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At least 415 records · Page 23Linked to original sources
PubMed1999

[Pathology of heart transplantation.(Morphological study of 1246 endomyocardial biopsies from 167 transplanted hearts). Causes of early, intermediate, and late deaths].

From January 1988 through October 1997, 167 cardiac transplants were performed. 1246 endomyocardial biopsies (EMBs) from 138 cardiac allograft recipients were investigated and graded according to the Working Formulation (WF) criteria. The specimens were inadequate in 44 EMBs (3.5%), while 598 (48%) showed no rejection. The grade of rejection was: mild (grade 1A and 1B) in 531 EMBs (42.6%), mild/moderate (grade 2) in 38 (3.1%), and moderate (grade 3A and 3B) in 35 (2.8%). The indications for transplantation were: dilated cardiomyopathy (46.1%); ischemic disease (37.1%); valvular disease (12%); hypertrophic cardiomyopathy (1.8%); myocarditis (1.2%); congenital cardiopathy (0.6%), restrictive cardiomyopathy (0.6%) and chronic rejection (0.6%). The most reliable histologic feature of acute rejection was the myocyte necrosis or damage in presence of pironinophilic mononuclear cell infiltrate, so our protocol requires multifocal or diffuse myocyte damage (rejection grade 3A and 3B) to perform an additional treatment, which was required in 35 cases (2.8%). An intermediate grade mild/moderate 2, was introduced from the WF to classify the EMBs in which the myocyte necrosis was scant or not clear; this grade in our series generally resolves without any additional treatment; in order to monitor the rejection another EMB was performed 5 days after in these patients. The EMBs showed also the following lesions other than acute rejection: Quilty A (79 patients; 57.25%), Quilty B (24 pts; 17.39%), early ischemic necrosis (43 pts; 31.15%) and late ischemic necrosis (5 pz; 3.62%). Quilty B and late ischemic necrosis were correlated with acute rejection (grade 2), furthermore the patients with graft vascular disease showed 3 or more episodes of acute rejection. These findings confirm the relationship between acute and chronic rejection. Furthermore, a relationship between chronic rejection (4 pts) and infection from hepatitis C (antibodies positive 3 pts/4) and cytomegalovirus (antibodies positive 4 pts/4) was found in our series. In the follow-up period (117 months), a 30.72% death rate was recorded; the main causes of death were: early failure of the transplanted heart (30 pts) in 4 of them associated with pulmonary hypertension, infections (6 pts), sudden death (4 pts), graft's vasculopathy (4 pts), acute pancreatitis (1 pts) pulmonary embolism (1 pts), lung (1 pts) and ovary (1 pts) carcinoma, acute rejection (1 pts), others (2 pts). In the early period (< 1 month), the most frequent cause of death was the early failure of the transplanted heart, while in the late period (> 1 year) the chronic rejection following by sudden death and tumours. The actuarial survival curve drops to 83.13% after the first post-operative month, abates to 75.30 at the end of the first year, and progressively decreases to 70.48% at the end of the fifth follow-up year. The mortality rate was 38.7% in pts transplanted for ischemic disease and 24.7% for dilated cardiomyopathy. Cardioplegia seems to play an important role in the success of the heart transplant.

Actuarial Analysis↗
PubMedDate not supplied

[Diagnostic value of myocardial biopsy in the diagnostic algorithm in cardiomyopathies in children].

