|Year : 2020 | Volume
| Issue : 2 | Page : 184-186
An interesting case of hypertrophic cardiomyopathy with dystrophic calcification
Anand Palakshachar, Darshan P Thakkar, Rangaraj Ramalingam, CN Manjunath
Department of Cardiology, Sri Jayadeva Institute of Cardiovascular Sciences and Research, Bengaluru, Karnataka, India
|Date of Submission||15-Jan-2020|
|Date of Decision||25-Mar-2020|
|Date of Acceptance||20-Mar-2020|
|Date of Web Publication||27-Aug-2020|
Dr. Darshan P Thakkar
Department of Cardiology, Sri Jayadeva Institute of Cardiovascular Sciences and Research, Bengaluru, Karnataka
Source of Support: None, Conflict of Interest: None
Hypertrophic cardiomyopathy (HCM) with left ventricular non-compaction (LVNC) is unusual. Atherosclerotic coronary artery disease in these is rare. We reported a case of HCM with LVNC presenting as stable angina pectoris. Multimodality imaging studies will help to diagnose and guide the management of this uncommon condition.
Keywords: Coronary artery disease, dystrophic calcification, hypertrophic cardiomyopathy, left ventricular noncompaction
|How to cite this article:|
Palakshachar A, Thakkar DP, Ramalingam R, Manjunath C N. An interesting case of hypertrophic cardiomyopathy with dystrophic calcification. J Pract Cardiovasc Sci 2020;6:184-6
|How to cite this URL:|
Palakshachar A, Thakkar DP, Ramalingam R, Manjunath C N. An interesting case of hypertrophic cardiomyopathy with dystrophic calcification. J Pract Cardiovasc Sci [serial online] 2020 [cited 2021 Oct 20];6:184-6. Available from: https://www.j-pcs.org/text.asp?2020/6/2/184/293532
| Introduction|| |
Hypertrophic cardiomyopathy (HCM) and left ventricular non compaction (LVNC) are two distinct genetic cardiomyopathies as categorized by the American Heart Association. HCM characterized by asymmetrical hypertrophy with or without obstruction. HCM can present as heart failure symptoms or sudden cardiac death due to ventricular tachycardia/fibrillation. LVNC is a rare cardiomyopathy due to embryonic arrest in the left ventricular (LV) development and myofiber arrangement. It classically presents as heart failure and cardioembolic events. Echocardiographic studies have reported prevalence between 0.014% and 1.3% in the general population. LVNC have been reported to share overlapping genetic mutation. Overlapping phenotypic and clinical presentation are very rare. We report a case of phenotypically overlapping HCM and LVNC presenting as stable angina pectoris due to calcified thrombus.
| Case Report|| |
A 59-year-old male was admitted with complaints of chest pain and dyspnea on exertion of New York Heart Association Class II for 3 months duration. Physical examination was unremarkable with regular pulse 78/min, blood pressure of 130/70mmHg. Jugular venous pulse was normal. Normal S1 and S2 with no murmurs.
Past medical history
There was no significant medical or family history.
Coronary artery disease (CAD) and cardiomyopathies.
Electrocardiogram suggestive of LV hypertrophy with strain pattern. Echocardiography showed asymmetrical septal hypertrophy involving mid and apical septum (septal thickness 24-mm and septal-to-posterior wall thickness ratio 1.4:1) without significant LV outflow systolic gradient and calcification at the apex with the involvement of papillary muscles [Figure 1]a, [Figure 1]b, [Figure 1]c.
|Figure 1: Multimodality imaging of a 59-year-old patient with hypertrophic cardiomyopathy with dystrophic calcification. (a) Apical 4C view suggestive of calcification at the apex of left ventricular involving papillary muscle. (b) PLAX view showing calcification involving papillary muscle with septal hypertrophy. (c) M mode transthoracic echocardiogram showing septal hypertrophy with normal left ventricular function. (d) Cardiac computed tomography suggestive of dystrophic calcification involving papillary muscles, left ventricular apex. (e) Cardiac computed tomography showing calcified lesions involving mid and distal right coronary artery. (f) Magnetic resonance imaging showed features suggestive of hypertrophic cardiomyopathy and features of noncompaction with apical area showing mixture of thrombus and calcification. (g and h) Coronary angiogram suggestive of 90%–95% calcified stenosis in the mid-right coronary artery with calcification at the left ventricular apex. (i and j) Intravascular ultrasound showing a mixture of calcification with organized thrombus in the mid-right coronary artery.|
Click here to view
Multislice dual-source computed tomography (CT) was done to assess the extent of calcification, which showed dystrophic calcification involving papillary muscles, LV apex and calcified lesions involving mid and the distal right coronary artery (RCA) [Figure 1]d and [Figure 1]e. Cardiac magnetic resonance imaging (MRI) showed asymmetrical septal hypertrophy and features of non-compacted myocardium with the apical area showing a mixture of thrombus and calcification [Figure 1]f and [Figure 1]j. Coronary angiogram was suggestive of calcified intermediate lesion in mid-RCA [Figure 1]g.
Since it was a nonobstructive HCM Treadmill test was done to find the functional significance of the lesion, which was positive for inducible ischemia.
Intravascular ultrasound (IVUS) was done to further characterize the lesion which was suggestive of mixture of organized thrombus and calcification [Figure 1]h and [Figure 1]i.
