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PubMed2002

[Echocardiographic evaluation of left atrial function in dilated cardiomyopathy with restricted and non-restricted Doppler transmitral flow].

UNLABELLED: Left atrial (LA) function is of great importance in left ventricular (LV) filling. There is evidence that echocardiographic Doppler evaluation of transmitral flow, routinely used for LV filling estimation, is dependent on LA function. Information regarding the relation of LA size and function to transmitral flow in heart failure is limited. We used 2D echocardiographic acoustic quantification methods to assess LA function in patients with dilated, (non-ischemic) cardiomyopathy (DCM) and a control group. The DCM group was divided into 2 subgroups: Group 1-with restrictive LV Doppler filling pattern-18 patients (DCM-R) and Group 2-with non-restrictive LV Doppler filling pattern-11 patients (DCM-NR) with similar heart rate, age and degree of mitral regurgitation. LA maximal area, total emptying fraction and absolute and fractional area change during rapid emptying and atrial contraction were calculated. The LA was enlarged only in DCM-R. Both DCM groups had decreased total emptying fractions and rapid emptying area changes compared to controls. An enlarged LA area and more decreased total emptying were found in DCM-R with high LV filling pressures compared to DCM-NR. The restrictive group had a significantly smaller LA rapid emptying area change, as well as a smaller LA area change and emptying fraction during atrial contraction compared to DCM-NR. Within < or = 2 hrs after the echocardiography study, cardiac catheterization was performed in the DCM group. We found significantly higher LV filling pressures and lower LV ejection fractions in DCM-R compared to DCM-NR. Significant correlations were found between LA function and invasive parameters like capillary wedge and LV enddiastolic pressures and LV EF. CONCLUSION: Patients with DCM-R had significantly enlarged LA areas with more depressed total emptying fractions and smaller LA area changes during contraction compared to DCM-NR. Thus, left atrial function plays an important role in LV filling and its dysfunction can be a marker of poor prognosis.

Adult↗
PubMed2000

Constrictive pericarditis post allogeneic bone marrow transplant for Philadelphia-positive acute lymphoblastic leukaemia.

We describe two cases of severe constrictive pericarditis arising after allogeneic BMT conditioning involving total body irradiation and melphalan to treat Philadelphia-chromosome positive ALL. Both patients required pericardectomy, resulting in marked improvement in ventricular filling. However, a degree of right-sided cardiac failure persisted in both patients secondary to restrictive cardiomyopathy. Constrictive pericarditis has not been previously described after BMT, but has been observed following thoracic radiotherapy for malignancy, usually involving a substantially higher radiation dose. Pericardial constriction and restrictive cardiomyopathy should be considered as causes of breathlessness and/or oedema occurring late after BMT. Bone Marrow Transplantation (2000) 25, 571-573.

Adult↗
PubMed2002

Diagnostic value of mitral annular velocity for constrictive pericarditis in the absence of respiratory variation in mitral inflow velocity.

Respiratory variation of 25% or more in transmitral early diastolic filling (E) velocity is a well-recognized diagnostic feature of constrictive pericarditis (CP) that is useful for distinguishing it from restrictive cardiomyopathy. However, a subset of patients with CP do not exhibit the typical respiratory change. Recent data showed that mitral annular (E') velocity measured by Doppler tissue echocardiography (DTE) is markedly reduced in patients with restrictive cardiomyopathy whereas E' velocity is well-preserved in CP. This study evaluated the role of DTE for the diagnosis of CP when there is no characteristic respiratory variation of E velocity. From September 1999 to March 2001, 19 patients (17 men, 2 women; mean age, 57 +/- 13 years) with surgically confirmed CP underwent comprehensive echocardiography preoperatively, including pulsed wave and DTE examination with simultaneous recording of respiration. Nine (47%) of the 19 patients had less than 25% respiratory variation in E velocity. There was no significant difference in mitral inflow peak velocity, deceleration time, early-to-late ventricular filling ratio, and E' velocity between patients with and patients without respiratory variation of E velocity of 25% or more. Regardless of the presence or absence of a significant respiratory variation of E velocity, E' velocity was relatively normal (mean, 12 +/- 4 cm/s) in all patients with CP. In conclusion, E' velocity is well preserved in patients with isolated CP even when there is no characteristic respiratory variation of E velocity. Thus, when the respiratory variation in Doppler E velocity is blunted or absent during the evaluation of suspected CP in patients with restrictive mitral inflow velocity, preserved E' velocity shown by DTE should support the diagnosis of CP over a primary myocardial disease.

