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At least 613 records · Page 34Linked to original sources
PubMed1997

[Doppler ultrasound of blood flow in the hepatic veins].

The blood flow in the hepatic veins can normally be studied easily by Doppler ultrasound. The pattern of blood flow in normal individuals is described, and its relation to the cardiac cycle and changing pressure in the right atrium. The blood flow shows variations in healthy persons, and may change in cases of heart disease and hepatic disease. Conditions such as atrial fibrillation, tricuspid regurgitation, abnormal relaxation, restrictive cardiomyopathy, constrictive pericarditis and cardiac tamponade are reflected in the hepatic veins, and the pattern of blood flow may help in diagnosis, and in grading the pathology. In cirrhosis and portal hypertension the heart-synchronous variation in velocity is reduced. This is due to increased resistance to blood flow across the liver and the pressure gradient becoming larger than the variations in pressure in the right atrium.

Blood Flow Velocity↗
PubMed1997

[Localizations and consequences].

The symptoms of amyloidosis depend on the type of precursor, the amount of deposits and their location. In systemic amyloidosis almost every organ may be involved. Cardiac involvement is severe, especially in AL amyloidosis, responsible for restrictive cardiomyopathy with right ventricular failure, leading rapidly to death. Renal amyloid deposition causes nephrotic syndrome with hypertension and renal failure. Neurological complications include peripheral neuropathy with dysautonomia cerebral involvement (dementia, cerebral haemorrhages). Arterial deposits are common in systemic senile amyloidosis, and may cause ischaemia. Osteo-articular damage is mainly seen in patients on long-term haemodialysis. Liver enlargement is often the only manifestation of hepatic amyloidosis. Digestive tract involvement includes macroglossia deposits in salivary glands and disturbances in gastrointestinal motility. Pulmonary amyloidosis causes nodular or interstitial infiltrates. Cutaneous lesions are various. Localized amyloidoses include goiter, breast and vesical involvement which can be difficult to differentiate from neoplasm, as well as ocular amyloidosis mimicking posterior uveitis.

Adult↗
PubMed1998

Third-degree atrioventricular block in a patient under chloroquine therapy.

The first case of chronic cardiac toxicity due to an antimalarial agent was reported in 1971 and since then several cases of heart failure, restrictive cardiomyopathy or atrioventricular block have been ascribed to this family of drugs. We report the case of a 43-year-old woman who developed juvenile chronic arthritis at the age of ten, followed in adulthood by sero-positive rheumatoid arthritis. In 1980 she was put under chloroquine sulfate (hydroxychloroquine was not available) in a dose of 200 mg/d (152.66 mg of chloroquine), with 10 mg/day of prednisone. She developed myalgia and increased skin pigmentation, but disregarded recommendations that these symptoms required discontinuation of chloroquine therapy. She was lost to follow-up, but continued the chloroquine therapy of her own accord. In December 1993, she developed a third-degree atrioventricular block with syncopes requiring implantation of a pacemaker. The rare but well-documented myopathy induced by antimalarial agents can produce early severe lesions of the cardiac muscle, which may have a predilection for the interventricular septum, explaining the risk of atrioventricular block. Although histologic studies were not performed in our patient, the clinical evidence of toxicity, absence of underlying heart disease and fairly young age of the patient pointed to chloroquine toxicity. Periodic cardiac investigations including electrocardiography may be warranted in patients under antimalarial therapy.

Adult↗
PubMed1998

Atrial septal aneurysm in a patient with pseudoxanthoma elasticum.

Pseudoxanthoma elasticum (PXE) is a rare heritable disorder of the connective tissue characterized by marked clinical heterogeneity. Common cardiovascular complications of PXE include renovascular hypertension, premature coronary artery disease and calcification of peripheral arteries frequently leading to claudicatio intermittens; fibrous thickening of the endocardium and atrioventricular valves, leading to restrictive cardiomyopathy and/or mitral valve prolapse have been also described. We herein report on a PXE patient who developed an atrial septal aneurysm as a previously undescribed cardiologic manifestation of her disease.

