Al-Mukhtar, Aws-

Almas, Talal

Haas, Donald C.

Inayat, Asad

Ullah, Waqas

English

Jefferson Digital Commons

Thomas Jefferson University Jefferson Digital Commons Division of Cardiology Faculty Papers Division of Cardiology 10-1-2020 Caseous mitral valve calcification and concurrent hypertrophic obstructive cardiomyopathy: A rare cause of stroke Waqas Ullah Thomas Jefferson University Donald C. Haas Thomas Jefferson University Talal Almas Royal College of Surgeons in Ireland Aws- Al-Mukhtar Imperial College London Asad Inayat Khyber Teaching Hospital Follow this and additional works at: https://jdc.jefferson.edu/cardiologyfp  Part of the Cardiology Commons Let us know how access to this document benefits you Recommended Citation Ullah, Waqas; Haas, Donald C.; Almas, Talal; Al-Mukhtar, Aws-; and Inayat, Asad, "Caseous mitral valve calcification and concurrent hypertrophic obstructive cardiomyopathy: A rare cause of stroke" (2020). Division of Cardiology Faculty Papers. Paper 68. https://jdc.jefferson.edu/cardiologyfp/68 This Article is brought to you for free and open access by the Jefferson Digital Commons. The Jefferson Digital Commons is a service of Thomas Jefferson University's Center for Teaching and Learning (CTL). The Commons is a showcase for Jefferson books and journals, peer-reviewed scholarly publications, unique historical collections from the University archives, and teaching tools. The Jefferson Digital Commons allows researchers and interested readers anywhere in the world to learn about and keep up to date with Jefferson scholarship. This article has been accepted for inclusion in Division of Cardiology Faculty Papers by an authorized administrator of the Jefferson Digital Commons. For more information, please contact: JeffersonDigitalCommons@jefferson.edu. CorrespondenceCaseous mitral valve calcification and concur-rent hypertrophic obstructive cardiomyopathy:A rare cause of strokeMitral annulus calcification (MAC) is a relatively common con-dition with a prevalence of about 14%, often seen at old age andresults from degenerative changes in the mitral valve fibrousskeleton [1]. An exceedingly rare and adverse variant of MAC isthe caseous calcification of the mitral annulus (CCMA), whichaccounts for only 0.6% of all MAC cases [2]. It can be easily con-fused with tumors, vegetations, or cysts; thus, often underdiag-nosed and mistreated. CCMA in association with othercomorbidities can potentially increase the risk of cerebrovascularaccidents (CVA). Here, we present the first case of CCMA with con-comitant hypertrophic obstructive cardiomyopathy (HOCM) pre-senting with cardioembolic stroke complicated by hemorrhagicconversion.1. Case presentationA 68-year-old woman with a past medical history of hyperten-sion and HOCM presented to the emergency department (ED) withsudden left-sided facial weakness and slurred speech for the last2 h. She also complained of sudden onset of dizziness and difficultyconcentrating, while she was working from home, attempting tosign into a zoom meeting. She noted that she was not able to signin, and was having trouble with her coordination and typing on hercomputer.In the ED, she was afebrile with a temperature of 98F, tachycar-diac (heart rate 120) and hypotensive (90/60 mmHg). Her oxygensaturation (SaO2) was 85% on room air. Her physical examinationwas significant for right middle lung rhonchi, facial droop and gar-bled speech. Her National Institutes of Health Stroke Scale (NIHSS)was 4. Her cardiovascular examination revealed a pansystolic mur-mur at the mitral area.Laboratory investigation was significant for leukocytosis (whiteblood cells count of 17.4  109/L). Rest of the blood work wasunremarkable. Her nasal swab real-time fluorescence polymerasechain reaction (RT-PCR) for severe acute respiratory syndromecoronavirus-2 (SARS-CoV-2) returned negative. The computedtomography (CT) head ruled out hemorrhagic stroke. Her brainmagnetic resonance imaging (MRI) was consistent with bihemi-spheric cortical and subcortical embolic strokes. Electrocardiogram(EKG) showed normal sinus rhythm. Transthoracic echocardio-gram (TTE) revealed a hyperdynamic anterior ventricle, systolicanterior motion of the mitral valve, peak resting left ventricularoutflow tract (LVOT) gradient of 84 mmHg and posterior mitralleaflet mass suggestive of vegetation complicated by moderate-to-severe mitral regurgitation. Her cardiac CT showed posteriorMV leaflet calcification (Fig. 1). Cardiac MRI was consistent withLVEF of 82%; focal HOCM with LVOT obstruction and systolic ante-rior motion of the mitral valve leaflet and moderate mitral valveregurgitation (Figs. 2, 3) There was no delayed myocardialenhancement to suggest scar, infarct, or infiltrative disorder.She was placed on supplemental oxygen at 2 L/min via nasalcannula and started on antibiotics for aspiration