European Journal of Echocardiography 2008 9(1):63-64; doi:10.1016/j.euje.2006.08.014
Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2006. For permissions please email: journals.permissions@oxfordjournals.org.
Discrete subaortic stenosis in elderly women
Pier Giorgio Masci*,
Francesca Rossi and
Francesco Chiarella
Unità Operativa di Cardiologia, A.O. Santa Corona, Via XXV Aprile 128, 17027 Pietra Ligure, Italy
Received 4 August 2006; accepted after revision 29 August 2006; online publish-ahead-of-print 11 October 2006.
* Corresponding author. Tel: +39 019 6234348; fax: +39 019 6234483. E-mail address: pgmasci{at}tiscali.it
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Abstract
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Discrete subaortic stenosis (DSS) is likely an acquired cardiac
disorder which requires anatomic precursors and a genetic background.
DSS occurs usually within the first decade, provoking rapidly
progressive left ventricular outflow tract obstruction and secondary
aortic regurgitation. DSS has been considered for a long time
exclusively a disease of infancy and childhood and few reports
and small series have described DSS in adulthood and only two
cases are reported in elderly. Our case describes a discrete
subaortic membranous ridge in an elderly woman with recent onset
of dyspnea.
Keywords: Discrete subaortic stenosis; Elderly
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Case report
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The patient, a Caucasian 77-year-old woman, has been admitted
to our hospital because of recent onset of exertional dyspnoea
and fatigue. Her previous clinical history was totally unremarkable.
At the presentation she was moderately dyspnoeic with crackles
at both pulmonary bases. An ejective systolic murmur (grade
4/6) was heard at the base, not modifying its intensity with
Valsava manoeuvre or with squatting. Transthoracic echocardiography
(TTE) showed normal left ventricle (LV) dimensions and moderate
wall hypertrophy which was more prominent at interventricular
septum (diastolic thickness =18 mm) (
Figure 1). An ejection
fraction of 60% was calculated according to Simpson's method.
On apical five chamber view a mean LV-aortic gradient of 58
mmHg was recorded at continuous (CW)-Doppler analysis. Colour-Doppler
analysis detected moderate eccentric aortic regurgitation (
Figure 2).
Systolic anterior motion (SAM) of mitral leaflets was not present
and pulsed-wave (PW) Doppler mapping of LVOT showed only a modest
acceleration of blood flow. Because parasternal views did not
permit a satisfactory evaluation of aortic valve, transoesophageal
echocardiography (TEE) was performed. This exam showed a moderate
degenerative process involving the three aortic cuspids with
mild commissural fusion. A slightly reduced anatomic aortic
valve area (=1.8 cm
2) was measured (
Figure 3). LVOT evaluation
showed a circumferential membrane at LVOT level located very
proximal to the aortic valve (
Figure 4). Considering the
clinical presentation and the echocardiographic findings the
patient was referred to a cardiac surgeon but she refused the
operation.
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Figure 4 Mid-oesophagus transoesophageal echocardiography view. Mild restrictive movement of aortic valve leaflets (arrows). The membranous ridge (*) is located in the left ventricle outflow tract (LVOT) slightly proximal to the aortic valve (AV). LV, left ventricle; LA, left atrium.
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Discussion
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Subaortic stenosis is likely an acquired cardiac disorder which
requires anatomic precursors at level of LVOT
1 and a genetic
background. Subaortic stenosis may manifest with different aspects
and grades of obstruction almost exclusively within the first
decade of life. Tunnel subaortic stenosis, where the LVOT is
hypoplasic with fibrous thickened endocardium, represents the
most extreme type. The other spectrum of subaortic stenosis
is the discrete variety (DSS), where LVOT is apparently normal
and the stenosis is caused by fibromuscular ridge just proximal
to the aortic valve.
