AIMS: To detect cardiac amyloidosis (CA) earlier, it is inevitable to improve diagnostic strategies. METHODS AND RESULTS: The impact of ECG, echocardiography including tissue Doppler imaging (TDI) and strain, and myocardial biopsies was evaluated in 30 patients (63% (n = 19) men, mean age 66 +/- 8 years, NYHA 3.0 +/- 0.5, 73% with prior myocardial decompensation), in whom we proved CA. Amyloid was confirmed by apple-green birefringence under polarised light, and the causing protein by immunohistochemical examinations. Genetic analyses excluded familial CA. All patients (AL-lambda (n = 22), AL-kappa (n = 3), senile amyloidosis (n = 5)) had echocardiographic signs of restrictive cardiomyopathy (RCM), typical TDI and strain parameters (E'septal; E' lateral < 8 cm/s; E/E' > 8; S' < or =9 cm/s; global longitudinal strain (GLS) -7.9 +/- 3.8%). Pericardial effusions were present in 63% of patients. ECGs were suspicious in many patients: 19 (63%) had low-voltage, 23 (77%) reduced R waves in V(1)-V(4), and 57% both. Abnormalities, retrospectively had been present for 0.5-4 years. Twenty (67%) patients died 232 +/- 268 [2-1020] days after CA was diagnosed, but 502 +/- 333 [30-1440] days after the first symptom. CONCLUSION: Accurate ECG evaluations, careful echocardiographic search for RCM, reduced strain/strain rates, and general indications to myocardial biopsies with correct analyses are needed to diagnose CA.
AIMS: To detect cardiac amyloidosis (CA) earlier, it is inevitable to improve diagnostic strategies. METHODS AND RESULTS: The impact of ECG, echocardiography including tissue Doppler imaging (TDI) and strain, and myocardial biopsies was evaluated in 30 patients (63% (n = 19) men, mean age 66 +/- 8 years, NYHA 3.0 +/- 0.5, 73% with prior myocardial decompensation), in whom we proved CA. Amyloid was confirmed by apple-green birefringence under polarised light, and the causing protein by immunohistochemical examinations. Genetic analyses excluded familial CA. All patients (AL-lambda (n = 22), AL-kappa (n = 3), senile amyloidosis (n = 5)) had echocardiographic signs of restrictive cardiomyopathy (RCM), typical TDI and strain parameters (E'septal; E' lateral < 8 cm/s; E/E' > 8; S' < or =9 cm/s; global longitudinal strain (GLS) -7.9 +/- 3.8%). Pericardial effusions were present in 63% of patients. ECGs were suspicious in many patients: 19 (63%) had low-voltage, 23 (77%) reduced R waves in V(1)-V(4), and 57% both. Abnormalities, retrospectively had been present for 0.5-4 years. Twenty (67%) patients died 232 +/- 268 [2-1020] days after CA was diagnosed, but 502 +/- 333 [30-1440] days after the first symptom. CONCLUSION: Accurate ECG evaluations, careful echocardiographic search for RCM, reduced strain/strain rates, and general indications to myocardial biopsies with correct analyses are needed to diagnose CA.
Authors: Wael A Aljaroudi; Milind Y Desai; W H Wilson Tang; Dermot Phelan; Manuel D Cerqueira; Wael A Jaber Journal: J Nucl Cardiol Date: 2014-04 Impact factor: 5.952