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PubMed2006

Pitfalls in the diagnosis of left ventricular hypertrabeculation/non-compaction.

Left ventricular hypertrabeculation/non-compaction (LVHT) is a cardiac abnormality, characterised by >3 trabeculations apically to the papillary muscles and intertrabecular spaces. LVHT may occur with other cardiac abnormalities, heart failure, electrocardiographic abnormalities and neuromuscular disorders. This study gives an overview about (1) patients with LVHT in whom LVHT was initially overlooked and (2) cardiac conditions that may lead to falsely diagnosed LVHT. In 50 reported cases, LVHT has been overlooked and misdiagnosed as dilated (n = 20), hypertrophic (n = 14) or restrictive cardiomyopathy (n = 2), endocardial fibroelastosis (n = 5), endomyocardial fibrosis (n = 1), myocarditis (n = 3), thrombus (n = 2), localised left ventricular hypertrophy (n = 1), left ventricular mass (n = 1) or myocardial/pericardial disease (n = 1). In 14 patients, LVHT was diagnosed only by transoesophageal echocardiography (n = 1), computed tomography (n = 2) ventriculography (n = 2), magnetic resonance imaging (n = 3) or pathoanatomic findings (n = 6). Falsely diagnosed LVHT comprises false tendons, aberrant bands, thrombi, apical hypertrophic cardiomyopathy, fibroma, obliterative processes, intramyocardial haematoma, cardiac metastases and intramyocardial abscesses. Echocardiographers should be more aware of LVHT and consider its differential diagnoses.

Diagnostic Errors↗
PubMed1999

Evaluation of the left ventricular systolic and diastolic functions by echocardiography in patients with acute leukemia.

Eighteen patients with acute leukemia were studied to evaluate their left ventricular systolic and diastolic function by M mode, two-dimensional and Doppler echocardiographic techniques. The study group was compared with another group of 18 patients of similar age with iron deficiency anemia. The latter group had a comparable heart rate and levels of Hb. There were no differences in systolic function, whereas the diastolic function was lower in patients with acute leukemia (E/A ratio = 1.1 +/- 0.3 vs. 1.42 +/- 0.5, p < 0.05). It is concluded that left ventricular diastolic dysfunction is more common in patients with acute leukemia and may be an indicator of restrictive cardiomyopathy.

Acute Disease↗
PubMed2003

Protein kinase Calpha negatively regulates systolic and diastolic function in pathological hypertrophy.

The protein kinase C (PKC) family is implicated in cardiac hypertrophy, contractile failure, and beta-adrenergic receptor (betaAR) dysfunction. Herein, we describe the effects of gain- and loss-of-PKCalpha function using transgenic expression of conventional PKC isoform translocation modifiers. In contrast to previously studied PKC isoforms, activation of PKCalpha failed to induce cardiac hypertrophy, but instead caused betaAR insensitivity and ventricular dysfunction. PKCalpha inhibition had opposite effects. Because PKCalpha is upregulated in human and experimental cardiac hypertrophy and failure, its effects were also assessed in the context of the Galphaq overexpression model (in which PKCalpha is transcriptionally upregulated). Normalization (inhibition) of PKCalpha activity in Galpha(q) hearts improved systolic and diastolic function, whereas further activation of PKCalpha caused a lethal restrictive cardiomyopathy with marked interstitial fibrosis. These results define pathological roles for PKCalpha as a negative regulator of ventricular systolic and diastolic function.

Adrenergic beta-Agonists↗
PubMed1984

Phonomechanocardiographic findings in endomyocardial fibrosis.

Endomyocardial fibrosis (EMF) is a severe restrictive cardiomyopathy characterized by the formation of fibrous tissue on the endocardium and to a lesser extent in the myocardium of one or both ventricles. The disease results in endocardial rigidity, atrioventricular valve dysfunction and progressive obliteration of the ventricular cavity. The clinical and pathological findings of EMF have been well established and definitive diagnosis can be made by selective cineangiocardiography. However, there are few reports concerning the phonomechanocardiographic (PMCG) patterns in this condition. In this paper we describe the PMCG findings in 12 patients with EMF which is now recognized with increasing frequency in the Northeast of Brazil.