pneumonia. Shewas emergently treated with tissue plasminogen activator (tPA),suspecting cerebrovascular accident (CVA) in the right middlecerebral artery (MCA) territory. She was admitted to the neurocrit-ical unit for close neurological monitoring, stroke workup, and fur-ther medical management.Patient developed a sudden onset of headaches 6 h later. Herneurological exam remained unchanged. A repeat CT head revealeda large right frontal and temporal intracerebral hemorrhage asso-ciated with right-sided subarachnoid hemorrhage (SAH) and rightto left midline shift. She was managed with supportive care andstarted on a nicardipine drip. She also received hypertonic salinefor osmotic diuresis for goal sodium level of 155–160 mEq/L forcerebral edema. She received a loop recorder and was ultimatelydischarged to a rehabilitation center with residual weakness. Onfollow-up, the biotel cardionet monitor recorded no episodes ofAF, heart block, pauses, or other arrhythmias.2. DiscussionCCMA is a distinctive and special variant of MAC that resultsfrom liquefactive necrosis of the posterior annulus of the mitralvalve and is usually considered a benign condition [1,2]. In rarecases, however, it may compromise the normal function of themitral valve, subsequently causing regurgitation or mitral stenosis[3]. The later can cause significant left ventricular inflow tractobstruction. Other rare valvular complications of CCMA may alsopredispose patients to left ventricular outflow obstruction (LVOT),atrial fibrillation (AF), and aortic valve abnormalities [4]. It can alsolead to the same hemodynamic consequences of constrictive peri-carditis. While all these complications significantly increase therisk of CVA, we believe that CCMA independently carries a higherrisk of cerebrovascular complications especially in presence ofHOCM [1,3,5,6].A review of 130 patients with CCMA reported a 19.2% preva-lence of CVAs, significantly higher than seen with MAC alone(11.8%) [7]. This highlights the importance of differentiating CCMAfrom MAC to prevent dire consequences. The suggestive echocar-diographic findings of CCMA include areas of decreased echogenic-ity, a central echolucent zone, and the absence of acousticshadowing. Microscopically, CCMA is characterized by liquefactionnecrosis with amorphous eosinophilic acellular material sur-rounded by lymphocytes and macrophages amid multiple smallcalcifications [1]. Intriguingly, only four of the reported 25(16.0%) patients with CCMA who suffered a CVA had a history ofhttps://doi.org/10.1016/j.ijcha.2020.1006472352-9067/ 2020 The Author(s). Published by Elsevier B.V.This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).IJC Heart & Vasculature 30 (2020) 100647Contents lists available at ScienceDirectIJC Heart & Vasculaturejournal homepage: www.journals .e lsevier .com/i jc -heart -and-vasculatureAF. This further indicates that CCMA might serve as an indepen-dent risk factor for CVA.The risk of stroke in patients with HOCM alone is merely 1.0%,which is largely attributed to the high rate of AF in these patients[8]. However, in our case, a biotel cardionet monitor recorded noepisodes of AF indicating that the hemodynamic instability cou-pled with cardiac embolization was the repercussions elicited byCCMA. We believe that embolization of the caseous necrotic debrisFig. 1. CT scan showing pleural effusion and mitral annular calcification.Fig. 2. Coronal section of cardiac MRI showing LVOT obstruction and mitral annular calcification.Correspondence IJC Heart & Vasculature 30 (2020) 1006472of the mitral annulus was the major cause of multifocal stroke inour case. The stroke risk in our case was further precipitated bythe LVOT obstruction induced hemodynamic instability in pres-ence of HOCM.Our case also highlights the importance of early recognition ofthis potentially fatal condition. Diagnosing CCMA is itself an oner-ous diagnostic challenge due to its rarity and perplexing clinicalmanifestations. Prompt detection and management of CCMA andHOCM may aid in foiling adverse outcomes, such as CVA. Lesionsafflicting the posterior mitral annulus on echocardiography shouldbe evaluated through further imaging to rule out CCMA so that thegrave ramifications elicited by it can be prevented. In our case, adefinitive diagnosis was made with MRI and CT scan which ruledout other causes and confirmed the presence of CCMA. This neces-sitates the need for multimodality imaging to yield a definitivediagnosis of CCMA. The patient also developed moderate to severemitral regurgitation due to prolonged CCMA. Previous studies haveshown that mitral regurgitation develops secondary to longstand-ing MAC [9]. This grave valvular abnormality, along with thepatient’s history of HOCM and LVOT culminated in hemodynamicinstability and CVA.It is unclear whether treating patients with CCMA