Concomitant Aortoplasty with Septal Myectomy for Supravalvar Aortic Stenosis in an Adult

doi:10.4236/wjcs.2012.21002

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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

Email: *[email protected]

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.

R. S. ELUMALAI ET AL.

(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.

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