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At least 559 records · Page 31Linked to original sources
PubMed2003

An unusual case of recurrent Loffler endomyocarditis of the aortic valve.

Idiopathic hypereosinophilic syndrome is a rare systemic disease with an unexplained elevated eosinophil count. Loffler endomyocarditis is hypereosinophilic syndrome with endocardial fibrosis and restrictive cardiomyopathy. The atrioventricular valves are frequently involved, causing valvular regurgitation. Previously, there has been one case report of combined aortic and mitral valve involvement with Loffler endomyocarditis that was treated with bivalvular replacement. We describe a previously healthy 50-year-old man diagnosed with Loffler endomyocarditis complicated by peripheral thromboembolism and severe aortic regurgitation due to valve fibrosis and fibrotic vegetation on the aortic valve. He underwent embolectomy and aortic valve replacement in addition to treatment for his hypereosinophilia. He later presented with cardiomyopathy with severe aortic insufficiency due to the destruction of the aortic valve prosthesis by sterile fibrinous vegetation. To our knowledge, this is the second case in the literature in which Loffler endomyocarditis involves the aortic valve and the first patient in whom only the aortic valve is involved.

Aortic Valve↗
PubMedDate not supplied

Radiofrequency catheter ablation of common atrial flutter--acute and follow-up results.

Atrial flutter with a structurally well-defined macro-reentrant circuit in the right atrium has recently become amenable to radiofrequency ablation with the recognition of isthmus as a narrow zone of slow conduction. This study describes 20 consecutive and symptomatic patients with atrial flutter (15 males, 5 females; mean age 38.5 +/- 10.2 years) who underwent radiofrequency ablation in our institute in the last 18 months. Fourteen patients had structurally normal hearts, while the remaining six patients had specific disorders (prior surgery for closure of atrial septal defect-2, idiopathic restrictive cardiomyopathy-1, primary sinus node dysfunction-2, tachycardiomyopathy-1). The endpoints of a complete isthmus block and conversion to sinus rhythm were achieved in 19 of the 20 patients. Total number of pulses needed to attain the endpoints was a mean of 4.2 (range 1-5), each pulse being delivered for 90 seconds. At a mean follow up of 9.4 +/- 3.2 months (range 6-12 months), recurrence of atrial flutter was seen in one patient, atrial fibrillation in two and sinus node reentrant tachycardia in one. These results are comparable to those reported in the literature. Achievement of a complete isthmus block appears to be an important endpoint in obtaining optimal results. The issues of alternative sites of ablation, long-term results and advantages of an 8 mm tip catheter need to be examined further. In conclusion, radiofrequency ablation appears to be the preferred mode of treatment for patients with atrial flutter with excellent short-term and mid-term results.

Adolescent↗
PubMed1998

Novel heart diseases requiring transplantation.

Cardiac transplantation represents an extraordinary opportunity to study in vivo the pathology of native disease in the terminal phase. The experience cumulated at the University of Padua in 418 orthotopic cardiac transplant procedures allowed to come across previously unrecognized disease: idiopathic left ventricular aneurysm, non-compacted (spongy) myocardium, restrictive cardiomyopathy. A discrepancy between pre- and post-transplant diagnosis was found in 8% of cases. Endomyocardial biopsy and coronary arteriography should be carried out in each patient with apparently dilated cardiomyopathy before proceeding to heart removal, since alternative therapeutic procedures may be available.

Journal Article↗
PubMed1999

[Pericardial and myocardial adiastole in rheumatoid polyarthritis].

The authors describe a case of clinical, echocardiographic and haemodynamic adiastole in a man with severe rheumatoid arthritis with a previous history of pericardial effusion. The adiastole was mixed, fibrous pericarditis, confirmed by ultra fast CT scan and at surgery; myocardial adiastole was suspected on finding thickening of the ventricular walls (in the absence of hypertension and coronary artery disease) and, unfortunately, confirmed by the persistence of adiastole despite very satisfactory pericardectomy. The authors underline the involvement of the three cardiac tunics in rheumatoid arthritis and the value of different diagnostic methods in the differentiation between constrictive pericarditis and restrictive cardiomyopathy.

Arrhythmias, Cardiac↗
PubMed2000

Cardiac damage following therapeutic chest irradiation. Importance, evaluation and treatment.

