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PubMed1998

[Association of pulmonary artery aneurysm, right heart thromboses and antiphospholipid antibodies in Behcet's disease].

INTRODUCTION: Cardiac thromboses are unusual in the course of Behçet's disease and are frequently associated with endomyocardial fibrosis of the right heart. Vascular pulmonary involvement with either pulmonary aneurysm or parenchyma alterations is also often observed. However, pathogenesis of thromboses occurring in the course of Behçet's disease is still unclear. CASE REPORT: The authors report the case of a 28-year-old man who presented with Behçet's disease accompanied by pulmonary aneurysm, multiple thrombi of the right heart and antiphospholipid antibodies. CONCLUSION: The choice of therapeutical strategies for curative treatment of this association as well as the interpretation of the role of antiphospholipid antibodies in this polyvascular disease raises questions.

Adult↗
PubMedDate not supplied

Pathology of the cardiomyopathies.

The following article reviews the anatomic and pathologic basis of various cardiomyopathies and illustrates the dilated, hypertrophic, and restrictive types in various echocardiographic planes. Two types of cardiomyopathy are defined: (1) heart muscle disease of unknown cause (idiopathic, primary) and (2) heart muscle disease of known cause or associated with disorders of other organ systems (secondary). Of the cardiomyopathies of unknown cause, the dilated type is the most common. The anatomic diagnosis of hypertrophic cardiomyopathy is based on several morphologic features, the most important of which are asymmetric septal hypertrophy, small left ventricular cavity, and myocardial fiber disarray. Endomyocardial fibrosis with or without peripheral eosinophilia represents the unknown type of restrictive or obliterative form of cardiomyopathy. Selective specific heart muscle diseases (amyloid, iron, myocarditis, toxic, storage disease) are briefly reviewed.

Cardiomyopathies↗
PubMed1999

Sudden infant death with anomalous origin of the left coronary artery from the pulmonary artery.

Autopsy of a 2-month-old boy revealed an anomalous origin of the left coronary artery from the pulmonary trunk. The endomyocardial fibrosis associated with myocardial ischemia was noted in the left ventricular wall of the enlarged heart. Death was attributed to silent heart failure due to the anomalous left coronary artery. Significant clinical symptoms are generally observed in patients with such anomalies, however, they might cause sudden death in childhood.

Journal Article↗
PubMed2001

A rat model of hypereosinophilic syndrome.

Hypereosinophilia-occurring rats without chemical and antigen treatment have been maintained in our laboratory. The rat, Matsumoto Eosinophilia Shinshu (mes), showed hypereosinophilia at the age of 9 weeks or older and developed eosinophil-related inflammatory lesions in many organs. These lesions included: aortitis, granulomatous lesion in the mesenteric lymph node, inflammatory fibroid polyp of the stomach and pulmonary vasculitis with septal infiltration. These lesions were involved with cellular infiltration of eosinophils and macrophages, and deposition of eosinophilic crystals which immunohistologically showed major basic protein and eosinophilic peroxidase derived from eosinophilic lysosomal constituents. Although the distribution of lesions in mes is a little different from that of hypereosinophilic syndrome (HES) in humans, in that endomyocardial fibrosis appears in HES while aortitis appears in mes, mes is probably comparable with HES. The present paper describes the pathological aspects of the lesions in mes and discusses the pathogenesis of tissue injury related to eosinophilic infiltration.

Animals↗
PubMed2004

Platelet-derived growth factor receptor inhibition to treat idiopathic hypereosinophilic syndrome.

Hypereosinophilic syndrome (HES) is a heterogeneous group of rare disorders characterized by peripheral blood and tissue eosinophilia leading to end-organ damage. Hypereosinophilic syndrome can be fatal, particularly in patients with endomyocardial fibrosis, and treatment has traditionally been palliative or preventive. The disease shares features with myeloproliferative disorders, such as chronic myeloid leukemia, including responsiveness to hydroxyurea and interferon. The tyrosine kinase inhibitor imatinib, a highly effective treatment for chronic myeloid leukemia, has shown efficacy in normalizing eosinophil counts and resolving signs and symptoms in some HES patients. Fusion of the Fip1-like 1 gene (FIP1L1) and the platelet-derived growth factor receptor alpha gene (PDGFRA) was discovered in the majority of patients with imatinib-sensitive HES, and all patients with the fusion responded to imatinib. The product of this fusion gene, FIP1L1-PDGFRalpha, is a constitutively active protein-tyrosine kinase capable of transforming hematopoietic cells. The efficacy of relatively low imatinib concentrations in HES, mediated by inhibition of FIP1L1-PDGFRalpha kinase activity, causally implicates FIP1L1-PDGFRA in the pathogenesis in certain HES patients.

