Zusammenfassung
Die Herzinsuffizienz (CHF) bei der kardialer Amyloidose geht mit einer diastolischen Dysfunktion einher, trotz schwere Symptomatik wurde eine normale oder allenfalls leichtgradig reduzierte linksventrikuläre Ejektionsfraktion (EF) beschrieben. Eine frühe Einschränkung der longitudinalen systolischen Funktion wurde durch den Einsatz des Gewebedopplers und die Bestimmung der myokardialen Verformung (“strain rate imaging”, SRI) beschrieben.
Unser Patient wurde mit einer symptomatischen Herzinsuffizienz und deutlich erhöhtem brain-natriuretic peptide (BNP) Plasmaspiegel zugewiesen. Die konventionellen Parameter der Bestimmung der systolischen Funktion waren normal, das mitrale Einstromprofl entsprach lediglich einer mäßigen diastolischen Dysfunktion. In der Myokardbiopsie konnte histologisch interstitielles Amyloid nachgewiesen werden. Durch die quantitative Bestimmung der myokardialen Geschwindigkeiten (TDI) und der Verformung (Strain) konnte im apikalen Schallfenster eine relevante Einschränkung der longitudinalen systolischen Funktion nachgewiesen werden.
Bei Patienten mit isolierter diastolischer Dysfunktion in der konventionellen Echokardiographie kann durch den ergänzenden Einsatz von myokardialem strain und strain rate frühzeitig auch eine systolische Funktionseinschränkung nachgewiesen werden.
Summary
Congestive heart failure (CHF) in cardiac amyloidosis has been attributed to the development of diastolic dysfunction, because severe CHF symptoms have been observed despite a normal or only mildly reduced LV ejection fraction (EF). An early impairment of longitudinal systolic function has been described by means of tissue Doppler-derived myocardial deformation imaging (‘strain rate imaging’, SRI).
Our patient presented with signs of CHF and significantly increased brain-natriuretic peptide (BNP) levels. Conventional measures of systolic contractile function were within the normal range and mitral inflow Doppler showed only moderate diastolic dysfunction. Histopathological examination by endomyocardial biopsy revealed interstitial deposition of amyloid fibers. Quantitative assessment of myocardial velocities (TDI) and deformation properties (Strain) from the apical view demonstrated a significant impairement of systolic longitudinal myocardial function.
In patients with isolated diastolic dysfunction detected by conventional Doppler echocardiography, the quantitative assessment of myocardial strain and strain rate can be helpful in the early detection of systolic dysfunction.
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Niedeggen, A., Breithardt, O.A. & Franke, A. Detection of early systolic dysfunction with strain rate imaging in a patient with light chain cardiomyopathy. ZS Kardiologie 94, 133–136 (2005). https://doi.org/10.1007/s00392-005-0175-8
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DOI: https://doi.org/10.1007/s00392-005-0175-8