prophylacti-cally with valve replacement can reduce the risk of stroke. Also, therisk of tPA associated hemorrhagic conversion of embolic stroke inthese patients warrants more studies on a larger scale. Currentstudies focused on the imperativeness of conservative manage-ment in treating early CCMA by controlling other cardiovascularcomorbidities (hypertension, dyslipidemia) and prevention ofcomplications such as arrhythmias [10,11]. Antithrombotic ther-apy is reserved for patients with CVA and cardioembolic complica-tions. Patients with symptomatic severe mitral regurgitation andmitral stenosis are treated with surgical mitral valve replacementas percutaneous mitral balloon valvuloplasty is generally noteffective. Due to a high risk of operative mortality a careful risk-benefit evaluation is recommended.3. ConclusionIn our case, CCMA along with concurrent HOCM and LVOTobstruction resulted in hemodynamic instability leading to car-dioembolic stroke and subsequent hemorrhagic conversion withtPA. We, therefore, advocate for early recognition and considera-tion of elective surgical resection of CCMA in patients with HOCM,even in asymptomatic patients who are good surgical candidates.Nevertheless, more studies are required to understand the patho-genesis and the precise clinical course of CCMA in patients withHOCM.4. Learning objectivesCCMA along with concurrent HOCM and LVOT obstruction canresult in hemodynamic instability, leading to cardioembolic strokeand subsequent hemorrhagic conversion with tPA. Early recogni-tion and consideration of elective surgical resection of CCMA inpatients with HOCM, even in asymptomatic patients who are goodsurgical candidates, therefore remains pivotal in avoiding potentialcomplicationsFundingNone.Declaration of Competing InterestThe authors report no relationships that could be construed asa conflict of interest.Fig. 3. Sagittal section of cardiac MRI showing LVOT obstruction and mitral annular calcification.Correspondence IJC Heart & Vasculature 30 (2020) 1006473AcknowledgementsNone.References[1] A. Goldberg, G. Singh, S. Boateng, M. Tracy, Caseous calcification of the mitralannulus causing multiple cardioembolic strokes, J. Am. Coll. Cardiol. 67 (13Supplement) (2016 Apr 5) 1184.[2] H. Arora, P. Madan, L. Simpson, R.F. Stainback, Caseous Calcification Of TheMitral Annulus, Tex Hear Inst. J. 35 (2) (2008) 211–213.[3] E. Curl, E. Riemer, Caseous calcification of the mitral annulus : case report andbrief review, Eur Heart J. 2 (2018) 1–5.[4] C. Report, K. Liu, J. Hollman, N. Deumite, Caseous mitral annular calcificationpresenting as a stroke: a cracking of the shell, Ann. Atheroscler. Res. [Internet]1 (2) (2018) 1–4.[5] G. Minardi, C. Manzara, G. Pulignano, P.G. Pino, H. Pavaci, M. Sordi, et al,Caseous calcification of the mitral annulus with mitral regurgitation andimpairment of functional capacity: A case report, J. Med. Case Rep. 2 (2008) 4–7.[6] D. Kim, C.Y. Shim, G.R. Hong, H. Jeong, J.W. Ha, Morphological and functionalcharacteristics of mitral annular calcification and their relationship to stroke,PLoS ONE 15 (1) (2020) 1–11.[7] C.A. Dietl, C.M. Hawthorn, V. Raizada, Risk of cerebral embolization withcaseous calcification of the mitral annulus, Open Cardiovascular Med. J. 10(2016) 221.[8] S. Haruki, Y. Minami, N. Hagiwara, Stroke and embolic events in hypertrophiccardiomyopathy, Stroke 47 (2016) 936–942.[9] N. Mallat, M. Limeme, H. Zaghouani, F. Mootemri, S. Majdoub, H. Amara, et al,Caseous calcification of the mitral annulus on MDCT: a rare intracardiac mass,Acta Radiol. Short Reports 2 (7) (2013), 204798161350217.[10] Y. Okada, Surgical management of mitral annular calcification, Gen. Thorac.Cardiovasc. Surg. 61 (11) (2013 Nov) 619–625.[11] L.S. Fong, A.J. McLaughlin, N.L. Okiwelu, I.A.J. Nordstrand, M. Newman, J.Passage, et al, Surgical management of caseous calcification of the mitralannulus, Ann. Thorac. Surg. 104 (3) (2017 Sep) e291–e293.Waqas Ullah⇑Donald HaasAbington Hospital - Jefferson Health, Abington, PA, United States⇑ Corresponding author.E-mail address: waqasullah.dr@gmail.com (W. Ullah)Talal AlmasRoyal College of Surgeons in Ireland, Dublin, IrelandAws- Al-MukhtarImperial College London, London, United KingdomAsad InayatKhyber Teaching Hospital, Peshawar, PakistanReceived 5 September 2020Received in revised form 12 September 2020Accepted 16 September 2020Available online 29 September 2020Correspondence IJC Heart & Vasculature 30 (2020) 1006474

Caseous mitral valve calcification and concurrent hypertrophic obstructive cardiomyopathy: A rare cause of stroke

https://jdc.jefferson.edu/cgi/viewcontent.cgi?article=1068&amp;context=cardiologyfp

Caseous mitral valve calcification and concurrent hypertrophic obstructive cardiomyopathy: A rare cause of stroke

Abstract

Similar works

Full text

Available Versions

Jefferson Digital Commons