Journal of Postgraduate Medicine
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CASE REPORT
 
 
Year : 1979  |  Volume : 25  |  Issue : 4  |  Page : 241-242  

Familial idiopathic hypertrophic subaortic stenosis

Lilam S Shah, AS Vengsarkar 
 Department of Cardiology, Seth G. S. Medical College and K.E.M. Hospital, Parel, Bombay-400 012., India

Correspondence Address:
Lilam S Shah
Department of Cardiology, Seth G. S. Medical College and K.E.M. Hospital, Parel, Bombay-400 012.
India

Abstract

Echocardiographic features of idiopathic hypertrophic subaortic stenosis (IHSS) are described. Systolic anterior motion (SAM) of anterior mitral leaflet and asymetric septal hypertrophy (ASH) are considered as the diagnostic criteria of IHSS. Effects of amyl nitrite and propranolol-a beta blocker are studied. Echocardio­graphic screening of family members revealed this as a case of fami­lial IHSS.



How to cite this article:
Shah LS, Vengsarkar A S. Familial idiopathic hypertrophic subaortic stenosis.J Postgrad Med 1979;25:241-242


How to cite this URL:
Shah LS, Vengsarkar A S. Familial idiopathic hypertrophic subaortic stenosis. J Postgrad Med [serial online] 1979 [cited 2020 Nov 26 ];25:241-242
Available from: https://www.jpgmonline.com/text.asp?1979/25/4/241/42229


Full Text

 Introduction



Echocardiography is one of the most useful non-invasive techniques for diagnosing idiopathic hypertrophic subaortic stenosis. This technique has furnished practical means for the evaluation of natural history of the disease, haemodynamic effects of various drugs and manoeuvres on left ventricular outflow tract obstruction and its prognostic im­plications.

 Case report



A 35 year old, female presented to us with the complaint of chest pain and palpitation of one year's duration prior to admission. Car­diovascular system examination revealed sustained apex suggesting left ventricular hypertrophy, and a long systolic cooing mur­mur of 4/6 grade, transmitted to the carotid and the apex. An ejection click was absent. On prompt squatting, the murmur decreased in intensity whereas while performing valsalva manuevre, and with amyl nitrite inhalation the murmur increased in intensity suggesting that the systolic murmur and the left ventri­cular hypertrophy could be because of dyna­ mic obstruction of left ventricular outflow tract.

Her echocardiogram revealed systolic anterior motion of anterior mitral leaflet, touching the interventricular septum, and obstructing the left ventricular outflow tract. Prolonged systo­lic mitral septal apposition indicated significant left ventricular outflow tract obstruction which was exaggerated with amyl nitrite and reduced with propranolol-a beta blocker. Another in­teresting feature was asymmetrical septal hypertrophy; septal thickness was 3.5 cm. and left ventricular posterior wall thickness was 1.5 cm. Septal thickness/left ventricular poste­rior wall thickness ratio was 2.3 (normal 0.8 to 1.3) (See [Figure 1] on page 242A) suggesting significant asymmetric septal hypertrophy. These two features of idiopathic hypertrophic subaortic stenosis helped us to diagnose the condition non-invasively. Systolic flutter and mid-systolic closure of the aortic valve leaflet were seen on aortic root echocardiogram (See [Figure 2] on page 242A). This patient's two brothers and one sister showed moderate systo­lic anterior motion of anterior mitral leaflet and asymmetric septal hypertrophy.

 Discussion



Over a period of last decade, various authors have studied the entity of IHSS non-invasively. Two diagnostic features of IHHS have been described [3],[4],[5],[6],[7] In our case both the diagnostic criteria are present i.e. SAM of anterior mitral leaflet and ASH. The severity of SAM suggest severe degree of left ventricular outflow tract obstruction. Left ventricular outflow gradient was measurer non-invasively by Henry et al. [2] The effect of drugs and manoeuvres on left ventricular outflow obstruction was studied by King et al . [3] Our case shower increase in left ventricular outflow tract obstruction by amyl nitrite and decrease by beta blockers. Further haemodynamic studies have been done by Feizi et al. [1] Systolic flutter of aortic leaflet and mid systolic closure is seen in our case.

References

1Feizi, O. and Emanuel, R.: Echoeardio. graphic spectrum of hypertrophic cardio. myopathy. Brit. Heart J. 37: 1286-1302 1975.
2Henry, W. L., Clark, C. E, Glancy, D. L. and Epstein, S. E.: Echocardiographic measurement of left ventricular outflow gradient in idiopathic hypertrophic sub­aortic stenosis. New Eng. J. Med., 288: 989-993, 1973.
3King, J. F., DeMaria, A. N., Reis, R. L., Botton, M. R., Dunn, M. I. and Mason, D. T.: Echocardiographic assessment of idiopathic hypertrophic subaortic steno­sis. Chest, 64: 723-731, 1973.
4Popp, R. L. and Harrison, D. C.: Ultra­sound in the diagnosis and evaluation of therapy of idiopathic hypertrophic sub­aortic stenosis. Circulation, 40: 905-914, 1969.
5Rossen, R. M., Goodman, D. J., Ingham, R. E. and Popp, R. L.: Echocardiographic criteria in the diagnosis of idiopathic hypertrophic subaortic stenosis. Circula­tion, 50: 747-751, 1974.
6Shah, P. M., Gramiak, R., Adelman, A. G. and Wigle, E. D.: Role of echocar­diography in diagnostic and haemodyna­mic assessment of hypertrophic subaortic stenosis. Circulation, 44: 891-898, 1971. .
7Shah, P. M., Gramiak, R. and Kramer, D. H.: Ultrasound localization of left ventricular outflow obstruction in hyper­trophic obstructive cardiomyopathy. Cir­culation, 40: 3-12, 1969

 
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