term has been expanded to include the larger category of myocardial disease secondary to coronary artery disease. Using this expanded definition, we have discussed the varied clinical presentations of congestive ischemic cardiomyopathy and restrictive ischemic cardiomyopathy (stiff heart syndrome and right ventricular infarction), and how the effects of ischemia on left ventricular systolic and diastolic performance may cause these varied presentations. The prognosis of any ischemic cardiomyopathy is related primarily to the degree of ventricular dysfunction and the extent of coronary artery disease. Therapy is aimed at preventing or ameliorating myocardial ischemia and halting the progression of, or even reversing, the deterioration in myocardial function.

Adrenergic beta-Antagonists↗

Atrio-ventricular regurgitations in constrictive pericarditis: incidence and post-operative outcome.

We studied 33 surgically proven cases of constrictive pericarditis during the period 1989-1991 by color Doppler echocardiography and angiography to look for incidence and postoperative outcome of atrioventricular regurgitation. The mean age was 27.2 + 16.5 years (21 males, 12 females). There was a very high incidence of mitral (79%, trivial in 13, mild in 11 and moderate in 2) and tricuspid (73%, trivial in 7, mild and moderate in 6 each and severe in 5) regurgitation. There was good correlation between 'color Doppler' and angiography for detection and quantification of these regurgitations (r = 0.89 for mitral and 0.76 for tricuspid regurgitation, respectively). There were no preoperative clinical or hemodynamic predictors for the incidence or severity of these regurgitations. Immediate postoperative (7-10 days) evaluation by color Doppler did not show any change in these regurgitations. A follow-up study (by color Doppler and angiography) in 18 patients at a mean period of 229 + 105 days revealed regression of these regurgitations by at least 1 grade in 50% of patients. Patients with persisting regurgitations had persisting hemodynamic abnormality and relatively longer duration of symptoms. The presence of atrio-ventricular regurgitations should not be taken as evidence favoring diagnosis of restrictive cardiomyopathy and against that of constrictive pericarditis. The mechanism of these regurgitations is not clear to us.

Adult↗

Radionuclide angiographic assessment of left ventricular diastolic filling in amyloid heart disease: a study of patients with familial amyloid polyneuropathy.

To assess left ventricular diastolic filling in amyloid heart disease, 17 patients with familial amyloid polyneuropathy and 20 normal subjects were examined by radionuclide angiography. None of the patients showed clinical evidence of restrictive cardiomyopathy. All but two patients had normal left ventricular ejection fraction. Peak filling rate was significantly lower and time to peak filling rate was significantly greater in patients than in normal subjects (2.60 +/- 0.52 versus 3.10 +/- 0.44 EDV/s, p less than 0.001, and 215 +/- 53 versus 147 +/- 18 ms, p less than 0.001, respectively). The mean left ventricular filling volume during rapid diastolic filling and atrial systole in patients was 54.5 +/- 19.5% and 44.2 +/- 21.6% of the stroke volume, respectively, compared with 83.8 +/- 6.6% (p less than 0.001) and 20.0 +/- 6.0% (p less than 0.001), respectively, in normal subjects. Although 10 of the 14 patients without clinical evidence of overt heart disease had normal ventricular wall thickness as well as normal ejection fraction, 8 of the 10 showed abnormal diastolic filling. In patients with familial amyloid polyneuropathy, indexes of diastolic filling were significantly related to ventricular wall thickness alone. The incidence and magnitude of abnormalities in time to peak filling rate and contribution of rapid filling as well as atrial systole to ventricular filling increased with age and duration of illness. Thus, abnormal diastolic filling can be seen even in the early stage of familial amyloid polyneuropathy and may be related to myocardial amyloid deposition as well as to fibrosis. Careful consideration should be given to age and duration of illness when diastolic filling is assessed in this disorder.

Adult↗

Spin echo MRI in the evaluation of pericardial disease.

