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Early and aggressive presentation of wild-type transthyretin amyloid cardiomyopathy:A case report

World Journal of Cardiology 2022年第12期14卷 657-664页

作者： ilham boda Hassan Farhoud Tarun Dalia Amandeep Goyal Zubair Shah Andrija Vidic Department of Internal Medicine University of Kansas Medical CenterKansas CityKS 66160United States School of Medicine University of Kansas Medical CenterKansas CityKS 66160United States Department of Cardiovascular Medicine University of Kansas Medical CenterKansas CityKS 66160United States

BACKGROUND Wild-type transthyretin amyloidosis(ATTRwt)is the most common form of transthyretin amyloid cardiomyopathy,occurring mostly over age of 60 years(mean age of 80 years).Mean survival without treatment is 3.6 years,making early detection *** report an unusual case of a 58-year-old patient with ATTRwt cardiomyopathy requiring heart *** SUMMARY A 58-year-old male presented with progressive fatigue,shortness of breath,weight gain,leg swelling,orthopnoea,and paroxysmal nocturnal dyspnoea for several *** ten months before this clinical presentation,the patient had first received a diagnosis of heart failure with reduced ejection fraction(EF)of 15% to 20%.The patient was started on appropriate guidelinedirected medical therapy with only mild improvement in his *** further investigation,echocardiogram,technetium pyrophosphate scan(Tc PYP),and cardiac magnetic resonance imaging(cMRI)suggested a diagnosis of amyloidosis,and ATTRwt was subsequently confirmed with native heart tissue biopsy,congo red staining,liquid chromatography-tandem mass spectrometry,and genetic *** patient was successfully treated with heart transplantation and is doing well *** Wild-type ATTR amyloidosis should be kept on differentials in all patients(even less than 60 years old)with non-ischemic cardiomyopathy,especially in the setting of increased ventricular wall thickness and other classic echocardiogram,cMRI,and Tc PYP *** diagnosis and management can be consequential in improving patient outcomes.

关键词： Wild-type Transthyretin Amyloidosis Young Heart failure Heart transplant Case report

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