Cardiac amyloidosis is an acquired heart disease secondary to the deposition of β-pleated amyloid proteins in heart tissue. Amyloid light chain (AL) amyloidosis is usually secondary to multiple myeloma and can rapidly deteriorate cardiac function, with high mortality. Up to 50% of AL patients have cardiac involvement presenting as heart failure, conduction abnormalities, and cardiomyopathies. One of the rare presentations is the likely simulation of disease with hypertrophic cardiomyopathies like left ventricular outflow tract (LVOT) obstruction due to the systolic anterior motion of the mitral valve and irregular septal hypertrophy secondary to amyloid deposits. We present a case of cardiac amyloidosis secondary to multiple myeloma who presented with dynamic LVOT obstruction resembling hypertrophic obstructive cardiomyopathy and complicated by acute pulmonary edema. These complicated cases can be initially treated for pulmonary edema with an elevation of the head of the bed, furosemide, and nitroglycerin intravenously. For multiple myeloma, chemotherapy was continued. Beta-blockers, calcium channel blockers and angiotensin-converting enzyme inhibitors, and aldosterone receptor blocker were avoided due to poor tolerability. After symptomatic control, the patient can likely be scheduled for septal myotomy and the placement of a pacemaker or implantable cardiac defibrillator to prevent any arrhythmias causing sudden cardiac death in these subsets of patients.
Peripheral artery disease (PAD) is a prevalent disorder in the United States, associated with significant morbidity and mortality. Fractional Flow Reserve (FFR) is a physiological test used to assess the hemodynamic significance of intermediate lesions on conventional angiography. It is well studied in coronary artery disease and is as an important tool to guide decisions regarding revascularization in a significant percentage of patients with intermediate lesions. As compared to coronary FFR, the use of FFR in peripheral artery disease (PFFR) is much less prevalent. Overall data regarding the use of the PFFR is sparse. There are limited studies that have shown the correlation of PFFR with non-invasive testing including ankle-brachial index (ABI) and Doppler Imaging. Unlike coronary FFR, the optimal pharmaceutical agents and doses to induce maximal hyperemia in the peripheral vascular bed are also not well established. Moreover, there are no established standardized procedural protocols for measuring PFFR. Various studies have employed varying techniques, hyperemic agents and doses. The aim of this literature review is to summarize the current evidence on PFFR, the correlation with noninvasive studies used in PAD and to increase awareness of the potential role of the PFFR in peripheral interventions.
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