Adult↗
PubMed2002

Long-term follow-up after total lymphoid irradiation in pediatric heart transplant recipients.

BACKGROUND: Total lymphoid irradiation (TLI) is used to treat recurrent allograft rejection. Short-term success and complication rates have been reported in pediatric and adult cardiac transplant populations. We report the long-term efficacy and safety of TLI in treating intractable rejection in pediatric patients. METHODS: Eight pediatric patients were treated with TLI (7 for recurrent rejection, 1 for risk of medication non-compliance). Therapy consisted of a mid-plane dose of 8 Gy administered with a 6-MeV linear accelerator using an anterior-posterior opposed technique. We reviewed outcomes for a total of 40 patient-years of follow-up. RESULTS: We encountered rejection (>Grade 2 by International Society for Heart and Lung Transplantation criteria) in 56.7% +/- 34.7% of biopsies performed within 90 days before TLI. Rejection rates dropped to 3.1% +/- 8.8% within the first 90 days (p < 0.005) after therapy and remained low at 5.6% +/- 1.3% (p < 0.05) during the first year after completion of TLI. Median time from TLI to the first subsequent rejection episode was 305 days (range, 77-1,920 days). Long-term follow-up (>3 years) of 5 patients demonstrated a continuing low incidence of rejection. Non-Hodgkin's lymphoma was diagnosed in 1 of 8 patients, graft coronary artery disease in 4 of 8 patients, and restrictive cardiomyopathy in 1 of 8 patients after TLI. CONCLUSIONS: Total lymphoid irradiation is an effective treatment for recurrent rejection and has short- and long-term efficacy. Morbid events may include cancer, graft coronary artery disease, and restrictive cardiomyopathy.

Adolescent↗
PubMed2005

Regressive aortic valve and infundibular tumors during idiopathic hypereosinophilic syndrome.

Fibroblastic endocarditis is a classic complication of prolonged hypereosinophilic syndrome, whatever the cause. In France, it is most frequently encountered in cases of idiopathic hypereosinophilic syndrome. It commonly involves the apex of the ventricles, with a clinical picture of restrictive cardiomyopathy of unfavorable prognosis, and the auriculoventricular valves. We report the case of a 77-year-old man in whom atypical cardiac involvement disclosed idiopathic hypereosinophilic syndrome. In addition to the usual features of obliteration of the apex and restrictive cardiomyopathy, echocardiographic examination showed severe left ventricular dysfunction and intracardiac tumors, one of which was unusually localized to an aortic valve. Treatment, which comprised strict control of the eosinophilic process, standard treatment for cardiac failure, and anticoagulation therapy, produced rapid and long-lasting improvement of his clinical status and left systolic and diastolic ventricular function, and on echocardiography the intracardiac tumors had totally disappeared. The patient suddenly died of septic shock 16 months after first being seen.

Aged↗
PubMed1993

[Obstructive cardiomyopathy, caused by amyloidosis, associated with bone marrow myeloma].

A case of a 40-year-old female is reported. Her symptoms started with abdominal complaints, loss of weight, general weakness, then gradually polyneuropathy, worsening hypotonia and systolic heart murmur developed. The bone marrow showed 20-25% infiltration by myeloma. An IgG lambda type paraproteinaemia was diagnosed using immunelectrophoresis. The ejection systolic murmur was caused by restrictive cardiomyopathy--characteristic of primary amyloidosis--with obstruction of the outflow tract of the left ventricle. Echocardiographic changes took 11 months to develop. The patient's status became dominated by severe hypotonia and she died as a result of heart failure. The post mortem examination confirmed amyloidosis with myeloma. Histopathological examination of the myocardium, coronary and other arteries revealed severe changes with AL-type amyloid deposits. The case demonstrates that amyloidosis can lead not only to restrictive cardiomyopathy but obstruction of the outflow tract of the left ventricle.

