Author + information
- Dany Debs, MD∗ (, )@debs_dany@FNabiMD,
- Basil Al-Sabeq, MD,
- Mahesh Ramchandani, MD,
- Mirette Fahim, MD and
- Faisal Nabi, MD
- Department of Cardiology, Houston Methodist DeBakey Heart and Vascular Center, Houston, TexasDepartment of Cardiology, Houston Methodist DeBakey Heart and Vascular Center, Houston, Texas
- ↵∗Address for correspondence:
Dr. Dany Debs, Department of Cardiology, Houston Methodist Hospital, 6550 Fannin Street, Smith Tower Suite #18-209, Houston, Texas 77030.
A 77-year-old woman presented for assessment of symptomatic mitral regurgitation. Multimodality cardiac imaging revealed severe mitral regurgitation secondary to mitral valve prolapse. Significant mitral annular calcification with dramatic intramyocardial calcification was also incidentally discovered. She was then given a diagnosis of idiopathic cardiac osseous metaplasia and was managed conservatively. (Level of Difficulty: Advanced.)
A 77-year-old female former smoker with a history of hypertension, dyslipidemia, and prior admissions for heart failure presented for management of symptomatic severe mitral regurgitation (MR). Transthoracic echocardiography showed severe mitral annular calcification (MAC) and P2 segment prolapse with moderate to severe eccentric MR directed anteriorly, despite normal left ventricular (LV) size and function of 65% to 69%. Cardiac magnetic resonance was performed to quantitate the volume of regurgitation, and it confirmed normal LV size and function, as well as P2 prolapse with severe MR (regurgitant volume, 50 ml; regurgitant fraction, 51%) directed anteromedially. Interestingly, however, on late gadolinium enhancement imaging, extensive, patchy non–coronary artery disease scar was seen involving the septum and lateral wall diffusely (Figure 1D), thus raising concerns for an infiltrative process or prior myocarditis. She was referred for surgical mitral valve replacement evaluation, and pre-operative cardiac computed tomography (CT) was ordered for coronary assessment. CT showed nonobstructive coronary arteries, but, of interest, severe, bulky MAC with extension of dense calcifications diffusely throughout the septal, anterior, and lateral LV myocardium (Figures 1A to 1C; Figures 1E and 1F are 3-dimensional cardiac CT reconstructions of these intramyocardial calcifications). Idiopathic cardiac osseous metaplasia was concurrently diagnosed.
Work-up for known causes of intramyocardial calcification (1) was undertaken, and results of tests for primary hyperparathyroidism (parathyroid hormone), chronic kidney disease (creatinine), infectious disease (white blood cell count and urine cultures), endomyocardial fibrosis (eosinophil count), and tuberculosis (interferon-gamma assay) were normal. Despite symptomatic, severe MR, the presence of extensive MAC with dramatic intramyocardial calcification did not allow for either percutaneous or surgical options. Hence the patient was managed conservatively.
Idiopathic cardiac osseous metaplasia is a rare condition often presenting challenges in clinical management and generally associated with poor outcomes. It often leads to conduction abnormalities and intractable heart failure. In our case, we believe that the intramyocardial calcifications, best seen by cardiac CT, were an extension of the exaggerated MAC. MAC is known to result from mitral valve leaflet stress (mitral valve prolapse) and risk factors for atherosclerosis (older age, hypertension, ex-smoker, dyslipidemia) (2). In retrospect, the scar seen on late gadolinium enhancement imaging was likely related to a fibrotic or inflammatory response to the profound calcification present (3). Furthermore, we postulate that intramyocardial calcifications appeared as a spiral following the normal course of myocardial fibers. Moreover, the constricting nature of the calcium may also have prevented the left ventricle from dilating, as is seen in most chronic, severe MR settings. Our case illustrates the value of multimodality imaging in assessing the severity, mechanism, and consequence of MR and the additional value of tissue characterization using different imaging techniques.
Dr. Ramchandani has received consulting fees from LivaNova. All other have reported that they have no relationships relevant to the contents of this paper to disclose.
- Received April 9, 2019.
- Revision received May 2, 2019.
- Accepted May 2, 2019.
- 2019 The Authors
- Nance J.W. Jr..,
- Crane G.M.,
- Halushka M.K.,
- Fishman E.K.,
- Zimmerman S.L.
- Abramowitz Y.,
- Jilaihawi H.,
- Chakravarty T.,
- Mack M.J.,
- Makkar R.R.
- Kirk R.S.,
- Russell J.G.