The purpose of this work was to show the importance of myocardial bioptate analysis using different methods in the diagram of diagnostic flow in primary cardiomyopathies in children. According to the guidelines of the Task Force on Cardiomyopathies of the WHO/ISFC, we identified 121 children (50 f and 71 m) as having cardiomyopathy, giving an average occurrence for all cardiomyopathies of 38.81 for each 10,000 pts examined in our outpatient clinics for paediatric cardiology. The dilated cardiomyopathy (DCM) was identified in 52 pts (42.9%), hypertrophic cardiomyopathy (HCM) in 43 pts (35.5%) and restrictive cardiomyopathy (RCM) in 6 pts (4.8%). We placed 11 pts (9.0%) in the group of specific cardiomyopathies. In nine pts (7.4%), it was impossible to classify the cardiomyopathy. Most of those with DCM had been diagnosed prior to the age of 3 years (RR 1.9, 95% CI 1.4-2.47). There were no statistically significant differences in the incidences of DCM as compared to HCM (Z 0.923, p < 0.1779), but we encountered a significantly lower occurrence of RCM (Z 6.044, p < 0.001). The biopsy of endocardium and myocardium was done to confirm the etiology of primary cardiomyopathy in 22 pts, 12 m and 10 f, age 1 to 17 (average age 9.5y). The bioptates were analysed by light microscope (Dallas criteria) in all pts, 13 bioptates by direct immunofluorescence, 8 by immunohystochemical method (two hystochemically by the method of coloring with Kongo red, one by the microscopy in polarised light), 7 by electron microscope, and 5 by PCR method where DNA and RNA of cardiotrophic viruses was used. Out of 10 pts with DCM, in 4 myonecrosis as a consequence of acute myocarditis and in 6 signs of late inflammatory processes, as a consequence of chronic immunologic myocarditis, were found. In 4 of them rebiopsy proved complete healing. In 5 pts with HCM the diagnosis was confirmed hystologically. One bioptate was analysed by electron microscope to rule out mitochondriopathy. Out of 4 pts with RCM due to inflammation, in 3 pathohistological findings proved diagnosis and in one showed primary amyloidosis. In one patient pathohystological finding showed fibroelastosis. In one patient heart tumor (fibroma) has been found.

Adolescent↗
PubMedDate not supplied

Cardiac amyloidosis: hemodynamic, echocardiographic and endomyocardial biopsy studies.

Six patients with cardiac amyloidosis (four males, two females; age 27-60 years) were evaluated by us. Four patients presented with congestive heart failure, while one patient each presented with effort angina and giddiness. Extracardiac clues to the diagnosis in the form of involvement of other systems were present in only two patients. The electrocardiogram was abnormal in four patients while three exhibited roentgenographic evidence of cardiomegaly or pulmonary venous hypertension. Echocardiography suggested the diagnosis of amyloidosis in only two patients, restrictive cardiomyopathy in two other patients and dilated and hypertrophic cardiomyopathy in one patient each. Cardiac catheterisation and angiography suggested restrictive heart disease in four patients and hypertrophic cardiomyopathy in one. One patient, whose initial haemodynamic study was normal, had features of dilated cardiomyopathy at repeat study after 11 months. Endomyocardial biopsies showed amyloid deposits in all patients. We emphasise the varied clinical manifestation of cardiac amyloidosis and the need for a high index of suspicion. The diagnosis can be safely and reliably confirmed by endomyocardial biopsy.

Adult↗
PubMed1994

Endomyocardial biopsy--technical aspects experience and current status. An Indian perspective.

Our study ellucidates the utility of endomyocardial biopsy (EMB) in various cardiac-muscle disorders seen in a tropical country like India. The procedure has been successfully performed in 501 patients (572 procedures) at our centre from April 1985 to December 1992. This included 60 infants and children. The indications were dilated cardiomyopathy (DCM) in 214, non-specific aortoarteritis in 91, rheumatic heart disease in 75, restrictive cardiomyopathy in 45, constrictive pericarditis in 14 and miscellaneous in 62 patients. There was no mortality, however, one patient developed cardiac tamponade and another sustained ventricular tachycardia requiring cardioversion. There was transient atrial fibrillation in six patients and all these had acute rheumatic heart disease. Transient complete heart block occurred in six patients with underlying left-bundle branch-block. Histological examination of EMB revealed myocarditis in 34/214 (15.4%) patients in DCM group and helped in following up these cases on immunosuppressive treatment. In the presence of restrictive haemodynamics it could identify amyloidosis in four patients. It was also helpful in differentiating between endomyocardial fibrosis and chronic constrictive pericarditis. In patients with non-specific aorto-arteritis significant histological changes of inflammatory myocarditis were observed in patients especially in congestive heart failure. Furthermore, it was helpful in identifying the nature of cardiac tumour in one patient. Its utility has also been evaluated in disorders, including rheumatic heart disease, peripartum cardiomyopathy and systemic disorders like systemic lupus erythematosis. Even in the absence of cardiac-transplant programmes at national level we have found EMB to be a useful investigation in a tropical country like India.