Benzamides↗
PubMed1997

[Löffler's parietal fibroplastic endocarditis. Echocardiographic course over 5 years].

HISTORY AND CLINICAL FINDINGS: A 28-year-old man, who had sustained a traumatic displaced fracture of the left lateral clavicle requiring operative realignment, was admitted to hospital because of the incidental finding of pathological electrocardiographic findings (suggestive of past anterior myocardial infarction). He had dyspnoea on effort and episodes of irregular tachycardia without other cardiac symptoms, as well as regular tachycardia (120/min) without pulse deficit. Body temperature was 37.3 degrees C, blood pressure 110/70 mm Hg. INVESTIGATIONS: Transoesophageal echocardiography showed regular myocardial contractility but a 3.27 cm cap-like area of echo-poor and echo-dense layers at the left ventricular apex. There was leucocytosis (25,400/microliter) with 45% eosinophilia (11,430/microliter). IgE was raised to 134.6 kU/l. Bone marrow smear showed marked eosinophilic but little neutrophilic hyperplasia. Parasitic infection was excluded. TREATMENT AND COURSE: The clinical and echocardiographic findings suggested the diagnosis of Löffler's eosinophilic endocarditis (endomyocardial fibrosis), later confirmed histologically. After initial treatment, first only with cortisone then also hydroxyurea, the eosinophilia finally responded to combined cortisone and interferon alpha 2. The dosage schedule was governed by the clinical and echocardiographic findings. In the further course of the disease there occurred progressive heart failure with severe tricuspid and mitral regurgitation, secondary pulmonary hypertension and severe fibrosis of both ventricles, 41 months after diagnosis the patient underwent tricuspid and mitral valve reconstruction with removal of the endocardial fibrotic layers. Follow-up examinations found the cardiac condition to be adequately controlled without further cardiac infiltration since 20 months. CONCLUSIONS: Echocardiography, in conjunction with the clinical findings, provides a firm foundation for successfully treating Löffler's endocarditis. In selected cases cardiac surgery can markedly improve the course.

Adult↗
PubMed1983

Acquired tricuspid incompetence in children: pitfalls in the clinical diagnosis of its aetiology.

Pitfalls in the clinical diagnosis of the aetiology of acquired tricuspid incompetence in children were exemplified in five patients. In three of them acute staphylococcal endocarditis, the cause of the valve defect, was obscured during life by co-existing sickle cell haemoglobinopathy, acute leukaemia and rheumatic mitral valvulitis, respectively. The fourth and fifth patients had clinical features suggestive of right ventricular endomyocardial fibrosis but necropsy revealed that the tricuspid incompetence was caused by dilated cardiomyopathy and constrictive pericarditis respectively. This series illustrates the need to suspect bacterial endocarditis if a child presents with signs of infection and valvular incompetence, and also dispels the widespread belief that pericardial constriction does not cause tricuspid incompetence.

Cardiomyopathies↗
PubMed2004

Rapid reversion of Loeffler's endocarditis by imatinib in early stage clonal hypereosinophilic syndrome.

Endomyocardial fibrosis (Loeffler's endocarditis) is the main cause of poor outcome in Hyper Eosinophilic Syndrome (HES) and Eosinophilic Leukemia (EL). Reversion of the cardiac damage has been seldom reported, and thrombi can superimpose on infiltrated walls, originating oembolic complications. The tyrosine kynase inhibitor imatinib has been recently employed in patients affected by HES or EL, with impressive results. We have treated with imatinib a young patient affected by Loeffler's endocarditis during EL. Loeffler's endocarditis was studied by transthoracic Doppler echocardiography with and without the contrast agent SonoVue. Cytogenetics, FISH and molecular analysis showed the presence of the FIP1L1/PDGFRA fusion gene, recently detected in a majority of HES patients. Standard echocardiography revealed a large infiltration of the apical region, with apparently pedunculate corpora floating in the LV chamber; after SonoVue injection, a thick endo-myocardial infiltration involving papillary muscles and tendinous chords appeared, which simulated mobile thrombi at standard echography. Treatment with low dose imatinib caused rapid regression of both eosinophilic proliferation and endomyocardiopathy. The fusion gene FIP1L1-PDGFRA was found significantly decreased after a few months of treatment. Using a contrast echocardiographic approach, we demonstrated the non-thrombotic origin of the "in plus" image in our patient and its rapid resolution following imatinib treatment. Imatinib is an excellent candidate for first line treatment of Loeffler's endocarditis, especially when the FIP1L1/PDGFA fusion gene is detected.