Adult↗
PubMed2006

Extensive myocardial infiltration by hemopoietic precursors in a patient with myelodysplastic syndrome.

BACKGROUND: Although myocardial infiltration with leukemic blasts is a known finding in patients with acute leukemia, this phenomenon in myelodysplasia is not reported in the literature. Cardiac symptoms in patients with myelodysplasia are often due to anemia and may be due to iron overload and side effects of therapy. CASE PRESENTATION: Herein we report the first case of neoplastic infiltration of the heart with associated myocardial necrosis in a patient with myelodysplasia. It was associated with unicellular and multifocal geographic areas of necrosis in the left ventricle and the interventricular septum. It is likely that cardiac compromise in our patient was due to a combination of restrictive cardiomyopathy due to leukemic infiltration, concomitant anemia, cardiac dilatation, conduction blocks and myocardial necrosis. Myocardial necrosis was most likely due to a combination of ischemic damage secondary to anemia and prolonged hypotension and extensive leukemic infiltration. Markedly rapid decrease in ejection fraction from 66% to 33% also suggests the role of ischemia, since leukemic infiltration is not expected to cause this degree of systolic dysfunction over a 24-hour period. The diagnosis was not suspected during life due to concomitant signs and symptoms of anemia, pulmonary infections, and pericardial and pleural effusions. The patient succumbed to cardiac failure. CONCLUSION: Hemopoietic cell infiltration was not considered in the differential diagnosis and contributed to this patient's morbidity and mortality. This case highlights the clinical importance of considering myocardial infiltration in patients with myelodysplasia and cardiac symptoms.

Journal Article↗
PubMed2005

Chronic progressive cardiac dysfunction years after doxorubicin therapy for childhood acute lymphoblastic leukemia.

PURPOSE: Cross-sectional studies show that cardiac abnormalities are common in long-term survivors of doxorubicin-treated childhood malignancies. Longitudinal data, however, are rare. METHODS: Serial echocardiograms (N = 499) were obtained from 115 doxorubicin-treated long-term survivors of childhood acute lymphoblastic leukemia (median age at diagnosis, 4.8 years; median follow-up after completion of doxorubicin, 11.8 years). Results were expressed as z scores to indicate the number of standard deviations (SDs) above (+) or below (-) the normal predicted value. Median individual and cumulative doxorubicin doses were 30 mg/m2 per dose and 352 mg/m2, respectively. RESULTS: Left ventricular fractional shortening was significantly reduced after doxorubicin therapy, and the reduction was related to cumulative dose. z scores for fractional shortening transiently improved before falling to -2.76 more than 12 years after diagnosis. Reduced fractional shortening was related to impaired contractility and increasing afterload, consequences of a progressive reduction of ventricular mass, and wall thickness relative to body-surface area. Left ventricular contractility fell significantly over time and was depressed at last follow-up in patients receiving more than 300 mg/m2 of doxorubicin. Systolic and diastolic blood pressures were below normal more than 9 years after diagnosis. Even patients receiving lower cumulative doxorubicin doses experienced reduced mass and dimension. Fractional shortening and dimension at the end of therapy predicted these parameters 11.8 years later. CONCLUSION: Cardiac abnormalities were persistent and progressive after doxorubicin therapy. Inadequate ventricular mass with chronic afterload excess was associated with progressive contractile deficit and possibly reduced cardiac output and restrictive cardiomyopathy. The deficits were worst after highest cumulative doses of doxorubicin, but appeared even after low doses.

Adolescent↗
PubMed1984

Ultrastructural features of the endomyocardium in patients with eosinophilic heart disease. An endomyocardial biopsy study.