Radiation induced heart disease, with its clinical manifestations, is becoming a growing problem. Its prevalence is increasing, keeping pace with the increased survival of many malignancies. The majority of patients with radiation induced heart disease is constituted by Hodgkin's disease survivors, followed by non Hodgkin's disease, esophageal carcinoma, thymoma, lung cancer, breast cancer and metastatic seminoma. Pericardial disease is the most well known expression of radiation induced heart disease, although the whole cardiac structure is compromised because of the structural and consequently functional impairment. Myocardial damage can lead to a congestive heart failure, typically due to a restrictive cardiomyopathy. Coronary artery obstructive disease frequently involves ostial coronary segments and the left main, for this reason it does appear particularly harmful. All patients undergoing chest irradiation require serial cardiological evaluation. Important risk factors of radiation induced heart disease are previous chemotherapy, radiation exposition exceeding 4000 Rad, administration next to the heart and on the left side of the chest must be taken into particular consideration. The cardiac damage limitation basically is founded on prevention. Significant results have been obtained with fractional exposition, high energy utilization and "split" zone covering. The radiotherapeutic technical improvement with the comprehensive individual patient risk evaluation will provide a substantial benefit for the future. The consultant cardiologist should cooperate with the oncologist and the radiotherapist, providing specific competence and continuative care.

Heart Injuries↗
PubMedDate not supplied

Endomyocardial fibrosis (Davies disease) coincidental with systemic lupus erythematosus.

This is the case of a 27 years-old woman with signs and symptoms of severe untreatable congestive heart failure, anemia, gingival mucosa ulcers, photosensitivity and alopecia. The electrocardiographic, echocardiographic, angiographic and hemodynamic data oriented the diagnosis of restrictive cardiomyopathy, mitral insufficiency secondary to mitral prolapse and bi-atrial dilation. The histologic study of the endomyocardial biopsy, performed during catheterization, showed signs of endomyocardial fibrosis, and immunological analysis was compatible with systemic lupus erythematosus. As far as we know, this is the first case of endomyocardial fibrosis (Davies disease) associated with systemic lupus erythematosus published in the medical literature. The etiology of Davies disease remains unrevealed and its association with systemic lupus erythematosus suggest a probable autoimmune origin.

Adult↗
PubMed2000

Constrictive pericarditis. Early experience in 12 patients in light of modern cardiology.

The last several decades have witnessed major advances in the understanding and management of constrictive pericarditis. The aim of the present study was to compare the diagnosis, treatment and outcome of constrictive pericarditis of 40 years ago to today. The study population consisted of 12 patients with a diagnosis of constrictive pericarditis who presented at the Institute of Cardiology of Beilinson Hospital, from 1961 to 1970. Their main physical findings, electrocardiographic and chest X-ray changes, and hemodynamic study results are discussed in relation to the surgical outcome of patients with constrictive pericarditis today. New noninvasive imaging modalities, such as M mode, two-dimensional and Doppler echocardiography, computed tomography and magnetic resonance imaging are presented, and their advantages and disadvantages in the diagnosis of constrictive pericarditis and its differentiation from restrictive cardiomyopathy are explained.

Adolescent↗
PubMed2001

Medium-term follow up of pulmonary autograft aortic valve replacement: technical advances and echocardiographic follow up.

BACKGROUND AND AIM OF THE STUDY: The Ross procedure (aortic valve replacement (AVR) with pulmonary autograft and pulmonary homograft replacement of pulmonary valve) was developed as a durable aortic valve substitute that avoids the need for anticoagulation and provides young patients with a long-lasting aortic valve substitute. Our seven-year follow up echocardiography data are reviewed. METHODS: Between May 1993 and March 2000, 40 adult patients (28 males, 12 females; mean age 33.3 years) underwent the Ross procedure at the Brigham and Women's Hospital for congenital aortic stenosis (n = 6), aortic insufficiency (n = 17) and mixed disease (n = 17). All patients had aortic root replacement with the pulmonary autograft and had no regurgitation after operation. Postoperative evaluation was conducted by transthoracic echocardiography, office visit and/or telephone interview. NYHA functional class, aortic and pulmonary valve function and aortic root dimensions were evaluated. RESULTS: One patient died postoperatively as a result of a low output state related to global left ventricular dysfunction. Four patients (10%) developed pulmonary homograft stenosis with a peak gradient >40mmHg; and six developed mild pulmonary stenosis. One patient had aortic insufficiency seven years postoperatively that required valve replacement. Eight patients developed mild dilatation (>37 mm) of the neoaortic root, and five of these had aortic insufficiency. One patient required transplantation at 40 months for restrictive cardiomyopathy. CONCLUSION: The Ross procedure is an effective means of AVR that can be accomplished with low perioperative morbidity and mortality if certain technical modifications are carried out. In this series of 40 patients with mid-term follow up, a significant number developed moderate pulmonary trunk stenosis, though echo characterization demonstrated good valve function.

