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PubMed2006

The systolic to diastolic duration ratio in children with heart failure secondary to restrictive cardiomyopathy.

Systole (S) and diastole (D) are fundamental to cardiac function, yet their durations are not routinely evaluated. We defined the S/D duration ratio in 11 children with restrictive cardiomyopathy (CM) and 31 control subjects, using tricuspid regurgitant flow duration. We correlated S/D ratio, S duration, and D duration with heart rate and compared groups. Heart rates of control subjects and patients with restrictive CM were similar (P = .995). The S/D ratio was higher in patients with restrictive CM than in control subjects (1.6 +/- 0.81 vs 0.8 +/- 0.19, P < .001) as a result of shortened D (0.43 +/- 0.13 vs 0.56 +/- 0.06, P < .01) and prolonged S (0.58 +/- 0.12 vs 0.44 +/- 0.06, P < .01). The S/D ratio was highly correlated to heart rate in restrictive CM (r = 0.95, P < .0001). The high S/D ratio found in restrictive CM, found also in dilated CM, suggests that this index is generic to heart failure rather than to a specific disease. The S/D ratio is easily measured using Doppler flow, enhancing echocardiographic assessment of D function in children.

Cardiomyopathy, Restrictive↗
PubMed1977

Primary restrictive cardiomyopathy. Non-tropical endomyocardial fibrosis and hypereosinophilic heart disease.

Eleven patients with a restrictive cardiomyopathy are described. Seven of these had biventricular disease and in them the haemodynamic abnormality resembled that in constrictive pericarditis but the condition was distinguishable because of unequal involvement of the two ventricles, murmurs of atrioventricular valve regurgitation, or characteristic ventricular deformity on angiography. Two of these patients had eosinophilia with the clinical features of Löffer's endocarditis. In 4 patients the disease was apparently confined to the left ventricle; they were investigated on account of atypical chest pain, third heart sound, or abnormal electrocardiogram. Simultaneous measurements of left ventricular pressure and volume throughout diastole were made in 3 patients and showed rapid but abruptly curtailed left ventricular filling. Transvenous endomyocardial biopsy in 2 patients showed fibrous tissue with collagen and irregular elastic fibrils. Surgical biopsy in 3 patients excluded pericardial constriction but was diagnostically unhelpful because of failure to obtain endocardium. Necropsy in one patient showed that the heart had features indistinguishable from tropical endomyocardial fibrosis. It is suggested that the spectrum of ventricular abnormalities in these patients results from endomyocardial fibrosis of varying severity and probably of differing cause. This study has shown that "tropical" endomyocardial fibrosis may occur outside the tropics and suggests that eosinophilia may play a leading role or associated part in the genesis of some cases.

Adult↗
PubMed1978

Restrictive cardiomyopathy in an infant with massive biatrial enlargement and normal ventricular size and pump function.

Restrictive cardiomyopathy in an infant with massive biatrial enlargement and normal ventricular size has been reported only once previously [3]. We now present a 13-month-old patient with severe and progressive congestive heart failure who demonstrated normal ventricular cavity size with biatrial enlargement. Ejection fraction and echocardiographic ventricular function studies were normal.

Adolescent↗
PubMed1990

Doppler echocardiography in dilated and restrictive cardiomyopathies.

Dilated cardiomyopathy is characterized by systolic dysfunction and cardiac enlargement of unknown origin. Various Doppler modalities are useful to detect and quantitate atrioventricular regurgitation, which is common and contributes to clinical symptoms. Pulsed Doppler assessment of mitral and tricuspid inflow velocities shows a spectrum of findings indicative of abnormal diastolic function and hemodynamic status. When mitral regurgitation is more than moderate and heart failure is severe, the ratio between early inflow E wave to atrial inflow A wave peak velocities is increased. Mitral deceleration time may be short. When mitral regurgitation is trivial and left atrial pressure is not increased, abnormal relaxation may be detected as a low E:A ratio. Mitral deceleration time and isovolumic relaxation time are prolonged. In restrictive cardiomyopathy, there is an abrupt limitation in early ventricular filling due to abnormal compliance of endocardial or endomyocardial origin. Mitral and tricuspid inflow velocities show normal to increased early peak velocity, rapid deceleration time, low peak atrial velocity, and an increased E:A ratio. Differentiation between restriction and constriction might be possible by the demonstration in pericardial constriction of inspiratory decreases in mitral early inflow peak velocities and in prolongation of isovolumic relaxation time, with reciprocal changes on tricuspid inflow velocity profiles. In constriction, these respiratory variations are caused by the ventricular limitation to accommodate changes in venous return due to the pericardial shell. Doppler abnormalities and two-dimensional echocardiographic assessment of ventricular and atrial size and ejection fraction provide the practicing physician with valuable diagnostic information.