Adult↗
PubMed1991

Pathomorphological aspects, aetiology and natural history of acquired mitral valve stenosis.

Commissural fusion, leaflet thickening and alteration of the subvalvular apparatus are dominant mechanisms causing clinically important mitral stenosis (MS) of rheumatic origin. Calcification and a consequent decrease in leaflet mobility are subsequent features in rheumatic MS and may be the primary mechanisms in MS of degenerative origin. In 1051 consecutive patients with pure or predominant MS requiring surgical intervention, aetiology was rheumatic in 76.9%, infective in 3.3%, degenerative (severe annular and leaflet calcification) in 2.7% and congenital (Lutembacher syndrome) in 1.2%; it was the result of systemic lupus erythematosus (n = 4), carcinoid heart disease (n = 2), endomyocardial fibrosis (n = 2) and rheumatoid arthritis (n = 2) in less than 1%, while in 14.5% of these patients aetiology remained unclassified. The natural history of rheumatic MS is characterized by an asymptomatic latent period, following the initial rheumatic fever (RF). In a prospective study of MS (n = 159) the mean interval between RF and the appearance of symptoms was 16.3 +/- 5.2 years. Twenty-five years after the initial RF 8% of the patients were still asymptomatic, 9% were class II (NYHA), 33% class III and 50% had been operated or were class IV. Progress from mild to severe disability took 9.2 +/- 4.3 years on average. When valve surgery was indicated but refused by the patients, survival with medical treatment was 0.44 +/- 0.06 after 5 years, 0.32 +/- 0.08 after 10 years and 0.19 +/- 0.09 after 15 years.(ABSTRACT TRUNCATED AT 250 WORDS)

Humans↗
PubMed1986

Loa loa infection in temporary residents of endemic regions: recognition of a hyperresponsive syndrome with characteristic clinical manifestations.

Loiasis in residents of endemic areas is generally manifested by microfilaremia, episodic angioedema, periocular migration of adult worms, modest eosinophilia, and variable antibody levels. In 20 temporary residents who acquired loiasis in West Africa, however, the clinical presentations were markedly different. Only three of the 20 had detectable microfilaremia. Furthermore, these patients often showed a state of marked immunological hyperresponsiveness manifested by very high titers of antibody to filariae, increased levels of serum IgE, and profound hypereosinophilia--to levels greater than 3,000/mm3 in 18 of the 20. These patients were also notable for the increased severity, frequency, and pruritic nature of their angioedema. Also, significant complications occurred in seven of the 20; one patient developed endomyocardial fibrosis and six, renal disease after treatment with diethylcarbamazine. Thirteen of the 20 developed subcutaneous nodules; adult parasites were recovered from three of five of these patients who underwent biopsy. All of the patients responded to therapy with diminution of their clinical symptoms and decreases in levels of eosinophils, IgE, and antibody to filariae.

Adult↗
PubMedDate not supplied

Resection of subvalvular aortic stenosis. Surgical and perioperative management in seven dogs.

