Home About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Subscribe Contacts Login 


 
 Table of Contents  
CASE REPORT
Year : 2013  |  Volume : 23  |  Issue : 3  |  Page : 81-83

Severe midventricular hypertrophic obstructive cardiomyopathy and apical aneurysm


Department of Cardiology, S Carlo Clinic, Paderno Dugnano, Milan, Italy

Date of Web Publication30-Dec-2013

Correspondence Address:
Giuseppe Gibelli
via Genova 13, 20090 Settala, Milan
Italy
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2211-4122.123954

Rights and Permissions
  Abstract 

A 40-year-old man was found to have hypertrophic cardiomyopathy (HCM) with severe mid ventricular obstruction. The obstruction produced two distinct left ventricular chambers with an estimated 60 mmHg continuous wave (CW) Doppler intraventricular gradient. Pulsed wave (PW) Doppler showed high velocity systodiastolic flow from apex to base and flow from base to apex confined mostly to the second half of diastole. Cardiac magnetic resonance (CMR) showed midventricular obstruction, due to septal, parietal, and to an hypertrophic, double posteromedial papillary muscle; an apical aneurysm was detected. Aneurysm is underdiagnosed by echocardiography in HCM and an accurate anatomic definition is needed if surgery is planned; thus, a CMR should always be obtained in these patients.

Keywords: Apical aneurysm of left ventricle, hypertrophic cardiomyopathy, midventricular obstruction


How to cite this article:
Gibelli G, Biasi S, Buonamici V. Severe midventricular hypertrophic obstructive cardiomyopathy and apical aneurysm. J Cardiovasc Echography 2013;23:81-3

How to cite this URL:
Gibelli G, Biasi S, Buonamici V. Severe midventricular hypertrophic obstructive cardiomyopathy and apical aneurysm. J Cardiovasc Echography [serial online] 2013 [cited 2019 Jul 16];23:81-3. Available from: http://www.jcecho.org/text.asp?2013/23/3/81/123954

A 40-year-old man underwent an echocardiogram because of dyspnea and chest pain on effort; Electrocardiogram (ECG) showed left ventricular hypertrophy with secondary changes of repolarization. Echocardiogram showd midventricular obstruction of the left ventricle (LV); continuous wave (CW) Doppler showed high velocity jet (3,8 m/s), originating at the level of obstruction, with a peak estimated gradient of about 60 mmHg; pulsed wave (PW) Doppler showed systolic flow in the apical chamber directed from apex to base, continuing well after the end of electrical systole and flow from base to apex mostly in the second half of diastole [Figure 1]a and b. Cardiac magnetic resonance (CMR) showed midventricular obstruction, due to hypertrophy of septum, LV lateral wall, and double posteromedial papillary muscle; producing two distinct proximal and distal chambers [Figure 2]a and b. Maximal septal thickness was 22 mm at midventricular level. Apical hypertrophy of the right ventricle was also shown [Figure 2]. An apical aneurism (AA) of the LV was found [Figure 2], [Figure 2], [Figure 3]. No LV outflow tract obstruction was present.
Figure 1: Echocardiogram two-dimensional (2D), continuous wave (CW) and pulsed wave (PW) Doppler; four chamber view. (a): CW 3.8 m/s jet directed towards left ventricle (LV) base is shown; flow velocity is maximal in proto- and end-systole, with marked attenuation in mid-systole, accordingly with midventricular obstruction. (b) PW, sampling in the apical chamber, just apical to obstruction. High velocity systolic flow directed from apex to base is seen; flow continues well after the end of electrocardiogram (ECG) T wave (thus during electrical diastole). Base to apex flow is mostly in end diastole (arrows)

Click here to view
Figure 2: Cine sequence, four chamber view. (a) End diastolic frame and (b) systolic frame. Midventricular hypertrophy with septal leftward bulging and hypertrophic double posteromedial papillary muscle are shown; severe systolic obstruction with double-chambered appearance of LV is caused by joining of septum and papillary muscle. Systolic expansion of tinned apex is also shown, as well as apical and mid right ventricle hypertrophy

Click here to view


Repeated Holter recordings showed only infrequent, isolated diurnal ventricular ectopic activity. No history of familiar sudden death was reported.