Adult↗
PubMed2004

Mitral valve repair in idiopathic hypereosinophilic syndrome.

Idiopathic hypereosinophilic syndrome is a rare systemic disease than can cause multiple organ failure by eosinophilic infiltration. Cardiac involvement is characterized by endocardial fibrosis and overlying thrombus, leading to restrictive cardiomyopathy and valvular dysfunction. Surgical experience of patients with mitral dysfunction caused by this syndrome is limited, and valvular replacement is most often performed. Mechanical valvular replacement has a high incidence of recurrent obstructive thrombosis, and replacement with a bioprosthesis is recommended, despite associated restrictive cardiomyopathy. A patient is described who presented with mitral insufficiency associated with idiopathic hypereosinophilic syndrome, and underwent mitral valve repair.

Aged↗
PubMedDate not supplied

Mitral valve replacement in idiopathic eosinophilic endocarditis without peripheral eosinophilia.

Idiopathic hypereosinophilic syndrome (IHS) is a rare systemic disease than can cause multiple organ failure by eosinophilic infiltration. Cardiac involvement is characterized by endocardial fibrosis and overlying thrombus leading to restrictive cardiomyopathy and valvular dysfunction. The absence of peripheral eosinophilia does not exclude eosinophilic cardiac involvement. Surgical experience of patients with mitral dysfunction caused by this syndrome is limited and valvular replacement is most often performed. Mechanical valvular replacement has a high incidence of recurrent obstructive thrombosis and replacement by bioprosthesis is recommended despite associated restrictive cardiomyopathy. This report describes a patient who presented mitral insufficiency caused by eosinophilic endocarditis without peripheral eosinophilia who underwent mitral valve replacement.

Bioprosthesis↗
PubMed1999

[Hereditary amyloid cardiomyopathy related to a mutation at transthyretin protein number 111. A clinical, genetic and echocardiographic study of an affected Danish family].

Amyloidosis is a group of diseases characterized by amyloid deposition in various tissues. The diseases can roughly be divided into hereditary and non-hereditary forms. The hereditary forms are related to a mutation in the serum protein transthyretin which is produced mainly in the liver. The inheritance is autosomal dominant. A family in Denmark has earlier been described as having inherited cardiac amyloidosis with a mutation at amino acid number 111 in the transthyretin protein. The family now has been re-examined because of new diagnostic and therapeutic possibilities. The aims of the study were to identify carriers and non-carriers of the mutant transthyretin methionine 111 linked familial amyloid disease, to detect early signs of the restrictive cardiomyopathy and other clinical manifestations of this disease. Clinical, echocardiographic and genetic examination was carried out. Out of 125 living family members, 99 were available for examination. Twenty-five persons were heterozygous carriers of the mutant transthyretin methionine 111 genotype, while 74 were non-carriers. Eight carriers, all above the age of 35, showed echocardiographic abnormalities suggestive of developing or manifest restrictive cardiomyopathy. Nine carriers had carpal tunnel syndrome as opposed to none of the non-carriers. It is concluded that for early detection of familial amyloid cardiomyopathy, echocardiography is the investigation of choice. The first sign is diastolic dysfunction detected as an abnormal relaxation pattern. Carpal tunnel syndrome appears to be the earliest presenting clinical symptom. Early liver transplantation seems to be curative.

Adolescent↗
PubMed2004

Echocardiographic features of atrial myocarditis with giant cells: a case report.

We report a case of restrictive cardiomyopathy in which a distinct endothelial thickening of the atrial wall and pulmonary vein orifices was noted on transesophageal echocardiography. Echocardiographically guided endomyocardial biopsy of the thickening revealed an inflammatory infiltrate that was rich in giant cells and provided important clues about an underlying immune mechanism for the pathogenesis. Positive results from the antineutrophil cytoplasmic autoantibody assay supported the diagnosis of Wegener's granulomatosis. After immunosuppressive therapy, the endomyocardial thickening completely resolved, but the restrictive cardiomyopathy did not reverse.