Adolescent↗
PubMed2004

Molecular and cellular aspects of troponin cardiomyopathies.

Advances in molecular genetics have led to the identification of mutations in each troponin subunit that cause different human cardiomyopathies. Mutations in the genes for cardiac troponin T (CTnT), troponin I (CTnI), and troponin C (CTnC) cause familial hypertrophic cardiomyopathy (FHC) and are associated with varying prognosis and mild-to-moderate hypertrophy. Mutations in CTnT and CTnC can also cause dilated cardiomyopathy (DCM), whereas mutations in CTnI can cause restrictive cardiomyopathy (RCM). All together, 60 mutations have so far been found in troponin subunits associated with cardiomyopathy. Recently, multiple cardiomyopathic phenotypes (either HCM or RCM), arising from a single nucleotide mutation in the same codon of CTnI, R145, have been documented. Although the clinical phenotypes of the cardiomyopathies vary, two common features are present in most cardiomyopathy patients: altered Ca(2+) sensitivity of force development and impaired energy metabolism. Here, we present the analyses of how these troponin mutations affect the in vitro contractile protein function and the hypotheses derived to explain the development of these disease states.

Cardiomyopathies↗
PubMed1977

Left ventricular endomyocardial biopsy. II: the value of light microscopy.

Histological sections of left ventricular endomyocardial biopsies have been examined by light microscopy. The biopsies were taken from patients with congestive, hypertrophic, or restrictive cardiomyopathy and from patients with either aortic stenosis or regurgitation. In congestive cardiomyopathy no specific features were found and similar abnormalities were seen in aortic valve disease. In only one of six cases of asymmetric septal hypertrophy were the characteristic histological appearances noted. In four out of five patients with a restrictive type of cardiomyopathy, amyloid was demonstrated. The muscle fibres in aortic stenosis had a greater cross-sectional area than those in aortic regurgitation. A greater degree of fibrosis was present in aortic stenosis than in aortic regurgitation. In aortic stenosis the amount of fibrous tissue was inversely related to function.

Aortic Stenosis, Subvalvular↗
PubMed2000

[Mutations in genes for sarcomeric proteins].

Idiopathic cardiomyopathy(ICM) is by definition of unknown etiology. There are four clinical types of ICM; hypertrophic cardiomyopathy(HCM) characterized by ventricular hypertrophy associated with reduced compliance of the heart and accompanied by myofibrillar disarray, dilated cardiomyopathy(DCM) characterized by dilated ventricles associated with systolic dysfunction, restricted cardiomyopathy (RCM) and arrhythmogenic right ventricular cardiomyopathy(ARVC). Recent molecular genetic analyses have now revealed disease-associated mutations in ICM, especially in familial HCM and familial DCM. Mutations in 9 different disease genes (MYH7, TNNT2, TPM1, MYBPC3, MYL3, MYL2, TNNI3, CACT and TTN) cause HCM, while mutations in 3 different genes(CACT, DES and DMD) cause DCM in adults. In this review, I will summarize our current data on sarcomere mutations found in Japanese ICM, especially in HCM and DCM.

Actins↗
PubMed1985

Mildly dilated congestive cardiomyopathy.

