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11/33. Arrhythmogenic right ventricular cardiomyopathy and atrial right-to-left shunt.

    A 47-year-old woman diagnosed with arrhythmogenic right ventricular cardiomyopathy, was studied at our institution with palpitations, progressive dyspnoea and cyanosis over a 3-year period. Her haemoglobin saturation was 76.1%. The transthoracic echocardiogram showed that both right atrium and ventricle were dilated, with diffuse akinetic and hypokinetic areas. Radionuclide scintigraphy, angiography and magnetic resonance imaging showed dilated and severe dysfunction of the right ventricle, with depressed ejection fraction (0.16), and bulging at the level of the right ventricular anterior wall areas. Doppler examination revealed a right-to-left interatrial shunt through a patent foramen ovale. Contrast transoesophageal echocardiography confirmed the right-to-left shunting. Considering the clinical symptoms were severe and refractory to medical treatment, she was referred for cardiac transplantation. Unfortunately the patient died suddenly a few weeks later.
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ranking = 1
keywords = arrhythmogenic right ventricular cardiomyopathy, right ventricular cardiomyopathy, arrhythmogenic, arrhythmogenic right, ventricular cardiomyopathy, cardiomyopathy
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12/33. Cardiac sarcoidosis mimicking right ventricular dysplasia.

    A 59-year-old woman with skin sarcoidosis was admitted to hospital for assessment of complete atrioventricular block. Cross-sectional echocardiography showed that the apical free wall of the right ventricle was thin and dyskinetic with dilation of the right ventricle. thallium-201 myocardial imaging revealed a normal distribution. Both gallium-67 and technetium-99m pyrophosphate scintigraphy revealed no abnormal uptake in the myocardium. Right ventriculography showed chamber dilation and dyskinesis of the apical free wall, whereas left ventriculography showed normokinesis, mimicking right ventricular dysplasia. Cardiac sarcoidosis was diagnosed on examination of an endomyocardial biopsy specimen from the right ventricle. A permanent pacemaker was implanted to manage the complete atrioventricular block. After steroid treatment, electrocardiography showed first-degree atrioventricular block and echocardiography revealed an improvement in the right ventricular chamber dilation. Reports of cardiac sarcoidosis mimicking right ventricular dysplasia are extremely rare and as this case shows, right ventricular involvement may be one of its manifestations.
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ranking = 3.5491247967614
keywords = ventricular dysplasia, right ventricular dysplasia, dysplasia
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13/33. Noncompaction of the ventricular myocardium: report of two cases with bicuspid aortic valve demonstrating poor prognosis and with prominent right ventricular involvement.

    Noncompaction of the ventricular myocardium is a rare, unclassified cardiomyopathy due to an arrest of myocardial morphogenesis. The characteristic echocardiographic findings consist of multiple, prominent myocardial trabeculations and deep intertrabecular spaces communicating with the left ventricular (LV) cavity. The disease typically involves the LV myocardium, but right ventricular (RV) involvement is not uncommon. The clinical manifestations include heart failure (HF) signs, ventricular arrhythmias and cardioembolic events. Noncompacted myocardium may occur as an isolated cardiac lesion, as well as it can be in association with congenital anomalies. We describe two illustrative cases of noncompaction of the ventricular myocardium, a 19-year-old male with bicuspid aortic valve and progressive worsening of HF, and a 61-year-old male with marked RV involvement in addition to LV apical involvement, both with the typical clinical and echocardiographic features of the disease.
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ranking = 0.0001496660542393
keywords = cardiomyopathy
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14/33. Reversal of refractory congestive heart failure after thiamine supplementation: report of a case and review of literature.

    patients with refractory heart failure comprise a very important subgroup of patients with congestive heart failure. Before assuming that this condition simply reflects advanced, perhaps terminal, myocardial dysfunction, potentially reversible factors should be sought carefully. We describe a 58-year-old Hispanic man with a diagnosis of idiopathic dilated cardiomyopathy who presented with symptoms of severe congestive heart failure, glossitis, and peripheral neuropathy. His hemodynamic profile was characterized by refractory low-output cardiac failure and decreased vascular resistance. thiamine deficiency was documented by a high thiamine pyrophosphate effect. His clinical condition was quickly reversed with thiamine administration. This response to thiamine administration supports the diagnosis and indicates that thiamine deficiency may play an important etiologic role in the deterioration of cardiac function in some patients with congestive heart failure.
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ranking = 0.0001496660542393
keywords = cardiomyopathy
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15/33. Misdiagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy.

