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|Year : 1979 | Volume
| 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
Lilam S Shah
Department of Cardiology, Seth G. S. Medical College and K.E.M. Hospital, Parel, Bombay-400 012.
Source of Support: None, Conflict of Interest: None
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;25:241-2
| :: 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 ,,,, 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.  The effect of drugs and manoeuvres on left ventricular outflow obstruction was studied by King et al .  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.  Systolic flutter of aortic leaflet and mid systolic closure is seen in our case.
| :: References|| |
|1.||Feizi, O. and Emanuel, R.: Echoeardio. graphic spectrum of hypertrophic cardio. myopathy. Brit. Heart J. 37: 1286-1302 1975. |
|2.||Henry, 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. |
|3.||King, 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. |
|4.||Popp, R. L. and Harrison, D. C.: Ultrasound in the diagnosis and evaluation of therapy of idiopathic hypertrophic subaortic stenosis. Circulation, 40: 905-914, 1969. |
|5.||Rossen, 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. |
|6.||Shah, 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. . |
|7.||Shah, P. M., Gramiak, R. and Kramer, D. H.: Ultrasound localization of left ventricular outflow obstruction in hypertrophic obstructive cardiomyopathy. Circulation, 40: 3-12, 1969 |
[Figure 1], [Figure 2]