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Large Caseous Mitral
Annular Calcification
with Mitral Stenosis, Dynamic Left
Ventricular Outflow Obstruction, and Syncope
Ibrahim Akpinar, MD
Turgut Karabag, MD
Muhammet Rasit Sayin, MD
Nesligul Yildirim, MD
Sait Mesut Dogan, MD
Mustafa Aydin, MD
A
68-year-old woman presented with exertional dyspnea, angina, and syncope. She was undergoing renal dialysis (3 times weekly, for one year) and was
taking nifedipine for hypertension. Laboratory results suggested secondary
hyperparathyroidism and hypercalcemia. Auscultation revealed a severe systolic murmur at the left upper parasternal border and a moderate diastolic murmur at the cardiac apex. Transthoracic echocardiography showed a 3.5  2.7-cm hyperechogenic,
ovoid, calcific mass covering the posterior periannular region (Fig. 1). Moderate mitral
inflow obstruction and severe left ventricular outflow tract (LVOT) obstruction were
detected between the calcific mass, the anterior mitral valve leaflet, and the patient’s
prominent, 20-mm-thick sigmoid interventricular septum (Figs. 2 and 3). Computed
tomography yielded further detail of the hyperechogenic mass (Fig. 4). During surgery, heavily calcified liquid was removed and a mechanical prosthetic valve was implanted to treat valvular and subvalvular narrowing and the caseous calcification of
the mitral annulus (CCMA) (Figs. 5 and 6). The posterior mitral valve leaflet had
been destroyed by calcific degeneration. The full contact between the middle anterior mitral valve leaflet and the large caseous calcification might have prevented any
mitral regurgitation (Fig. 7).
Comment
Section Editor:
Raymond F. Stainback, MD,
Department of Adult
Cardiology, Texas Heart
Institute at St. Luke’s
Episcopal Hospital, 6624
Fannin St., Suite 2480,
Houston, TX 77030
From: Department of
Cardiology, Faculty of
Medicine, Zonguldak
Karaelmas University,
Zonguldak 67600, Turkey
In one study, CCMA constituted only 0.64% of all mitral annular calcification diagnoses.1 Echocardiographic images of CCMA are often heterogeneous because of calcium and lipid deposits, and the masses display hyperechogenic and hypoechogenic
areas.2 Although the appearance of CCMA can mimic that of tumors, abscesses, and
cysts, surgery might not be needed when there is no marked obstruction.2,3
To our knowledge, few reports have described combined LVOT and mitral inflow
obstruction related to CCMA. When a patient with CCMA has a sigmoid septum,
the physician should suspect mitral inflow and dynamic LVOT obstruction and carefully evaluate the results of continuous-wave Doppler echocardiography. Our patient
had no mitral regurgitation, and the LVOT obstruction lacked the characteristic latepeaking spectral Doppler signal—perhaps explained by dynamic LVOT obstruction
Address for reprints:
Ibrahim Akpinar, MD,
Department of Cardiology,
Faculty of Medicine,
Zonguldak Karaelmas
University, Kozlu/Zonguldak
67600, Turkey
E-mail: dr.ibrahimakpinar@
gmail.com
© 2012 by the Texas Heart ®
Institute, Houston
910
Caseous Calcification of the Mitral Annulus
Fig. 1 Transthoracic echocardiogram (apical 5-chamber
view) shows caseous calcification of the mitral valve (arrow).
AMVL = anterior mitral valve
leaflet
Real-time motion image is availClick
here for real-time
able
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journal. image: Fig. 1.
Volume 39, Number 6, 2012
that might have developed between the tip of the anterior mitral valve leaflet and the sigmoid septum during
early systolic movement. In our patient and in others,
the chief factor in the growth of the calcification adjacent to the posterior leaflet was probably hyperparathyroidism secondary to chronic renal failure.4 In such
patients, meticulous echocardiographic examinations
can reveal the progression of caseous calcification.
Fig. 2 Transthoracic echocardiogram with continuous-wave
Doppler shows mitral inflow obstruction between the caseous
calcification and the anterior mitral valve leaflet (maximum transmitral gradient, 15 mmHg; mean gradient, 7 mmHg).
Fig. 5 Intraoperative photograph shows the caseous calcification (arrow).
Fig. 3 Transthoracic echocardiogram with continuous-wave
Doppler shows left ventricular outflow obstruction between the
tip of the anterior mitral valve leaflet and the sigmoid interventricular septum (maximum outflow gradient, 155 mmHg; mean
gradient, 61 mmHg).
Fig. 4 Computed tomogram (64-slice) shows a heterogeneous,
hyperdense mass with smooth borders (arrow).
LA = left atrium; LV = left ventricle; RA = right atrium; RV = right
ventricle
Texas Heart Institute Journal
Fig. 6 Photomicrograph reveals caseous calcification and
degenerative necrosis of the posterior mitral valve leaflet (arrow)
(H & E; orig. ×200).
V = valve tissue
Caseous Calcification of the Mitral Annulus
911
References
1. Deluca G, Correale M, Ieva R, Del Salvatore B, Gramenzi
S, Di Biase M. The incidence and clinical course of caseous
calcification of the mitral annulus: a prospective echocardiographic study. J Am Soc Echocardiogr 2008;21(7):828-33.
2. Chahal M, Temesy-Armos P, Stewart WJ. Big MAC: caseous
calcification of the mitral annulus referred for possible cardiac
tumor. Echocardiography 2011;28(4):E76-8.
3. Teja K, Gibson RS, Nolan SP. Atrial extension of mitral annular calcification mimicking intracardiac tumor. Clin Cardiol
1987;10(9):546-8.
4. Isotalo PA, Walley VM. Coronary artery erosion and dissection: an unusual complication of mitral annular calcification.
Cardiovasc Pathol 1999;8(3):141-4.
Fig. 7 Transthoracic color-flow Doppler echocardiogram (apical
5-chamber view) shows no mitral regurgitation. During systole,
as the middle part of the anterior mitral valve leaflet completely contacted the large caseous calcification, dynamic left ventricular outflow tract obstruction probably occurred between the tip
of that leaflet and the prominent sigmoid interventricular septum
(arrow).
Ao = aorta; AV = aortic valve
Real-time motion image is available at www.texasheart.org/
Click here for real-time motion image: Fig.
journal.
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Caseous Calcification of the Mitral Annulus
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Volume 39, Number 6, 2012