Cardiomyopathy, Dilated↗
PubMed1983

Cardiac ultrastructure in primary restrictive cardiomyopathy.

A five-year clinical follow-up and the results of myocardial biopsies are described in a patient with primary restrictive cardiomyopathy. Histologic examination of a right ventricular endomyocardial biopsy taken early in the course of the illness was not contributory. Histologic examination of a left ventricular endomyocardial biopsy five years later showed hypertrophy and disarray of myocytes, thickening of the endocardium, and interstitial fibrosis. Connective tissue was compact and regularly oriented in the endocardium, but tangled and irregularly oriented in the interstitium. It is concluded that the irregular network of collagen fibrils and elastic fibers limits diastolic relaxation and prevents ventricular dilatation; that the coexisting hypertrophy results from an attempt to maintain normal pump function; and that the myocyte disarray is a consequence of abnormal mechanical forces generated under conditions of severe fibrosis.

Biopsy↗
PubMed1988

The surgical treatment of restrictive cardiomyopathy in pseudoxanthoma elasticum.

A patient with pseudoxanthoma elasticum presented in pulmonary oedema with restrictive left ventricular cardiomyopathy caused by calcified endocardial bands that were confirmed on echocardiography and at catheterisation. The bands were resected as far as possible and the involved mitral valve was replaced by a heterograft. A year later calcification of the heterograft forced its replacement by a St Jude prosthesis. Relief of symptoms has been good in the medium term.

Adult↗
PubMed1986

Ultrastructural aspects of endomyocardial biopsy in restrictive cardiomyopathy: evaluation of elastogenesis.

Besides elastic and collagen fibres, numerous smooth muscle (SM) cells were found in the thickened endocardium in 3 cases of restrictive cardiomyopathy and in 1 case of congenital endocardial fibroelastosis. In 3 other cases of congenital endocardial fibroelastosis, fibroblast-like cells were present besides numerous fibres. The former seemed to originate from SM cells. The authors present their opinion that both in congenital and acquired endocardial fibroelastosis, the lesion develops essentially in the same way. The basic phenomenon consists in increased elastoplastic activity of SM cells. In contrast, the aetiology (stimulus) of higher, elastogenous activity of SM cells remains obscure and seems to be different in individual cases.

Adolescent↗
PubMed1980

Restrictive cardiomyopathy in pseudoxanthoma elasticum.

A unique case of severe and diffuse endocardial fibroelastosis covering the entire left ventricle and associated with pseudoxanthoma elasticum is presented. The clinical picture was that of an apparently primary restrictive cardiomyopathy. Specific histologic lesions could be recognized in the endocardium.

Adult↗
PubMed1983

[Diagnosis of restrictive cardiomyopathy in the hypereosinophilia syndrome by 2-dimensional echocardiography].

The cardiac manifestation of the hypereosinophilic syndrome was diagnosed by means of echocardiography in a 36-year-old man. The following two-dimensional echocardiographic characteristics indicated a restrictive cardiomyopathy in this patient: Enlargement of the left atrium. Thrombotic obliteration of the left ventricle from the apex up to the subvalvular region. Embedding of the chordae tendineae and papillary muscles by the thrombotic mass. Normal left ventricular ejection fraction. The echocardiographic findings were verified by autopsy.

Adult↗
PubMed2002

[A case of familial restrictive cardiomyopathy].

According to estimates of WHO experts cases with diagnosed cardiomyopathy account for 40-60 per 100,000. Restrictive cardiopathy (RCP) is encountered in 5% of all the diagnosed cases of cardiomyopathy. Two patients (a mother and her daughter) with suspected of family RCP were examined using ECG, Holter ECG monitoring, echo-CG, histological tests, x-ray, blood biochemical tests. Echo-CG was most informative for verification of RCP diagnosis. The daughter had edema, enlarged liver, arterial hypertension, cardiac arrhythmia. The mother had arrhythmia, dyslipidemia. Based on the above symptoms, the patients received combined drug therapy with positive results.

Adult↗
PubMed1990

Long-term follow-up of a child with idiopathic restrictive cardiomyopathy.

