[Stiff heart syndrome].
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Cardiomyopathy, Restrictive↗
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Left ventricular volume reduction for end-stage heart failure: new horizon or false dawn?
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Cardiomyopathy, Restrictive↗
Xq28-linked noncompaction of the left ventricular myocardium: prenatal diagnosis and pathologic analysis of affected individuals.
Isolated noncompaction of the left ventricular myocardium (INVM) is characterized by the presence of numerous prominent trabeculations and deep intertrabecular recesses within the left ventricle, sometimes also affecting the right ventricle and interventricular septum. Familial occurrence of this disorder was described previously. We present a family in which 6 affected individuals demonstrated X-linked recessive inheritance of this trait. Affected relatives presented postnatally with left ventricular failure and arrhythmias, associated with the pathognomonic echocardiographic findings of INVM. The usual findings of Barth syndrome (neutropenia, growth retardation, elevated urinary organic acids, low carnitine levels, and mitochondrial abnormalities) were either absent or found inconsistently. Fetal echocardiograms obtained between 24-30 weeks of gestation in 3 of the affected males showed a dilated left ventricle in one heart, but were not otherwise diagnostic of INVM in any of the cases. Four of the affected individuals died during infancy, one is in cardiac failure at age 8 months, and one is alive following cardiac transplant at age 9 months. The hearts from infants who died or underwent transplantation appeared, on gross examination, to be enlarged, with coarse, deep ventricular trabeculations and prominent endocardial fibroelastosis. Histologically, there were loosely organized fascicles of myocytes in subepicardial and midmyocardial zones of both ventricles, and the myocytes showed thin, often angulated fibers with prominent central clearing and reduced numbers of filaments. Markedly elongated mitochondria were present in some ventricular myocytes from one specimen, but this finding was not reproducible. Genetic linkage analysis has localized INVM to the Xq28 region, where other myopathies with cardiac involvement have been located.
Adolescent↗
[Cardiomyopathies].
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Arrhythmogenic Right Ventricular Dysplasia↗
A 50-year-old male with diabetes mellitus, peripheral neuropathy, hypothyroidism, obesity, sleep disorder, pickwickian syndrome, and heart failure.
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Cardiomyopathy, Restrictive↗
[Changes of the interventricular heart septum in various forms of cardiomyopathy].
A pathomorphological study included hearts of 52 patients who died of various forms of cardiomyopathy, 10 hearts of subjects who died of chance causes (control) and 30 hearts in 3 groups of comparison for revealing changes in the interventricular septum in the dilatation, hypertrophic and restrictive forms of cardiomyopathy. Use was made of postmortem coronarography and cardioventriculography, the WHO standard anatomical method, histological and histochemical methods and separate weighing of cardiac portions. A complicated structure of the interventricular septum pathognomonic for each form of cardiomyopathy was revealed which had an influence on the intracardiac hemodynamics.
Adult↗
Hypertrophic cardiomyopathy mimicking pericardial constriction or myocardial restriction.
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Cardiomyopathies↗
Persistence of restrictive left ventricular filling pattern in dilated cardiomyopathy: an ominous prognostic sign.
OBJECTIVES: We sought to assess the prognostic implications of the evolution of restrictive left ventricular filling pattern (RFP) in dilated cardiomyopathy (DCM). BACKGROUND: Previous work has demonstrated that a RFP in DCM is associated with a poor prognosis. Few data are available on the prognostic implications of the evolution of this pattern. METHODS: The evolution of left ventricular filling was studied by Doppler echocardiography in 110 patients with DCM. According to the left ventricular filling pattern at presentation and after 3 months of treatment, the patients were classified into three groups: Group 1A (n = 24) had persistent restrictive filling; Group 1B (n = 29) had reversible restrictive filling; and Group 2 (n = 57) had nonrestrictive filling. RESULTS: During follow-up (41 +/- 20 months), mortality plus heart transplantations was significantly higher in Group 1A than in Groups 1B and 2 (p < 0.0001). On multivariate analysis, the model incorporating E wave deceleration time at 3 months was more powerful at predicting mortality with respect to this variable at baseline (p = 0.0039). Clinical improvement at 1 and 2 years was significantly more frequent in Groups 1B and 2 than in Group 1A (p < 0.0001 at 2 years). CONCLUSIONS: In patients with DCM, the persistence of restrictive filling at 3 months is associated with a high mortality and transplantation rate. The patients with reversible restrictive filling have a high probability of improvement and excellent survival. Doppler echocardiographic reevaluation of these patients after 3 months of therapy gives additional prognostic information with respect to the initial study.
Adult↗
Value and limitations of two dimensional echocardiography in assessment of cardiomyopathy.
