European Journal of Echocardiography 2008 9(1):145-147; doi:10.1016/j.euje.2007.04.011
Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2007. For permissions please email: journals.permissions@oxfordjournals.org
Giant Eustachian valve and left ventricular systolic dysfunction in a patient with non-dilated amyloid cardiomyopathy
Sandeep Arora*,
Madhavi Kadiyala and
LeRoy Moore
Department of Cardiovascular Disease, Western Pennsylvania Hospital/Temple University Program, 4800 Friendship Avenue, Pittsburgh, PA 15224, USA
Received 7 April 2007; accepted after revision 28 April 2007; online publish-ahead-of-print 30 June 2007.
* Corresponding author. Department of Cardiovascular Disease, Suite 3411 North, Western Pennsylvania Hospital/Temple University Program, 4800 Friendship Avenue, Pittsburgh, PA 15224, USA. Tel: +1 412 578 6934, +1 412 486 3819 (Res); fax: +1 412 578 6804.E-mail address: sandeeparora24{at}hotmail.com
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Abstract
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Amyloid cardiomyopathy is characterized by non-dilated thick-walled
left ventricular, thickening of interventricular septum and
right ventricular free wall, biatrial enlargement associated
with granular ‘sparkling’ appearance of the myocardium.
Typically, decreased left ventricular compliance results in
abnormal diastolic functions but left ventricular systolic functions
are preserved until late in the course of the illness when left
ventricle starts to dilate culminating into dilated cardiomyopathy.
We present a 77-year-old patient who had typical echocardiographic
features of amyloid heart disease, a giant Eustachian valve
resembling cor triatriatum dexter and left ventricular systolic
dysfunction without associated left ventricular dilatation.
Keywords: Restrictive cardiomyopathy; Eustachian valve; Systolic function
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Introduction
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We present a patient with typical echocardiographic features
of amyloid cardiomyopathy who had left ventricular systolic
dysfunction in the absence of ventricular dilatation. This was
also associated with a giant Eustachian valve giving the appearance
of divided right atrial cavity.
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Case report
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A 77-year-old female with history of hypertension and chronic
atrial fibrillation was brought to the hospital because of swelling
and pain in bilateral lower extremities for five days. This
was associated with progressively increasing shortness of breath
for the last few months. She denied any chest pain, fevers,
chills or cough. There was no known history of coronary artery
disease or prior myocardial infarction. On examination, she
had pulse rate of 88/min, blood pressure of 112/74 mmHg, respiratory
rate of 22/min and oxygen saturation of 90% on room air. Jugular
venous pressure was elevated around 5 cm above the sternal angle.
Cardiovascular examination revealed normal heart sounds with
a pansystolic murmur at the apex radiating to the axilla. She
also had a holosystolic murmur at left sternal border which
increased on inspiration. On auscultation of the chest, she
had crackles and decreased air entry at both lung bases. Rest
of the systemic examination showed hepatomegaly and bilateral
erythematous, tender and swollen lower extremities suggestive
of chronic edema with superimposed cellulites.
Laboratory investigations showed mild anemia with elevated white blood cell count. Chest X-ray was remarkable for bilateral vascular congestion and pleural effusions without significant cardiomegaly. EKG showed low voltage complexes in the precordial leads. She was diagnosed with congestive heart failure with cellulites of lower extremities and was started on diuretic therapy and appropriate antibiotics. An echocardiogram was ordered to further evaluate heart failure.
Transthoracic echocardiogram revealed non-dilated thick-walled left ventricle, interventricular septum and right ventricle free wall, biatrial enlargement and thickened mitral and tricuspid valve leaflets associated with moderate mitral and tricuspid regurgitation and trace pericardial effusion (Figures 1 and 2). Ventricular walls had reflactile ‘sparkling’ appearance typical of amyloid heart disease (Figure 1). Doppler examination of mitral-inflow velocity showed prominent E waves with steep deceleration slope and a small Em wave on tissue Doppler, a pattern suggestive of restrictive heart disease. There was a giant Eustachian valve in the right atrial cavity extending from mid-interatrial septum to just above the inferior vena cava simulating a cor triatriatum dexter (Figure 1). This was also associated with prominent chiari network and a small patent foramen ovale with left to right shunting (Video clip 1). Echocardiography also revealed akinetic anterior-lateral, anterior, anterior-septal and inferior-septal walls with severe left ventricular systolic dysfunction and estimated left ventricular ejection fraction of 30% (Video clips 2 and 3).