Echocardiography, Transesophageal↗
PubMed1999

Quantification of left ventricular diastolic wall motion by Doppler tissue imaging in healthy cats and cats with cardiomyopathy.

OBJECTIVE: To assess Doppler tissue imaging (DTI) for evaluating left ventricular diastolic wall motion in healthy cats and cats with cardiomyopathy. ANIMALS: 20 healthy cats, 9 cats with hypertrophic cardiomyopathy (HCM), and 9 cats with unclassified cardiomyopathy (UCM). PROCEDURE: A pulsed wave DTI sample gate was positioned at a subendocardial region of the left ventricular free wall in the short axis view and at the lateral mitral annulus in the apical 4-chamber view. Indices of diastolic wall motion were measured, including peak diastolic velocity (PDV), mean rate of acceleration and deceleration of the maximal diastolic waveform (MDWaccel and MDWdecel, respectively), and isovolumetric relaxation time (IVRT). RESULTS: The PDV of cats with HCM and 6 of 9 cats with UCM was significantly decreased, compared with that of healthy cats. In the 3 cats with UCM that had a PDV that was not different from healthy cats, MDWaccel and MDWdecel were greater, and IVRT was shorter than those of healthy cats. The IVRT in cats with HCM was longer than that of other cats. CONCLUSIONS AND CLINICAL RELEVANCE: Indices of diastolic function in cats with HCM, and in many cats with UCM, differed from those of healthy cats and were similar to those reported in humans with HCM and restrictive cardiomyopathy, respectively. However, the hemodynamic abnormality was not the same for all cats with UCM; some cats with an enlarged left atrium and a normal left ventricle (ie, UCM) had abnormal left ventricular wall motion consistent with restrictive cardiomyopathy while others did not.

Animals↗
PubMed1997

[Isolated cardiac amyloidosis. Presentation of a clinical case].

A 48-year old patient affected by congestive heart failure came to our observation for cardiac arrest due to ventricular fibrillation. After cardiopulmonary resuscitation and defibrillation he underwent complete evaluation. Echo Doppler findings were consistent with restrictive cardiomyopathy. Laboratory findings revealed monoclonal gammopathy and plasma cells dyscrasia. Diagnosis of amyloidosis was then suspected and biopsies of different organs and tissues were performed. Presence of amyloid deposits was found only in myocardial specimens from the right ventricle. Medical treatment with drugs of various classes, administered during hemodynamic invasive monitoring, was uneffective in improving the hemodynamic and clinical status of the patient and he entered in a heart transplantation list. He died six months later, while awaiting for transplantation. Although isolated cardiac amyloidosis is quite rare, we believe that this condition has ever to be kept in mind during differential diagnosis of restrictive cardiomyopathies and we remark that endomyocardial biopsy was mandatory and necessary for certain diagnosis in this case; in addition, the unefficacy of drugs nowadays available for treatment of congestive heart failure in amyloid cardiomyopathy is confirmed.

Amyloidosis↗
PubMed1989

Impaired left ventricular diastolic filling in patients with familial amyloid polyneuropathy: a pulsed Doppler echocardiographic study.

To assess left ventricular diastolic filling in patients with amyloid heart disease 12 patients with familial amyloid polyneuropathy and 15 normal subjects were studied by pulsed Doppler echocardiography. None of the patients had clinical evidence of overt heart disease or restrictive cardiomyopathy and only two of them showed ventricular wall thickening. The peak flow velocity of rapid diastolic filling and the acceleration rate of early diastolic inflow were significantly lower in patients with familial amyloid polyneuropathy than in controls. The pressure half time was significantly longer in patients than in controls. In addition, the peak flow velocity during atrial contraction and the ratio of atrial peak flow velocity to rapid diastolic peak flow velocity were significantly greater in patients than in controls. Although there were no significant correlations between measurements of diastolic filling and clinical findings in patients with familial amyloid polyneuropathy, the ratio of atrial peak flow velocity to rapid diastolic peak flow velocity was significantly related to left ventricular posterior wall thickness. These findings suggest that in patients with cardiac amyloidosis without restrictive cardiomyopathy, abnormal left ventricular diastolic filling, manifested by a reduction in the rate and volume of rapid diastolic filling with enhanced atrial contraction, can be seen even in the early stage of the disease.