Primary myocardial diseases have traditionally been classified into congestive, hypertrophic and restrictive varieties. M mode echocardiography has proved extremely valuable in distinguishing congestive cardiomyopathy with major ventricular dilation and poor contractile performance from hypertrophic cardiomyopathy with predominant left ventricular hypertrophy (septum greater than posterior wall) and normal pump performance, and restrictive disorders with symmetric ventricular wall thickening and normal or diminished contractile function. The contributions of two dimensional echocardiography to the evaluation of patients with congestive or restrictive cardiomyopathy have been limited to certain specific situations, such as the recognition of left ventricular mural thrombi. Twenty-five patients with hypertrophic cardiomyopathy were studied to assess two dimensional echocardiography in the evaluation of patients with this disorder. Two dimensional echocardiography indicated that hypertrophy of the interventricular septum is not uniform from apex to base in all patients but may be greatest in the apical, mid or basal third. In addition, the anterior free wall of the left ventricle was involved in the hypertrophic process in approximately 50 percent of patients. Two dimensional echocardiography documented that the location of systolic anterior motion of the mitral valve in patients with hypertrophic cardiomyopathy is most often at the junction of the mitral valve leaflets and chordae tendineae, although the chordae themselves and even th papillary muscles may be involved in this movement. The heterogeneity of these characteristics may enable patients with hypertrophic cardiomyopathy to be classified into subsets of patients in whom meaningful therapeutic and prognostic implications may be derived. Finally, data derived from two dimensional echocardiography have shown that, although the left ventricle in hypertrophic cardiomyopathy conforms in some degree to the configuration of a catenoid, this geometric conformation is unlikely to account for the genesis of this order.
Cardiomegaly↗
[Doppler echocardiographic investigation of cardiomyopathies].
There are three types of cardiomyopathy: hypertrophic, dilated and restrictive. The diagnosis and prognosis of these three types may be assessed from Doppler echocardiographic data. In hypertrophic cardiomyopathy, the diagnostic criterion is parietal hypertrophy. This hypertrophy is asymmetrical and usually affects the interventricular septum. Ventricular outflow obstruction is not necessarily present. The left ventricle is small and analysis of mitral inflow usually shows abnormal relaxation (E/A ratio < 1). The prognosis of this type of cardiomyopathy seems to be related to ventricular arrhythmias and the relationship between the hypertrophy and the presence of arrhythmias remains controversial. Dilated cardiomyopathy is characterized by ventricular walls of normal or decreased thickness, an increase in left ventricular dimensions and a reduction in the ejection fraction. An end-diastolic left ventricular dimension > 70 mm and an ejection fraction < 25% are poor prognostic factors. Left ventricular filling is abnormal and severe cases show a restrictive type of profile; in this case, an E/A ratio > 2 carries a poor prognostic. A mitral deceleration time of E wave < 150 msec usually indicates a bad outcome. Restrictive types of cardiomyopathy are more rare, amyloidosis being the commonest cause. Symmetrical wall thickening and a small ventricular chamber are observed. In advanced stages with abnormal systolic function, an E/A mitral ratio > 2 and deceleration time < 150 msec, the outcome is rapidly fatal.
Adult↗
[Endomyocardial biopsy in children and adolescents].
24 endomyocardial biopsies were performed in children aged 4-18 (x = 12.9) without any complications. The endomyocardial biopsy was performed in 12 patients with the heart failure of unknown origin (6 - restrictive heart disease, 4 - dilated cardiomyopathy with arrhythmias, 2-post-inflammatory dilated cardiomyopathy), in 5 patients with a clinical diagnosis of myocarditis, in 1 with the hypertrophic cardiomyopathy, in 2 cases of the dilated cardiomyopathy without heart failure and in 1 girl with the recurrent exudative pericarditis. Active myocarditis with fibrosis was stated in 2 cases of a restrictive heart disease, mild inflammatory state in 2 patients with a clinical diagnosis of the myocarditis and in 3 others with the dilated cardiomyopathy. All of 7 patients with the myocarditis underwent the immunosuppressive therapy. In the patient with endomyocardial fibrosis the result of left ventricular biopsy corresponded with angiocardiographic diagnosis. Non-specific changes in biopsies were stated in 2 children with the restrictive cardiomyopathy and in 5 with the dilated cardiomyopathy. Bioptic, morphologic lesions in patients with the dilated cardiomyopathy did not correlate with hemodynamic parameters of contractility. Biopsies were normal in 6 subjects. Endomyocardial biopsy influenced on making a decision of the therapy in 9 of 21 patients (42.8%). Diagnosis was verified in 7 patients (38.3%) basing on endomyocardial biopsy. In 18 of 24 subjects (75%) endomyocardial biopsy contributed to the interpretation of the disease pathology.
Adolescent↗
Nutritional influences on aging of Fischer 344 rats: II. Pathology.
