Background: Patients with aortic stenosis (AS) may develop heart failure even in the absence of severe valve stenosis. Our aim was to assess the contribution of systemic arterial properties and the global left ventricular afterload to graded heart failure symptoms in AS.Methods: We retrospectively reviewed medical records of 157 consecutive subjects (mean age, 71±10 years; 79 women and 78 men) hospitalized owing to moderate-to-severe degenerative AS. Exclusion criteria included more than mild aortic insufficiency or disease of another valve, atrial fibrillation, coronary artery disease, severe respiratory disease or anemia. Heart failure symptoms were graded by NYHA class at admission. Systemic arterial compliance (SAC) and valvulo-arterial impedance (Zva) were derived from routine echocardiography and blood pressure.Results: Sixty-one patients were asymptomatic, 49 presented mild (NYHA II) and 47 moderate-to-severe (NYHA III-IV) heart failure symptoms. Mild symptoms were associated with lower SAC and transvalvular gradients, while more severe exercise intolerance coincided with older age, lower systolic blood pressure, smaller aortic valve area and depressed ejection fraction. By multiple ordinal logistic regression, the severity of heart failure symptoms was related to older age, depressed ejection fraction and lower SAC. Each decrease in SAC by 0.1 ml/m² per mmHg was associated with an increased adjusted odds ratio (OR) of a patient being in one higher category of heart failure symptoms graded as no symptoms, mild exercise intolerance and advanced exercise intolerance (OR: 1.16 [95% CI, 1.01-1.35], P=0.045).Conclusions: Depressed SAC may enhance exercise intolerance irrespective of stenosis severity or left ventricular systolic function in moderate-to-severe AS. This finding supports the importance of non-valvular factors for symptomatic status in AS.
Primary systemic amyloidosis (AL amyloidosis) is the most common subtype of amyloidosis in developed countries. Amyloid fibrils deposition results from an abnormal secondary structure of immunoglobulin light chains produced by a plasma cell clone. The most common accompanying plasma cell dyscrasia is monoclonal gammopathy of undetermined significance, while multiple myeloma coexists in only 10-15% of patients. The kidneys and the heart are the most frequently affected organs. Patients usually present with concentric left ventricular concentric thickening displaying a restrictive filling pattern with well-preserved systolic function. Clinical suspicion of AL amyloidosis should be raised in older adults and elderly patients with diastolic heart failure accompanied by heavy proteinuria, upon detection of thick-walled heart on echocardiography with low-voltage QRS on ECG ("red-flags" for amyloidosis"), in non-diabetic subjects with peripheral neuropathy or autonomic neuropathy, commonly with severe postural hypotension. Classical clinical stigmata, i.e. periorbital purpura, macroglossia, carpal tunnel syndrome, are not frequent (10-20%) but can guide diagnosis. In the presence of typical echocardiographic features recommended diagnostic steps include identification of monoclonal gammopathy (serum and urine immunofixation and serum free light-chain kappa to lambda ratio) and confirmation of amyloid deposition, preferentially in a non-cardiac tissue such as periumbilical fat or minor salivary glands. Bone marrow biopsy, serum calcium assay and skeletal survey are mandatory to exclude multiple myeloma. Prognosis is mainly dependent on cardiac involvement, being determined by cardiac biomarkers and the difference between involved and uninvolved light chains. Intensive chemotherapy is the therapy of choice in intermediate-risk patients with AL amyloidosis. An improved overall survival was reported in patients with a complete haematological response and an adequate cardiac response, especially a fall in circulating levels of B-type natriuretic peptides.
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