Adult↗
PubMed2000

[Functional heart murmur and echocardiography: a solved problem?].

BACKGROUND: Is it really vanished the need for echocardiography in the differential diagnosis of functional pediatric murmurs? METHODS: To this aim 260 children (132 males, 128 females; age range 1-84 months) were examined with auscultatory cardiac murmur. All children underwent a clinical and instrumental evaluation (ECG and echocardiography) from two pediatric cardiologists with independent assessment. RESULTS: Two hundred and ten patients (Group A) were evaluated clinically with functional murmur, 45 patients (Group B) with pathological murmur, 5 patients (Group C) with uncertain pathology. ECG showed changes in 1 Group B patient. Echocardiography showed pathology in 15 Group A patients (6 patients with patent foramen ovale, 5 patients with interatrial defect, 2 patients with bicuspid aortic valve, 1 patient with mitral insufficiency, 1 patient with restrictive cardiomyopathy). Pathology was excluded in 3 Group B patients, and in 2 Group C patients. In our clinical analysis echocardiography showed sensitivity 75%, specificity 97%, positive predictive value 90%, and negative predictive value 92%. CONCLUSIONS: In our experience we suggest to perform echocardiographic examination in children with functional murmur for complete assessment.

Child↗
PubMed2001

Infusion of light chains from patients with cardiac amyloidosis causes diastolic dysfunction in isolated mouse hearts.

BACKGROUND: Primary (AL) amyloidosis is a plasma cell dyscrasia characterized by clonal production of immunoglobulin light chains (LC) resulting in the subsequent systemic deposition of extracellular amyloid fibrils. Cardiac involvement is marked by the hemodynamic pattern of impaired diastolic filling and restrictive cardiomyopathy. Although cardiac death in patients with AL amyloidosis is usually associated with extensive myocardial infiltration, the infiltration alone does not correlated with the degree of heart failure or survival. We hypothesized that circulating monoclonal LC may directly impair cardiac function, in addition to any mechanical effects of amyloid fibril deposition. Therefore, we examined the effects of amyloid LC proteins on diastolic and systolic cardiac function, as measured in an isolated mouse heart model. METHODS AND RESULTS: LC were obtained from patients with nonamyloid disease or from those with noncardiac, mild cardiac, and severe cardiac involved AL amyloidosis. Saline or LC (100 microgram/mL) was infused into a Langendorff-perfused, isovolumically contracting mouse heart. Saline and control, noncardiac, and mild-cardiac LC infusions did not alter ex vivo cardiac function. In contrast, infusion of sever cardiac LC resulted in marked impairment of ventricular relaxation with preservation of contractile function. CONCLUSION: These results demonstrate that infusion of LC from patients with AL amyloidosis result in diastolic dysfunction similar to that observed in patients with cardiac involved AL amyloidosis, and they suggest that amyloid LC proteins may contribute directly to the pathogenesis and the rapid progression of amyloid cardiomyopathy, independent of extracellular fibril deposition.

Amyloidosis↗
PubMed2002

[Myocardial pyrophosphate uptake in cardiac amyloidosis: report of case].

We report a previously healthy 73 years old woman, who was hospitalised with increasing dyspnea and signs of congestive heart failure. Echocardiography showed a normal left ventricular cavity with increased echogenicity of its walls and severe pulmonary hypertension. A lung ventilation/perfusion scintigraphy concluded that there was a low probability for pulmonary embolism. Coronary angiography was normal. A restrictive cardiomyopathy due to amyloid deposits was suspected. Myocardial pyrophosphate scintigraphy showed intense pyrophosphate uptake in the left ventricle wall. An abdominal fat tissue biopsy was positive for amyloid deposits.

Aged↗
PubMedDate not supplied

Tissue Doppler echocardiography: principles and applications.

Tissue velocity imaging is an important development in the field of cardiac ultrasound that provides quantitative information for analysis of myocardial motion independent of the quality of gray-scale 2-D echocardiography data. It holds promise to reduce inter- and intraobserver variability in regional wall motion interpretation and is likely to improve the accuracy and reproducibility of stress echocardiography and myocardial viability assessment. It also enables regional diastolic function assessment independent of the loading conditions and offers a practical clinical tool to differentiate pathologic from physiologic myocardial hypertrophy, restrictive cardiomyopathy from constrictive pericarditis and for monitoring and selecting therapies in patients with advanced heart failure. The use of tissue velocity data for myocardial strain and strain rate imaging is likely to circumvent the limitations of tissue velocity in differentiating active and passive motion of a myocardial segment. However, its incremental utility and exact role in improving the diagnostic yield and clinical outcome needs to be addressed in future studies.