Adult↗
PubMed1980

Primary cardiomyopathies: classification, pathophysiology, clinical recognition and management.

In recent years, the definition of cardiomyopathy has been restricted to the idiopathic forms of myocardial disease and has been grouped into three general categories: (1) congestive or dilated cardiomyopathy, (2) hypertrophic cardiomyopathy, and (3) restrictive cardiomyopathy. The history and physical examination and echocardiography appear to be the most helpful in the clinical recognition of these disorders. In general, the prognosis of patients with congestive cardiomyopathy is guarded. Their survival primarily depends on the natural history and progression of the disease as well as the response to treatment with anticongestive drugs. The recent advent of vasodilator therapy has provided additional help to the patient with refractory congestive heart failure. Hypertrophic cardiomyopathy appears to be much more frequently recognized since the advent of echocardiography. The young patient has a much higher incidence of serious complications (combined right and left ventricular outflow obstruction, more generalized hypertrophic cardiomyopathy, and sudden death), and the patient presenting in later life appears to have fewer associated complications. There is a familial association with hypertrophic cardiomyopathy, although the true incidence and full expression of the association is not well understood at present. The majority of adult patients acieve prolonged survival with medical and/or surgical treatment. The restrictive cardiomyopathies are probably the least well understood. All three subgroups (Löffler's endocarditis, primary restrictive cardiomyopathy, and endomyocardial fibrosis) have a guarded prognosis. The acute form (Löffler's endocarditis) is characterized by a debilitating illness with death usually within a couple of months; however, a small percentage of these patients can have long survival and may actually evolve into a more chronic form, i.e., primary restrictive cardiomyopathy or endomyocardial fibrosis. The latter two restrictive myopathies usually have a slow indolent course, and their prognosis is primarily determined by the amount of myocardial damage and endocardial fibrosis.

Adolescent↗
PubMedDate not supplied

Heart transplantation: hemodynamics over a five-year period.

Long-term hemodynamic evaluation after heart transplantation has rarely been reported. Fifty-seven heart transplant recipients (mean age, 42.1 years: range, 22 to 56 years) were investigated with annual cardiac catheterization. Mean follow-up was 2.2 years (range, 1 to 7 years). Years 1 through 5 were evaluated statistically. Hemodynamic variables did not change significantly during the 5-year period. Mean aortic pressure (105 +/- 16 to 115 +/- 17 mm Hg, minimum and maximum annual average value +/- SD of the 5-year period) and LVEDP (13 +/- 6 to 15 +/- 7 mm Hg) were slightly elevated. Borderline values were found for mean pulmonary artery pressure (18 +/- 4 to 21 +/- 5 mm Hg), systemic vascular resistance (1407 +/- 227 to 1487 +/- 409 dynes.sec.cm-5), pulmonary vascular resistance (86 +/- 42 to 118 +/- 66 dynes.sec.cm-5), and heart rate (85 +/- 17 to 95 +/- 14 beats/min). Mean right atrial pressure (5 +/- 2 to 8 +/- 4 mm Hg), cardiac index (2.8 +/- 0.6 to 3.5 +/- 1.1 L/min/m2), end-diastolic volume (111 +/- 37 to 137 +/- 35 ml) and ejection fraction (69% +/- 10% to 75% +/- 9%) were in the normal range. Pressure-volume loops for each year excluded the development of either restrictive or dilated cardiomyopathy with time. Contractility, as measured by imposing afterload stress, was normal in 12 of 14 representative patients. Prevalence of coronary abnormalities (circumscript coronary stenoses, diffuse vessel obliteration or dilated angiopathy) increased from 14.9% in the first to 66.7% in the fifth year. Of the patients, 93% received antihypertensive drugs.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed2001

Cardiomyopathies in adolescents: dilated, hypertrophic, and restrictive.

