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A woman with Noonan syndrome had clinical and haemodynamic features of restrictive cardiomyopathy. There was no ventricular hypertrophy on echocardiography but myocardial biopsies showed myocyte hypertrophy without pathological disarray. This case illustrates the overlap of the cardiac phenotypes of Noonan syndrome, restrictive cardiomyopathy, and hypertrophic cardiomyopathy.
Constrictive pericarditis (CP) and restrictive cardiomyopathy have the same pathophysiological disorder (impaired diastolic filling) and a similar presentation. To determine the value of a noninvasive technique, CT, in differentiating between the two, we reviewed the CT scans of 212 patients with clinical signs and symptoms of CP and/or restrictive cardiomyopathy, after 26 cases that did not proceed to surgery were excluded. Computed tomography showed pericardial thickening (greater than 3 mm) in 157 patients with proven tuberculous CP. In two children with proven constriction, the pericardium measured between 2 and 3 mm. We determined that pericardial thickening, in the correct clinical setting, is the only CT sign necessary to diagnose CP. Other CT signs, inferior vena cava dilatation (97%), and deviation of the interventricular septum (15%) were confirmatory. Computed tomography accurately differentiates between CP and restrictive myocardial disease, making more invasive modalities such as cardiac catheterization and endomyocardial biopsy, with few exceptions, unnecessary.
This report describes the evaluation of 1,278 patients referred to The Johns Hopkins Hospital with dilated cardiomyopathy. After a careful history and physical examination, selected laboratory tests, and endomyocardial biopsy, a specific diagnosis was made in 49% of cases. In 16% of cases the biopsy demonstrated a specific histologic diagnosis. Myocarditis and coronary artery disease were the most frequent specific diagnoses; 51% of patients were classified as idiopathic. Thus a rigorous and systematic search can demonstrate an underlying cause for approximately one-half of patients with unexplained cardiomyopathy. Endomyocardial biopsy plays a crucial role in this evaluation. Six cases are presented which demonstrate the utility of endomyocardial biopsy in specific clinical situations. In addition to its routine use in monitoring rejection in heart transplant recipients, endomyocardial biopsy is indicated in the evaluation of possible infiltrative cardiomyopathy, in differentiating restrictive cardiomyopathy from constrictive pericarditis, and in diagnosing and monitoring doxorubicin cardiotoxicity. The importance of diagnosing myocarditis remains controversial, and disagreement persists about the utility of immunosuppressive therapy in these patients. A combination of clinical and histologic features can divide patients with myocarditis into 4 subgroups--acute, fulminant, chronic active, and chronic persistent. This classification provides prognostic information and may identify those patients who may respond to immunosuppression, as well as those likely to have adverse outcomes from such treatment. The continued development of novel molecular techniques may allow endomyocardial biopsy to provide greater prognostic and therapeutic information in the future.
Cardiomyopathies are diseases of the myocardium associated with cardiac dysfunction, and are classified as dilated cardiomyopathy (DCM), hypertropic cardiomyopathy (HCM) and restrictive cardiomyopathy. Heart failure and sudden death are the two major complications. Also, since DCM is the primary indication for heart transplantation and HCM the primary cause of sudden death in young athletes, the socioeconomic impact of these diseases is important. Recently, the role of the genetic background in both monogenic and multifactorial cardiomyopathies has been studied, which has led to a better understanding of the underlying mechanisms that promote the development and progression of these diseases. Preliminary data suggest interactions between pharmacological treatment and genetic polymorphisms, which appear to be the first steps towards the application of pharmacogenetics in heart failure.
