|Year : 2022 | Volume
| Issue : 4 | Page : 192-195
Mitral annular calcification leading to severe mitral stenosis in a patient with severe calcific aortic stenosis and complete heart block: Different shades of calcium in heart
Najeeb Ullah Sofi, Santosh Kumar Sinha, Mohit Sachan
Department of Cardiology, LPS Institute of Cardiology, GSVM, Kanpur, Uttar Pradesh, India
|Date of Submission||24-Jul-2022|
|Date of Decision||01-Aug-2022|
|Date of Acceptance||02-Aug-2022|
|Date of Web Publication||19-Dec-2022|
Dr. Najeeb Ullah Sofi
GT Road, Swaroop Nagar, Kanpur - 208 002, Uttar Pradesh
Source of Support: None, Conflict of Interest: None
Calcium deposition in the heart can present in various ways. Mitral annular calcification (MAC) can cause mitral regurgitation but severe mitral stenosis has been reported very rarely. Mitral stenosis in the Indian subcontinent is mostly caused by rheumatic heart disease; however, here, we present a case of severe mitral stenosis due to MAC in a hypertensive and diabetic female that also had severe calcific aortic stenosis and conduction defect. Clinicians need to be aware of other causes of acquired mitral stenosis that include systemic lupus erythematosus, antiphospholipid antibody syndrome, carcinoid syndrome, mucopolysaccharidosis, Whipple disease, radiation, and MAC. Although MAC usually causes mitral regurgitation, severe mitral stenosis has been reported very rarely. However, when MAC is the cause of severe mitral stenosis, those patients are poor candidates for mitral valve replacement. Differentiating the etiology of mitral stenosis is of therapeutic and prognostic significance.
Keywords: Mitral annular calcification, mitral stenosis, rheumatic heart disease
|How to cite this article:|
Sofi NU, Sinha SK, Sachan M. Mitral annular calcification leading to severe mitral stenosis in a patient with severe calcific aortic stenosis and complete heart block: Different shades of calcium in heart. J Indian coll cardiol 2022;12:192-5
|How to cite this URL:|
Sofi NU, Sinha SK, Sachan M. Mitral annular calcification leading to severe mitral stenosis in a patient with severe calcific aortic stenosis and complete heart block: Different shades of calcium in heart. J Indian coll cardiol [serial online] 2022 [cited 2023 Feb 8];12:192-5. Available from: https://www.joicc.org/text.asp?2022/12/4/192/364213
| Introduction|| |
Calcium deposition in the heart can be present in different ways [Table 1]. Mitral annular calcification (MAC) can cause mitral regurgitation but severe mitral stenosis has been reported very rarely. Mitral stenosis in the Indian subcontinent is mostly caused by rheumatic heart disease; however, here, we present a case of severe mitral stenosis due to MAC in a hypertensive and diabetic female that also had severe calcific aortic stenosis and conduction defect.
| Case Report|| |
A 65-year-old hypertensive and diabetic female had a dual-chamber pacemaker implanted for complete heart block 4 years back. Echocardiography at that time revealed mild MAC and moderate aortic stenosis with a mean gradient of 27 mmHg across the aortic valve. The patient recently visited our clinic for complaints of dyspnea and angina on exertion that had progressed over a year. On examination, the patient had a pulse rate of 86/min and blood pressure of 136/88. A cardiac examination revealed a long mid-diastolic murmur at the apex and an ejection systolic murmur at the aortic area.
Chest X-ray revealed a dual-chamber pacemaker with leads properly placed and mitral and aortic calcification [Figure 1]. Electrocardiogram showed left bundle branch block with strain pattern. Troponin I was negative. Her serum creatinine was 1.09 mg/dl and serum calcium was 9.07 mg/dl.
|Figure 1: Chest X-ray showing dual-chamber pacemaker, mitral (arrow), and aortic calcification (arrowhead)|
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Echocardiography revealed concentric left ventricular (LV) hypertrophy, severe MAC involving leaflets causing severe mitral stenosis, and moderate mitral regurgitation [Figure 2] and [Figure 3]. Mitral valve area by planimetry was 1.2 cm2 and pressure half time (PHT) was 1.1 cm2. The mitral valve mean pressure gradient was 9.08 mmHg [Figure 4], [Figure 5], [Figure 6]. The aortic valve was severely calcified, velocity across the aortic valve was 5.24 m/s, and the mean gradient across the valve was 71.62 mmHg [Figure 7]. LV ejection fraction was 60%. The patient is planned to be discussed at the heart team meeting.