The aim of this study was to explore the effects of nutritional manipulations on the occurrence and progression of age-related pathologic lesions in male Fischer 344 rats. The following nutritional regimens were studied: (a) ad libitum feeding, (b) food restriction initiated at 6 weeks of age, (c) food restriction initiated at 6 months of age, (d) food restriction limited to a period of early life (6 weeks to 6 months of age), (e) protein restriction without caloric restriction. The major age-related lesions observed were chronic nephropathy, cardiomyopathy, and neoplasia. Food restriction initiated at 6 months of age was as effective as food restriction initiated at 6 weeks of age in slowing the progression of chronic nephropathy and cardiomyopathy and in delaying the occurrence of neoplasia. Food restriction limited to early life was much less effective. Protein restriction in the absence of caloric restriction did not delay the occurrence of neoplasia, but it did retard chronic nephropathy and cardiomyopathy, although much less effectively than caloric restriction involving a similar level of protein restriction.
Aging↗
Safety and utility of endomyocardial biopsy in infants, children and adolescents: a review of 66 procedures in 53 patients.
The benefits and risks of endomyocardial biopsy in infants, children and adolescents were determined by reviewing the indications for and complications and results of 66 procedures in 53 patients aged 2 months to 20 years. One patient had a pneumothorax, and three had a right ventricular perforation. Ventricular tachycardia developed in four patients; it was treated with lidocaine in three and was self-limited in one. The procedure was unsuccessful in two patients. Among 25 patients with a prebiopsy diagnosis of idiopathic dilated cardiomyopathy, microscopic features were consistent with cardiomyopathy in 24 (96%) and were normal in 1. Of nine patients with clinically suspected myocarditis, only two (22%) had microscopic evidence of inflammation, and seven had chronic nonspecific features suggestive of dilated cardiomyopathy. Of eight patients with unexplained arrhythmias, six (75%) had microscopic findings compatible with dilated cardiomyopathy and two had myocarditis. Biopsy tissue samples from seven patients with nondilated forms of cardiomyopathy (four hypertrophic, three restrictive) were consistent with the clinical diagnosis in six and were inadequate in one. Cardiac biopsies were also performed in four patients with other disorders. Among the 51 patients with adequate biopsy specimens, microscopic features were considered diagnostic in 5, confirmatory in 44 and not helpful in 2 with normal tissue. The results indicate that endomyocardial biopsy is safe in infants, children and adolescents. It is useful for the evaluation of cardiomyopathy and specific secondary forms of myocardial disease. There seems to be little correlation, however, between clinical and tissue diagnoses of myocarditis.
Adolescent↗
Relation of transmitral flow velocity patterns to left ventricular diastolic function: new insights from a combined hemodynamic and Doppler echocardiographic study.
In an effort to determine what clinically useful information regarding left ventricular diastolic function can be inferred noninvasively with pulsed wave Doppler echocardiography, mitral flow velocity patterns and measured variables were correlated with hemodynamic findings in 70 patients: 30 with coronary artery disease, 20 with idiopathic congestive cardiomyopathy, 14 with a restrictive myocardial process and 6 without significant cardiac disease. The effect of sudden changes in hemodynamics on the mitral flow velocity pattern was also investigated in a subgroup of patients who had simultaneous recording of mitral flow velocity and left ventricular pressure before and after left ventriculography. Mitral flow velocity recordings from 30 healthy adults served as a reference group. This analysis suggests that 1) the majority of patients with these cardiac disorders demonstrate abnormal mitral flow velocity patterns or variables; 2) markedly different flow velocity patterns can be seen in patients with impaired left ventricular relaxation; 3) the different mitral patterns appear to relate more to myocardial function and hemodynamic status than to the type of disease process present; 4) certain mitral patterns suggest different filling pressures and rates of early diastolic left ventricular filling; 5) an increase in left atrial pressure can "normalize" an abnormal mitral flow velocity pattern and "mask" a left ventricular relaxation abnormality; and 6) the different patterns appear to represent a dynamic continuum with the potential to change from one to another as a result of disease progression, medical therapy or sudden changes in hemodynamics. It is concluded that, despite the indirect method of estimation and certain limitations, mitral flow velocity recordings have clinical potential in assessing left ventricular diastolic function that merits further investigation.
Adult↗
Epidemiology of anthracycline cardiotoxicity in children and adults.
