World Journal of Cardiovascular Surgery, 2012, 2, 5-6
http://dx.doi.org/10.4236/wjcs.2012.21002 Published Online March 2012 (http://www.SciRP.org/journal/wjcs) 5
Concomitant Aortoplasty with Septal Myectomy for
Supravalvar Aortic Stenosis in an Adult
Raja Saravanan Elumalai1*, Kirthivasan Vaidyanathan1, Madhu Sankar Nainar2,
Karthik Vaidyanathan2, Govini Balasubramaniam2, Susan George3
1Department of Cardiac Anaesthesiology, Global Hospital and Health City, Perumbakkam, India
2Department of Cardiothor ac ic Surgery, Global Hospital and Health City, Perumbakkam, India
3Department of Cardiology, Global Hospital and Heal th City, Perumbakkam, India
Received December 3, 2011; revised January 13, 2012; accepted February 13, 2012
ABSTRACT
Survival of patients with severe congenital aortic stenosis beyond third decade is uncommon. This report describes a 31
year old patient who underwent successful aortoplasty and in addition septal myectomy for complete relief of left ven-
tricular outflow ob struction.
Keywords: Aortoplasty; Myectomy; Supravalvar Aortic Stenosis
1. Introduction
Congenital Supravalvar Aortic Stenosis is a rare form of
obstructive lesion of the left ventricular outflow tract.
The defining feature of the malformation is an aortic
narrowing at the level of the sino tubular junction. The
commonly associated lesions include peripheral pulmo-
nary artery lesions, coronary lesion s, abnormalities of the
aortic leaflets and diffuse narrowing of ascending aorta.
Survival beyond three decades is uncommon and this
report describes a case of supravalva r aortic stenosis in a
31 year old patient who underwent successful aortoplasty
and septal myectomy.
2. Case Summary
A 31 years old lady with symptoms of class III dyspnea,
intermittent chest pain and syncopal attacks of 2 years
duration was referred to our institute. Two Dimensional
Echocardiography revealed discrete hour glass type of
Supravalvular Aortic Stenosis with severe left v entricular
hypertrophy (Figure 1). Left ventricle to aorta peak gra-
dient was 163 mm Hg with a mean of 101 mm Hg. She
underwent surgical repair using cardiopulmonary bypass
and blood cardioplegic arrest.
Inverted Y shaped Aortotomy was done with limbs
extending into non and right coronary sinus. The aortic
valve was tricuspid and competent Intraluminally the
posterior ridge above left coronary sinus was excised.
There was severe obstructive hypertrophy of septum and
hence septal myectomy was done. The aortotomy was
(RV: Right ventricle, LV: Left ventricle, AV: Aortic valve, LA: Left
atrium, ASC AO: Ascending aorta, SUPRAVALV.AS: Supravalvular
aortic stenosis)
Figure 1. Preoperative echo showing hour glass lesion of
aorta.
closed with inverted Y shaped pericardial patch extend-
ing into the non coronary sinus and right coronary si-
nuses. The post operative recovery was uneventful .Pre
discharge echocardiography (Figure 2(a)) and CT an-
giographic study (Figure 2(b)) showed a wide open
LVOT with a peak gradient of 20 mm Hg and mean of
12 mm Hg.
Ethics Committee approval and patient consent for
publication of this data was obtained.
3. Discussion
Congenital supravalvular ao rtic stenosis often presents in
*Corresponding a uthor.