Open heart surgery was performed during cardiopulmonary bypass (CPB) to surgically correct subvalvular aortic stenosis in seven dogs. After initiation of total CPB, cardiac arrest was induced by antegrade and retrograde administration of blood cardioplegia. The subvalvular fibrous stenosis was resected through a transverse aortotomy. Intraoperatively and postoperatively, dobutamine, nitroprusside, lidocaine, blood(-products), and crystalloid solutions were used to manage hypotension and optimize cardiac index. Aortic cross-clamp time varied from 73 to 166 minutes, and duration of CPB varied from 130 to 210 minutes. Iatrogenic incision into the mitral valve in two dogs was the most significant intraoperative complication. Postoperative complications included: hypoproteinemia (n = 7), premature ventricular depolarization (n = 6), increased systemic vascular resistance index (n = 5), increased O2 extraction (n = 3), pulmonary edema (n = 2), and decreased cardiac index (n = 1). All seven dogs were discharged alive and in stable condition. Six dogs are alive and in stable condition after a mean follow up of 15.8 months. This is the first detailed report of CPB in a series of clinical veterinary patients. Using the techniques described in this paper, open heart surgery of considerable duration can be performed successfully in dogs with significant myocardial hypertrophy and endomyocardial fibrosis secondary to subvalvular aortic stenosis.

Anesthesia, General↗
PubMed1997

Factors predisposing to the development of atrial fibrillation.

Atrial fibrillation (AF) is in most patients (approximately 70%) associated with organic heart disease including valvular heart disease, coronary artery disease, hypertension, hypertrophic cardiomyopathy, dilated cardiomyopathy, and congenital heart disease, mostly atrial septal defect in adults. In many chronic conditions, determining whether AF is the result or is unrelated to the underlying heart disease, remains unclear. The list of possible etiologies also include cardiac amyloidosis, hemochromatosis and endomyocardial fibrosis. Other heart diseases, such as mitral valve prolapse (without mitral regurgitation), calcifications of the mitral annulus, atrial myxoma, pheochomocytoma, and idiopathic dilated right atrium may present with AF. Atrial fibrillation may occur in the absence of detectable organic heart disease, the so-called "lone AF", in about 30% of cases. The term "idiopathic AF" implies the absence of any detectable etiology including hyperthyroidism, chronic obstructive lung disease, overt sinus node dysfunction, and overt or concealed preexcitation (Wolff-Parkinson-White syndrome), only to mention a few of other uncommon causes of AF. The autonomous nervous system may contribute to the occurrence of AF in some patients. AF occurs commonly. In patients with valvular heart disease, AF is common, particularly when the mitral valve is involved. The occurrence of AF is unrelated to the severity of mitral stenosis or mitral regurgitation but is more common in patients with enlarged left atrium and congestive heart failure. In patients with coronary artery disease, AF occurs predominantly in older patients, males, and patients with left ventricular dysfunction, Important predictive factors of AF include hypertension, left ventricular hypertrophy and diabetes. The risk of the development of AF, in an individual patient, is often difficult to assess. Increasing age, presence of valvular heart disease, and congestive heart failure increase the risk of atrial fibrillation.

Animals↗
PubMed1998

Echocardiographic Aspects of Restrictive Cardiomyopathy: Their Relationship with Pathophysiology.

BACKGROUND AND METHODS: We studied 17 patients with restrictive cardiomyopathy; eight had biventricular restriction (type A), four had left ventricular restriction (type B), and five had only right ventricular restriction (type C). RESULTS: Type A disease was characterized by pulmonary and systemic venous congestion. The restrictive pattern was found in the inlet of both ventricles. Both atria were enormous, with small or normal-size ventricles. Differential diagnosis included constrictive pericarditis and systolic pump dysfunction. Type B restriction disease was characterized by venous pulmonary congestion, pulmonary hypertension, and important dilatation of the left atrium and right cavities with a small or normal-size left ventricle; the restrictive pattern was found only in the affected left ventricle. CONCLUSIONS: The clinical picture resembles that of rheumatic mitral valve disease with right ventricular failure. Type C disease had restriction only in the inlet of right ventricle, with giant right atrium, systemic venous hypertension with low flow, and normal pressure of pulmonary artery and left heart. Differential diagnosis included Ebstein's anomaly of tricuspid valve. The etiology of type A disease was amyloid, endomyocardial fibrosis of ventricles and idiopathic interstitial fibrosis. Asymmetric types were always caused by Davies' disease.

Journal Article↗
PubMed1979

Congenital heart block and maternal systemic lupus erythematosus.

The association between infants with congenital heart block (CHB) and the presence or later development of maternal systemic lupus erythematosus or other connective-tissue disease (CTD) was reviewed in 67 cases. In 24 cases CHB was diagnosed at or before birth. Of nine necropsies on affected infants, seven showed endomyocardial fibrosis. The results suggest that one in three mothers who deliver babies with CHB have or will develop CTD. The association is probably explained by placental transfer of a maternal antibody. Awareness of the association may lead to prevention of the birth of children with CHB and better neonatal care of affected children.

