intracardiac flow assessment in cardiac amyloidosis

Conditions: Amyloidosis Cardiac; Healthy Adults

Summary: the primary objective of this study is to define the intracardiac flow imaging biomarkers in cardiac amyloidosis.


Eligibility criteria:
inclusion criteria: * subject is clinically stable without cardio-vascular-related hospitalizations within 6 weeks prior to enrollment as assessed by the investigators. * subject is able to provide written informed consent and is willing and able to complete study procedures. * currently in sinus rhythm by clinical assessment or documented electrocardiographic studies. * subject and disease characteristics noted by medical record review: * healthy control volunteers must also meet the following criteria: karnofsky performance scale \> 80%; ecog status 0 or 1. * attr cardiac amyloidosis based on meeting all the following criteria: diagnosis of amyloidosis within five years prior to study screening; documentation of absence of al, heavy chain disease, multiple myeloma or malignant lymphoproliferative disorders; transthyretin amyloid deposits in cardiac tissue or technetium (99mtc) pyrophosphate scintigraphy with grade 2 or 3 cardiac uptake or transthyretin amyloid deposits in non-cardiac tissue with echocardiographic evidence of cardiac involvement or an end-diastolic mean wall thickness \>12mm or transthyretin amyloid deposits in non-cardiac tissue with cmr diagnostic of amyloidosis * al with cardiac involvement based on meeting all the following criteria: diagnosis of amyloidosis within five years prior to study screening; histopathologic diagnosis of amyloidosis with al protein identification; documented clinical signs or symptoms consistent with heart failure; cardiac involvement as defined by: amyloid deposits in cardiac deposits or echocardiography with an end-diastolic mean wall thickness \> 12 mm in the absence of other causes or elevated nt-probnp (\>332 ng/l) in the absence of renal failure or atrial fibrillation or cmr diagnostic of amyloidosis; * al without cardiac involvement based on meeting all the following criteria: diagnosis of amyloidosis within five years prior to study screening; histopathologic diagnosis of amyloidosis with al protein identification; no documented clinical signs and symptoms consistent with heart failure from al; absence of cardiac involvement as defined by: echocardiography with an end-diastolic mean wall thickness \< 13 mm if the subject does not have other causes for increased wall thickness and nt-probnp \<333 ng/l if the subject does not have renal failure or atrial fibrillation and no cmr diagnostic of amyloidosis if cmr is available prior to screening. exclusion criteria: * unable to consent or unable to complete all study procedures. * unable to ambulate for 6 minutes (confirmed at study coordinator visit). * unable to maintain in supine position for 30 minutes. * unable to maintain breath-holding for 10 seconds (confirmed at study coordinator visit). * contraindications for safe cmr scanning (e.g., uncontrolled claustrophobia, cochlear implant, implanted neural stimulator). * presence of implantable cardiac pacemaker or defibrillator. * history of complex congenital heart disease, intracardiac shunt (except for patent foramen ovale), prosthetic valves, prosthesis in the main pulmonary artery or ascending thoracic aorta. * significant artifact from prior mri studies. * pregnant or breast-feeding women. * weight equal to or greater than 155 kg. * maximum body side-to-side or anterior-posterior diameter equal to or greater than 70 cm. * documented non-sinus rhythm within 1 week prior to screening. * for healthy controls, the following exclusion criteria apply, confirmed per chart review and/or patient report: * history of cardiomyopathy or structural heart disease; * history of valvular disease of greater than mild severity; * history of coronary artery disease or coronary heart disease; * history of cardiac or thoracic surgery. * history of symptomatic, persistent atrial tachyarrhythmia, ventricular tachyarrhythmia, or bradyarrhythmia; * left ventricular hypertrophy or abnormally increased myocardial thickness by prior echocardiography, cardiac computed tomography, or cmr; * acute kidney injury, or chronic renal disease with glomerular filtration rate \< 45 ml/min/1.73m\^2 as per medical record review. * uncontrolled hypertension of systolic blood pressure \> 150 mmhg or diastolic blood pressure \> 90 mmhg as per medical record review; * taking three or more anti-hypertensive medications; * type 1 diabetes, or uncontrolled type 2 diabetes mellitus of hemoglobin a1c greater than 8, as per medical record review; * taking three or more diabetic medications; * history of confirmed stroke or transient ischemic attack, as per medical record review; * current cigarette smoker; * history of plasma cell dyscrasia or chronic malignant hematologic diagnosis; * bmi \> 35 kg/m\^2.