Anthracyclines, potent cytotoxic agents used to treat a broad spectrum of malignancies, are limited in their use by an attendant risk of cardiotoxicity. Malignancies affect all age ranges, and anthracyclines are used in all age ranges, thereby exposing a broad population of patients to the development of heart disease. For some treated patients, anthracyclines affect cardiac muscle, resulting in cardiomyopathy. The type and degree of cardiomyopathy, as well as when during or after treatment the condition occurs, are dependent on what risk factors are present. Age is a major risk factor. Children and adults may develop restrictive and dilated cardiomyopathy. The length of subsequent survival and amount of subsequent somatic growth may influence late anthracycline-associated cardiac outcome. Early cardiotoxicity, occurring during or within 1 year of completion of treatment, is the largest risk factor for the development of late cardiotoxicity, which occurs beyond a year of completion of treatment. Risk factors, which appear to be specific for early cardiotoxicity in children, include black race, trisomy 21, and the use of amsacrine therapy after anthracycline therapy. More cardiotoxic effects are seen in survivors of childhood cancer, the longer from completion of treatment a patient is followed. Cumulative as well as peak anthracycline doses affect adults and children alike, and cardiotoxicity occurs early and late. In adults, left ventricular contractility is affected by anthracyclines. Children may manifest impairment of left ventricular contractility and increased afterload due to thinning of left ventricular walls. Patients with an early presentation of depressed left ventricular contractility are likely to show progression of cardiac disease with time. In addition, female gender appears to affect early and late cardiotoxicity in both adults and children, although this risk factor has been described predominantly in the survivors of childhood cancer. Thus, although anthracycline chemotherapy has improved overall survivorship of patients with cancer, there is a significant risk of cardiotoxicity associated with this class of drugs.
Adult↗
[Idiopathic dilated cardiomyopathies in children. Development and prognostic factors].
This review is a retrospective study of the cases of 103 patients under 20 years of age with idiopathic dilated cardiomyopathy and represents the whole experience of the Department of Paediatric Cardiology of the Hospital of Cardiovascular Disease, Lyon, between January 1970 and December 1988. The aetiological investigations were constantly negative: hypertrophic, restrictive and secondary cardiomyopathies were excluded. The clinical, radiological, electrocardiographic and echocardiographic data of each patient was noted at the time of admission and at the end of the clinical course. There were many more infants in this series. The onset of the disease was usually sudden. All patients were given medical treatment. The study period ranged over 19 years. Forty one patients (39.8%) died mainly of terminal cardiac failure. Sixty two patients are still alive, of whom 39 are completely cured, 2 have undergone cardiac transplantation, and 21 have a variable degree of chronic invalidity. The six months survival was 77.5%; the 1 year survival was 70% and the 5 year survival was 60%. Over one third of deaths (36.6%) occurred during the first month, 56% during the first 3 months and 70% during the first year. A statistical analysis comparing the initial criteria to death by the chi 2 test revealed the following prognostic factors: age at presentation (32.9% of deaths in patients under 2 years of age compared to 56.6% in the group over 2 years of age, p less than 0.025) and a family history of myocardial disease. The other clinical, radiological and echocardiographic parameters had no predictive value in this series and it was not possible to identify potential candidates for cardiac transplantation.
Adolescent↗
Cardiomyopathy in children.
The cardiomyopathies are a group of disorders of the myocardium associated with a decrease in cardiac function. This article describes the three functional types: (1) dilated or congestive; (2) obstructive, as in hypertrophic cardiomyopathy, and (3) restrictive as in infiltrative disorders such as the storage diseases.
Anti-Arrhythmia Agents↗
Polymorphisms in cytokine genes do not predict progression to end-stage heart failure in children.
UNLABELLED: A number of cytokines have been implicated in the pathophysiology of congestive heart failure. Genetic polymorphisms of several cytokine genes are known to result in altered gene expression, enabling us to characterize patients as "high" or "low" producers of specific cytokines. We speculate that the cytokine genotypes for a population of children who underwent heart transplantation for end-stage ventricular failure due to cardiomyopathy or congenital heart disease would be enriched for "high producers" of pro-inflammatory cytokines and "low producers" of anti-inflammatory cytokines. METHODS: Cytokine genotyping was performed for the following cytokines on 94 transplanted children using polymerase chain reaction-sequence specific technique: tumor necrosis factor-alpha (-308), interleukin 10 (-1082, -819, -592), interleukin 6 (-174), transforming growth factor-beta1 (codons 10 & 25), and interferon-gamma (+874). Patients with ventricular failure after transplantation for dilated cardiomyopathy, numbering 37, or for congenital heart disease, numbering 34, were compared to 15 children transplanted for structural disease, such as hypoplastic left heart syndrome, without ventricular failure, and to data from healthy children. An additional 8 children with restrictive or hypertrophic cardiomyopathy were also studied. RESULTS: No differences in genotypic distribution were seen between the groups, and all patients were comparable to genotypic distributions as assessed from published normal data. CONCLUSION: No evidence is found to support the hypothesis that these polymorphisms for cytokine genes influence progression to end-stage heart failure in children undergoing transplantation because of cardiomyopathy or congenital heart disease.
Child↗