    INTRODUCTION: diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) has major implications for the management of patients and their first-degree relatives. diagnosis is based on a set of criteria proposed by the International Task Force for cardiomyopathies. We report our experience in providing a re-evaluation for patients who previously have been diagnosed with ARVD/C. methods AND RESULTS: We studied 89 patients who requested a re-evaluation for diagnosis of ARVD/C at our center. Each of these patients had been diagnosed with ARVD/C at their initial evaluation. Each patient was re-evaluated with clinical history, physical examination, and noninvasive testing at our center. Invasive testing, which included electrophysiologic testing, right ventricular angiography, and endomyocardial biopsy, was performed when clinically indicated. Sixty (92%) of the 65 patients who had undergone magnetic resonance imaging (MRI) at an outside institution were reported to have an abnormal MRI consistent with ARVD/C. Among these patients, the only abnormality identified was the qualitative finding of intramyocardial fat/wall thinning in 46 patients. On re-evaluation, these qualitative findings were not confirmed. None of these 46 patients ultimately were diagnosed with ARVD/C. Among the entire patient group, only 24 (27%) of the 89 patients met the Task Force criteria for ARVD/C. CONCLUSION: This study demonstrates that the high frequency of "misdiagnosis" of ARVD/C is due to over-reliance on the presence of intramyocardial fat/wall thinning on MRI, incomplete diagnostic testing, and lack of awareness of the Task Force criteria. diagnosis of ARVD/C cannot rely solely upon qualitative features on MRI.
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ranking = 3.928604804762
keywords = ventricular dysplasia, right ventricular dysplasia, arrhythmogenic, arrhythmogenic right, arrhythmogenic right ventricular dysplasia, cardiomyopathy, dysplasia
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16/33. Isolated right ventricular failure in scleroderma heart disease.

    Scleroderma is reported to have numerous cardiac manifestations. Right ventricular failure (RVF) is a well-recognized cardiac complication of scleroderma and most often is related to underlying pulmonary hypertension (PH). Causes of PH include both interstitial lung disease and pulmonary artery vasculopathy. Direct involvement of the ventricle by myocardial fibrosis or coronary vasospasm could also cause a diffuse bilateral cardiomyopathy. We describe a case of predominant RVF in the absence of significant PH in a patient with longstanding scleroderma.
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ranking = 0.0001496660542393
keywords = cardiomyopathy
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17/33. Detection of early systolic dysfunction with strain rate imaging in a patient with light chain cardiomyopathy.

    Congestive heart failure (CHF) in cardiac amyloidosis has been attributed to the development of diastolic dysfunction, because severe CHF symptoms have been observed despite a normal or only mildly reduced LV ejection fraction (EF). An early impairment of longitudinal systolic function has been described by means of tissue Doppler-derived myocardial deformation imaging ('strain rate imaging', SRI).Our patient presented with signs of CHF and significantly increased brain-natriuretic peptide (BNP) levels. Conventional measures of systolic contractile function were within the normal range and mitral inflow Doppler showed only moderate diastolic dysfunction. Histopathological examination by endomyocardial biopsy revealed interstitial deposition of amyloid fibers. Quantitative assessment of myocardial velocities (TDI) and deformation properties (Strain) from the apical view demonstrated a significant impairement of systolic longitudinal myocardial function.In patients with isolated diastolic dysfunction detected by conventional Doppler echocardiography, the quantitative assessment of myocardial strain and strain rate can be helpful in the early detection of systolic dysfunction.
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ranking = 0.00059866421695721
keywords = cardiomyopathy
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18/33. arrhythmogenic right ventricular dysplasia presenting as right ventricular outflow tract tachycardia.

    A case of a 51-year old male is presented. A left bundle branch block inferior axis tachycardia was manifest. At electrophysiological study this tachycardia was inducible and was ablated in the septal right ventricular outflow tract (RVOT). Two other tachycardias were identified both with right bundle branch block (RBBB) morphology raising the suspicion of diffuse pathology. Arrythmogenic right ventricular dysplasia (ARVD) was confirmed by right ventricular angiography and magnetic resonance imaging (MRI). An implantable cardioverter defibrillator (ICD) was implanted and an appropriate shock was later delivered.
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ranking = 2.9576039973012
keywords = ventricular dysplasia, right ventricular dysplasia, dysplasia
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19/33. Right ventricular noncompaction in a neonate with complex congenital heart disease.

    Ventricular noncompaction is a rare unclassified cardiomyopathy occurring because of arrest of the normal intrauterine compaction of the loose luminal component of the ventricular myocardium. There is limited data regarding its diagnosis and outcome in children. It is recognised, however, that right ventricular involvement is extremely rare. We report a case in which only the right ventricular myocardium was noncompacted, a situation which led to heart failure soon after birth.
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ranking = 0.0001496660542393
keywords = cardiomyopathy
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20/33. Reversible right ventricular dysfunction in patients with hiv infection.

    Human immunodeficiency virus-related cardiomyopathy is characterized by global left ventricular (LV) dysfunction commonly associated with biventricular dilation. Human immunodeficiency virus (hiv) cardiomyopathy carries a poor prognosis, and the role of antiretroviral therapy in the reversal of heart failure is not very clear. We report two patients with hiv infection who presented with severe right ventricular (RV) dysfunction in the absence of pulmonary parenchymal, pulmonary arterial and left ventricular myocardial involvement. During the period of intensive antiretroviral therapy, the symptoms of right heart failure progressively and remarkably improved. This was accompanied by normalization of right ventricular size and RV function documented by repeat echocardiograms. Given that the serologic tests for opportunistic infections were negative, and the RV function improvement correlated with a decrement in the viral load, it is likely that the cardiomyopathy was due to direct infection by hiv. These cases illustrate that there can be isolated involvement of the right heart in the absence of lung, significant pulmonary vascular and left ventricular disease, and also that the antiretroviral therapy might reverse the cardiomyopathy.
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ranking = 0.00059866421695721
keywords = cardiomyopathy
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