Circulation: Heart Failure. 2008;1:290-292
doi: 10.1161/CIRCHEARTFAILURE.108.799437
Images and Case Reports in Heart Failure |
Endocardial Calcification of Left Atrium, Tracheobronchopathia Osteoplastica, and Calcified Aortic Arch in a Patient With Dyspnea
Ulrike M. Müller, MD
;
Stephan Gielen, MD
;
Gerhard C. Schuler, MD
and
Matthias Gutberlet, MD
From the Departments of Internal Medicine Cardiology, (U.M.M., S.G., G.C.S.), and Diagnostic and Interventional Radiology (M.G.), University of Leipzig, Heart Centre, Leipzig, Germany.
Correspondence to Ulrike M. Müller, MD, Department of Internal Medicine/Cardiology, University of Leipzig, Heart Centre, Strümpellstr. 39, D-04289 Leipzig, Germany. E-mail ulrike.mueller{at}herzzentrum-leipzig.de
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Introduction
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A 69-year-old female patient was admitted to our hospital for
further cardiac evaluation with symptoms of dyspnea (New York
Heart Association class III) and peripheral edema. Her history
revealed artificial mitral valve replacement due to mitral stenosis
8 years earlier, severe chronic pulmonary hypertension, and
severe tricuspid valve incompetence.
Electrocardiogram showed normofrequent atrial fibrillation. Transthoracic echocardiography indicated moderate biatrial enlargement, although both ventricles had normal dimensions and normal global systolic function. The transmitral prosthetic valve gradient was 14/6 mm Hg. We documented severe tricuspid regurgitation and only mild mitral regurgitation. Nevertheless, echocardiography was difficult to perform because of lack of an adequate acoustic window and metal artifacts of the mitral valve prosthesis. Reflections at the epicardial surface close to the mitral valve were therefore interpreted as calcifications of pericardial layer, and constrictive pericarditis was expected to be the most likely cause of the patient’s symptoms and diastolic left ventricle dysfunction.
By right heart catheterization, severe postcapillary hypertension was confirmed (systolic/diastolic/mean pulmonary artery pressure, 65/23/43 mm Hg; mean pulmonary wedge pressure, 29 mm Hg). In all cardiac cavities, an end-diastolic pressure equilibration was measured (Figure 1). Pressure curves did not show a dip-plateau phenomenon. Therefore, constrictive pericarditis was excluded. However, in a 64-row multidetector computed tomography scan, the endocardial layer of the left atrium (LA) and the aortic arch were heavily calcified (Figure 2), whereas the pericardium itself did not show any relevant calcifications. As an additional finding, the patient showed tracheobronchopathia osteoplastica (Figure 2A and 2B). Cardiac MRI was performed to further assess ventricular and atrial function (Figure 3). Any acute or persistent cardiac inflammation or interstitial storage cardiomyopathy could be excluded by edema-sensitive T2-weighted short T1 inversion recovery (STIR) images and T1-weighted images early and late after intravenous Gd-DTPA according to the protocol previously described.1 The dynamic cine MRI study showed no relevant changes in LA volumes during the heart cycle, indicating a stiff LA due to the heavily isolated calcification of the endocardial surface of the LA (Figure 3).
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Figure 1. End-diastolic pressure equilibration in all cardiac cavities. Pressure curves of left ventricle and left atrium (A), right ventricle and left ventricle (B), right atrium and left ventricle (C).
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Figure 2. A, Conventional-plane x-ray film of the thorax showing sternal cerclages after mitral valve replacement and the typical findings of a tracheobronchopathia osteoplastica (arrows). B, Three-dimensional reconstruction of a multidetector computed tomography data set (64 rows) of the isolated calcified LA in a frontal view. C, Axial 2D reconstruction of the isolated calcification of the LA (black arrows) at the endocardial surface and the open mechanical mitral valve (black broken arrow). D, Three-dimensional reconstruction of a multidetector computed tomography data set (64 rows) of the isolated calcified LA in a dorsal view and left anterior oblique view (E), displaying the opened artificial mitral valve and isolated calcifications at the LA. LV indicates left ventricle; RA, right atrium, RV, right ventricle.
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Figure 3. Cine MRI of a 4-chamber view of the heart during diastole (A) and systole (B), showing contraction of the ventricles and differences in size of the right atrium but not of the isolated calcified LA. Dephasing artifacts at the tricuspid valve indicate tricuspid incompetence. Metal artifacts are obvious at the artificial mitral valve.
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The patient’s symptoms were explained as combination of
elevated LA pressure and pulmonary hypertension. The singular
calcification of the stiff LA led most likely to reduced filling
of the LA itself. In combination with diastolic dysfunction
and former mitral stenosis, it might be the main cause for backward
failure and secondary pulmonary hypertension, tricuspid incompetence,
and right heart failure. Therapeutic management included medication
for diastolic dysfunction and decrease of afterload with diuretics
(hydrochlorothiazide, furosemide), angiotensin-converting enzyme
inhibitors and β-blockers. A surgical approach was abandoned
because of low probability of success.
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Discussion
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Two rare conditions were found in 1 patient: a calcified LA
and a tracheobronchopathia osteoplastica. The presence of LA
calcification, also known as "porcelain atrium" or "coconut
atrium," has been reported as a result of extensive rheumatic
pancarditis.
2,3 The etiology of tracheobronchopathia osteoplastica,
a mostly asymptomatic condition of the tracheobronchial tree
with a probably benign course, is still unknown.
4 Several other
mechanisms may account for endocardial LA calcification, including
postoperative complication after mitral valve replacement due
to hematoma and inflammation; atrial wall response to chronic
strain forces present in the setting of mitral disease, idiopathic,
or in the context of rheumatic fever in combination with tracheobronchopathia
osteoplastica and calcification of the aorta; ischemic myocardial
damage with dystrophic calcification in remodeling; or hypercalcemia
and hyperphosphataemia (eg, in metastatic calcification or in
combination with renal failure). In our patient, malignancies,
electrolyte disturbances, or renal failure could be excluded.
Although the patient was unable to recall a history of rheumatic
disease, which may have been oligosymptomatic, it seems to be
the most likely diagnosis.
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Acknowledgments
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Disclosures
None.
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References
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1. Gutberlet M, Spors B, Thoma T, Bertram H, Denecke T, Felix R, Noutsias M, Schultheiss HP, Kühl U. Suspected chronic myocarditis at cardiac mr: diagnostic accuracy and association with immunohistologically detected inflammation and viral persistence.
Radiology. 2008; 246: 401–409.
[Abstract/Free Full Text]2. Pulikal G, Marshall A. Images in clinical medicine. Complete calcification of a rheumatic left atrium. N Engl J Med. 2006; 354: 2262.[Free Full Text]
3. Vallejo JL, Merino C, Gonzalex-Santos JM, Bastida E, Albertos J, Riesgo MJ, González de Diego F. Massive calcification of the left atrium: surgical implications. Ann Thorax Surg. 1995; 60: 1226–1229.[CrossRef]
4. Thomas D, Stonell C, Hasan K. Tracheobronchopathia osteoplastica: incidental finding at tracheal intubation. Br J Anaesth. 2001; 87: 515–517.[Abstract/Free Full Text]
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Introduction
Discussion