Significant obstruction of the right and left ventricular outflow tract in a patient with biventricular hypertrophic cardiomyopathy.
Echocardiography demonstrated pronounced asymmetric left ventricular (LV) hypertrophy and thickened right ventricular muscular components in a 54-year-old woman with a history of dyspnoea (NYHA III), and recurrent syncopes. Left ventricular outflow peak gradient was 80 mmHg at rest and 125 mmHg during Valsalva manoeuvre. Cardiac cine and gadolinium-enhanced T1 weighted magnetic resonance imaging (MRI) provided striking images of a right ventricular outflow tract obstruction and a markedly abnormal gadolinium uptake in the interventricular septum consistent with myocardial fibrosis. Right and left heart catherization, with simultaneous measurement of aortic and LV pressures revealed a 150 mmHg subaortic gradient and a 130 mmHg subpulmonic gradient at rest. Impediment to right ventricular (RV) outflow was due to massive hypertrophy of the crista supraventricularis with an 'hour-glass' deformity. A surgical intervention with LV septal myotomy-myectomy and RV ventriculotomy was performed successfully. Hypertrophic obstructive cardiomyopathy with significant RV and LV outflow tract obstruction is a very rare finding. Echocardiography and MRI can be used in combination for non-invasive evaluation of morphological and haemodynamic information because mechanisms of obstruction are different in each ventricle.
Butz
Thomas
T
Department of Cardiology, Heart Center North Rhine-Westphalia, Ruhr University Bochum, Georgstrasse 11, D-32545 Bad Oeynhausen, Germany. akohlstaedt@hdz-nrw.de
Horstkotte
Dieter
D
Langer
Christoph
C
Esdorn
Hermann
H
Kleikamp
Georg
G
Körfer
Reiner
R
Faber
Lothar
L
D002312:Cardiomyopathy, Hypertrophic / Q000150:complications / Q000000981:diagnostic imaging* / Q000601:surgery; D003287:Contrast Media; D004452:Echocardiography; D005260:Female; D019786:Gadolinium DTPA; D006801:Humans; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D014694:Ventricular Outflow Obstruction / Q000150:complications / Q000000981:diagnostic imaging* / Q000601:surgery
D003287:Contrast Media; D019786:Gadolinium DTPA