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Figure 1 (A) Para-sternal long axis view showing ‘sparkling’ appearance of the myocardium, non-dilated left ventricles, dilated left atrium, thickened mitral valve leaflets and pleural effusion (PL). (B) Apical four-chamber view showing thickened left ventricle and right ventricular free wall and a giant Eustachian valve (arrow) giving the appearance of divided right atrium. LA, left atrium; LV, left ventricle; RV, right ventricle; AO, aorta; EV, Eustachian valve; PL, pleural effusion.
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Figure 2 (A) Para-sternal short axis view with thickened left ventricles. (B) Subcostal view showing a small pericardial effusion and a giant Eustachian valve (arrow) extending from interatrial septum to just above inferior vena cava. LA, left atrium; EV, Eustachian valve; PE, pericardial effusion.
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Patient underwent MRI of her lower extremities to evaluate possible
necrotizing fasciitis. Unfortunately she developed ventricular
fibrillation and cardiac arrest while undergoing MRI and died
in spite of aggressive resuscitative measures. Family did not
request autopsy.
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Discussion
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We present a patient of restrictive cardiomyopathy who had severe
left ventricular systolic dysfunction in absence of left ventricular
dilatation. Though myocardial biopsy was not done, echocardiographic
features were quite suggestive of amyloid infiltration. These
include thickened left ventricular and right ventricular free
walls with ‘sparkling’ appearance of the myocardium,
biatrial enlargement, thickening of valvular leaflets, mitral
and tricuspid regurgitation and restrictive pattern on Doppler
inflow velocities.
1 Also, our patient had few unusual features.
Patients with amyloid cardiomyopathy usually have non-dilated
left ventricles with preserved systolic functions until late
in the course of their disease. While these patients may progress
and develop dilated cardiomyopathy, our patient had abnormal
left ventricular systolic functions without any anatomic/ hemodynamic
pattern of left ventricular dilatation.
Patho-physiological mechanisms responsible for left ventricular systolic dysfunction in our patient are not clear. Though it may be attributed to concomitant coronary artery disease, myocardial ischemia with or without prior myocardial infarction there was no clinical or electrocardiographic evidence of significant coronary artery disease. Moreover, absence of myocardial thinning makes prior myocardial infarction a less likely etiology for left ventricular dysfunction. It is possible that extensive infiltration of amyloid protein by itself may result in impaired myocardial contractility in the presence of normal myocardial perfusion.
Our patient also had a giant Eustachian valve extending from inferior vena cava to mid portion of the interatrial septum giving the appearance of a divided right atrium. This type of Eustachian valve has been described only rarely before.2 Such a giant valve should be differentiated from cor triatriatum dexter, another rare cardiac malformation which can resemble the former echocardiographically. This is especially important due to the fact that the latter entity may require surgical correction. Cor-triatriatum dexter forms a large obstructive (though perforated) membrane or septum across the right atrium thus dividing it into two distinct chambers. The upper chamber receives blood from both inferior and superior vena cava and directs it towards interatrial septum with shunting of blood from right to left atrium through atrial septal defect, which frequently co-exists in these patients. This results in mixing of desaturated blood with oxygenated blood in the left atrium causing cyanosis. Our patient had none of these features and had a free flow of blood across the Eustachian valve. Though there was a communication between the two atria in our patient, color Doppler demonstrated predominantly left to right shunting across the interatrial septum.
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Supplementary material
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Supplementary date associated with this article can be found in the online version.
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References
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- Shah KB, Inoue Y, Mehra MR. Amyloidosis and the heart: a comprehensive review. Arch Intern Med (2006) 166:1805–13.[Abstract/Free Full Text]
- Yavuz T, Nazli C, Kinay O, Kutsal A. Giant Eustachian valve with echocardiographic appearance of divided right atrium. Tex Heart Inst J (2002) 29:36–38.
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Abstract
Introduction