In a series of studies with endomyocardial biopsy, 7 adult cases with cardiac disease and eosinophilia were studied clinically and electro-microscopy. Degranulation of the eosinophils in the peripheral blood was observed ultrastructurally in 3 of the 4 cases studied. The clinical expression of the 7 patients were, restrictive cardiomyopathy in 2, dilated cardiomyopathy in 2 and sick sinus syndrome in 1 and others in 2. Endocardial thickening was observed in 5 cases, one of whom showed a marked cellular infiltration with macrophages, plasma cells, lymphocytes and mast cells. One other case showed cell debris of degranulated eosinophils. Degeneration of the myocytes was manifested by an increase in Z-bands of the myofibrils as well as streaming (1 case), disarrangement of the myofibrils, and mitochondrial change (1 case) which was characterized by giant mitochondria (1 case) as well as by a numerical increase in mitochondria. Increase of atrial granules and mitochondria in a right atrial biopsy of a case with sick sinus syndrome was noteworthy. Basal lamina layering of the capillaries of the myocardium and pyknosis of an endothelial cell cytoplasm were also noted in each case. These observations may reveal that various disease processes are taking place in the endocardium and adjacent myocardium.

Adult↗
PubMed1986

Histological findings of the right and left ventricular myocardium and clinical follow up in idiopathic ventricular tachycardia.

In order to evaluate the etiology of so-called idiopathic ventricular tachycardia, endomyocardial biopsies were performed in four patients with electrocardiographically documented recurrent and sustained ventricular tachycardia. During the episodes of ventricular tachycardia, standard ECG showed a QRS pattern of right bundle branch block with left axis deviation in two patients and left bundle branch block in two patients. The episodes were associated with palpitation, dyspnea and hypotension in all cases. No organic heart disease was detected by physical examination, chest X-ray films, echocardiograms, left ventriculograms or coronary cineangiograms. His bundle electrograms showed blocks at various sites in the atrioventricular conduction system. The biopsy specimens revealed nonspecific myocardial degeneration in the right and left ventricles. These findings suggest mild but wide-spread myocardial damage in both the working myocardium and the conduction system. The clinical course of these patients appeared benign according to follow-up data of one to nine years' duration. None developed overt clinical signs of dilated, hypertrophic or restrictive cardiomyopathy.

Adult↗
PubMed1993

Left ventricular systolic dysfunction precipitated by verapamil in cardiac amyloidosis.

Cardiac amyloidosis produces a restrictive cardiomyopathy with impaired diastolic function. We report a case in which low-dose verapamil resulted in marked worsening of congestive heart failure, as a result of a profound negative inotropic effect. Withdrawal of verapamil therapy demonstrated a return of systolic function to normal with improvement in heart failure. We postulate that patients with cardiac amyloidosis may be exceptionally sensitive to the negative inotropic effects of calcium-channel blockers either because of abnormal binding to amyloid fibrils or because their usual vasodilator effects are blunted.

Amyloidosis↗
PubMed2005

Pitfalls in diagnosis and clinical, echocardiographic, and hemodynamic findings in endomyocardial fibrosis: a 25-year experience.

Endomyocardial fibrosis (EMF) is a fascinating disease entity of unknown etiology. It is prevalent in the tropical zone. Its essential features are the formation of fibrous tissue on the endocardium and to a lesser extent in the myocardium of the inflow tract and apex of one or both ventricles. It results in endocardial rigidity, atrioventricular valve incompetence secondary to papillary muscle involvement, and progressive reduction of the cavity of the involved ventricle leading to restriction in filling and atrial enlargement. This article will present 21 patients with EMF who were initially referred to our hospital from 1979 to 2004 with different diagnoses: rheumatic heart disease with mitral and or tricuspid regurgitation (n = 9), constrictive pericarditis (n = 6), restrictive cardiomyopathy (n = 1), hypertrophic cardiomyopathy apical type (n = 2), dilated cardiomyopathy (n = 2), and Ebstein malfunction of the tricuspid valve (n = 1). The clinical, echocardiographic, hemodynamic, and angiographic findings in these 21 patients are presented; echocardiographic findings lead to the right diagnosis. The presence of a small ventricle with obliteration of the apex and large atrium shown on two-dimensional echocardiography is highly suggestive of EMF.

Adolescent↗
PubMed1981

Motion of pulmonic valve and constrictive pericarditis.