C
opyright © 2012 SciRes. WJCS

R. S. ELUMALAI ET AL.
6
(a)
(b)
Figure 2. (a) Postoperative echo showing Left Ventricular
Outflow Tract; (b) Postoperative 64 slice Computed Tomo-
graphy of Aortic arch.
childhood and if not corrected by surgery can lead to
heart failure and death. The defining feature is a focal or
diffuse narrowing starting at the Sino tubular junction
and often involving the entire ascending aorta with rare
involvement of the aortic arch and occasionally the pe-
ripheral arterial system [1]. This disorder occurs in an
autosomal inherited from and in a rare sporadic form it is
frequently associated with Williams-Beuren syndrome
[2]. The underlying cause has been identified as a muta-
tion of the elastin gene on chromosome 7q11.23 [3]. The
first repor t was in 187 8 b y Arch er w ho d e scribe d an elas-
tic band stretching across distal to the aortic cusps [4].
irst successful surgical repair with standard patch was
described by McGoon in 1961 [5]. Doty et al described
extended aortoplasty w ith bifur cated patch in 1977 which
allows more symmetric enlargement of the ao rtic root [6].
Brom described enlarging all the 3 sinuses with ascend-
ing aorta enlarg ement by an additional patch [7]. Surgical
treatment of supravalvar aortic stenosis by augmenting
the aortic root in 2 or 3 sinuses of Valsalva is associated
with a lower mortality rate and fewer reoperations as it
preserves the anatomy of the aortic root with better
physiologic flow. Currently, septal myectomy is recom-
mended in patients undergoing aortic valve replacement
for aortic stenosis with severe basal septal hypertrophy,
as it leads to better regression of left ventricular hyper-
trophy due to more effective reduction of left ventricular
outflow tract gradien t [8]. The p rogno sis of suprav alvular
aortic Stenosis is poor and survival to adulthood without
intervention is unusual. The outflow obstruction can be
relieved by patch aortoplasty and septal myectomy with
good results.
F
REFERENCES
[1] A. Wessel, R. Pankau, D. Kececioglu, W. Ruschewski, J.
H. Buersch, “Three Decades of Follow-Up of Aortic and
Pulmonary Vascular Lesions in the Williams-Beuren
Syndrome,” American Journal of Genetics, Vol. 52, 1994,
pp. 297-301. doi:10.1002/ajmg.1320520309
[2] C. A. Morris and C. B. Mervis, “Williams Syndrome and
Related Disorders,” Annual Review of Genomics and
Human Genetics, Vol. 1, 2000, pp. 461-484.
[3] M. T. Keating, “Genetic Approaches to Cardiovascular
Disease. Supravalvular Aortic Stenosis, Williams Syn-
drome, and Long-QT Syndrome,” Circulation, Vol. 92,
No. 1, 1995, pp. 142-147.
[4] R. S. Archer, “Note on a Congenital Band Stretching
across the Origin of the Aorta,” Dublin Journal of Medi-
cal Science, Vol. 65, 1878, pp. 405-406.
[5] D. C. McGoon, H. T. Mankin, P. Vlad and J. W. Kirklin,
“The Surgical Treatment of Supravalvular Aortic Steno-
sis,” Journal of Thoracic and Cardiovascular Surgery,
Vol. 41, 1961, pp. 125-133.
[6] D. B. Doty, D. B. Polansky and C. B. Jenson, “Supraval-
vular Aortic Stenosis Repair by Extended Aortoplasty,”
Journal of Thoracic and Cardiovascular Surgery, Vol. 74,
1977, pp. 362-371.
[7] M. G. Hazekamp, A. P. Kappetein, P. H. Schoof, J. Ot-
tenkamp, M. Witsenburg, H. A. Huysmans and A. J. J. C.
Bogers, “Brom’s Three-Patch Technique for Repair of
Supravalvular Aortic Stenosis,” Journal of Thoracic and
Cardiovascular Surgery, Vol. 118, No. 2, 1999, pp. 252-
258. doi:10.1016/S0022-5223(99)70215-1
[8] N. Kayalar, H. Schaff, R. C. Daly, J. A. Dearani and S. J.
Park, “Concomitant Septal Myectomy at the Time of
Aortic Valve Replacement for Severe Aortic Stenosis,”
Annals of Thoracic Surgery, Vol. 89, No. 2, 2010, pp.
459-464. doi:10.1016/j.athoracsur.2009.10.065