multimodality imaging of funnel-shaped subaortic membrane

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Sep 4, 2014 - 1Department of Cardiology, Sint-Jan Hospital Bruges, Ruddershove 10, Bruges 8000, Belgium; 2Department of Cardiology, University ...
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CARDIOVASCULAR FLASHLIGHT

doi:10.1093/eurheartj/ehu334 Online publish-ahead-of-print 4 September 2014

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Moderate gradient with disproportional dyspnoea: multimodality imaging of funnel-shaped subaortic membrane Daan Cottens1,2, Ben Corteville1,3, Annick Vanvinckenroye1, Marc Schepens4, Luc Missault1, and Philippe Debonnaire1* 1

Department of Cardiology, Sint-Jan Hospital Bruges, Ruddershove 10, Bruges 8000, Belgium; 2Department of Cardiology, University Hospitals Leuven, Leuven, Belgium; Department of Cardiology, University Hospital Ghent, Ghent, Belgium; and 4Department of Cardiac Surgery, Sint-Jan Hospital Bruges, Bruges, Belgium

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* Corresponding author. Tel: +32 50452670, Fax: +32 50452679, Email: [email protected]

A 47-year-old man without medical history presented with new onset angina and progressive excertional dyspnoea, New York Heart Association class III. A crescendo murmur corresponded to systolic turbulent flow in the left ventricular outflow tract (LVOT), coinciding with a thickened sharp-edged basal septum of 14 mm with ring-like structure on transthoracic (Panel A and B) and transoesophageal echocardiography (Panel C). Pseudonormal diastolic LV function and normal aortic valve morphology and function were noted. The ascending aorta measured 46 mm. Moderate subvalvular LVOT obstruction at rest with a peak gradient of 36 mmHg was found. Computed cardiac tomography (Panel D) and LV angiography (Panel E) both demonstrated a large LVOT subvalvular membrane with supra membraneous cage-like cavity and excluded coronary artery stenosis. Severe dyspnoea and immediate tachycardia (144 b.p.m.) limited bicycle exercise test at only 100 W. A metoprolol 100 mg trial reduced resting gradient to 21 mmHg, however, without providing symptomatic relief as severe dyspnoea and tachycardia re-occurred after 20 squads with a peak gradient increase to 28 mmHg. Subaortic valvular membrane is a rare entity requiring surgery if symptomatic and/or peak gradient ≥50 mmHg. We speculate unfavourable diastology caused excertional steep LV end-diastolic pressure increase transmitted to the pulmonary vasculature, leading to symptoms disproportional to LVOT gradient. The patient underwent uneventful resection of the funnel-shaped complete subvalvular membrane (Panel F, arrows) with limited Morrow procedure and supracoronary aorta ascendens replacement by a 24 mm woven, collagen-coated graft, leading to complete resolution of complains, LVOT gradient and exercise capacity restoration 2 months later.

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