Three major types of cardiomyopathies affect adolescents: dilated, hypertrophic, and restrictive. At the present time the etiologies of the majority of these cardiomyopathies in children remain elusive. Treatment of these diseases is generally directed toward improving symptoms, survival, myocardial performance, and hemodynamics. Most treatment strategies are extrapolated from studies in adults with these types of cardiomyopathies, although prospective, randomized therapeutic trials are currently underway in children. All three types of cardiomyopathy can be associated with a wide range of symptoms (from none to severe) and also are associated with sudden death. For those in whom medical management fails, heart transplantation is a therapeutic option with an excellent intermediate-term success rate.

Adolescent↗
PubMed1996

[Myocardiopathies (XI). Restrictive myocardiopathy: concept, classification, and clinical approach].

Currently, cardiomyopathies are defined as "heart muscle diseases of unknown etiology". Restrictive cardiomyopathy is the rarest type of this disease, and the only one with no uniformly accepted diagnostic criteria. Its importance lies primarily in properly distinguishing it from constrictive pericarditis because of the direct consequences of mistakenly treating a case of constrictive pericarditis as if it were inoperable. Restrictive cardiomyopathy must be considered within the broader spectrum of diastolic dysfunction. Diagnostic criteria used show a general consensus for the diagnosis of restrictive cardiomyopathy in patients with clinical signs of heart failure in the presence of a nondilated, nonhypertrophic left ventricle with preserved contractility but abnormal diastolic function. Characteristically, right and left ventricular filling pressures are elevated and a dip and plateau diastolic pressure pattern appears. Restrictive cardiomyopathy may be either idiopathic and thus be a true cardiomyopathy in the strict sense of the term or secondary to an infiltrative disease of the myocardium. In this article we review the clinical and hemodynamic features of this disease and the different imaging techniques used in patients to help differentiate restrictive cardiomyopathy from constrictive pericarditis. Finally we study some types of restrictive cardiomyopathies.

Cardiac Catheterization↗
PubMed1993

A unified perspective on copper deficiency and cardiomyopathy.

Dietary copper restriction in rats results in cardiomyopathy. In rats fed copper-restricted diets from weaning for 5 to 8 weeks, a concentric hypertrophy is apparent, whereas postweaning copper restriction does produce cardiomyopathy without apparent hypertrophy. Both sets of circumstances appear to affect the integrity of the basal laminae of cardiac myocytes and capillaries. In rats fed copper-restricted diets from weaning, decreases in cytochrome c oxidase are related not only to copper's role as a coenzyme, but also to a marked decrease in the nuclear encoded subunits of the enzyme complex. Decreased levels of the delta-subunit of ATP synthase have been observed. However, such aberrations in mitochondrial enzymes, as well as morphologic alterations, apparently do not affect cardiac levels of ATP. This review suggests mechanisms of cardiac adaptation and initiation factors leading to cardiac hypertrophy. We present a hypothetical working model explaining the events leading to cardiac failure in the copper-deficient rat heart based on the present body of knowledge, and compare the pathology with other models of cardiomyopathies.

Animals↗
PubMed1998

Congenital myopathy with mosaic fibers and interlacing sarcomeres: a new structural myopathy.

A 44-year-old man presenting with dyspnoic attacks was found to be affected with congenital myopathy, rigid spine, restrictive respiratory insufficiency and cardiomyopathy. Muscle biopsy showed type 1 fiber predominance (65.7%) and hypotrophy, and characteristic changes in 43.9% of the type 1 fibers, consisting in alternating pale and dark staining on alkaline ATPase reacted sections in a mosaic pattern. Ultrastructural examination demonstrated bands of myofibrils at right angles or skew to the remaining myofibrils transversing the fibers. Myofibrillar disarray was always associated with loss of the Z-discs and actin filaments, and often with aggregation of mitochondria. The muscle biopsy findings in this patient suggest a new entity of congenital myopathy with clinical features of rigid spine, cardiomyopathy and restrictive respiratory insufficiency, characterized by peculiar abnormalities of ATPase staining in a mosaic pattern and, ultrastructurally, by zones of disorientation of the sarcomeres.