Coronary Disease↗
PubMed2002

[Use of myocardial tissue Doppler imaging in cardiology].

Tissue Doppler imaging is a new promising method of non-invasive assessment of cardiac function. This method has heralded a renaissance of interest in longitudinal ventricular function. There is evidence that long-axis function is a more sensitive index of myocardial contractility than conventional echocardiographic parameters, and that therefore, this technique could contribute to early diagnosis of systolic dysfunction. Tissue Doppler imaging can be successfully applied in the assessment of regional myocardial function. It has been proven that myocardial velocities truly reflect structural myocardial changes (the degree of myocardial fibrosis) and beta-adrenoreceptor density. The use of tissue Doppler imaging in concert with stress echocardiography is also extremely encouraging and is considered as the most likely solution to the problem of quantitative analysis of stress echocardiography. The method additionally opens new horizons in non-invasive assessment of diastolic function of the heart. Myocardial velocities have been shown to have important advantages over conventional Doppler indices of left ventricular diastolic filling. Frustration among cardiologists caused by unreliability of the traditional approach in the assessment of diastolic properties of the heart seems to have been overcome. Tissue Doppler imaging allows the examination of right ventricular systolic and diastolic function, the differentiation between constrictive pericarditis and restrictive cardiomyopathy and the estimation of pressures in cardiac chambers and in the pulmonary artery. It can also establish the presence of accessory conduction pathways, the diagnosis of rejection of transplanted hearts and can be used for identification of indications for biventricular pacing. Although many clinicians believe that tissue Doppler imaging is still an experimental technique, it can now be introduced into the routine practice of echocardiographic laboratories. The wider use of the method will help to develop its benefits and better understand and overcome limitations.

Cardiology↗
PubMed1975

The role of myocardial biopsy in cardiac diagnosis.

With the advent of cardiac catheterization, cardioangiography, and selective coronary arteriography, specific types of cardiac disease can be recognized and clearly defined. This is appropriate because myocardial biopsy alone rarely plays a major role in cardiac diagnosis. Excluding Aschoff's nodules in patients with rheumatic valve disease, the light microscopic findings in patients with rheumatic heart disease, congenital heart disease, pericardial disease, hypertensive and arteriosclerotic heart disease are similar and nonspecific. In these, interstitial fibrosis and/or myocardial hypertrophy is the dominant tissue diagnosis. Occasionally a pericardial and myocardial specimen is helpful to distinguish constrictive pericarditis and restrictive cardiomyopathy. Myocardial biopsy has provided the only method for diagnosis in a small number of patients with normal hemodynamics, normal coronary arteriograms and normal ventriculograms. The patients were studied because of chest pain and/or cardiac arrhythmias. Supraventricular and/or ventricular arrhythmias were encountered. In these patients the tissue diagnosis was interstital fibrosis and/or myocardial hypertrophy. These findings are consistent with primary myocardial disease which was not recognized clinically or by angiographic studies. The procedure seems to play a major role in the diagnosis of specific types of primary myocardial disease. It is valuable in the recognition of glycogen storage disease, amyloidosis, hemochromatosis, and myocarditis. On the basis of current experience, the indications for myocardial biopsies depend on the need for a tissue diagnosis in determining the management of the patient and the availability of adequately trained personnel to perform the procedure and manage the complications.

Angiocardiography↗
PubMed2000

Endomyocardial fibrosis in China.

PURPOSE: To introduce the epidemical, pathological, and clinical characteristics as well as the diagnostic and therapeutical experiences of endomyocardial fibrosis (EMF) in China. DATA SOURCES: A CMBdisc search was done of the Chinese-language literature published from January 1983 through June 1997 about EMF and/or restrictive cardiomyopathy. A manual search was then done for other contributions, including abstracts, between January 1965 and June 1997. RESULTS: Eighty-seven Chinese cases of EMF were collected in this paper. There were 49 men and 38 women, with a mean age of 28 +/- 13 years (range, 8 to 68 years). The distribution of the cases is mainly in the south of China. Combined right and left ventricular disease occurs in 48 percent of cases, with pure right ventricular involvement occurring in 12 percent and pure left ventricular involvement in the remaining 10 percent of patients who are examined postmortem. The diagnosis of EMF was confirmed in 21 cases at autopsy, and in 66 cases by echocardiography, angiocardiography, and/or endomyocardial biopsy which showed the characteristic changes. Clinically, right-sided disease is the commonest variety. Endocardiectomy and tricuspid (n - 7) or mitral (n - 1) valves replacement have been performed in 8 patients. There were 2 operative deaths. Six patients had a satisfactory recovery postoperatively and living well in the follow-up duration. CONCLUSION: EMF has been diagnosed clinically and confirmed at necropsy in a number of cases in the south of China. The etiology, incidence and epidemiology are still unknown. The pathological and clinical features are similar to those in tropical areas, but right ventricular involvement is the commonest type in our country.

