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Archives of cardiovascular diseases
Volume 103, n° 11-12
pages 629-630 (novembre 2010)
Doi : 10.1016/j.acvd.2010.03.008
Received : 2 Mars 2010 ;  accepted : 3 Mars 2010
Biventricular dysplasia presenting as an acute coronary syndrome
Dysplasie biventriculaire se présentant comme un syndrome coronaire aigu
 

Virginie Brandao Carreira a, Darach O h-Icí b, Jérôme Garot b, , Rémy Cohen a
a Service de cardiologie, centre hospitalier de Lagny–Marne-La-Vallée, Massy, France 
b CMR Department, Institut cardiovasculaire Paris Sud, hôpital privé Jacques-Cartier, 6, avenue du Noyer-Lambert, 91300 Massy, France 

Corresponding author. Fax: +33 1 60 13 48 04.

Keywords : Cardiovascular magnetic resonance imaging, Arrhythmogenic right ventricular dysplasia, Acute coronary syndrome

Mots clés : IRM cardiovasculaire, Dysplasie arythmogène du ventricule droit, Syndrome coronaire aigu

Abbreviations : CMR, ECG, LAD, LV, MRI, RV


A 45-year-old man without past medical history was referred for acute chest pain and suspected non-ST-segment elevation myocardial infarction. His cardiovascular risk factors were diabetes, obesity and current smoking. Physical examination was normal. The 12-lead ECG demonstrated ST-segment depression in leads V2 to V6 (Figure 1A). Troponin I was elevated at 10μg/L. Quantitative coronary angiography revealed a 65% diameter stenosis of the mid portion of the LAD coronary artery (Figure 1B). LV angiography showed severe LV dysfunction involving the anterior wall (Figure 1B, Video A). Because of the clinical presentation associated with the ECG and the biological and angiographic features, percutaneous coronary angioplasty of the LAD coronary artery was performed successfully with stent implantation. The following day, the patient developed sustained ventricular tachycardia that resolved spontaneously (Figure 1C). Transthoracic echocardiography revealed severe global LV dysfunction with moderate RV dilatation. Because of the discrepancy between the moderate troponin elevation and the severe LV dysfunction, the patient underwent CMR imaging (Siemens Espree® 1.5T, Erlangen, Germany). Cine MRI acquired before contrast injection showed dilated and severely hypokinetic left and right ventricles (Figure 1D). The RV myocardium was thin and showed segmental outward systolic wall motion particularly in the anterior wall of the RV outflow tract (Figure 1D, Video B). T1-weighted black-blood fast spin echo images revealed the presence of intramyocardial areas of hypersignal within the RV and LV myocardium, indicative of intramyocardial fat (Figure 1E). Late-enhanced CMR imaging acquired 10minutes after contrast injection demonstrated multiple intramyocardial foci of late enhancement in the region of the RV outflow tract, consistent with the presence of myocardial fibrosis (Figure 1E). Based on CMR imaging findings, the diagnosis of biventricular dysplasia with fibro-fatty replacement was made without evidence for signs of recent myocardial infarction.



Figure 1


Figure 1. 

A. Electrocardiogram on admission showing diffuse ST-segment depression in the anterior leads. B. Coronary angiography showing left anterior descending coronary artery stenosis (arrow). Left ventricular (LV) angiography at end-diastole and end-systole with marked hypokinesis in the anterior wall (arrows). C. Sustained ventricular tachycardia. D. Steady-state free precession cine magnetic resonance imaging (cine-MRI) at end-diastole and end-systole in the four-chamber view, demonstrating dilated and severely hypokinetic left and right ventricles, along with unusual areas of hyposignal within the LV myocardium suggestive of intra-myocardial fat (arrows). Cine-MRI of right heart chambers showing outward systolic wall motion of the right ventricular (RV) outflow tract. E. Black-blood T1-weighted cardiac magnetic resonance imaging (CMRI) showing hypersignal indicative of intramyocardial fat (arrows). Corresponding areas of myocardial fibrosis on late-enhanced-CMRI acquired 10min after gadolinium injection (0.1mM) (arrows). Note the presence of myocardial late enhancement (fibrosis) in the RV outflow tract. RA: right atrium; RV: right ventricle; PA: pulmonary artery.

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This case raises the issue of the coexistence of biventricular dysplasia with acute coronary syndrome, or more likely, biventricular dysplasia mimicking an acute coronary syndrome during acute progression of the disease.

Conflicts of interest

None.


Appendix A. Supplementary data

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 Video A 
Video A. 

Left ventricular angiogram in the 30̊ right anterior oblique projection showing severe anterior hypokinesis.

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 Video B 
Video B. 

Cine magnetic resonance imaging of the right heart chambers showing a dilated and severely hypokinetic right ventricle, with outward systolic wall motion of the right ventricular outflow tract, at the site of myocardial fibro-fatty replacement.



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