Adult↗
PubMed1992

Familial cardiomyopathy with variable hypertrophic and restrictive features and common HLA haplotype.

We describe a familial cardiomyopathy affecting three generations of family members with similar electrocardiographic abnormalities and human leukocyte antigen (HLA) haplotype. Autopsy findings in two of our patients were diagnostic of hypertrophic cardiomyopathy, although surviving affected members do not show ventricular hypertrophy on echocardiography. One patient's echocardiogram revealed giant atria in the absence of ventricular hypertrophy, suggesting a restrictive form of cardiomyopathy. In this kindred, an HLA haplotype appearing in affected, but not in healthy, family members may predict occurrence of disease during its presymptomatic phase in subsequent generations.

Adolescent↗
PubMed1992

Cardiomyopathies and specific heart muscle diseases. Definitions, terminology, classifications and new and old approaches.

Cardiomyopathies are defined as 'heart muscle diseases of unknown cause' and classified into hypertrophic, dilated and restrictive types, respectively. Hypertrophic cardiomyopathy is notable for massive ventricular hypertrophy without obvious cause, impaired diastolic and systolic function, a tendency for sudden death and a familial propensity. Dilated cardiomyopathy by contrast, demonstrates severe systolic failure progressing to congestive heart failure, with usually no familial tendency. Restrictive cardiomyopathy and diastolic heart disease represent syndromes with restriction to ventricular filling due to restrictive forces in the endomyocardium (and in constrictive pericarditis in the pericardium). The commonest cause of restrictive cardiomyopathy is endomyocardial fibrosis now usually known as hypereosinophilic endomyocardial disease. Specific heart muscle diseases are those conditions in which myocardial disease is due to a known cause: they usually produce systolic failure though occasionally a restrictive syndrome is evident. Amyloid heart disease occupies a place intermediate between cardiomyopathies and specific heart muscle diseases. The major features of the above conditions are described and current and future advances noted. Examples are the identification of the gene probably responsible for hypertrophic cardiomyopathy located on chromosome 14, and the identification of virus RNA particles in the myocardium in both myocarditis and in dilated cardiomyopathy, which strengthens the growing evidence suggesting that some cases of dilated cardiomyopathy may be due to previous myocarditis.

Cardiomyopathies↗
PubMedDate not supplied

[Current knowledge of cardiomyopathy].

In 1961, 1964 and 1970, Goodwin proposed a somewhat unwield definition of cardiomyopathies, which was subsequently shortened to "Heart muscle disease of unknown cause" and separated from heart muscle diseases due to conditions involving organs other than the heart: the "specific heart muscle diseases". The cardiomyopathies thus defined were divided into hypertrophic, dilated and restrictive types and their classification was confirmed by the WHO/ISFC Task Force and the report of the WHO Expert Committee on cardiomyopathies in 1984. The cardiomyopathies are now classified by the dominant pathophysiology or where possible by aetiological/pathogenic factors. The modifications proposed by Dr Peter Richardson are timely and reflect revised ways of looking at problems that still face us. They are classified as dilated cardiomyopathy, hypertrophic cardiomyopathy, restrictive cardiomyopathy, and arrhythmogenic right ventricular cardiomyopathy. Unclassified cardiomyopathies include a few cases that do not fit readily with any group. The term specific cardiomyopathies is now used to describe heart muscle diseases which are associated with specific cardiac or systemic disorders.

Cardiomyopathies↗
PubMedDate not supplied

Effect of protein restriction during brooding on spontaneous turkey cardiomyopathy.