China↗
PubMed2003

Laparoscopically assisted vaginal hysterectomy with bilateral salpingo-oophorectomy due to endometrial cancer in a heart transplant recipient. A case report.

INTRODUCTION: Transplant recipients have a higher incidence of cancer compared with the general population. This increased risk is related to the intensity and chronicity of immunosuppression that these patients receive. In this report, we present a case of a heart transplant woman with endometrial cancer who was diagnosed six months after transplantation. CASE REPORT: A 49-year-old woman who had undergone a heart transplant was referred to our department in May 2002 for final treatment. The diagnosis of endometrial cancer was established on the basis of the histopathology findings of the fractional curettage. Her heart transplant had occurred six months before, as a result of idiopathic restrictive cardiomyopathy. The patient received triple immunosuppression with cyclosporin, azathioprine and prednisone and she displayed no signs of acute graft rejection features. Laparoscopically assisted vaginal hysterectomy with adnexa was performed without any complications. Duration of surgery was 85 minutes. The patient was operated on under general anesthesia and intraabdominal pressure was automatically maintained at 10 mmHg with a carbon dioxide insufflator (AESCULAP, Germany). Immunosuppressive therapy was continued without modification. The postoperative course was uncomplicated in our patient. No significant changes in heart rate or blood pressure were observed. The patient was discharged from the hospital on the 11th postoperative day. Microscopic appearance revealed Stage I endometrial cancer. The patient is in good physical condition with normal heart performance and without disease recurrence. CONCLUSION: In our opinion LAVH was a justifiable form of surgical management in the treatment of a heart transplant recipient with an early-stage endometrial cancer.

Adenocarcinoma↗
PubMed2004

[Immunoglobulin light chain amyloidosis: recent molecular, clinical and therapeutic approach].

AL amyloidosis is a rare disorder characterised by tissue deposition of a fibrillary proteinaceous material, formed from monoclonal immunoglobulin light (or exceptionally heavy) chains. Although it may complicate multiple myeloma or B-cell lymphomas, AL amyloidosis is often associated with a low burden of clonal plasma cells ("primitive" AL amyloidosis). The mechanisms involved in the formation of AL amyloid deposits remain unclear, but are probably related to structural peculiarities of monoclonal immunoglobulin light chains. AL amyloidosis is usually a systemic disease, often revealed by renal involvement, the most common complication of the disease. The longterm prognosis of AL amyloidosis is poor, mainly related to amyloid restrictive cardiomyopathy leading to congestive heart failure. Oral melphalan and prednisone is considered the standard treatment for AL amyloidosis, but with limited increase in the median survival. High-dose intra-venous melphalan with autologous stem cell transplantation is an effective treatment, aimed at eliminating the clonaly expanded plasma cells, which has been shown to induce complete hematologic remissions and to prolong survival. However, the tolerability of such treatment is low, limiting its use to selected patients. The development of new drugs, able to interfere with amyloid fibril deposition, may provide a new therapeutic approach.

Amyloidosis↗
PubMedDate not supplied

[Heart conduction disorders in long-term treatment with chloroquine. Two new cases].

Cardiac complications are exceptional in long-term chloroquine therapy; congestive heart failure and restrictive cardiomyopathy may develop, but disorders of conduction are more frequent. The characteristics of these disorders emerge from 12 cases in the literature and from 2 personal cases. The usual disorder is fascicular block which may become a complete, syncopal, atrioventricular block, as in one of our 2 patients. The time elapsed between the beginning of treatment and the occurrence of these disorders (2 to 30 years) and the total dose of chloroquine received (100 to 2,500 g) are extremely variable. Retinopathy or neuromyopathy is present in 64 and 35 percent of the cases respectively. The diagnosis is confirmed by endomyocardial biopsy with electron microscopic study which shows vaculoar myopathy with numerous large secondary lysosomes containing a dense material of lamellar structure (myelinic figures, curvilinear bodies). Regression of heart conduction disorders after withdrawal of chloroquine seems to be inconstant and incomplete. The rare occurrence of this complication raises the question of genetic predisposition. We believe that chloroquine therapy should be contra-indicated in patients with a history of conduction disorders and that a 6-monthly electrocardiographic control of these patients would be justified.

Biopsy↗