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OBJECTIVE--To determine whether standard manual frequency filters in the ON and OFF settings affected P-QRS-T voltages, discover whether recorded P-QRS-T voltages vary between commercial electrocardiographs, assess effects of frequency filters on base-line artifact, and evaluate ECG frequency content by high-fidelity recordings subjected to digital filters with variable frequencies. DESIGN--Sequential 10-lead ECG were recorded in 30 cats, using 3 commercial electrocardiographs to assess effects of manual frequency filters on the P-QRS-T wave forms. Three clinically normal cats were evaluated for ECG frequency content. ANIMALS--Thirty cats (13 with hypertrophic cardiomyopathy; 4 with restrictive cardiomyopathy; 3 hyperthyroid; 1 with ventricular septal defect; 1 with aortic stenosis; and 8 with no detectable cardiovascular disease). Three additional clinically normal cats were studied for effects of frequency filters on the ECG frequency content. PROCEDURES--Ten-lead ECG were recorded on each cat by use of 3 commercial electrocardiographs sequentially. For each machine, a recording was made with manual filters ON, immediately followed by a recording with manual filters OFF. High-fidelity lead-II ECG recordings were made with filters set with their rolloff frequency at 0.1 Hz and 3.0 kHz; output voltage (0.2 mV/V) was fed to an analog-to-digital converter, then to attendant software, which sampled the signal at 6 kHz with a 12-bit sampler, and were digitally filtered at various corner frequencies. RESULTS--Voltages recorded by all 3 electrocardiographs were greatest when filters were OFF (most prominent on R- and S-wave voltages). In all recorded leads, R-wave voltage was significantly greater when filters were OFF than ON. Comparison of voltages indicated significant (P < 0.05) differences between R-wave voltages recorded in all leads with manual filters ON, but not with filters OFF. With filters ON, each electrocardiograph produced a smaller percentage of recordings with moderate to severe baseline artifact than with filters OFF. R-Wave amplitudes of high-fidelity lead-II ECG were significantly decreased with digital filters set at corner frequencies < 150 Hz. CONCLUSIONS--Significant (P < 0.05) voltage attenuation was recorded by each of the 3 commercial electrocardiographs when frequency filters were ON, compared with OFF. Comparison of waveform voltages among electrocardiographs with filters ON indicated significant variation in R-wave amplitudes in all leads. With manual filters ON, each electrocardiograph recorded a smaller percentage of recordings with baseline artifact than with filters OFF. Substantial frequency components > or = 150 Hz are present in the feline ECG waveform. Thus, filters with frequencies < 150 Hz markedly attenuate the feline R wave. CLINICAL RELEVANCE--Attenuation of feline ECG signals occurs with use of commercial electrocardiographs and varies greatly between manufacturers. This is attributable largely to internal manual frequency filters. These consequences may be important when applying standard feline reference values or when equivocal voltage measurements are recorded.
No heart transplants have been performed in Japan due to various obstacles since the only operation performed in 1968. Since 1981, a number of patients requiring heart transplants have been accepted by foreign transplantation centers in England, the U.S.A., and Germany. This report describes an investigation of the postoperative course of these Japanese heart transplant patients and discusses the problems regarding transplantation in Japan. Of the 21 transplant patients, the diagnosis was dilated cardiomyopathy in 17, restrictive cardiomyopathy in 1, hypertrophic cardiomyopathy in 2, and congenital muscle dystrophy in 1. All patients survived surgery but three died in the long-term period. The causes of death were acute rejection (after 3 months), chronic rejection (after 50 months) and infection (after 30 months). The actuarial survival curve of these patients was 95.0% for one-year survival and 86.4% for three-year survival. The postoperative functional class was NYHA classification I in all patients (100%). Ninety-three percent of patients returned to work. Immunosuppressive therapies included triple drug therapy in 14 patients (66.7%), double drug therapy in 4 (19.0%), ciclosporin alone in 2 (9.5%) and FK506 in 2 (5.0%). The incidence of acute rejection was 1.56 episodes per patient per year within 3 months and 2.9 episodes per patient per year within 1 year. The postoperative courses of Japanese patients who underwent heart transplantation at foreign transplantation centers were satisfactory. These results will encourage heart transplantation in Japan.
The results of endomyocardial biopsy (EMB) are presented in 174 patients. Techniques and apparatus are described. EMB is safe and useful for diagnosing inflammatory myocarditis and as a guide to therapy. Unsuspected lymphocytic myocarditis was found in 17% of patients with dilated cardiomyopathy. The results confirm that the EMB findings in dilated cardiomyopathy are nonspecific and that restrictive cardiomyopathy is not confined to hypereosinophilic states and amyloid heart disease.