Extreme respiratory variation in the depth of the "a" wave of the pulmonic valve echo was demonstrated in a patient with constrictive pericarditis; a mechanism for this finding is offered. Disparity in systolic and diastolic ventricular function in constriction is also useful in ruling out restrictive cardiomyopathy.

Aged↗
PubMed1986

Utility of endomyocardial biopsy in the diagnosis of cardiac sarcoidosis.

Cardiac involvement in sarcoidosis can be demonstrated in about 25 percent of autopsied cases, but antemortem diagnosis is uncommon. To evaluate the usefulness of the endomyocardial biopsy in detecting cardiac sarcoid disease, the medical records of ten patients with sarcoidosis who underwent endomyocardial biopsy for routine clinical indications were reviewed. The patients fell into two groups: patients with known sarcoidosis and presumed cardiac involvement (n = 8), and patients in whom the biopsy finding of sarcoid disease was unexpected (n = 2). Four patients in the first group had positive endomyocardial biopsy results (granulomas and/or marked mononuclear cell infiltrate) and were treated with glucocorticoid therapy with improvement in three; the fourth was disabled with lung disease. The diagnoses of three other patients were revised on the basis of the biopsy results; their therapy was tailored accordingly. The remaining patient may represent a false-negative biopsy result, based on clinical criteria. The two patients in the second group presented with symptomatic ventricular tachycardia and restrictive cardiomyopathy respectively, and in neither case was sarcoidosis considered prior to biopsy results. Overall, a change in treatment strategy based on biopsy results occurred in eight of ten cases. Thus, endomyocardial biopsy is useful for the diagnosis of cardiac sarcoidosis; treatment strategies may be affected by biopsy findings; and rarely, endomyocardial biopsy can provide the first clinical evidence of sarcoid disease that is otherwise occult.

Adrenal Cortex Hormones↗
PubMed2000

Selenium supplementation of children in a selenium-deficient area in China: blood selenium levels and glutathione peroxidase activities.

Keshan disease is a cardiomyopathy restricted to the endemic areas of China and seen in residents having an extremely low selenium (Se) status. Prophylactic administration of sodium selenite has been shown to decrease significantly the incidence of acute and subacute cases. The aim offthe study was to assess the relative bioavailability of selenite versus organic Se-yeast in a Se-deficient area in China with a randomized double-blind double-dummy design. Healthy children (n=30) between 14 and 16 yr of age were randomized into three equal groups receiving either 200 microg/d selenite Se or 200 microg/d Se-yeast or placebo for 12 wk. Blood was drawn at baseline, 4, 8, and 12 wk and 4 wk postsupplementation. The plasma Se concentration (mean +/- SD) was 0.16+/-0.03 micromol/L at baseline. Selenite and Se-yeast supplementation increased plasma Se to plateau values, 1.0+/-0.2 and 1.3+/-0.2 micromol/L, respectively. In red cells, Se-yeast increased the selenium level sixfold and selenite threefold compared to placebo. The relative bioavailability of Se-yeast versus selenite measured as glutathione peroxidase (GSHPx) activity was similar in plasma, red blood cells, and platelets. GSHPx activity reached maximal levels in plasma and platelets of 300% and 200%, respectively, after 8 wk compared to the placebo group, but continued to increase in red cells for 16 wk. Our study showed that although both forms of Se were equally effective in raising GSHPx activity, Se-yeast provided a longer lasting body pool of Se. Se-yeast may be a better alternative to selenite in the prophylaxis of Keshan disease with respect to building up of body stores.

Adolescent↗
PubMed1991

Endomyocardial biopsy in children. Usefulness in various myocardial disorders.

Endomyocardial biopsy studies in adults have demonstrated the usefulness of this method. It is possible that studies will be more productive in determining the etiology and clinical status in patients with clinically diagnosed myocardial diseases. A prospective study conducted over 16 months included 17 children, aged 14 months to 18 years, with the diagnosis of dilated, restrictive cardiomyopathy and myocarditis. In 16 patients right, and in 1 patient left heart endomyocardial biopsies were performed. The specimens were evaluated by light and electron microscopy. There were no serious complications after the procedure. In 1 of 17 children histology showed no myocardial tissue. Electron microscopy evaluations were currently available in 9 patients. Endomyocardial biopsy findings were found to be diagnostic in 41.2%, helpful in 29.4% and of no help in 29.4% of patients. In conclusion, endomyocardial biopsy technique is highly sensitive in children with myocardial disorders. In future it will be the major diagnostic tool for invasive but safe detection of myocardial disease.