Adult↗
PubMed1991

Endomyocardial biopsy in infants and children with cardiomyopathy.

We reviewed our experience of endomyocardial biopsy performed on 21 symptomatic infants and children with cardiomyopathy. Clinical congestive cardiomyopathy was noted in 18 patients, 2 had hypertrophic cardiomyopathy, and 1 a restrictive cardiomyopathy. The biopsy findings led to a diagnosis of hemochromatosis in one patient, Adriamycin cardiomyopathy in another, and lymphocytic myocarditis in a third. Five patients had features of endocardial fibroelastosis, one endomyocardial fibrosis, and a further one, a mitochrondrial abnormality. In 11 patients normal or nonspecific features were seen. There were 2 myocardial perforations, both patients being successfully resuscitated. Endomyocardial biopsy, although occasionally hazardous, may sometimes provide valuable information which may lead to a diagnosis, facilitate treatment, and be of prognostic value. Despite the low positive yield, it may still be indicated in selected patients, in view of the seriousness and often poor prognosis of this disorder: 5 of our study group subsequently died.

Adolescent↗
PubMed2004

Regression of dietary copper restriction-induced cardiomyopathy by copper repletion in mice.

Dietary copper deficiency (CuD)(3) leads to cardiac hypertrophy in various animal models. We showed recently that heart failure develops after hypertrophy in FVB mice fed a CuD diet. The present study was undertaken to determine whether CuD-induced cardiac failure is reversible upon copper repletion (CuR). Dams of FVB mice were fed a CuD diet (0.3 mg/kg) starting from d 3 postdelivery; the weanling pups were fed the same diet until CuR with 6.0 mg/kg Cu in the diet at 4 or 5 wk of age. CuR at 4 wk of age prevented the body weight loss; at 5 wk of age, it resulted in the regaining of the lost weight caused by CuD. A significant regression of CuD-induced cardiac hypertrophy was observed in the CuR mice. Histopathological examination revealed that CuR eliminated CuD-caused lipid deposition in the myocardium, and electron microscopy demonstrated that CuD-induced ultrastructural changes such as mitochondrial swelling and organelle structural disarray were all reversed in the CuR mice. Hemodynamic analysis showed that the CuD-depressed systolic and diastolic parameters such as the maximal rate of left ventricular pressure rise (+dP/dt) and decline (-dP/dt), and the contraction and relaxation times were completely recovered in the CuR mice. Furthermore, the CuD-blunted myocardial responses to the beta-adrenergic agonist, isoproterenol, were also restored in the CuR mice. This study thus demonstrates for the first time that CuR results in the regression of heart failure induced by CuD as demonstrated by the reversal of depressed cardiac hemodynamic and contractile function and the restored responsiveness to beta-adrenergic stimulation.

Adrenergic beta-Agonists↗
PubMed1990

Cardiomyopathy and multicore myopathy with accumulation of intermediate filaments.

A girl affected by a restrictive cardiomyopathy with neuromuscular involvement is described. Morphological examination showed a pattern of multicore myopathy and with electron microscopy a sarcoplasmic accumulation of electron dense granular and filamentous material was demonstrated both in skeletal muscle and heart. This peculiar electron dense material corresponded to increased desmin in muscle and cardiac fibres and was demonstrated immunohistochemically.

Adolescent↗
PubMedDate not supplied

[Pathologic diagnostic criteria of cardiomyopathy in children].

Pathologic diagnostic criteria of the hypertrophic and dilated cardiomyopathy and comparative histologic characteristics of chronic non-specific myocarditis and restrictive cardiomyopathy are presented. The basis of the idiopathic cardiomyopathies is according to the authors, destructive and degenerative processes in cardiomyocytes, their combination with a zonal-segmentary muscle hypertrophy, dilatation and myocardium sclerosis. Dependence of the cardiomyopathy upon the mitochondrial dysfunction and the aggravation of morphological changes by viruses is emphasized.

Adolescent↗