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 ...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 imperative.We report an unusual case of a 58-year-old patient with ATTRwt cardiomyopathy requiring heart transplantation.CASE SUMMARY A 58-year-old male presented with progressive fatigue,shortness of breath,weight gain,leg swelling,orthopnoea,and paroxysmal nocturnal dyspnoea for several months.Approximately 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 EF.Upon 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 testing.The patient was successfully treated with heart transplantation and is doing well post-transplant.CONCLUSION 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 findings.Early diagnosis and management can be consequential in improving patient outcomes.展开更多
BACKGROUND Q fever myocarditis is a rare disease manifestation of Q fever infection caused by Coxiella burnetii.It is associated with significant morbidity and mortality if left untreated.Prior studies have reported m...BACKGROUND Q fever myocarditis is a rare disease manifestation of Q fever infection caused by Coxiella burnetii.It is associated with significant morbidity and mortality if left untreated.Prior studies have reported myocarditis in patients with acute Q fever.We present the first case of chronic myocarditis in an end-stage heart failure patient with chronic Q fever infection.CASE SUMMARY A 69-year-old male was admitted with dyspnea on exertion,hypotension and bilateral lower extremity edema for a few months.He has a past medical history of ischemic cardiomyopathy with left ventricular ejection fraction of 25%,implantable cardioverter defibrillator in place,bioprosthetic aortic valve and mitral valve replacement.He continued to have shortness of breath despite diuresis along with low grade fevers.Initial infectious work up came back negative.On further questioning,the patient was found to have close contact with farm animals and the recurrent fevers prompted the work-up for Q fever.Q fever serologies and cardiac positron emission tomography confirmed the diagnosis of chronic Q fever myocarditis.He was then successfully treated with doxycycline and hydroxychloroquine for 18 mo.CONCLUSION Chronic Q fever myocarditis,if left untreated,carries a poor prognosis.It should be kept in differentials,especially in patients with recurrent fevers and contact with farm animals.展开更多
文摘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 imperative.We report an unusual case of a 58-year-old patient with ATTRwt cardiomyopathy requiring heart transplantation.CASE SUMMARY A 58-year-old male presented with progressive fatigue,shortness of breath,weight gain,leg swelling,orthopnoea,and paroxysmal nocturnal dyspnoea for several months.Approximately 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 EF.Upon 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 testing.The patient was successfully treated with heart transplantation and is doing well post-transplant.CONCLUSION 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 findings.Early diagnosis and management can be consequential in improving patient outcomes.
文摘BACKGROUND Q fever myocarditis is a rare disease manifestation of Q fever infection caused by Coxiella burnetii.It is associated with significant morbidity and mortality if left untreated.Prior studies have reported myocarditis in patients with acute Q fever.We present the first case of chronic myocarditis in an end-stage heart failure patient with chronic Q fever infection.CASE SUMMARY A 69-year-old male was admitted with dyspnea on exertion,hypotension and bilateral lower extremity edema for a few months.He has a past medical history of ischemic cardiomyopathy with left ventricular ejection fraction of 25%,implantable cardioverter defibrillator in place,bioprosthetic aortic valve and mitral valve replacement.He continued to have shortness of breath despite diuresis along with low grade fevers.Initial infectious work up came back negative.On further questioning,the patient was found to have close contact with farm animals and the recurrent fevers prompted the work-up for Q fever.Q fever serologies and cardiac positron emission tomography confirmed the diagnosis of chronic Q fever myocarditis.He was then successfully treated with doxycycline and hydroxychloroquine for 18 mo.CONCLUSION Chronic Q fever myocarditis,if left untreated,carries a poor prognosis.It should be kept in differentials,especially in patients with recurrent fevers and contact with farm animals.
