|INTERESTING CASE REPORT
|Year : 2019 | Volume
| Issue : 1 | Page : 36-37
“Inverted” Asymmetrical hypertrophy of left ventricle: Unusual variant of an uncommon disease
Shaheen Ahmad1, Mukta Agarwal2, Rahul Ranjan1, Rashid Anwar1
1 Department of Cardiology, All India Institute of Medical Sciences, Patna, Bihar, India
2 Department of Obstetrics and Gynecology, All India Institute of Medical Sciences, Patna, Bihar, India
|Date of Web Publication||15-Mar-2019|
Department of Cardiology, All India Institute of Medical Sciences, Patna - 801 505, Bihar
Source of Support: None, Conflict of Interest: None
Hypertrophic cardiomyopathy (HCM) is a rare genetic disease. Usual presentation is asymmetrical hypertrophy of interventricular septum as compared to the posterior wall of the left ventricle. We present a rare variant of HCM in which the posterior wall of the left ventricle is more hypertrophied than ventricular septum (inverted asymmetry). Very few cases are reported in literature.
Keywords: Hypertrophic cardiomyopathy, inverted asymmetrical hypertrophy, posterior wall hypertrophy
|How to cite this article:|
Ahmad S, Agarwal M, Ranjan R, Anwar R. “Inverted” Asymmetrical hypertrophy of left ventricle: Unusual variant of an uncommon disease. J Indian Acad Echocardiogr Cardiovasc Imaging 2019;3:36-7
|How to cite this URL:|
Ahmad S, Agarwal M, Ranjan R, Anwar R. “Inverted” Asymmetrical hypertrophy of left ventricle: Unusual variant of an uncommon disease. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2019 [cited 2019 Sep 19];3:36-7. Available from: http://www.jiaecho.org/text.asp?2019/3/1/36/254255
| Introduction|| |
Hypertrophic cardiomyopathy (HCM) is a genetic disorder. The clinical spectrum of HCM is complex and includes a variety of phenotypes, which leads to different types of manifestations. Although most of the patients are asymptomatic, 25% will develop symptoms or risk of arrhythmias and sudden cardiac death. Interventricular septum usually shows the greatest magnitude of hypertrophy followed by the anterior free wall. The posterior left ventricular (LV) free wall is the least affected region in HCM. We are reporting a rare phenotype of rare genetic disease in which “inverted” asymmetry of LV hypertrophy was seen.
| Clinical Presentation|| |
A 26-year-old nondiabetic normotensive pregnant female with 37 weeks of gestation presented in labor pain to the emergency room. Her vitals were normal with pallor on general physical examination. The chest was clear and no murmur was heard on cardiovascular auscultation. Her electrocardiogram showed extreme LV hypertrophy with deep T-wave inversion in precordial and lateral leads [Figure 1]. Her echocardiography (echo) was done to find the cause of extreme hypertrophy. Echo showed concentric LV hypertrophy, disproportionately more in the posterior wall as compared to the interventricular septum [Figure 2] and [Figure 3]. No LV outflow gradient (LVOT) was present on echo Doppler assessment of LVOT [Figure 4]. She was diagnosed as a case of nonobstructive hypertrophy with asymmetric posterior wall hypertrophy phenotype, a rare variety of HCM. She was managed with urgent lower segment cesarean section for obstetric indication. Postoperatively, the mother and child were stable. The patient was discharged on β-blocker in stable condition. Echocardiographic screening of first-degree relatives was advised.
|Figure 1: Electrocardiogram showing extreme hypertrophy of the left ventricle with deep T-wave inversion in precordial and lateral leads|
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|Figure 2: Parasternal long-axis view showing asymmetrical hypertrophy of the posterior wall of the left ventricle. LA: Left atrium, LV: Left ventricle, Ao: Aorta, PW: Posterior wall, IVS: Inter ventricular septum, PLAX: Parasternal long axis|
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|Figure 3: Parasternal short-axis view showing asymmetrical hypertrophy of the posterior wall of the left ventricle. AW: Anterior wall, SEP: Septum, LAT: Lateral, PW: Posterior wall, PSAX: Parasternal short axis|
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|Figure 4: Doppler assessment of the left ventricular outflow tract showing the absence of the left ventricular outflow tract gradient|
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| Discussion|| |
HCM is a genetic disorder with a variety of phenotypes. Hypertrophy in HCM is usually diffuse, disproportionately more in the interventricular septum. This asymmetrical hypertrophy of septum as compared to the posterior wall is classical for HCM. Of all phenotypic presentations, involvement of posterior wall is the rarest. Unlike common phenotypic variants of HCM, our patient showed predominant hypertrophy of the posterior LV free wall as compared to the interventricular septum. This is an inverted pattern of asymmetric LV hypertrophy.
Lewis et al. reported 17 cases of HCM in which the posterior wall of the left ventricle was disproportionately more hypertrophied than the interventricular septum. After extensive review of literature, we did not find any major article on inverted asymmetrical hypertrophy of the left ventricle except the work of Lewis andMaron. Despite posterior wall hypertrophy, systolic anterior motion of the anterior mitral leaflet and LVOT obstruction usually present in this subset due to the anterior displacement of the mitral valve. But our patient did not show any LVOT gradient or mitral regurgitation. Patients with this variant of HCM usually present with severe symptoms and represent a clinically severe form of HCM, but our patient was relatively stable possibly because of the absence of LVOT obstruction. Findings shown by Lewis andMaron underscore the recognized limitation of using asymmetric hypertrophy of the ventricular septum relative to the posterior free wall as the sole morphologic marker of HCM.
| Conclusion|| |
HCM has multiple phenotypic variants. We reported our case as one of the rare variants of HCM, which showed inverted asymmetrical hypertrophy of the left ventricle.
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.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
Maron BJ, Casey SA, Poliac LC, Gohman TE, Almquist AK, Aeppli DM, et al.
Clinical course of hypertrophic cardiomyopathy in a regional United States cohort. JAMA 1999;281:650-5.
Klues HG, Schiffers A, Maron BJ. Phenotypic spectrum and patterns of left ventricular hypertrophy in hypertrophic cardiomyopathy: Morphologic observations and significance as assessed by two-dimensional echocardiography in 600 patients. J Am Coll Cardiol 1995;26:1699-708.
Lewis JF, Maron BJ. Hypertrophic cardiomyopathy characterized by marked hypertrophy of the posterior left ventricular free wall: Significance and clinical implications. J Am Coll Cardiol 1991;18:421-8.
[Figure 1], [Figure 2], [Figure 3], [Figure 4]