ECHOCARDIOGRAPHY
Midventricular obstruction causing abnormal flow patterns in hypertrophic cardiomyopathy
P.A. van der Wouw, A.C. Brauns
In rare cases of hypertophic cardiomyopathy a midventricular obstruction can exist, dividing the left ventricle into an apical and a basal part. Abnormal flow between these two compartments can be detected by Doppler echocardiography. The apical chamber can develop into an apical aneurysm. We describe two patients with midventricular obstruction and two distinct flow patterns in the left ventricle. Both patients (female, 70 and 82 years) presented with chest pain. One ofthem had a left bundle branch block, the other had an abnormal ECG consistent with left ventricular hypertrophy. Both had normal coronary arteries on angiography. The upper panel of figure 1 shows a continuous wave Doppler signal through the left ventricle. It clearly demonstrates an abnormal flow that is directed from the apex towards the base ofthe heart. The flow starts in the second halfof systole and continues through diastole up to the start of the A wave. After the A wave, it appears again as a protosystolic flow from apex to base. The lower panel shows a dynamic gradient of 43 mmHg in the outflow tract and the same abnormal midventricular flow in a pulsed Doppler signal employing a high pulse repetition frequency (HPRF) to depict high velocity signals. In figure 2, an angiogram ofthe left ventride, the doublechambered left ventricle can be appreciated. Patient 2 was different in that the abnormal midventricular flow was directed from base to apex, as can be seen in the upper panel offigure 3, a pulsed Doppler reading from the mid left ventricle. The lower panel shows the pulsed Doppler signal obtained from the mitral valve orifice in which the abnormal flow is absent. More images from these cases as well as video clips can be found at www.cardiologie.nl under 'werkgroep echocardiografie'. c
OV r~~
-
Figure 1. Continuous wave (upperpanel) andpulsd wave Dopper signak (lower panel) obtainedfrom the apex Arrows in the upper panel point at the abnormal stolic and diastolic flow in the mid
ventricle. The arrow in the lowerpanel shows the dynamicgradient in the kft ventrcular outflow tract. References 1
PA. van der Wouw A.C. Brauns Department of Cardiology, Onze Lieve Vrouwe Gasthuis, PO Box 95500, 1090 HM Amsterdam
Correspondence to: P.A. van der Wouw E-mail: [email protected]
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2
3
Tse HF, Ho HH. Sudden cardiac death caused by hypertrophic cardiomyopathy associated with midventricular obstruction and apical aneurysm. Heart2003;89:178. Gowda RM, Konka S, Khan IA. Hour-glass left ventricle: Midventricular hypertrophy and apical aneurysm in elderly hyper-
trophic cardiomyopathy. AmJGeriatr Cardiol2002;11:270-1. Sniderman AD, McCormick M, Musgrave R, Sniderman S, Patton RP Midventricular diastolic pulse Doppler flow velocity profiles in the normal and abnormal left ventricle. AmJ Cardiol 1997;80: 498-505.
Ncthcdands Heart Journal, Vohlme 12, Number 6, June 2004
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ECHOCARDIOGRAPHY
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Fi,gure 2. An,gio,gram of the letventricke showing bour-gl1ass confi,guration of the ventricke and midventricular obliteration. 4
5
Yamanari H, Morita H, Mouta S, Sakuragi S, Ohe T. Uniquely abnormal intracavitary flow during late systole and relaxation in hypertrophic obstructive cardiomyopathy with midventricular obstruction. Clin Cardiol 1996;19:913-5. Sutsch G, Jenni R, Krayenbuhl HP. Left ventricular flow from apex to base during systole and isovolumic relaxation in a patient with hypertrophic cardiomyopathy and midventricular obstruction. Eur HeartJ 199;12:1132-9.
Figqure 3. Pulsed Doppker tracings from the mid ventricle (upper panel) and the mitral valve orifice (lower panel) obtained from the apex. Arrows point at the dynamicgradient in systoe and the abnormal apical directedflow in early diastoe that is absentin the mitral orifice.
This section is coordinated by the working group on echocardiography and edited by M.J.M. Cramer.
