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PubMed2003

The epidemiology of childhood cardiomyopathy in Australia.

BACKGROUND: The incidence and age distribution of primary cardiomyopathy in children are not well defined. We undertook a population-based, retrospective cohort study in Australia to document the epidemiology of childhood cardiomyopathy. METHODS: We analyzed all cases of primary cardiomyopathy in children who presented between 1987 and 1996 and who were younger than 10 years of age. Children were recruited from multiple sources, and cases of cardiomyopathy were classified according to World Health Organization guidelines. RESULTS: Over the 10-year period, 314 new cases of primary cardiomyopathy were identified, for an annual incidence of 1.24 per 100,000 children younger than 10 years of age (95 percent confidence interval, 1.11 to 1.38). Dilated cardiomyopathy made up 58.6 percent of cases, hypertrophic cardiomyopathy 25.5 percent, restrictive cardiomyopathy 2.5 percent, and left ventricular noncompaction 9.2 percent of cases. The incidence of all types of cardiomyopathy except restrictive declined rapidly after infancy. In 11 cases (3.5 percent), sudden death was the first symptom. There was a male predominance among children with hypertrophic and unclassified cardiomyopathy. Indigenous children had a higher incidence of dilated cardiomyopathy than nonindigenous children (relative risk, 2.67; 95 percent confidence interval, 1.42 to 4.63) and a higher rate of death as the presenting symptom (16.7 percent vs. 2.6 percent, P=0.02). Lymphocytic myocarditis was present in 25 of 62 children with dilated cardiomyopathy (40.3 percent) who underwent cardiac histologic examination within two months after presentation. CONCLUSIONS: Lymphocytic myocarditis and left ventricular noncompaction are important causes of childhood cardiomyopathy in Australia. The timing and severity of presentation in children with cardiomyopathy are related to the type of cardiomyopathy, as well as to genetic and ethnic factors.

Age Distribution↗
PubMed2005

[MR imaging in cardiomyopathies].

According to the WHO classification, cardiomyopathies are a group of diseases which are associated with myocardial dysfunction and can be classified either as primary or secondary cardiomyopathies. Genetic disorders have been identified in certain primary cardiomyopathies, however often the etiology remains unknown. The term "secondary cardiomyopathy" is used to specify diseases with the clinical indications of a cardiomyopathy, but can be attributed to a certain pathophysiological mechanism such as exposure to toxic substances, metabolic syndromes or systemic diseases. Based on morphological and functional criteria, primary cardiomyopathies are divided into dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), arrhythmogenic right ventricular cardiomyopathy (ARVC) and restrictive cardiomyopathy (RCM). During the last two decades MR imaging has emerged to a well established diagnostic tool for the understanding and treatment of cardiomyopathies. Morphological and functional information can be achieved with a high level of accuracy and reproducibility. Tissue alteration of the myocardium can be detected assessing regional contrast enhancement, T1- and T2-signal intensities and chemical shift phenomena. This article describes characteristic aspects of MR imaging for the diagnosis of primary and secondary cardiomyopathies.

Adult↗
PubMed2000

Iron-overload cardiomyopathy: evidence for a free radical--mediated mechanism of injury and dysfunction in a murine model.

Iron-overload cardiomyopathy is a restrictive cardiomyopathy that manifests itself as systolic or diastolic dysfunction secondary to increased deposition of iron in the heart and occurs with common genetic disorders such as primary hemochromatosis and beta-thalassemia major. Although the exact mechanism of iron-induced heart failure remains to be elucidated, the toxicity of iron in biological systems is believed to be attributed to its ability to catalyze the generation of oxygen-free radicals. In the current investigation, the dose-dependent effects of chronic iron-loading on heart tissue concentrations of iron, glutathione peroxidase (GPx) activity, free-radical production, and cardiac dysfunction were investigated in a murine model of iron-overload cardiomyopathy. It was shown that chronic iron-overload results in dose-dependent (a) increases in myocardial iron burden, (b) decreases in the protective antioxidant enzyme GPx activity, (c) increased free-radical production, and (d) increased mortality. These findings show that the mechanism of iron-induced heart dysfunction involves in part free radical-mediated processes.

Animals↗
PubMed1992

[Endomyocardial biopsy in childhood].