Midventricular obstruction of the LV is an increasingly encountered phenotypic variant of hypertrophic cardiomyopathy (HCM). [1],[2] Abnormal and hypertrophic papillary muscles commonly are seen in these cases [1],[3] impinging in a ventricular cavity reduced by mid ventricular septal and free wall hypertrophy. Apical dilation is common and AA occurs in severe cases; [2],[4],[5],[6],[7],[8],[9],[10] such as the present one. AA occurs uncommonly in HCM patients as a whole, but is common in midventricular obstruction cases. Maron et al., [5] found an AA in 28 (2%) of 1,299 HCM patients, but each one of these 28 patients had midventricular (19, 68%) or apical (9, 32%) hypertrophy. Maximal wall thickness was similar in AA patients with midventricular and apical hypertrophy (18 and 20 mm, respectively). AA is thought to be the result of repeated ischemia resulting from reduced capillary density, hyperplasia of the arterial media, increased perivascular fibrosis, and myocardial bridging. [5],[6],[7] AA should be looked for in every case of midventricular HCM because it portends a severe arrhythmic risk, most probably due to reentry through the border zones of the AA, containing fibrous tissue interspersed with vital muscle. [8],[9],[10] Thrombotic apposition in AA is not uncommon [1],[5],[8] and can be associated with embolism. [5]
Figure 3: Inversion recovery-contrast enhanced 3D sequence, four chamber view. Late enhancement of thinned left ventricular apex is shown. Intermediate intensity signal of border zone is shown (arrows). No thrombus is detected

Click here to view


AA in HCM is considered a potential sudden death risk modifier that can contribute to resolution of ICD decision. [5],[6],[7],[8],[9],[10]

At the site of obstruction a flow disturbance is seen, that can even present as paradoxic flow, that is, systolic flow towards the apex and diastolic flow from the apex to base. [11],[12] Paradoxic flow is related to the diskynesia of the apex due to the AA. In the present case a mixed pattern was seen, with systolic flow in the apical chamber directed from apex to base continuing well after the end of electrical systole and flow from base to apex mostly in the second half of diastole [Figure 1]b. This pattern, representing an almost continuous flow, is similar to the one described by Hsieh et al., [13] Flow from apex to base during systole and isovolumic relaxation has been described in midventricular obstruction-no AA HCM, [14] thus a continuum of flow patterns seems to exist in this disease, spanning from totally paradoxic flow to delayed empting and filling of the apical chamber. The variable pattern of flow arguably is the end result of the complex interaction between variable obstruction severity, AA, delayed relaxation, and contraction of apical chamber.

An issue is the relatively poor sensitivity of echocardiography in imaging AA, as an AA was recognized by echocardiography in only 16 of 28 patients (57%), but by CMR in the 12 patients undetected by echocardiography. [5]

Surgical treatment by transaortic midseptal and papillary muscle reduction has been used in some cases. [15] More recently an apical approach to septal surgical remodeling, alone or combined with other procedures including AA repair, has been utilized with excellent results and no mortality. [16] Intraventricular gradient was reduced from 64 ± 32 mmHg before myectomy to 6 ± 12 mmHg. [16]

In the present case, a temptative treatment with propranolol 160 mg/day in divided doses reduced symptoms but not the gradient. The patient is now on list for surgery.