Five patients with only mildly dilated ventricles but other features typical of congestive cardiomyopathy underwent cardiac transplantation for class IV NYHA heart failure. The findings of clinical studies, cardiac catheterization, endomyocardial biopsy, and pathologic examination of the removed hearts in this group with mildly dilated congestive cardiomyopathy (MDCM) were compared with similar data in four patients with idiopathic restrictive cardiomyopathy (IRCM) and in 10 patients with typical dilated congestive cardiomyopathy (DCM). In comparison with the other groups, patients with MDCM had a higher incidence of familial cardiomyopathy (p = .02) and a shorter symptomatic period than patients with IRCM (p less than .02). Patients with both MDCM and DCM had globular hearts (with predominant left ventricular dilatation), congestive hemodynamics and poor left ventricular contractility (ejection fraction 12% to 19%), and high incidence of ventricular thrombi. Patients with IRCM showed normal ventricular size, marked atrial dilatation, restrictive hemodynamics, mild-to-moderate decrease in left ventricular contractility (ejection fraction 29% to 55%), and absence of ventricular thrombi. Cardiac index, ventricular wall thickness, and light microscopic findings were similar in the three groups of patients. Electron microscopy showed no myofibrillar loss in patients with IRCM but mild (partial) or moderate-to-severe (almost total) myofibrillar loss in those with MDCM and DCM, respectively. We conclude that end-stage congestive cardiomyopathy may occur without significant ventricular dilatation and patients with MDCM have heart sizes intermediate between those found in IRCM and DCM but their clinical, hemodynamic, and pathologic findings are virtually identical to those of patients with typical DCM.

Adult↗
PubMed2003

Feline idiopathic cardiomyopathy: a retrospective study of 106 cats (1994-2001).

The case records of 106 cats with idiopathic cardiomyopathy that presented to the Feline Centre of the University of Bristol between September 1994 and September 2001 were reviewed retrospectively. Hypertrophic cardiomyopathy (HCM) was the most common form seen (57.5%), followed by restrictive cardiomyopathy (RCM) (20.7%), dilated cardiomyopathy (DCM) (10.4%) and unclassified cardiomyopathy (UCM) (10.4%). One cat showed echocardiographic changes compatible with a moderator band cardiomyopathy (MBCM). Most affected cats were domestic short hairs (DSH) (57.5%). The mean (+/-SD, range) age of cats with cardiomyopathy at presentation was 6.8 (4.3, 0.5-16) years, with an equal distribution of males and females. Clinical findings, electrocardiographic changes and radiographic abnormalities were also reviewed. The median survival time for 73 cats for which follow-up data was available was 300 days. A greater survival time was observed for cats with UCM (925 days) when compared with those with HCM (492 days), RCM (132 days) or DCM (11 days).

Animals↗
PubMed1994

Low frequency of detection by nested polymerase chain reaction of enterovirus ribonucleic acid in endomyocardial tissue of patients with idiopathic dilated cardiomyopathy.

OBJECTIVES: The purpose of this study was to determine the prevalence of enteroviral infection in the myocardium of patients with idiopathic dilated cardiomyopathy by using a highly sensitive and specific detection technique. BACKGROUND: Recent molecular studies have suggested that enteroviral persistence (in particular, coxsackieviruses type B) may underlie idiopathic myocarditis and dilated cardiomyopathy. METHODS: The method used to detect enterovirus-specific ribonucleic acids (RNAs) is based on reverse transcription and nested polymerase chain reaction amplification with four pairs of primers from the conserved 5' noncoding region of the enteroviral genome. Several members of the Enterovirus genus are detectable by this assay (coxsackieviruses B1 to B6; polioviruses 1 to 3; echoviruses 9, 19 and 31), with a sensitivity threshold close to the detection of a single molecule of viral RNA in 1 mg of tissue sample. Endomyocardial tissue samples from 84 subjects were analyzed (77 samples obtained from left endomyocardial biopsies, 7 from explanted hearts). The subjects comprised 63 study patients (53 with dilated cardiomyopathy, 3 with idiopathic myocarditis, 1 with right ventricular dysplasia, 1 with restrictive cardiomyopathy, 1 with eosinophilic myocarditis, 1 with primary ventricular fibrillation and 3 with myocarditis of known etiology) and 21 control subjects with other diseases. RESULTS: Positive signals were obtained only in samples from six study patients (four with dilated cardiomyopathy, one with right ventricular dysplasia and one with myocarditis). Samples from control subjects, uninfected rat myocardium and cultured cell lines yielded systematically negative results. Moreover, the nucleotide sequence analysis of the amplification products from patients with positive samples raised doubts about the true positivity of these samples. CONCLUSIONS: This study suggests that the persistence of enteroviral RNA in dilated cardiomyopathy is not a major cause of the disease and that a careful analysis of polymerase chain reaction amplification products is essential in any study in which this technique is pushed to high sensitivity thresholds.

