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Abstract
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' ≤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 V1–V4, 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.
| Abbreviations | ||
| CA | = | cardiac amyloidosis |
| TDI | = | tissue Doppler echocardiography |
| NYHA | = | New York Heart Association |
| ECG | = | electrocardiogram |
| RCM | = | restrictive cardiomyopathy |
| E | = | early peak diastolic transmitral inflow velocity |
| E′ | = | peak early diastolic velocity of the mitral annulus |
| E/E′ | = | ratio of early transmitral inflow velocity E to early diastolic velocity of the mitral annulus |
| GLS | = | global longitudinal strain |
| A' | = | peak late diastolic velocity of the mitral annulus |
| S' | = | peak systolic velocity of the mitral annulus |
| DET | = | deceleration time |
| E/A | = | ratio of early transmitral inflow velocity E to late transmitral inflow velocity A |
| 2D | = | two-dimensional |
| PSS | = | peak systolic strain |
| PSSR | = | peak systolic strain rate |
| 4 CH | = | apical four chamber view |
| NT proBNP | = | N-terminal fragments of brain type natriuretic peptide |
| A | = | amyloidosis |
| SSA | = | senile systemic amyloidosis |
| CHF | = | congestive heart failure |
| HCM | = | hypertrophic cardiomyopathy |
| ARVD | = | arrhythmogenic right ventricular dysplasia |
| SD | = | standard deviation |
| VO2 | = | respiratory oxygen uptake |
| LVEDD | = | left ventricular enddiastolic dimension |
| LA | = | left atrium |
| LVPW | = | left ventricular posterior wall diameter |
| EF | = | ejection fraction |
| RVEDP | = | right ventricular enddiastolic pressure |
| PAm | = | mean pulmonary artery pressure |
| PCW | = | mean pulmonary capillary wedge pressure |
| Abbreviations | ||
| CA | = | cardiac amyloidosis |
| TDI | = | tissue Doppler echocardiography |
| NYHA | = | New York Heart Association |
| ECG | = | electrocardiogram |
| RCM | = | restrictive cardiomyopathy |
| E | = | early peak diastolic transmitral inflow velocity |
| E′ | = | peak early diastolic velocity of the mitral annulus |
| E/E′ | = | ratio of early transmitral inflow velocity E to early diastolic velocity of the mitral annulus |
| GLS | = | global longitudinal strain |
| A' | = | peak late diastolic velocity of the mitral annulus |
| S' | = | peak systolic velocity of the mitral annulus |
| DET | = | deceleration time |
| E/A | = | ratio of early transmitral inflow velocity E to late transmitral inflow velocity A |
| 2D | = | two-dimensional |
| PSS | = | peak systolic strain |
| PSSR | = | peak systolic strain rate |
| 4 CH | = | apical four chamber view |
| NT proBNP | = | N-terminal fragments of brain type natriuretic peptide |
| A | = | amyloidosis |
| SSA | = | senile systemic amyloidosis |
| CHF | = | congestive heart failure |
| HCM | = | hypertrophic cardiomyopathy |
| ARVD | = | arrhythmogenic right ventricular dysplasia |
| SD | = | standard deviation |
| VO2 | = | respiratory oxygen uptake |
| LVEDD | = | left ventricular enddiastolic dimension |
| LA | = | left atrium |
| LVPW | = | left ventricular posterior wall diameter |
| EF | = | ejection fraction |
| RVEDP | = | right ventricular enddiastolic pressure |
| PAm | = | mean pulmonary artery pressure |
| PCW | = | mean pulmonary capillary wedge pressure |