The experience with endomyocardial biopsy in pediatric age is still limited. From February 1986 to August 1990, 144 right ventricle endomyocardial biopsies were performed in 84 patients (age range 33 days--14 years, median age 31 months, weight range 3--57 kgs). Clinical diagnosis was: dilated cardiomyopathy in 50 patients; graft reject in 19; hypertrophic cardiomyopathy in 4; restrictive cardiomyopathy in 5; heart tumor in 3; ventricular arrhythmia in 3. The bioptome was introduced directly, without the use of a long sheath. There were no major complications; 2 patients experienced complete transient atrioventricular block and in 1 case right ventricular perforation occurred. In 11/45 patients (27%) with the clinical diagnosis of dilated cardiomyopathy and available myocardial specimens, acute myocarditis was diagnosed. In 47/65 procedures in the transplanted patients, a moderate to severe rejection was diagnosed. In the remaining patients, endomyocardial biopsy did not help the clinical diagnosis. We conclude that the right ventricular endomyocardial biopsy is a safe procedure in pediatric age; its utility is mostly limited to the diagnosis of acute myocarditis and graft reject after cardiac transplantation.

Adolescent↗
PubMedDate not supplied

The value of echocardiography in the early diagnosis of myocardial impairment due to connective tissue diseases.

The study comprised 14 patients with systemic lupus erythematosus (SLE) and 12 patients with diffuse scleroderma. Echocardiography ascertained 5 cases of congestive and 1 case of restrictive cardiomyopathy among the patients with SLE, as well as 6 cases with restrictive cardiomyopathy among those with scleroderma. The authors specify two important echocardiographic variables: the E-septum distance and the septal and wall motion after amyl-nitrite test, which reveal the intrinsic contractile properties of the myocardium, allowing an early diagnosis of myocardial impairment, as well as the selection of patients for an efficient treatment with vasodilator drugs.

Cardiomyopathy, Dilated↗
PubMed1997

Newer findings on a unified perspective of copper restriction and cardiomyopathy.

The cuproenzymes lysyl oxidase, cytochrome-c oxidase, and superoxide dismutase are key factors in understanding the cardiac hypertrophy and cardiomyopathy associated with dietary copper restriction. The role of copper in cardiac lipid and energy metabolism as a consequence of changes in some of these enzyme activities in comparison with what is known about normal cardiac substrate utilization is discussed here. While the decrease in the nuclear encoded subunits of cytochrome-c oxidase in hearts from copper-deficient rats is known, new evidence suggests that other factors, such as ATP synthase metabolism may be exerting an influence upon this observation. While this review focuses on newer knowledge about energy and fatty acid metabolism in copper deficiency, the extracellular matrix is considered as well. This complex interplay of extracellular and cellular events in copper restriction is outlined as a model for further studies of this unique model of concentric hypertrophy.

Animals↗
PubMed1978

Functional differentiation of various types of cardiomyopathies by echocardiography.

Echocardiography was used to compare the left ventricular function in 35 cases of congestive cardiomyopathy (COCM), 16 cases of obstructive and 28 cases of non-obstructive asymmetric septal hypertrophy, 43 cases of scleroderma heart disease, 21 cases of ischaemic cardiomyopathy (ICM), 2 cases of restrictive cardiomyopathy, and one case of cardiac amyloidosis. COCM was characterized by left ventricular dilatation, decreased contractility and signs of elevated end-diastolic pressure. In asymmetric septal hypertrophy there was a decreased distensibility of the obstructed left ventricle, both in early and end-diastole, but the pump function remained normal. In scleroderma and amyloid heart disease both the contractility and distensibility of the small, stiff heart were reduced. In restrictive cardiomyopathy the only abnormality was the impaired end-diastolic distensibility. Reduced contractility confined primarily to the interventricular septum and impaired early diastolic distensibility are the characteristic features distinguishing ischaemic cardiomyopathy from COCM. However, echocardiography is not suitable for individual differentiation of ICM and COCM. The COCM can be differentiated from left ventricular aneurysm by M-mode sector scanning technique.

Adolescent↗
PubMed1983

Atrial systolic notch on the interventricular septal echogram: an echocardiographic sign of constrictive pericarditis.

