[Augmentation of left ventricular outflow tract gradient by digitalis glycosides in a case of cardiac (AL-) amyloidosis].
METHODS
A 61-year-old man suffering from histologically confirmed amyloidosis associated with coagulation disturbances presented with exercise induced shortness of breath and symptoms of cardiac asthma after four cycles of chemotherapy with melphalan and prednisolone. As a result, treatment with digitoxin was initiated. In addition furosemide and an oral nitrate were administered.


METHODS
Disparity between electrocardiogram and echocardiographic findings was observed in that, while the electrocardiogram showed loss of 'R wave in precordial leads V(2 - 4), excessive thickening of both left and right ventricular wall was shown in the echocardiogram. Doppler-echocardiography revealed a left ventricular outflow tract obstruction at rest with a peak pressure gradient of 64 mm Hg which rose to 145 mm Hg during Valsalva manoeuvre. Colour Doppler presented a moderate mitral insufficiency and the transmitral Doppler flow studies detected a restrictive left ventricular filling pattern.


METHODS
Digitalis therapy was stopped because of the outflow tract obstruction complicating cardiac amyloidosis. Cardiac symptoms abated over the following weeks. This improvement was reflected in a significant reduction of the outflow tract gradient, the gradients now being 16 mm Hg under resting conditions and a maximum of 36 mm Hg during the Valsalva manoeuvre. The transmitral Doppler flow pattern showed a pseudonormalisation and the mitral regurgitation regressed nearly completely. The patient was free from cardiac complaints until his sudden death 21 months after the diagnosis of cardiac amyloidosis.


CONCLUSIONS
Cardiac amyloidosis can present with left ventricular outflow tract obstruction mimicking hypertrophic obstructive cardiomyopathy. This fact must be borne in mind to avoid therapy with preload-reducing or positive inotropic drugs and especially glycosides.
Petzsch
M
M
Abteilung Kardiologie, Klinik und Poliklinik für Innere Medizin, Universität Rostock. michael.petzsch@med.uni-rostock.de.
Pulya
K
K
Anders
O
O
D000686:Amyloidosis / Q000188:drug therapy* / Q000503:physiopathology; D001250:Asthma, Exercise-Induced / Q000188:drug therapy; D002316:Cardiotonic Agents / Q000009:adverse effects; D004074:Digitoxin / Q000009:adverse effects*; D004232:Diuretics / Q000627:therapeutic use; D018618:Echocardiography, Doppler, Color / Q000187:drug effects; D004562:Electrocardiography / Q000187:drug effects; D005665:Furosemide / Q000627:therapeutic use; D006331:Heart Diseases / Q000188:drug therapy* / Q000503:physiopathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D014665:Vasodilator Agents / Q000627:therapeutic use
D002316:Cardiotonic Agents; D004232:Diuretics; D014665:Vasodilator Agents; D005665:Furosemide; D004074:Digitoxin
Aggravierter linksventrikulärer Ausflusstraktgradient unter Digitalis-Therapie bei kardialer (AL-) Amyloidose.