2 Rarely the two forms may coexist.
In children DSS is often associated with subtle morphologic and functional alterations of LVOT, such as small dimensions and steep aortoseptal angle.3 These abnormalities have been reported to alter shear stress stimulating cellular proliferation in LVOT. Under genetic predisposition, the altered septal shear stress may promote fibromuscular ridge growth and may be responsible for the rapid progression of subaortic stenosis. Therefore, DSS in children has been regarded as an adversely progressive disease and some investigators have proposed surgical repair irrespective of the trans-stenosis gradient.4
On the other hand very little is known about DSS in adulthood and only a few anecdotal cases5,6 have been described in elderly. Oliver et al.7 published a single centre retrospective analysis on DSS in adults. According to their data, DSS was present in 6.5% of 2057 consecutive adults with congenital heart disease (mean age, 31 ± 17 years) and DSS was the only congenital abnormality in 66% of cases. At the opposite of what was described for children, in adults DSS seems to remain stable or progress slowly.
Transthoracic and transoesophageal Doppler echocardiography have improved diagnostic accuracy of DSS even for small membrane not causing hemodynamically significant pressure gradient. Nevertheless making a differential diagnosis of DSS versus obstructive subaortic hypertrophic cardiomyopathy (HOCM) can be particularly complicated. In fact patients with DSS may develop severe LV wall hypertrophy prevalently located at septal level which is sometimes associated with SAM of mitral apparatus. In the literature, it has been reported that some adult patients who received the diagnosis of HOCM were instead affected by DSS which was overlooked at first echocardiographic evaluation.8 In our clinical case, although a predominant hypertrophy of interventricular septum was detected, the diagnosis of HOCM was unlikely because of the non-dynamic nature of LV-aortic gradient and the negative familial history for hypertrophic cardiomyopathy.
In conclusion, we reported a rare case of DSS in an elderly woman who started to complain of symptoms of LV dysfunction. Our suggestion for early cardiac surgery was mainly dictated by the severity of LVOT obstruction and patient's clinical features. Indeed, according to the literature a watchful approach to DSS in asymptomatic adults and elderly without severe LVOT obstruction seems to be plausible and wise.
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References
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- Firpo C, Azcarate Mj, Quero-Jimenez M, Saravalli O. Discrete subaortic stenosis (DSS) in childhood: a congenital or acquired disease? Follow-up in 65 patients. Eur Heart J (1990) 11:1033–40.[Abstract/Free Full Text]
- Kelly DT, Wulfsberg BA, Rowe R. Discrete subaortic stenosis. Circulation (1972) 46:309–22.[Abstract/Free Full Text]
- Sigfusson G, Tacy TA, Van Aucker MD, Cape EG. Abnormalities of the left ventricular outflow tract associated with discrete subaortic stenosis in children: an echocardiographic study. J Am Coll Cardiol (1997) 30:255–9.[Abstract]
- Brauner R, Laks H, Drikwater DC Jr, Shvarts O, Eghbali K, Galindo A. Benefits of early surgical repair in fixed subaortic stenosis. J Am Coll Cardiol (1997) 30:1835–42.[Abstract]
- Onbasili AO, Tekten T, Ceyhan C. Subaortic stenosis caused by flail discrete membrane in older patient. Heart (2004) 90:399.[Free Full Text]
- Izgi A, Kirma C, Tanalp AC, Mansuroglu D, Ipek G. Cystic subaortic discrete membrane in elderly women. J Heart Valve Dis (2004) 13:925–6.[Web of Science][Medline]
- Oliver JM, Gonzalez A, Gallego P, Sanchez-Recalde A, Benito F, Mesa JM. Discrete subaortic stenosis in adults: increased prevalence and slow rate of progression of the obstruction and aortic regurgiation. J Am Coll Cardiol (2001) 38:835–42.[Abstract/Free Full Text]
- Bruce CJ, Nishimura RA, Tajik J, Schaff VH, Danielson GK. Fixed left ventricular outflow tract obstruction in presumed hypertrophic obstructive cardiomyopathy: implication for therapy. Ann Thorac Surg (1999) 68:100–4.[Abstract/Free Full Text]
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