Adolescent↗
PubMed2005

[Biventricular endomyocardiofibrosis associated with renal amyloidosis].

Endomyocardiofibrosis is a restrictive cardiomyopathy characterized by fibrotic involvement of the endocardium and adjacent myocardium, and by diastolic dysfunction caused by changes in distensibility making ventricular filling inadequate while preserving the systolic function. Clinically, it appears as heart failure, but etiological symptomatic discernment, suspicion and a clinical examination would be necessary in order to make a correct etiological diagnosis. The case of a patient with biventricular endomyocardial fibrosis associated with renal amyloidosis is presented.

Amyloidosis↗
PubMedDate not supplied

[A historical perspective of tissue Doppler--when the starlight illuminates the myocardial function].

In 1842, an Austrian professor of mathematics and practical geometry, Christian Doppler, presented results of his investigations in the field of astronomy, without any clue that they would become important principles of modern ultrasound diagnostics. Almost a century later, at the University of Osaka, Japan, S. Satomura first applied these principles to measure the blood flow velocities in peripheral and extracranial brain-supplying vessels. The father of tissue Doppler imaging is Karl Isaaz, a French scientist, who was the first to realize the importance of clinical and diagnostic potentials of tissue Doppler imaging. Today, this noninvasive echocardiographic method is widely used in evaluation of myocardial function, especially in diagnosing of diastolic heart failure and early diagnosis of coronary artery disease, but also in diffrential diagnosis of restrictive cardiomyopathy and constrictive pericarditis and aberrant myocardial conduction. It is also an antecedent of "stain rate", a new sensitive method for diagnosing myocardial diseases.

Astronomy↗
PubMed2000

Heart failure caused by a novel amyloidogenic mutation of the transthyretin gene: ATTR Ala45Ser.

Cardiac failure in transthyretin (TTR) amyloidosis patients has been shown to be caused by different mutations in the TTR gene. In the present case, a 73-year-old man from Northern Sweden was evaluated for heart failure. Amyloid deposits were found in subcutaneous fat and in intestinal biopsies. The presence of a variant form of TTR was detected in the plasma by electrospray ionisation mass spectrometry (ESI-MS). The mutation was located by single-strand conformation polymorphism (SSCP) analysis of the TTR gene where a band shift was seen in exon 2. Direct sequencing of exon 2 revealed a single base-pair substitution (G1724T). This transversion results in an amino acid substitution at codon 45, alanine to serine (ATTR Ala45Ser). Mass spectrometry analysis excluded that the variant is a polymorphism, since no similar shift in molecular weight has been present in more than 200 control samples. Congo red and immunostaining of duodenum biopsy specimens confirmed the presence of systemic ATTR amyloidosis, and clinical examination, including echocardiography, found evidence of a restrictive cardiomyopathy. He had 10 years previously been operated for a bilateral carpal tunnel syndrome, but otherwise no symptoms were present that could be attributed to his systemic amyloidosis. No axonal polyneuropathy was noted at nerve conduction studies. This novel mutation is the second amyloidogenic TTR mutation found in the Swedish population.

Aged↗
PubMed2002

Transthyretin Thr60Ala Appalachian-type mutation in a Japanese family with familial amyloidotic polyneuropathy.

A Japanese case with familial amyloidotic polyneuropathy (FAP) associated with the transthyretin mutation Thr60Ala (Appalachian-type mutation) is described This is the first reported case of a non-Caucasian harboring this type of TTR mutation. The patient developed severe late-onset restrictive cardiomyopathy as well as sensorimotor and autonomic polyneuropathy, which were essentially similar to the previously reported clinical pictures of Appalachian-type FAP.

Aged↗