Netherlands Heart Joumal, Volumc 12, Number 6, June 2004
301
Midventricular obstruction causing abnormal flow patterns in hypertrophic cardiomyopathy
P.A. van der Wouw, A.C. Brauns
In rare cases of hypertophic cardiomyopathy a midventricular obstruction can exist, dividing the left ventricle into an apical and a basal part. Abnormal flow between these two compartments can be detected by Doppler echocardiography. The apical chamber can develop into an apical aneurysm. We describe two patients with midventricular obstruction and two distinct flow patterns in the left ventricle. Both patients (female, 70 and 82 years) presented with chest pain. One ofthem had a left bundle branch block, the other had an abnormal ECG consistent with left ventricular hypertrophy. Both had normal coronary arteries on angiography. The upper panel of figure 1 shows a continuous wave Doppler signal through the left ventricle. It clearly demonstrates an abnormal flow that is directed from the apex towards the base ofthe heart. The flow starts in the second halfof systole and continues through diastole up to the start of the A wave. After the A wave, it appears again as a protosystolic flow from apex to base. The lower panel shows a dynamic gradient of 43 mmHg in the outflow tract and the same abnormal midventricular flow in a pulsed Doppler signal employing a high pulse repetition frequency (HPRF) to depict high velocity signals. In figure 2, an angiogram ofthe left ventride, the doublechambered left ventricle can be appreciated. Patient 2 was different in that the abnormal midventricular flow was directed from base to apex, as can be seen in the upper panel offigure 3, a pulsed Doppler reading from the mid left ventricle. The lower panel shows the pulsed Doppler signal obtained from the mitral valve orifice in which the abnormal flow is absent. More images from these cases as well as video clips can be found at www.cardiologie.nl under 'werkgroep echocardiografie'. c
OV r~~
-
Figure 1. Continuous wave (upperpanel) andpulsd wave Dopper signak (lower panel) obtainedfrom the apex Arrows in the upper panel point at the abnormal stolic and diastolic flow in the mid
ventricle. The arrow in the lowerpanel shows the dynamicgradient in the kft ventrcular outflow tract. References 1
PA. van der Wouw A.C. Brauns Department of Cardiology, Onze Lieve Vrouwe Gasthuis, PO Box 95500, 1090 HM Amsterdam
Correspondence to: P.A. van der Wouw E-mail: [email protected]
300
r --t>~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~------
2
3
Tse HF, Ho HH. Sudden cardiac death caused by hypertrophic cardiomyopathy associated with midventricular obstruction and apical aneurysm. Heart2003;89:178. Gowda RM, Konka S, Khan IA. Hour-glass left ventricle: Midventricular hypertrophy and apical aneurysm in elderly hyper-
trophic cardiomyopathy. AmJGeriatr Cardiol2002;11:270-1. Sniderman AD, McCormick M, Musgrave R, Sniderman S, Patton RP Midventricular diastolic pulse Doppler flow velocity profiles in the normal and abnormal left ventricle. AmJ Cardiol 1997;80: 498-505.
Ncthcdands Heart Journal, Vohlme 12, Number 6, June 2004
l*I_e,~ ~ .
I ft ~~~~~~~~~~~~~~~~~...
if.S.",t.......
ECHOCARDIOGRAPHY
HA
Fi,gure 2. An,gio,gram of the letventricke showing bour-gl1ass confi,guration of the ventricke and midventricular obliteration. 4
5
Yamanari H, Morita H, Mouta S, Sakuragi S, Ohe T. Uniquely abnormal intracavitary flow during late systole and relaxation in hypertrophic obstructive cardiomyopathy with midventricular obstruction. Clin Cardiol 1996;19:913-5. Sutsch G, Jenni R, Krayenbuhl HP. Left ventricular flow from apex to base during systole and isovolumic relaxation in a patient with hypertrophic cardiomyopathy and midventricular obstruction. Eur HeartJ 199;12:1132-9.
Figqure 3. Pulsed Doppker tracings from the mid ventricle (upper panel) and the mitral valve orifice (lower panel) obtained from the apex. Arrows point at the dynamicgradient in systoe and the abnormal apical directedflow in early diastoe that is absentin the mitral orifice.
This section is coordinated by the working group on echocardiography and edited by M.J.M. Cramer.
Netherlands Heart Joumal, Volumc 12, Number 6, June 2004
301