Adolescent↗
PubMed1990

Familial restrictive cardiomyopathy with atrioventricular block and skeletal myopathy.

Five generations of an Italian family with an autosomal dominant restrictive cardiomyopathy are described. Members of four generations were examined. Symptoms usually developed in the third or fourth decade but the disease did occur in childhood. Initially the condition was characterised by normal ventricular size and systolic function with increased diastolic filling pressures in both ventricles and consequent bi-atrial enlargement. Cardiac catheterisation showed a left ventricular filling pattern of "dip and plateau". The electrocardiogram typically showed non-specific changes in the ST segment and T wave and changes indicating considerable atrial enlargement, which were confirmed by echocardiography. Light microscopy of two endocardial biopsy specimens showed no specific features but excluded the endomyocardial fibrosis of eosinophilic heart disease, amyloid, and specific heart muscle diseases. At necropsy in one case examined under light microscopy extensive patchy fibrosis was found throughout the endocardium, myocardium, and subepicardium, but there were no features typical of eosinophilic heart disease. Histopathological and biochemical examination of skeletal muscle identified no abnormality. The disease often had an insidious course over five to ten years after presentation. Bundle branch blocks, leading to complete atrioventricular block, however, often occurred and may be the first manifestation. Some individuals who survived into the fifth decade developed a progressive, non-wasting skeletal myopathy.

Adolescent↗
PubMed2006

Pitfalls in the diagnosis of left ventricular hypertrabeculation/non-compaction.

Left ventricular hypertrabeculation/non-compaction (LVHT) is a cardiac abnormality, characterised by >3 trabeculations apically to the papillary muscles and intertrabecular spaces. LVHT may occur with other cardiac abnormalities, heart failure, electrocardiographic abnormalities and neuromuscular disorders. This study gives an overview about (1) patients with LVHT in whom LVHT was initially overlooked and (2) cardiac conditions that may lead to falsely diagnosed LVHT. In 50 reported cases, LVHT has been overlooked and misdiagnosed as dilated (n = 20), hypertrophic (n = 14) or restrictive cardiomyopathy (n = 2), endocardial fibroelastosis (n = 5), endomyocardial fibrosis (n = 1), myocarditis (n = 3), thrombus (n = 2), localised left ventricular hypertrophy (n = 1), left ventricular mass (n = 1) or myocardial/pericardial disease (n = 1). In 14 patients, LVHT was diagnosed only by transoesophageal echocardiography (n = 1), computed tomography (n = 2) ventriculography (n = 2), magnetic resonance imaging (n = 3) or pathoanatomic findings (n = 6). Falsely diagnosed LVHT comprises false tendons, aberrant bands, thrombi, apical hypertrophic cardiomyopathy, fibroma, obliterative processes, intramyocardial haematoma, cardiac metastases and intramyocardial abscesses. Echocardiographers should be more aware of LVHT and consider its differential diagnoses.

Diagnostic Errors↗
PubMed2000

Aetiology of heart failure as seen from a National Cardiac Referral Centre in Africa.

572 consecutive patients with heart failure referred to the National Cardiothoracic Centre, Accra, Ghana, over a 4-year period were evaluated for the aetiology of heart failure using two-dimensional Doppler echocardiography with colour flow. The mean age of the subjects with heart failure was 42.3 +/- 0.9 years. The male to female ratio was 1.2:1.0. Combined heart failure was seen in 50.5% of subjects. Peak incidence of heart failure occurred in the 5th decade. The main causes of heart failure were hypertension (21.3%; n = 122), rheumatic heart disease (20.1%; n = 115) and cardiomyopathy (16.8%; n = 96). Congenital heart disease and coronary artery disease accounted for 9.8 and 10% of cases, respectively. The commonest rheumatic valvular lesion was mitral regurgitation (78%). Dilated cardiomyopathy was the commonest form of idiopathic cardiomyopathy (67.7%; n = 65). Endomyocardial fibrosis and hypertrophic cardiomyopathy accounted for 22.9% (n = 22) and 9.4% (n = 9), respectively, of cardiomyopathies.

Adolescent↗