The clinical significance of the mode of left ventricular (LV) diastolic filling in hypertrophic cardiomyopathy was studied by the LV inflow velocity patterns (LVIF) of pulsed Doppler echocardiography and LV early diastolic filling rates (V2) of radionuclide (RI) angiography. The relationship between the deceleration time (DT) obtained from LVIF and the V2 was evaluated in 34 patients with nonobstructive hypertrophic cardiomyopathy (HCM) and in nine with obstructive hypertrophic cardiomyopathy (HOCM), and the results were compared with those of 10 patients with dilated cardiomyopathy (DCM), of two with restrictive cardiomyopathy (RCM), and of 19 normal subjects. HCM was subdivided into the following groups according to V1-DT relationships: Group 1 with prolonged DT and decreased V1, Group 2 with normal or short DT and normal V1, and Group 3 with normal or short DT and decreased V1. There were significant negative correlations between V2 and DT in Groups 1 and 2 of HCM, normal and HOCM. However, there were significant positive correlations in Group 3 of HCM, DCM and RCM. Nearly all patients in Group 3 had decreased LV ejection fraction and % fractional shortening, distinct B-B' step formation of the mitral valve echogram, and huge A wave of the apexcardiogram. These findings suggested that the LV rapid filling interval gradually became shorter because the LV contraction is decreased as myocardial fibrosis develops in HCM, and that we must pay attention to the diagnosis of such abnormalities, similar to those of DCM or RCM.
Heart failure, a major contributor to cardiovascular disease morbidity and mortality, is newly diagnosed in approximately 400,000 patients each year, and is particularly prevalent in individuals over age 65 years. Average mortality rates 5 years after diagnosis are 45-60%, and may be as high as 50% after 1 year for those with New York Heart Association class IV heart disease. Heart failure occurs when myocardial muscle dysfunction prevents the heart from pumping enough blood at normal cardiac pressures to meet the metabolic needs of the body, especially during exercise, and compensatory hemodynamic and neurohormonal mechanisms are overwhelmed or maladaptive. Pathologic classifications are broadly based on the presence of systolic (dilated cardiomyopathy) or diastolic (hypertrophic or restrictive cardiomyopathies) dysfunction. The etiologies of heart failure may include inadequate coronary blood flow, pressure or volume overload, cardiomyopathy, or pericardial disease. Coronary artery disease, idiopathic dilated cardiomyopathy, and hypertension are the most frequent causes, and certain drugs may also worsen myocardial function. When contractility is reduced, stroke volume and cardiac output are decreased, and alterations in the kidneys may induce fluid retention to compensate for the perceived low output and reduced circulating blood volume. Fluid retention in turn causes preload or filling pressure to increase and symptoms of pulmonary congestion to emerge. Depressed contractility also results in a reduction in blood pressure, leading to compensatory neurohormonal activation and vasoconstriction, which significantly elevate afterload and further reduce stroke volume. The overall approach to heart failure includes defining the etiology, identifying precipitant factors, and assessing the severity of myocardial dysfunction and clinical symptoms.(ABSTRACT TRUNCATED AT 250 WORDS)
BACKGROUND: Infiltrative cardiomyopathies are characterized by diastolic dysfunction. In monoclonal plasma cell dyscrasias, organ compromise may be produced by tissue deposition of monoclonal immunoglobulins or their constituent peptides independently of the effects of unbridled plasma cell proliferation. The deposits may be fibrillar, as in light chain amyloid (AL) or nonfibrillar, as in light chain deposition disease (LCDD). AL disease of the heart is a restrictive cardiomyopathy. We hypothesized that, despite differences in physical properties, nonamyloidotic light chain deposition in the myocardium could produce similar clinical and physiological abnormalities. METHODS: Cardiac tissue from five patients with LCDD and cardiac dysfunction was examined by immunohistochemical and electron microscopic techniques. Hospital charts, electrocardiograms, echocardiograms and cardiac catheterization results were reviewed. In two cases, the original echocardiograms were reanalyzed. RESULTS: The five patients with nonamyloidotic light chain deposits in the myocardium had either mechanical or electrocardiographic abnormalities. In four with adequate clinical documentation, the diastolic dysfunction and conduction abnormalities were similar or identical to that described in cardiac AL disease. CONCLUSIONS: Although nonamyloidotic immunoglobulin light chain deposits in the myocardium differ in distribution and ultrastructural organization from the fibrillar deposits of AL disease, an analogous pattern of diastolic dysfunction and conduction disturbances results. The diagnosis should be considered in patients with a plasmacytic dyscrasia and restrictive cardiomyopathy in whom Congo red staining of endomyocardial biopsy tissue is negative. The diagnosis can be established by using the appropriate immunohistochemical and ultrastructural tissue examinations.
