1/14. A fatal case of idiopathic restrictive cardiomyopathy.We describe the clinical features of idiopathic restrictive cardiomyopathy in a female infant. A marked elevation of left ventricular end-diastolic pressure, and profoundly abnormal myocardial relaxation, were detected with the use of Doppler blood flow echocardiography, coupled with the relatively new technique of Doppler tissue echocardiography. There was no clinical evidence of ongoing heart failure, but she had signs of myocardial ischaemia, and unfortunately died suddenly at the age of 13 months.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
2/14. 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.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
3/14. Interpretation of cardiac pathophysiology from pressure waveform analysis: pericardial compressive hemodynamics, Part III.Failure of right atrial pressure to normalize after pericardial pressure is relieved suggests persistent effusive-constrictive physiology, myocardial failure, or cardiomyopathy which may have indistinguishable hemodynamic pressure waveforms. Clinical characteristics, ancillary testing, and endomyocardial biopsy may be required to obtain a definite diagnosis in such cases. The hemodynamic waveforms obtained during pericardiocentesis provide insight into the pathophysiologic processes, producing symptoms of dyspnea in this interesting patient group.- - - - - - - - - - ranking = 7keywords = pressure (Clic here for more details about this article) |
4/14. Heterotopic heart transplant combined with postoperative Sildenafil use for the treatment of restrictive cardiomyopathy.We report successful management of a 22-month-old child with restrictive cardiomyopathy and severe pulmonary hypertension using the heterotopic heart transplant technique. Additional lessons learned from postoperative management, including the novel use of Sildenafil (Viagra, Pfizer, NY) for controlling pulmonary arterial pressure are described.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
5/14. Progressive restrictive cardiomyopathy--a case report.We present a case of restrictive cardiomyopathy which progressed over a 10 month period. A 69-year-old female was admitted because of acute inferior myocardial infarction; hemodynamically, she was in Forrester subset I. cardiac catheterization performed 4 weeks post-infarction showed markedly increased left ventricular end-diastolic and pulmonary wedge pressures. Left ventricular ejection fraction was 66% with postero-basal akinesis and minimal mitral regurgitation. The right coronary artery was completely occluded with good collateral circulation from the intact left coronary artery. Doppler echocardiography 4 weeks post-infarction showed pseudo-normalization of the A/E ratio of peak mitral flow velocity in atrial systole (A) to peak mitral flow velocity in early diastole (E). Preload reduction by nitroglycerin increased the A wave. Ten months post-infarction, the patient was re-admitted due to congestive heart failure. The A/E ratio was unchanged, however the A wave no longer increased after the same dose of nitroglycerin. We have hypothesized that dehydration and/or the vigorous use of a nitrate, in the acute phase of myocardial infarction, masked an underlying restrictive cardiomyopathy which progressed in the 10 months post-infarction.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
6/14. An autopsy case of cardiomyopathy with restrictive physiology in a child.An autopsy case of cardiomyopathy with restrictive physiology associated with subendocardial myocardial necrosis and fibrosis in a seven-year-old child is described. cardiac catheterization showed high left ventricular end-diastolic pressure with a dip-and-plateau pattern. Macroscopically, marked dilatation with fibroelastosis in both atria and mild dilatation in both ventricles were observed. Histologic examination revealed extensive subendocardial necrosis with marked disorganization of myocardial cells and moderate arteriolosclerosis. We speculate that these extensive myocardial lesions, which contributed to the restrictive hemodynamic changes, could have been induced by anoxia or a Ca2 transport abnormality of unknown cause.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
7/14. Analysis of left ventricular diastolic function by the pressure-volume relation in cases with idiopathic restrictive cardiomyopathy and amyloid restrictive cardiomyopathy.To assess left ventricular (LV) diastolic function in idiopathic restrictive cardiomyopathy (IRCM) and amyloid restrictive cardiomyopathy (ARCM), we analyzed LV cineangiograms and pressures (tip manometer) in two patients, one with IRCM and the other with ARCM. LV volume and instantaneous rate of change of volume were derived from frame-by-frame analyses of angiograms. LV relaxation was assessed by the time constant of isovolumic pressure decay. LV compliance was evaluated by the pressure-volume relationship. From these analyses, we conclude as follows: (1) In restrictive cardiomyopathy (RCM), LV diastolic dysfunction may be characterized by impaired relaxation and decreased compliance; (2) LV diastolic filling was minimally impaired or normal in both conditions; (3) The ventricular diastolic pressure wave form of "dip and plateau" seemed not to be a characteristic sign in RCM.- - - - - - - - - - ranking = 8keywords = pressure (Clic here for more details about this article) |
8/14. Restrictive cardiomyopathy following acute myocarditis--a case report.A fifty-five-year-old man developed intractable heart failure four weeks after upper respiratory infection. His central venous pressure was as high as 300 mmH2O. Two-dimensional echocardiogram revealed no muscular hypertrophy of the ventricles, no pericardial thickness, and no pericardial effusion. On catheterization, hemodynamic data were compatible with restrictive cardiomyopathy. Transvenous endomyocardial biopsy of the left ventricle was performed. Histologic examination strongly suggested that cardiomyopathy developed after acute myocarditis.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
9/14. 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.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
10/14. Restrictive cardiomyopathy in children. Ultrastructural findings.Restrictive cardiomyopathy is usually related to fibrosis of the endocardium or to an infiltrative disorder. However, in few cases, it can be due to isolated pathology of the myocytes but such alterations are not well characterized. This paper reports the disease in two 7 year old patients. There was severe venous congestion and catheterisation revealed increased end diastolic pressure in the ventricles. Both pericardial and myocardial biopsies were performed, as the clinical and haemodynamic data were indistinguishable from constrictive pericarditis. The structure of the pericardium was normal. The endocardium was not thickened. The interstitium of the myocardial tissue was not increased. Electron microscopic examination revealed intracellular masses of disorganized myofilaments. These large deposits may have produced decrease compliance of the myocytes and of the ventricular walls.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
| | Next -> |