|Figure 3: PSLA view showing concentric LVH (arrowhead) and aortic (star) and mitral calcification (arrow). PSLA: Parasternal Long Axis, LVH: Left Ventricular Hypertrophy|
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|Figure 4: MAC (arrows) and mitral valve area by planimetry 1.2 cm2. MAC: Mitral annular calcification|
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| Discussion|| |
Severe mitral stenosis in the Indian subcontinent and other endemic zones is mostly due to rheumatic etiology. Radiation, systemic lupus erythematosus, antiphospholipid antibody Syndrome (APLA), carcinoid syndrome, mucopolysaccharidosis, Whipple disease, and the MAC are rare causes of mitral stenosis. In the Western world, MAC is a common cause of mitral stenosis due to the elderly population and the rarity of rheumatic disease, but it rarely causes severe mitral stenosis.
Pressman et al. in their study of 37 patients with MAC found that mitral valve gradient progressed in half of the patients. They observed that, even though severe mitral stenosis is unusual in patients with MAC, the preexisting gradient at rest and severe overall cardiac calcification appear to be a risk for the development of severe mitral stenosis in these patients. Hypertension, aortic stenosis, hypertrophic obstructive cardiomyopathy, and renal failure are other risk factors for MAC.,
Echocardiographically, MAC is graded according to the annular circumference surrounded by calcium. Calcium occupying 1/3–1/2 of the annular circumference is considered moderate, smaller accumulations are regarded as mild, and more than 50% of accumulations are considered severe. In addition, MAC that projects into the LV inlet or is >4 mm in thickness (measured in the anteroposterior direction in the short-axis view) is also regarded as severe.
Transmitral gradient (TMG) in mitral stenosis due to MAC is not proportional to the severity of the mitral valve area due to the multiple confounding factors that lead to an increase in diastolic pressure of the left ventricle. Reduced LV end-diastolic volume due to LV concentric remodeling and reduced mitral annular translational motion, may decrease LV stroke volume and reduce TMG.
As most patients with MAC are frail, elderly, and likely to have one or more comorbidities, including diabetes mellitus, hypertension, coronary artery disease, and renal failure, they are poor candidates for mitral valve replacement (MVR). Transcatheter MVR (TMVR) in patients with calcific MS is currently performed using transcatheter heart valves designed for use in the aortic position. This strategy is unfortunately associated with several complications including suboptimal seating due to calcium, leading to paravalvular leak, device migration into the left atrium, and LV outflow tract obstruction. In the largest series of valve-in-MAC procedures reported to date 30-day mortality was 25%. In light of these risks, TMVR is generally reserved for those deemed to be at high surgical risk.
Our patient had a complete heart block likely due to calcium deposition and was diagnosed with moderate aortic stenosis with a gradient of 27 mmHg and MAC 4 years before. Her disease progressed in 4 years and now had severe aortic stenosis and severe mitral stenosis. Management of such patients is challenging and surgical or transcatheter valve replacement is associated with high mortality. It is important that physicians working in endemic zones of rheumatic disease be aware of other causes of mitral stenosis as the management and prognosis of these cases are different compared to that of rheumatic heart disease.
| Conclusions|| |
Calcium deposition in the heart can take various forms and one is mitral stenosis. Severe mitral stenosis in developing nations and endemic zones of rheumatic fever is almost always considered to be due to rheumatic etiology until and unless proven otherwise. However, clinicians need to be aware of other causes of acquired mitral stenosis that include systemic lupus erythematosus, Whipple disease, carcinoid syndrome, mucopolysaccharidosis, and MAC.
MAC can cause mitral regurgitation but severe mitral stenosis has been reported very rarely. However, when MAC is the cause of severe mitral stenosis, those patients are poor candidates for MVR. Differentiating the etiology of mitral stenosis is of therapeutic and prognostic significance.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]