Cardiomyopathies are an important cause of congestive heart failure in the elderly, and the magnitude of the problem is compounded by changing population demographics and the frequency of congestive heart failure in the elderly. Although the data are far from complete, differences in the clinical presentations and natural history of the cardiomyopathies in older and younger patients are becoming more clearly appreciated. Dilated cardiomyopathy (DCM) is clearly more common than previously appreciated, and elderly patients have a worse prognosis than their younger counterparts with this disease. The medical management of DCM is often more difficult in the elderly, and the problem is compounded by the relatively infrequent use of cardiac transplantation as a therapeutic option. Hypertrophic cardiomyopathy is also more common than previously appreciated, and changes in left ventricular structure often create difficulties in differentiating pathologic states from physiologic. Fortunately, the prognosis for HOCM is more favorable in the elderly than in younger patients and may be partly accounted for by the different structure of the left ventricle. If needed, surgery is an option for elderly patients with medically refractory HOCM, but particular attention must be paid to the presence and severity of associated cardiovascular disease. Restrictive cardiomyopathy with diastolic dysfunction is increasingly recognized as a cause of congestive heart failure. The differentiation from systolic dysfunction is crucial, because the treatments are so markedly different. Age-related changes in diastolic function are becoming more apparent and better characterized, but standardization of age-related "normal" values is still not available. The diagnosis of restrictive heart disease should stimulate a search for an underlying cause, inasmuch as restrictive cardiomyopathy remains a diagnosis of exclusion. Advances in noninvasive imaging have led to a resurgence of interest and have enhanced our knowledge of the cardiomyopathies. Further investigation should proceed in conjunction with studies aimed at defining the characteristics and variables of "normal" aging. For the present, the enigmatic, poorly identified "cardiomyopathies" remain a problem for both young and old.
Feline and canine cardiomyopathies (primary myocardial diseases) were reviewed and divided into three groups based on the clinical, hemodynamic, angiocardiographic, and pathologic findings: (1) feline and canine hypertrophic cardiomyopathy, (2) feline and canine congestive (dilated) cardiomyopathy, and (3) feline restrictive cardiomyopathy. All three groups consisted predominantly of mature adult male cats and dogs. Cardiomyopathy in the hamster and turkey was also reviewed. The most common presenting signs were dyspnea and/or thromboembolism in the cat, systolic murmurs with gallop rhythms on auscultation, cardiomegaly with (groups 1 and 3) or without (group 2) pulmonary edema, abnormal electrocardiograms, elevated left ventricular end-diastolic pressures, and angiocardiographic evidence of mitral regurgitation with left ventricular concentric hypertrophy (group 1), left ventricular dilatation (group 2), or midventricular stenosis (group 3). Some cats in groups 1 and 3 also had evidence of left ventricular outflow obstruction. The principal pathologic findings in all of the cats and dogs were left atrial dilation, hypertrophy, increased septal:left ventricular free wall thickness ratio with disorganization of cardiac muscle cells (group 1); dilatation of the four chambers with degeneration of cardiac muscle cells (group 2); and extensive endocardial fibrosis and adhesion of the left ventricle (group 3). Aortic thromboembolism was commonly observed in the cats of all three groups. These clinical and pathologic findings indicate that cardiomyopathy in the cat or dog is similar to the three forms of cardiomyopathy in humans (hypertrophic, congestive, and restrictive).
Acute decompensating cardiomyopathy induced by cyclophosphamide is usually irreversible. To investigate the clinical course and the outcome of therapy, 13 patients (1.7%) with grade III acute cardiomyopathy and hypotension who were treated with ablative transplant regimens between January 1980 and September 1995 were analyzed. Eight of nine patients died of acute fatal restrictive cardiomyopathy with unresponsive hypotension (ARCH), whereas three of four patients who survived the initial episode died of subacute congestive heart failure (SCHF). Acute fatal restrictive cardiomyopathy was characterized with extreme sensitivity to volume overload, myocardial edema and a rapidly fatal course. It was associated with progressive, unresponsive hypotension, reduced left ventricular stroke work index (LVSWI: 29.29 +/- 9.74 g-m/beat/m2) and markedly reduced systemic and pulmonary vascular resistance indices (SVRI: 429.72 +/- 168.84, PVRI: 58.63 +/- 45.08 dyne.sec/cm5.m2). Subacute CHF was identified by myocardial edema, dilated chambers and biventricular pump failure represented by decreases in fractional shortening (FS: 19.5 +/- 4.9%). Of 10 patients who received conventional therapy, nine died and one sustained chronic CHF. One of three patients with ARCH on antioxidant therapy of ascorbic acid and theophylline survived the episode. The data suggests peripheral vascular collapse may also be responsible for fatal ARCH.