Adult↗
PubMed1982

Right Ventricular endomyocardial biopsy: clinicopathologic correlates in 100 consecutive patients.

The first 100 consecutive patients to undergo right ventricular endomyocardial biopsy at the Mayo Clinic were divided retrospectively into five groups, depending on the prebiopsy clinical diagnosis, and the biopsies were review histologically in a single-blind format. Group 1 consisted of 34 patients with a diagnosis of unexplained congestive heart failure and a dilated heart; of these, 4 (12%) had active myocarditis by biopsy. Of the six patients in group 2 with a clinical diagnosis of myocarditis, only one (17%) had biopsy evidence of inflammation, but two (33%) had changes that, in the clinical setting, were suggestive of cardiomyopathy. Group 3 consisted of 27 patients with dysrhythmia, syncope, or cardiac arrest but without congestive heart failure; of these, 4 (15%) had active myocarditis by biopsy, and 8 (30%) had changes that, with the clinical history, were consistent with cardiomyopathy. Group 4 included 19 patients with unexplained congestive heart failure and a nondilated heart; 4 (21%) had cardiac amyloid on biopsy. Group 5 was a diverse group of 14 patients with possible cardiac involvement by a known systemic disease; myocardial disease was documented by biopsy in 7 (50%). On the basis of these findings, we recommend endomyocardial biopsy (1) in patients with dilated cardiomyopathy in whom myocarditis is suspected, (2) in patients with the clinical diagnosis of active myocarditis in whom tissue documentation is indicated before treatment with anti-inflammatory or immunosuppressive drugs, (3) in patients with clinically unexplained life-threatening dysrhythmias in whom myocarditis or cardiomyopathy may be present, and (4) in patients with apparent hypertrophic or restrictive cardiomyopathy in whom cardiac amyloid may be present.

Adolescent↗
PubMed1976

[Morpho-functional changes of left ventricle produced by sublingual nitroglycerin (author's transl)].

Two successive left ventriculargraphs [in basal conditions and after Nitroglycerin (TNG)] were performed with the same technical procedures in 20 patients before coronary arteriography. End-diastolic and end-systolic volumes and ejection fraction were studied following Greene's modified method. Diastolic and systolic ventricular projected images were divided into four zones and their variations were evaluated after TNG. Two patients did not show any significant abnormality (pseudoangor); two others suffered from cardiomyopathy (myocardosis) with mitral regurgitation and no coronary artery disease; two had restrictive cardiomyopathy. Fourteen had a coronary artery disease: seven of them had signs of end-systolic mitral regurgitation. End-diastolic and end-systolic volumes decreased and ejection fraction increased after TNG in all of these cases except in the two suffering from cardiomyopathy (ESV increased and EF decreased). Mitral regurgitation disappeared in all of them. In patients with coronary artery disease and mitral regurgitation the volume variations were slighter, and the increase of EF was larger than in the other cases. Finally, a decrease in asynergic and hypokinetc zones was observed, whereas no variations were seen in diskinetic zones. TNG can improve left ventricular kinetics by decreasing the pre-load, the after-load and myocardial oxygen consumption. Demonstration of this improvement could be of prognostic value.

Administration, Oral↗
PubMed2000

Single large-scale mitochondrial DNA deletion in a patient with encephalopathy, cardiomyopathy, and prominent intestinal pseudo-obstruction.