Interventricular septal motion during ventricular diastole was analyzed using M-mode echocardiography in 13 patients with constrictive pericarditis and 12 patients with restrictive cardiomyopathy. In seven of eight patients with constrictive pericarditis in sinus rhythm, an abnormal "atrial systolic" notch was observed consisting of abrupt initial posterior motion toward the left ventricle approximately at the middle of the P wave and subsequent anterior motion at the end of the P wave and termination before the R wave. This notch was absent during atrial premature beats that were recorded in two patients. The atrial systolic notch was not observed in any patient with restrictive cardiomyopathy. The septal notch in early ventricular diastole previously described in constrictive pericarditis was seen in 62% of patients with constrictive pericarditis and 25% of patients with restrictive cardiomyopathy. Thus, an abnormal atrial systolic notch may be an additional useful sign to differentiate constrictive pericarditis from restrictive cardiomyopathy. The mechanism may be related to transient late diastolic pressure gradients between both ventricles resulting from asynchrony of left and right atrial contractions.

Diastole↗
PubMed1996

[Restrictive myocardiopathy in children. A study of 4 patients].

Restrictive cardiomyopathy is an unusual form of cardiomyopathy during childhood. Only occasional cases or reduced populations have been reported in international journals. The purpose of this report is to document the clinical, echocardiographic and hemodynamic profile and outcome of restrictive cardiomyopathy in a group of four children (two boys and two girls). The mean age at admission was 3.5 +/- 2.7 years (range 1(3/12) to 7(10/12) years). The diagnosis was made by clinical, echocardiographic and hemodynamic criteria. The main symptoms were bronchial disease (two patients), hepatomegaly and a cardiac murmur. All of the patients had impaired growth and physical development, normal or near-normal Rx cardiothoracic ratio, evidence of congestive heart failure and only one of them showed a cardiac murmur. The most common ECG finding was biatrial enlargement and incomplete right-bundle branch block. The echocardiographic feature was severe biatrial dilatation in the presence of normal ventricular cavity size. Left ventricular shortening was normal and there was diastolic dysfunction. Doppler mitral flow was restrictive, with an E/A ration > 1.5. Marked elevation of left ventricular end-diastolic pressure was noted in cardiac catheterization (x = 32 +/- 7 mmHg). Right ventricular end-diastolic pressure was elevated, but less than the left side (x = 19 +/- 12 mmHg). The outcome was very poor. Two patients died, one remains with heart failure NYHA II and the other with heart failure NYHA IV. We conclude that restrictive cardiomyopathy during childhood may simulate a bronchial or liver disease, so we must bear this in mind in order to make the differential diagnosis. Because of the poor prognosis and lack of specific treatment, the best therapeutic option may be heart transplantation.

Cardiomyopathy, Restrictive↗
PubMed1984

The septal arteries in the differential diagnosis of constrictive pericarditis.

Angiographic study of the motion of the septal and left marginal arteries was performed in patients with restriction in ventricular diastolic filling in order to separate patients with constrictive pericarditis from those with restrictive cardiomyopathy. Twelve patients with constrictive pericarditis (group I) and 10 patients with restrictive cardiomyopathy (group II) were evaluated and compared with 21 patients with normal coronary angiograms (group III). The displacement of the septal arteries (23 +/- 2.04 mm) was abnormally exaggerated in group I and normal (9 +/- 0.81 mm) in groups II and III. The displacement of the left marginal arteries as seen by the "corrugating index" was similar in all groups. We conclude that study of the displacement of the septal arteries is a useful angiographic sign that helps to separate constrictive pericarditis from restrictive cardiomyopathy and normals.

Cardiomyopathies↗
PubMed1995

Constrictive pericarditis: its history and current status.

The diagnosis of constrictive pericarditis remains a challenge because it is often mimicked by restrictive cardiomyopathy. The last few years have seen numerous advances in our ability to differentiate between these two conditions which often have similar physical findings and hemodynamics. This review begins with a brief history of constrictive pericarditis; this is followed by an extensive discussion of newer etiologies, and then the classical clinical history and physical examination findings are described. Radiologic, electrocardiographic, and angiographic findings are discussed. The hemodynamics of constrictive pericarditis are reviewed. Recent results of echocardiographic and echo-Doppler investigations are presented. Emphasis is placed upon the limitations of M-mode echocardiography in the diagnosis of constrictive pericarditis. The value of echocardiographic Doppler studies of mitral and tricuspid flow velocity patterns, as well as of those in the pulmonary veins and hepatic veins, is described. Nuclear ventriculograms and angiocardiograms tend to show more rapid ventricular filling in constrictive pericarditis than in restrictive cardiomyopathy. Although only a small number of patients has been studied, these evaluations seem to have merit in separating restrictive cardiomyopathy from constrictive pericarditis. The role of computed tomography scanning and magnetic resonance imaging studies of pericardial thickness in confirming the presence of constrictive pericarditis is discussed. Abnormal pericardial thickening (> 3 mm) confirms the diagnosis of constrictive pericarditis, but only if the characteristic hemodynamic pattern is present. The usefulness of endomyocardial biopsy in recognizing specific varieties of restrictive cardiomyopathy is presented.(ABSTRACT TRUNCATED AT 250 WORDS)

Diagnosis, Differential↗
PubMed1986

MRI of the abnormal pericardium.