Studies were done on blood eosinophils from four patients with raised blood eosinophil counts and heart failure. In three of the patients cardiological studies demonstrated the distinctive endocardial lesions and restrictive cardiomyopathy of Löffler's endocarditis and endomyocardial fibrosis. The fourth patient died with similar symptoms and signs. In blood films it was found that all four had more than 1 X 10(9) eosinophils per litre which were vacuolated and contained reduced numbers of crystalloid granules which were also shown to have ultrastructural changes. Unlike eosinophils from normal individuals the patients' eosinophils possessed receptors for rabbit IgG-coated erythrocytes and actively phagocytosed erythrocytes coated with rabbit IgG or human C3b. It is concluded that in these patients, a large proportion of the circulating eosinophils had developed characteristics of mature or stimulated eosinophils. This enabled them to respond to soluble substances in the bloodstream by forming endocytic vacuoles which led to degranulation of the crystalloid granules. These studies, taken in conjunction with other recent work in this field, support the concept that the restrictive cardiomyopathy of hypereosinophilic states, including Löffler's endocarditis and endomyocardial fibrosis, is a result of prolonged release of products from degranulated eosinophils while they are in the circulation.
Constrictive pericarditis is a uncommom disease in children. We have now encountered pericardial thickening as the cause of severe constrictive physiology in two patients, one also having haemodynamic features of restrictive cardiomyopathy. Both patients, who had refractory ascites and evidence of increased systemic venous pressure, underwent Doppler echocardiography, cardiac catheterisation, and magnetic resonance imaging. Resonance imaging failed to show any thickning of the pericardium, but cardiac catheterisation revealed diastolic equalisation of pressures in all four chambers, with only mild elevation of pulmonary pressure in the first patient, but nearly equalisation of diastolic pressure, and a very high pulmonary arterial pressure with a difference of 7 mm Hg between the end diastolic pressures in the two ventricles in the second patient. Doppler revealed a restrictive pattern of mitral inflow, with high E and small A velocities and a short deceleration time. The clinical background did not suggest pericardial disease in either of the patients. We conclude that a careful search is needed to uncover constrictive pericarditis when there is no previous disease which may suggest late pericardial constriction. The haemodynamic features of restrictive cardiomyopathy can co-exist with pericardial restriction, and differentiation between the two entities is critical in view of the diverse management and prognosis of the two conditions.
Amyloidosis is a systemic disease in which clinical manifestations are caused by the replacement of normal tissue with insoluble amyloid fibrils. Cardiac involvement causes a restrictive cardiomyopathy and is associated with poor functional outcomes. Cardiac magnetic resonance imaging and measurement of B-type natriuretic peptide are particularly helpful in distinguishing restrictive cardiomyopathy from constrictive pericarditis, but a tissue biopsy is required to make the diagnosis of amyloidosis. Although standard treatment options for congestive heart failure may provide symptomatic relief in cardiac amyloidosis, prognosis remains dismal. Judicious diuretic use remains the mainstay of therapy, but achieving optimal fluid balance is difficult because patients are usually "preload dependent." Angiotensin-converting enzyme inhibitors in low doses are often helpful but may lead to orthostatic hypotension, particularly in patients who also have involvement of the autonomic nervous system. beta Blockers may be useful if given relatively early in the disease process, but should be used with caution in patients with advanced disease because they may exacerbate symptoms. Therapy aimed at the underlying disease process in primary systemic amyloidosis is based on treatment regimens used in multiple myeloma, such as melphalan and prednisone. These offer limited benefit when cardiac involvement is significant, but newer treatments, including the novel anthracycline 4'-iodo-4'-deoxydoxorubicin, potentially combined with autologous stem cell transplantation, offer some hope for the future.
Doppler echocardiography is useful in assessing diastolic dysfunction. Pulsed-wave Doppler echocardiographic interrogation of the atrioventricular valves and the central veins has been used in conjunction with respiratory monitoring to characterize abnormal diastolic function of the heart in diseases such as constrictive pericarditis, restrictive cardiomyopathy, and cardiac tamponade. Constrictive pericarditis has distinct Doppler flows with marked respiratory variation because the pericardial shell separates the intracardiac pressures from intrathoracic pressure. This is not the case with restrictive cardiomyopathy, as in cardiac amyloidosis. Cardiac amyloidosis shows a spectrum of the Doppler flows which evolve as the disease progresses from abnormal relaxation in the early phase to pseudo-normal in the intermediate phase and to restrictive in the advanced phase. Doppler echocardiography can be used to assess the hemodynamic significance of pericardial effusion and to detect cardiac tamponade.