As in other pathological processes of the heart, the contribution of MRI is not limited to optimal anatomical evaluation but also offers functional or etiological information (approach of tissue characterization). In this work we report on our experience in evaluating normal and pathological pericardium with MRI spin echo sequences. The results with 41 patients, including pericardial effusion, thickening, and mass are discussed and confronted with previously reported data. Contrary to classical echocardiographic notions, pericardial effusions are more frequently in the anterior than posterior location (26/30 vs. 19/30 p less than 0.03). Not only does MRI provide a precise and positive diagnosis, but also a rather clear distinction appears in spin echo sequences between hemopericardium with high signal intensity and other effusions with lower signal (high intensity found in 6/6 patients with hemopericardium versus only 8/24 in the remaining group; p less than 0.01). When constriction signs are present, MRI solves the difficult differential diagnosis between pericardial thickening and restrictive cardiomyopathy. In paracardiac primary or metastatic tumors pericardial involvement is clearly demonstrated. Distinction between paracardiac, respectively pericardial cyst or false aneurysm is possible. We conclude that MRI is a new imaging method, complementary to echocardiography and/or computed tomography in the evaluation of pericardial pathology especially when hemopericardium, constrictive pericarditis, or tumoral etiologies are suspected. Important technological improvements are needed to solve the problem of long acquisition time and to improve the possibilities of tissue discrimination.

Aged↗

Accuracy and pitfalls of early diastolic motion of the mitral annulus for diagnosing constrictive pericarditis by tissue Doppler imaging.

Mitral annular velocities are reportedly useful in diagnosing constrictive pericarditis (CP); however, their exact efficacy in larger clinical settings remains unevaluated. This study reexamined the role of longitudinal tissue Doppler imaging in diagnosing CP in clinical practice. Tissue velocity imaging (GE Vingmed System Five) was performed in 122 subjects (87 referred with clinically suspected CP and 35 age- and sex-matched controls). Of the 87 subjects with suspected CP, 45 (51.7%) had CP confirmed at surgery, 11 (12.6%) had restrictive heart disease, 20 (23.0%) had right heart failure due to cor pulmonale, and the other 11 (12.6%) had old pericardial effusions and no hemodynamic evidence of constriction on follow-up echocardiography. Of the 45 patients with CP, mitral early diastolic (Ea) annular velocities from septal and lateral regions were normal (>/=8 cm/s) in 40 (88.9%) and decreased (<8 cm/s) in 1 or both regions in 5 (3 with left ventricular systolic dysfunction, 2 with extensive mitral annular calcification). Of 11 patients with restrictive cardiomyopathy, 8 (72.7%) had reduced Ea (<8 cm/s) and 3 showed normal Ea velocity in 1 or both corners of the mitral annulus. All except 2 patients with right-sided heart failure from cor pulmonale and those with previous pericardial effusion had normal Ea velocities. A normal Ea velocity improved recognition of CP, particularly in the presence of nondiagnostic 2-dimensional or transmitral flow-Doppler imaging. The overall sensitivity and specificity for diagnosing CP using tissue Doppler incrementally with M-mode, 2-dimensional, and transmitral flow Doppler were 88.8% and 94.8%, respectively. Mitral annular velocities help with diagnosis and differentiation of CP in most cases, except in the presence of extensive annular calcification, left ventricular systolic dysfunction, or segmental nonuniformity in myocardial velocities.

Adult↗

Late constrictive involvement of the pericardium in a case of previous myocarditis.

Constrictive pericarditis (CP) is a highly relevant disease clinically because pericardiectomy represents the only curative therapeutic approach. Previous cardiac surgery or mediastinal radiation may cause CP, however, infectious agents account for a substantial portion of CP. In this report, we present a patient with previous biopsy-proven myocarditis and positive seroconversion against coxsackievirus B3 without clinical evidence of acute pericardial involvement who developed CP after a prolonged period of time. This suggests that infectious particles primarily infecting the myocardium may lead to chronic inflammatory responses of the pericardium, thus causing CP even at late clinical stages. This case emphasizes the important fact that primary myocarditis may not only cause systolic ventricular impairment but may also induce diastolic dysfunction of the heart, either as restrictive cardiomyopathy or, as in this case, through inflammatory involvement of the pericardium, leading to CP.

Antibodies, Viral↗