66-year-old man was admitted to a cardiology unit with diagnosis of congestive heart failure, having complained of worsening asthenia and myalgia for several months before referral. Family history was aspecific. Echocardiography showed a moderately dilated and uniformly hypokinetic left ventricle (end-diastolic diameter 68 mm; ejection fraction 35%); wall thickness was normal. Coronary angiography showed normal epicardial arteries. After temporary improvement during treatment with beta-blockers and ACE-inhibitors, heart failure became severe and left ventricular ejection fraction fell to 20%. Suspected myocarditis prompted a right ventricular endomyocardial biopsy (Panel A), which excluded inflammation but identified amyloid infiltration of small intramural vessels without interstitial involvement. A left ventricular assist device was urgently implanted: histopathological examination of the excised left ventricular apex confirmed obstructive intramural coronary amyloidosis without interstitial deposits and with foci of coagulative necrosis. The patient died a few days later because of gastroenteric haemorrhage (autopsy was not performed) (Panels B–D). This case documents the existence of isolated intramural coronary obstruction as a peculiar phenotype of cardiac amyloidosis (distinct from the more common amyloidotic cardiomyopathy) (Panels E–I). This rare type of amyloidotic cardiac involvement—which in this patient led to a mistaken clinical diagnosis of myocarditis—must be recognized as one of the possible causes of acute or rapidly progressive heart failure.
Obstructive intramural coronary amyloidosis: a distinct phenotype of cardiac amyloidosis that can cause acute heart failure
Rapezzi C;
2006
Abstract
66-year-old man was admitted to a cardiology unit with diagnosis of congestive heart failure, having complained of worsening asthenia and myalgia for several months before referral. Family history was aspecific. Echocardiography showed a moderately dilated and uniformly hypokinetic left ventricle (end-diastolic diameter 68 mm; ejection fraction 35%); wall thickness was normal. Coronary angiography showed normal epicardial arteries. After temporary improvement during treatment with beta-blockers and ACE-inhibitors, heart failure became severe and left ventricular ejection fraction fell to 20%. Suspected myocarditis prompted a right ventricular endomyocardial biopsy (Panel A), which excluded inflammation but identified amyloid infiltration of small intramural vessels without interstitial involvement. A left ventricular assist device was urgently implanted: histopathological examination of the excised left ventricular apex confirmed obstructive intramural coronary amyloidosis without interstitial deposits and with foci of coagulative necrosis. The patient died a few days later because of gastroenteric haemorrhage (autopsy was not performed) (Panels B–D). This case documents the existence of isolated intramural coronary obstruction as a peculiar phenotype of cardiac amyloidosis (distinct from the more common amyloidotic cardiomyopathy) (Panels E–I). This rare type of amyloidotic cardiac involvement—which in this patient led to a mistaken clinical diagnosis of myocarditis—must be recognized as one of the possible causes of acute or rapidly progressive heart failure.I documenti in SFERA sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