 
  References Top

1.Maron MS. Clinical utility of cardiovascular magnetic resonance in hypertrophic cardiomyopathy. J Cardiovasc Magn Reson 2012;14:13.  Back to cited text no. 1
    
2.Noureldin RA, Liu S, Nacif MS, Judge DP, Halushka MK, Abraham TP, et al. The diagnosis of hypertrophic cardiomyopathy by cardiovascular magnetic resonance. J Cardiovasc Magn Resone 2012;14:17.  Back to cited text no. 2
    
3.de Gregorio C. Left ventricular dynamic obstruction by atypical papillary muscle morphology: Is this finding so unusual in clinical practice? J Am Soc Echocardiogr 2006;20:100-101.  Back to cited text no. 3
    
4.Spirito P, Maron BJ, Bonow RO, Epstein SE. Occurrence and significance of progressive left ventricular wall thinning and relative cavity dilatation in hypertrophic cardiomyopathy. Am J Cardiol 1987;60:123-9.  Back to cited text no. 4
    
5.Maron MS, Finley JJ, Bos JM, H. Hauser TH, Manning WJ, Haas TS, et al. Prevalence, clinical significance, and natural history of left ventricular apical aneurysms in hypertrophic cardiomyopathy. Circulation 2008;118:1541-9.  Back to cited text no. 5
    
6.Shirani J, Pick R, Roberts WC, Maron BJ. Morphology and significance of the left ventricular collagen network in young patients with hypertrophic cardiomyopathy and sudden cardiac death. J Am Coll Cardiol 2000;35:36-44.  Back to cited text no. 6
    
7.Hughes SE. The pathology of hypertrophic cardiomyopathy. Histopathology 2004;44:412-27.  Back to cited text no. 7
    
8.Holloway CJ, Betts TR, Neubauer S, Myerson SG. Hypertrophic cardiomyopathy complicated by large apical aneurysm and thrombus, presenting as ventricular tachycardia. J Am Coll Cardiol 2010;56:1961.  Back to cited text no. 8
    
9.Shenoy C, Maron MS. Hypertrophic cardiomyopathy with left ventricular apical aneurysm in brothers. Am J Cardiol 2011;108:612-3.  Back to cited text no. 9
    
10.2011 ACCF/AHA guideline for the diagnosis and treatment of hypertrophic cardiomyopathy. Circulation 2011;124:e783-831.  Back to cited text no. 10
    
11.Mixon TA, Giebel DW. Abnormal diastolic flow demonstrated by color M mode echocardiography in hypertrophic cardiomyopathy with mid-ventricular cavity obliteration. Echocardiography 2004;21:49-52.  Back to cited text no. 11
    
12.de Gregorio C, Recupero A, Grimaldi P, Coglitore S. Can transthoracic live 3-dimensional echocardiography improve the recognition of midventricular obliteration in hypertrophic obstructive cardiomyopathy? J Am Soc Echocardiogr 2006;19:1190.e1-4.  Back to cited text no. 12
    
13.Hsieh BP, Tauras J, Taub C. Continuous apex to left ventricle blood flow pattern in hypertrophic cardiomyopathy with apical aneurysm and midventricular obstruction. Echocardiography 2012;29:E131-3.  Back to cited text no. 13
    
14.Sütsch G, Jenni R, Krayenbühl HP. Left ventricular flow from apex to base during systole and isovolumic relaxation in a patient with hypertrophic cardiomyopathy and midventricular obstruction. Eur Heart J 1991;12:1132-9.  Back to cited text no. 14
    
15.Maron BJ, Nishimura RA, Danielson GK. Pitfalls in clinical recognition and a novel operative approach for hypertrophic cardiomyopathy with severe outflow obstruction due to anomalous papillary muscle. Circulation 1998;98:2505-8.  Back to cited text no. 15
    
16.Kunkala MR, Schaff HV, Nishimura RA, Abel MD, Sorajja P, Dearani JA, et al. Transapical approach to myectomy for midventricular obstruction in hypertrophic cardiomyopathy. Ann Thorac Surg 2013;96:564-70.  Back to cited text no. 16
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

Top
 
 
  Search
 
Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

 
  In this article
Abstract
References
Article Figures

 Article Access Statistics
    Viewed3044    
    Printed91    
    Emailed0    
    PDF Downloaded112    
    Comments [Add]    

Recommend this journal


[TAG2]
[TAG3]
[TAG4]