To evaluate the use of MRI in the diagnosis of pericardial disease, 63 patients with pericardial abnormalities or clinically suspected pericardial disease were studied retrospectively. Twenty-three patients had pericardial effusion, 19 patients had pericardial thickening, and 11 patients were referred for evaluation of masses with possible pericardial involvement. The other 10 patients were referred for differentiation of constrictive pericarditis from restrictive cardiomyopathy and eventually were found to have pericardial hematoma or normal pericardium as assessed by MRI. The calculated size of pericardial effusion by MRI showed a good correlation with semiquantitative echocardiographic estimations. MRI could demonstrate fibrinous adhesions in patients with uremic pericarditis. It was also of great value in the differential diagnosis of constrictive pericarditis vs restrictive cardiomyopathy. Pericardial thickness of more than 4 mm was found in patients with constrictive pericarditis. Normal pericardial thickness was demonstrated by MRI in the three patients with restrictive cardiomyopathy. MRI diagnosed hemopericardium correctly as the cause of constrictive symptoms in two patients. Pericardial thickening in patients after cardiac surgery was commonly found by MRI and usually was not associated with clinical signs of constrictive pericarditis. MRI proved to be useful in the diagnosis of pericardial cysts and in the evaluation of paracardiac masses with possible pericardial involvement. MRI is an important technique in the evaluation of the pericardium. It can provide important additional information when diagnosis cannot be made adequately by other noninvasive imaging techniques.

Adolescent↗
PubMed1998

[The echocardiographic examination in adult cardiomyopathy].

Echocardiography allows distinction between the diastolic dysfunction of hypertrophic or restrictive cardiomyopathies and the systolic dysfunction of dilated cardiomyopathy. The diagnosis and prognosis may be deduced from echocardiographic parameters. In hypertrophic cardiomyopathy systolic function is normal and there is asymmetric left ventricular hypertrophy (> 13 mm) associated with a reduced diastolic dimension and atrial dilatation resulting from diastolic dysfunction. The prognosis could be related to the severity of left ventricular hypertrophy; right ventricular hypertrophy is uncommon and its severity seems to be related to that of left ventricular hypertrophy. Restrictive cardiomyopathies are less common and amyloidosis is the commonest cause. Symmetric hypertrophy with reduced diastolic dimensions is observed; right ventricular involvement occurs in about 30% of cases. The prognosis seems to be related to the degree of parietal infiltration and, at advanced stages, systolic function is abnormal (fractional shortening < 20% with a left ventricular diastolic dimension > or = 55 mm) and rapidly fatal. Dilated cardiomyopathy is diagnosed when wall thickness is normal but left ventricular diastolic dimensions > 27 mm/m2 and ejection fraction < 45%. Right and left ventricular dimensions of the same size, left ventricular diastolic dimensions > 70 mm and left ventricular ejection fractions < 20% are poor prognostic indicators.

Adult↗
PubMed1999

[Heterotropic heart transplantation in young patients with elevated pulmonary vascular resistance].

UNLABELLED: Right heart failure due to elevated PVR is one of the major causes of mortality and morbidity after orthotopic heart transplantation. In 5 patients (median age 14 years) with dilated or restrictive cardiomyopathy and important elevation of the PVR, a heterotopic heart transplantation was performed using the technique reported by Yacoub (the donor pulmonary artery was implanted on the recipient right atrium). All the patients presented with at least two of the following parameters: PVR/m2 > 6 U, transpulmonary gradient > 15 mmHg, mean pulmonary pressure > 50 mmHg. One patient with restrictive cardiomyopathy died three months after transplantation of severe failure of the native right ventricle. The other four patients, with a mean follow-up of 29 months, are in good clinical and hemodynamic condition and later post-operative catheterizations showed a progressive reduction of the pulmonary pressure. CONCLUSIONS: Our experience suggests that this type of heterotopic heart transplantation can be performed successfully in patients with secondary pulmonary hypertension. Particular attention should be paid to patients with restrictive cardiomyopathy and important right ventricle dysfunction, in which a complete heterotopic heart transplantation could be a better solution.

