Rationale:Cardiac amyloidosis, considered for the last years to be a rare disease, is one of the determinants of HFpEF. The non-specific clinical presentation and the difficulties related to endomyocardial biopsy have made cardiac amyloidosis an underdiagnosed clinical entity. Improvement of non-invasive diagnostic techniques and the development of new therapies increased clinical awareness for this form of restrictive cardiomyopathy. We here summarize echocardiography and 99mTc-HDP scintigraphy findings in 6 cases of cardiac amyloidosis and review the literature data of this progressive and fatal cardiomyopathy.Patients concerns:The main clinical manifestations were fatigue, low exercise tolerance and edemas. The right heart failure symptoms usually dominated the clinical picture.Diagnoses:All cases were evaluated by echocardiography; 3 cases were further examined by bone scintigraphy and 4 cases a peripheral biopsy was performed. Electrocardiography showed low-voltage QRS complexes and “pseudo-infarct” pattern in the precordial leads, contrary to the echocardiographic aspect, which revealed thickening of ventricle walls. Biatrial dilation and diastolic disfunction were observed. Impaired systolic function was detected in advanced stages of the disease. 99mTc-HDP scintigraphy revealed cardiac uptake of radiopharmaceutical and managed to confirm the diagnosis in 1 case of cardiac amyloidosis in which salivary gland biopsy was negative.Interventions:The treatment was based on managing fluid balance, with the mainstream therapy represented by diuretics. Neurohormonal agents, usually used in heart failure treatment were avoided, due to poor tolerance and worsening of disease course. The management of these 6 cases was challenging due to the refractory manifestation of congestive heart failure.Outcomes:During follow-up, 4 of the 6 patients from the current study died in the first year after the final diagnosis was established.Lessons:Nuclear imaging of cardiac amyloidosis has a revolutionary development nowadays. Bone scintigraphy presents promising results for identifying patients at early stages of disease and to differentiate between cardiac amyloidosis types. Further studies are necessary for the standardization of imaging protocol and development of non-invasive diagnostic tools, especially in assessing the response to treatment and disease progression, for which little is known.
Alcoholic cardiomyopathy is one of the main causes of dilated cardiomyopathy. The aim of this study was to review biochemical and electrocardiographic parameters on a group of patients diagnosed with alcoholic cardiomyopathy in order to see which correlates with a poor outcome. The analysis showed different degrees of hyponatremia, without a statistically significant correlation to outcome. We did not find a positive connection between the impaired renal function and severe evolution. On the other hand, LBBB presence was strongly correlated with the poor outcome (p[0.00001). We emphasize the importance of alcoholic dilated cardiomyopathy proportions in the study group and the prognostic value of LBBB presence for negative outcome.
Treatment of patients with immune thrombocytopenic purpura (ITP) associated with recurrent venous and arterial thrombosis can represent a major challenge. We present the rare case of a 56-year-old female who was first diagnosed with severe ITP at the age of 36. She required corticosteroid therapy and splenectomy in evolution. However, in the last three years she had several episodes of recurrent venous thromboembolism for which she required different anticoagulant therapies despite severe thrombocythemia. The patient also developed acute myocardial infarction treated by primary percutaneous coronary intervention that was complicated with acute intrastent thrombosis. Thus, maximal antiplatelet therapy was mandatory. For ITP, the patient received intravenous steroids, platelet transfusion as well as eltrombopag. Moreover, the patient also suffered from a haemorrhagic uterine fibroma that required surgery. Thus, a close multidisciplinary approach was needed for the successful treatment of this patient.
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