The lesion was treated with coronary angioplasty with drug-eluting stent under IVUS guidance [Figure 2]a and [Figure 2]b. Patient was discharged on dual antiplatelets, statin, beta-blocker, angiotensin-converting enzyme – inhibitor, and anticoagulation. On 6 months follow–up, patient is asymptomatic with no limitation of activity.
|Figure 2: Multimodality imaging of a 59-year-old patient with hypertrophic cardiomyopathy with dystrophic calcification. (a) Coronary angioplasty is done with 3.5 mm × 15 mm drug-eluting stent in mid right coronary artery. (b) Final intravascular ultrasound imaging showing well-apposed stent struts.|
Click here to view
| Discussion|| |
HCM is a predominantly genetic cardiac disease related to sarcomere protein mutation with variable localization of myocardial hypertrophy. About one-third of patients have the nonobstructive form of HCM.
Prior reports describe cases of hypertrophic cardiomyopathy with calcified myocardium; however, the mechanism remains unknown. The vast majority of HCM patients also exhibit structurally abnormal intramural coronary arterioles with thickened vessel walls caused by media smooth muscle hyperplasia. These microvascular changes cause narrowing of the vessel lumen, which is likely responsible for an impaired vasodilator response and blunting of the coronary flow reserve. Some studies postulate that increased LV filling pressure in the setting of known microvascular disease associated with hypertrophic cardiomyopathy leads to decreased microvascular perfusion pressure. These abnormalities are believed to cause “small-vessel” ischemia, which, over extended periods, results in myocyte death and a repair process characterized by replacement myocardial fibrosis and dystrophic calcification.
The circumferential mid and apical septum and papillary muscle calcification, the most striking finding in this case, heralds the ultimate diagnostic impression. The calcified sub-endocardium can represent a marker of chronically infarcted myocardial tissue, a finding optimally demonstrated by cardiac CT, which necessitated evaluation of the coronary arteries.
Focal calcification of the coronary artery with calcification of mid and apical septum with hypertrophic cardiomyopathy makes the case unusual.
MRI was done to further delineate LV myocardial fibrosis, extent of thrombus formation and to rule out overlapping cardiomyopathies such as LVNC and LV endomyocardial fibrosis. Cardiac MRI revealed Asymmetrical septal hypertrophy with features of LV non compaction with ratio of compacted to non-compacted myocardium of >2.3. There were prominent trabaculations and thrombus formation in the non compacted layers at apex with calcification.
These overlapping cardiomyopathies have been reported previously. LV Non-compaction can present as thromboembolic events, including myocardial infarction. In our case, possible thromboembolic coronary artery disease was kept since IVUS characterize the lesion as mixture of thrombus and calcification rather than classical atherosclerotic lesion. IVUS also very useful in the intervention from stent sizing to optimal expansion, as in this case.
On 6 months follow-up, patient is currently asymptomatic.
| Conclusion|| |
HCM and LVNC along with dystrophic calcification with coronary artery disease are unusual. These overlapping cardiomyopathies are difficult to diagnose. Multimodality imaging studies will help to come to the conclusion. HCM is frequently accompanied by microvascular ischemia, and it is associated with a higher cardiovascular risk.
CAD might aggravate the ischemia and fibrosis of HCM and lead to worse clinical outcomes.
Our case had macrovascular disease in RCA hypothesized to be due to embolization from LV and recanalization and calcification of organized thrombus.
Invasive coronary angiogram is a luminogram with less specificity to delineate the nature of plaque. Hence, vascular imaging such as IVUS can facilitate detailed assessment and characterization of CAD and aid optimization of revascularization.
As per institute's guidelines, ethical clearance not required for case/image reports. However the authors certify that they have obtained all appropriate patient consent forms. In the form the patient has given his/her consent for his/her images and other clinical information to be reported in the journal. The patient understand that his/her name and initials will not be published and due efforts will be made to conceal his/her identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Maron BJ, Towbin JA, Thiene G, Antzelevitch C, Corrado D, Arnett D, et al
. Contemporary definitions and classification of the cardiomyopathies: An American Heart Association Scientific statement from the council on clinical cardiology, heart failure and transplantation committee; quality of care and outcomes research and functional genomics and translational biology interdisciplinary working groups; and council on epidemiology and prevention. Circulation 2006;113:1807-16.
Yuan L, Xie M, Cheng TO, Wang X, Zhu F, Kong X, et al
. Left ventricular noncompaction associated with hypertrophic cardiomyopathy: Echocardiographic diagnosis and genetic analysis of a new pedigree in China. Int J Cardiol 2014;174:249-59.
Maron BJ, Ommen SR, Semsarian C, Spirito P, Olivotto I, Maron MS. Hypertrophic cardiomyopathy: Present and future, with translation into contemporary cardiovascular medicine. J Am Coll Cardiol 2014;64:83-99.
Maron BJ, Wolfson JK, Epstein SE, Roberts WC. Intramural (“small vessel”) coronary artery disease in hypertrophic cardiomyopathy. J Am Coll Cardiol 1986;8:545-57.
Nance JW Jr., Crane GM, Halushka MK, Fishman EK, Zimmerman SL. Myocardial calcifications: Pathophysiology, etiologies, differential diagnoses, and imaging findings. J Cardiovasc Comput Tomogr 2015;9:58-67.
Zuccarino F, Vollmer I, Sanchez G, Navallas M, Pugliese F, Gayete A. Left ventricular noncompaction: Imaging findings and diagnostic criteria. AJR Am J Roentgenol 2015;204:W519-30.
Witzenbichler B, Maehara A, Weisz G, Neumann FJ, Rinaldi MJ, Metzger DC, et al
. Relationship between intravascular ultrasound guidance and clinical outcomes after drug-eluting stents: The assessment of dual antiplatelet therapy with drug-eluting stents (ADAPT-DES) study. Circulation 2014;129:463-70.
[Figure 1], [Figure 2]