A ten-year-old male with palpebral edema was proved to have a restrictive hemodynamic pattern by cardiac catheterization. Sequential echocardiographic examination revealed uniformly depressed left ventricular diastolic properties, well-maintained left ventricular systolic function, and a progression of obliterative change of the left ventricular cavity. Myocardial biopsy at 17 years of age disclosed a normal endocardium and marked interstitial fibrosis of the myocardium. Consequently, a decrease of cardiac output and a marked biatrial enlargement have developed in association with persistent atrial fibrillation.

Adolescent↗
PubMed1987

Restrictive cardiomyopathy caused by adipositas cordis.

Adipositas cordis is a very rare disorder, characterized by fatty infiltration of myofibers. It has been related to dilated or hypertrophic cardiomyopathy and obesity. In this report, however, we present a non-obese patient, presenting with a restrictive syndrome and showing massive infiltration of adipocytes in the interventricular septum, proving adipositas cordis.

Cardiomyopathies↗
PubMed2004

[Restrictive cardiomyopathy as a manifestation of primary amyloidosis].

Primary amyloidosis is a rare disease, cardiac involvement occurs in up to 40% of patients. Diffuse amyloid deposits cause an impairment of myocardial systolic and diastolic function. In this paper we are presenting a case of a 54-year-old woman. The woman was admitted because of progressive fatigue, dyspnoea, chest pain, later she experienced hypotension, dyspepsia, and enterorrhagia. ECG showed decrease in QRS amplitude. We have found an echocardiographic evidence of wall hypertrophy. Right cardiac catheterization showed a restrictive situation. Immunobinding of serum and urine revealed monoclonal kappa light chains. The diagnosis was determined by rectal biopsy. Unfortunately, amyloid deposits caused progressive heart failure, hemorrhage, and death just before the diagnosis of primary amyloidosis could be determined on the basis of results of the immunofixations of serum and urine proteins (detection of the monoclonal light chains kappa) and from biopsy specimens taken from rectum (amyloid deposits).

Amyloidosis↗
PubMed2001

The in vivo role of p38 MAP kinases in cardiac remodeling and restrictive cardiomyopathy.

Stress-induced mitogen-activated protein kinase (MAP) p38 is activated in various forms of heart failure, yet its effects on the intact heart remain to be established. Targeted activation of p38 MAP kinase in ventricular myocytes was achieved in vivo by using a gene-switch transgenic strategy with activated mutants of upstream kinases MKK3bE and MKK6bE. Transgene expression resulted in significant induction of p38 kinase activity and premature death at 7-9 weeks. Both groups of transgenic hearts exhibited marked interstitial fibrosis and expression of fetal marker genes characteristic of cardiac failure, but no significant hypertrophy at the organ level. Echocardiographic and pressure-volume analyses revealed a similar extent of systolic contractile depression and restrictive diastolic abnormalities related to markedly increased passive chamber stiffness. However, MKK3bE-expressing hearts had increased end-systolic chamber volumes and a thinned ventricular wall, associated with heterogeneous myocyte atrophy, whereas MKK6bE hearts had reduced end-diastolic ventricular cavity size, a modest increase in myocyte size, and no significant myocyte atrophy. These data provide in vivo evidence for a negative inotropic and restrictive diastolic effect from p38 MAP kinase activation in ventricular myocytes and reveal specific roles of p38 pathway in the development of ventricular end-systolic remodeling.

Animals↗
PubMed1978

Angiocardiographic differentiation of constrictive pericarditis and restrictive cardiomyopathy due to amyloidosis.

The cineangiocardiograms and coronary angiograms of two cases of amyloidosis of the heart were compared to six cases of constrictive pericarditis. Three angiographic differentiating points were seen: (1) right ventricular free wall motion showed diastolic restriction in both disorders, whereas the crista supraventricularis, which moved normally in constrictive pericarditis, demonstrated restriction in amyloidosis; (2) ventricles in cases of constrictive pericarditis showed subtle further expansion during atrial systole after initial rapid filling (atrial kick), while in both cases of amyloidosis there was no motion during atrial systole; (3) pericardial thickening in constrictive pericarditis was demonstrated by failure of the distal coronary arteries to reach the surface of the cardiac image. In amyloidosis, the distal coronary arteries normally reached the periphery of the image. All three signs may be useful in differentiation, but the first is the easiest to evaluate. The right anterior oblique or posteroanterior view is the recommended projection.

Amyloidosis↗