We studied a 62 year-old woman with a clinical phenotype characterized by encephalopathy, restrictive cardiomyopathy, and prominent intestinal pseudo-obstruction. Muscle morphology showed ragged red fibres with ultrastructurally abnormal mitochondrial whereas muscle respiratory chain was normal. Molecular genetics revealed the 'common deletion' in mtDNA, which represented 40% of total mtDNA. These data expand and confirm the wide clinical spectrum of mitochondrial disorders associated with single large-scale mtDNA deletions.

Cardiomyopathies↗
PubMedDate not supplied

Discordance between pre and post cardiac transplant diagnosis: implications for pre- and postoperative decision making.

A correct clinical diagnosis in end-stage patients undergoing cardiac transplantation may have important prognostic and therapeutic implications. A retrospective clinico-pathologic study was carried out in 257 patients who had undergone cardiac transplantation at the University of Padua. A discrepancy between clinical and pathological diagnosis was found in 20 cases (8%). Among 126 patients with the clinical diagnosis of dilated cardiomyopathy, seven were found eventually to have ischemic heart disease (IHD), five myocarditis, one arrhythmogenic right ventricular cardiomyopathy (ARVC), and one non-compacted myocardium. Among the 87 patients with clinical diagnosis of IHD, three turned out to be dilated cardiomyopathy and one granulomatous myocarditis. Among the 10 patients with the clinical diagnosis of hypertrophic-restrictive cardiomyopathy, one had ARVC and one had cardiac fibroma. Altogether, only 24.5% underwent endomyocardial biopsy (EMB) and 75% coronary angiography before transplantation. Missed diagnosis of myocarditis occurred in patients in whom EMB was not carried out. EMB and coronary angiography might be indicated routinely in patients with apparent dilated cardiomyopathy, before proceeding to cardiectomy.

Adolescent↗
PubMed1995

Selecting candidates for cardiac transplantation. How to assess exclusion criteria and predict who will benefit.

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.

Cardiac Output, Low↗
PubMed1992

Peripheral neuropathy with giant axons and cardiomyopathy associated with desmin type intermediate filaments in skeletal muscle.

A sporadic case (female, aged 14 years) is reported who was affected by myopathy, restrictive cardiomyopathy and sensory motor polyneuropathy. A muscle biopsy showed accumulation of osmiophilic granular and filamentous material on electron microscopy, which stained positively in immunofluorescence for desmin. Increased desmin phosphorylated isoforms have been demonstrated by one- and two-dimensional electrophoresis. Sural nerve biopsy showed a peripheral neuropathy with giant axons, filled with closely packed neurofilaments. Clinical and morphological aspects of this new disease entity are discussed with regards to the classical form of giant axonal neuropathy and to other conditions of peripheral neuropathy with giant axons.

Adolescent↗
PubMed1989

[The use of endomyocardial biopsy in diagnosing cardiomyopathy and myocarditis].

The data available in the literature on history and improvement of a method of endomyocardial biopsy, indications and contraindications for its use and its value in the diagnosis of various types of cardiomyopathies and myocarditis are presented and generalized. The paper also gives a morphological picture of dilated, hypertrophic, and restrictive cardiomyopathies, as well as myocarditis occurring in dilated cardiomyopathy. It was concluded that endomyocardial biopsies were safe and highly diagnostically significant.

Biopsy↗
PubMed1992

A case of pediatric cardiomyopathy with severely restrictive physiology.

A rare case of a 6-year-old male with idiopathic familial cardiomyopathy manifesting severely restrictive physiology is reported. The patient showed congestive heart failure with dilatation of both atria with a normal ventricular cavity. A square-root configuration was revealed in the ventricular pressure tracings. His elder brother had died of hypertrophic cardiomyopathy at the age of 3 years. Endomyocardial biopsy disclosed marked disorganization of muscle bundles with hypertrophy of the myocytes and interstitial fibrosis. The patient died suddenly during hospitalization. Autopsy revealed diffuse hypertrophy of both the ventricular walls and the ventricular septum with extensive myocardial disorganization and interstitial fibrosis. These advanced myopathic changes in the myocardium may have been related to the restrictive physiology in this case.

Bundle-Branch Block↗