The effect of early protein restriction on poult performance and mortality due to spontaneous turkey cardiomyopathy were examined in a facility that historically had a high incidence of the condition. Two thousand male turkey poults were divided into two equal subgroups for the first 4 weeks of life: one received standard commercial rations for the first 4 weeks (high-protein subgroup), and the other received rations with a protein content approximately 70% of the first subgroup (low-protein subgroup). Rations were the same after 4 weeks of age (standard commercial rations). At 16 weeks of age, turkeys in the low-protein subgroup weighed an average of 12.32 kilograms (27.1 pounds), whereas turkeys in the high-protein subgroup weighed an average of 12.73 kilograms (28.0 pounds). Total mortality for the low-protein subgroup was 10.1%, whereas total mortality for the high-protein subgroup was 15.7%. Total mortality due to spontaneous turkey cardiomyopathy in the high-protein subgroup was greater than twice that in the low-protein subgroup (10.4% versus 4.6%). These results show that lowering the protein content of the feed in the first 4 weeks significantly reduces mortality due to spontaneous turkey cardiomyopathy, but body weight gain is also reduced.

Animals↗
PubMed2005

MRI to evaluate left atrial and ventricular reverse remodeling after restrictive mitral annuloplasty in dilated cardiomyopathy.

BACKGROUND: Data on reverse remodeling of the left atrium (LA) and left ventricle (LV) after restrictive annuloplasty in patients with dilated cardiomyopathy are scarce, and follow-up studies are performed with echocardiography. METHODS AND RESULTS: Twenty patients with dilated cardiomyopathy and severe mitral regurgitation selected for restrictive mitral annuloplasty underwent serial MRI studies (within 1 week before surgery, and 2 months [n =18] and 1 year [n =13] after surgery). Early mortality was 10%; all patients were free from endocarditis and thromboembolism. New York Heart Association class improved from 3.2+/-0.4 to 1.2+/-0.9. Only 1 patient developed recurrent severe mitral regurgitation during follow-up and it was re-repaired. LA end-systolic volumes decreased significantly over time (from 165+/-48 mL to 109+/-23 mL to 111+/-28 mL; P < 0.01), as did LA end-diastolic volumes (from 92+/-32 mL to 71+/-22 mL to 75+/-17 mL; P = 0.01). LV end-diastolic volumes decreased significantly (from 244+/-56 mL to 184+/-54 mL to 195+/-67 mL; P < 0.01), whereas end-systolic volumes did not change significantly. LV ejection fraction increased significantly (from 35+/-8% to 46+/-13% to 46+/-15%; P < 0.01) and LV mass decreased significantly (from 150+/-43 grams to 132+/-39 grams to 136+/-33 grams; P = 0.02). CONCLUSIONS: Restrictive annuloplasty in patients with dilated cardiomyopathy yielded excellent clinical results associated with significant LA and LV reverse remodeling over time as demonstrated by MRI.

Adult↗
PubMed1988

[Dilated cardiomyopathy. Role of radioisotope technics].

Radionuclide techniques are easily obtainable, noninvasive examinations that provide useful information in the evaluation, diagnosis and management of patients with dilated cardiomyopathy. The gated blood pool scan allows the assessment of ventricular size, configuration, and wall and septal thickness. These data allow the functional class of the cardiomyopathy (congestive, restrictive or hypertrophic) to be defined. Often Thallium-201 myocardial perfusion imaging adds further information and is particularly useful in distinguishing congestive cardiomyopathy from severe coronary artery disease and in depicting septal abnormalities in hypertrophic cardiomyopathy. Useful as these techniques are, they are not substitutes for conventional approaches to diagnosis. Careful history taking and physical examination, as well as scrutiny of the electrocardiogram, chest X-ray and echocardiogram should be standard practice for the evaluation of patients with suspected cardiomyopathy. Judicious use of noninvasive techniques may obviate the need for cardiac catheterization in many patients.

Adult↗