Adolescent↗
PubMed1980

Primary cardiac amyloidosis: A case presentation.

A young man with primary cardiac amyloidosis presented clinically as a case of 'restrictive' cardiomyopathy with an initial diagnosis of constrictive pericarditis, probably of tuberculous origin. Cardiac catheterization showed features compatible with restrictive cardiomyopathy or constrictive pericarditis but pericardial biopsy was negative. Percutaneous right ventricular endomyocardial biopsies established amyloid infiltration of the myocardium, but rectal, tongue and liver biopsies were all negative for amyloidosis. Bence-Jones protein was absent from the urine. Serum electrophoresis, immunophoresis, and bone marrow examination all failed to detect possible multiple myelomatosis as a cause of secondary amyloidosis. Respiratory function tests showed a restrictive picture, probably secondary to chronic congestive cardiac failure. Thus, this case is the second example of primary cardiac amyloidosis in the world literature which has been dianosed by right ventricular endomyocardial biopsy. Phonocardiographic features of early aortic valve closure are reported for the first time in amyloid restrictive cardiomyopathy. Since tuberculous constrictive pericarditis is an important cause of cardiac disease in this country, with effective therapy, the authors stress more frequent use of endomyocardial biopsy as a safe diagnostic tool to exclude other similar disease states. The patient was offered the possibility of a cardiac transplantation in view of the localized nature of his disease, but refused. He now continues to respond unsatisfactorily to antifailure therapy with, presumably, a poor prognosis.

Adult↗
PubMed1997

[Cardiac amyloidosis. A disease with many faces and different prognosis].

PURPOSE: To identify the principal forms of cardiac amiloydosis presentation in a terciary hospital. METHODS: Eight cases with cardiac amyloidosis were identified. Five were women, their ages ranged from 23 to 83 years (mean 62). After a medical history and clinical examination the patients were submitted to complementary tests: electrocardiogram (EKG), echocardiogram (ECHO), scintigraphy with technecium pirophosphate and cardiac biopsy these results allowed the identification of their clinical situation. RESULTS: Seven patients referred dyspnea, 6 were in heart failure, 1 patient had syncope. The EKG identified complete atrioventricular (AV) block in 4 patients, and antero septal inactive area in the other 4. The ECHO showed normal cardiac diameter in all (mean left ventricular diastolic diameter of 46.8) and slight reduction of left ventricular ejection fraction; hypertrophy of the left ventricular septal and posterior walls in all cases, in 7 cases there was a hyper refractile granular sparkling ECHO. Two different groups were identified: one with complete AV block and the second with restrictive cardiomyopathy. The prognosis was different in these two groups. Those with complete AV block evolved better after pacemaker implantation and those with restrictive cardiomyopathy had refractory heart failure and 3 of them died. CONCLUSION: The increased free wall and septal thickness, the slight systolic dysfunction and the infiltration aspect at ECHO allow us to identify the great majority of the cases. Those patients with restrictive cardiomyopathy evolve with refractory heart failure and most of them die in a few months.

Adult↗
PubMed2005

[Diagnosis difficulty in occult constrictive pericarditis].

We report the case of a 42 years woman known to have a cardiac heart failure attributed to restrictive cardiomyopathy for want of any other plausible diagnosis. Evolution and repeted investigations finally permitted to rectify the diagnosis by revealing a constrictive pericarditis, remained occult 9 years during. The differentiation of restrictive cardiomyopathy and constrictive pericarditis has been a perennial problem in clinical cardiology. Diagnosis of constrictive pericarditis is based on associated signs sometimes too poor to go straight to thoracotomy. We discuss the mean to approach more precisely this uncommon pattern named occult constrictive pericarditis.

Adult↗
PubMed1991

Fatty infiltration: another restrictive cardiomyopathic pattern.

Restrictive cardiomyopathies have been shown to occur as result of infiltrative processes from a variety of sources. The current report describes an obese male, who was found to have hemodynamic evidence of a restrictive cardiac process. His pericardium was proven to be normal and an incisional biopsy obtained of the myocardium during coronary artery bypass surgery demonstrated histologic evidence of fatty infiltration of myocardium. Review of the restrictive and pathology literature is discussed and indicates that this is the first report to demonstrate the association between fatty infiltration and hemodynamic findings consistent with a restrictive cardiomyopathy.

Adipose Tissue↗