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Table of Contents
CASE REPORT
Year : 2019  |  Volume : 20  |  Issue : 1  |  Page : 21-24  

Successful percutaneous balloon dilatation of supravalvular aortic membrane


Department of Cardiology, Grant Medical College and J.J. Group of Hospitals, Mumbai, Maharashtra, India

Date of Web Publication7-May-2019

Correspondence Address:
Dr. Gurkirat Singh
Department of Cardiology, 4th Floor, Main Hospital Building, J.J. Hospital Campus, Byculla, Mumbai - 400 008, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/HEARTVIEWS.HEARTVIEWS_98_18

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   Abstract 


Supravalvular aortic stenosis is the least common type of left ventricular outflow tract obstruction. Primary balloon dilatation of membranous supravalvular aortic stenosis was performed in a 10-year-old male child with a remarkable reduction in systolic pressure gradient. Balloon dilatation is a feasible treatment modality for membranous supravalvular aortic stenosis. It provides good immediate results and sustained relief of stenosis.

Keywords: Balloon dilatation, membranous obstruction, supravalvular aortic stenosis


How to cite this article:
Singh G, Khemani H, Singla R, Bansal NO. Successful percutaneous balloon dilatation of supravalvular aortic membrane. Heart Views 2019;20:21-4

How to cite this URL:
Singh G, Khemani H, Singla R, Bansal NO. Successful percutaneous balloon dilatation of supravalvular aortic membrane. Heart Views [serial online] 2019 [cited 2019 Jun 18];20:21-4. Available from: http://www.heartviews.org/text.asp?2019/20/1/21/257796


   Introduction Top


Supravalvular aortic stenosis is the least common form of left ventricular (LV) outflow obstruction, occurring in approximately 1 in 20,000 live births.[1],[2] It has a slight male predominance (52%–55%).[3],[4] It is caused by narrowing of the ascending aorta just above the aortic sinuses, distal to the coronary artery orifices. Membranous type is the rarest. Supravalvar aortic stenosis is often associated with Williams–Beuren syndrome and homozygous familial hypercholesterolemia, although it may also occur independently in both familial and sporadic patterns. We report a case of 10-year-old male child with membranous type of supravalvular aortic stenosis. There was a significant reduction in the systolic gradient after balloon dilatation.


   Case Presentation Top


A 10-year-old male was admitted to our hospital with effort intolerance for 3 years. On examination, pulse was 88 beats per minute and blood pressure was 102/60 mmHg. On cardiovascular system examination, apex beat was forceful and sustained, systolic thrill was noted in the first and second right intercostal space. A harsh, loud V/VI grade systolic murmur was heard in the right first and second intercostal space radiating to right carotid more than left carotid artery. Electrocardiogram showed LV hypertrophy [Figure 1].
Figure 1: Electrocardiogram showing left ventricular hypertrophy

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Transthoracic echocardiography showed LV hypertrophy; a membrane was noted at the sinotubular junction. Peak gradient was 106 mmHg. The membrane was confirmed on transesophageal echocardiography [Figure 2].
Figure 2: Transesophageal echocardiography showing supravalvular membrane (marked by arrow)

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Cardiac catheterization showed systolic LV pressure of 236 mmHg [Figure 3]. The aortic systolic pressure was 106 mmHg [Figure 4]. Gradient across the membrane was 130 mmHg. The position of the membrane was confirmed on left ventriculogram [Figure 5]. It was decided to dilate the membrane.
Figure 3: Left ventricular pressure before balloon dilatation. Left ventricular systolic pressure was 236 mm Hg

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Figure 4: Aortic pressure before balloon dilatation. Aortic pressure was 106/60 mmHg

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Figure 5: Left ventriculogram (in the left anterior oblique/cranial view) showing membrane at the sinotubular junction

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TYSHAK 16 mm × 40 mm balloon was used based on the measurement of aortic annulus [Figure 6]. After inflation, LV systolic pressure reduced to 130 mmHg [Figure 7] and systolic aortic pressure was 116 mmHg with gradient reduced to 14 mmHg from 130 mmHg. The procedure was uneventful.
Figure 6: Balloon dilatation of the membrane with TYSHAK 16 mm × 40 mm balloon

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Figure 7: Left ventricular systolic pressure after the procedure. Left ventricular systolic is 130 mmHg

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The patient was discharged after 2 days. The patient is asymptomatic on subsequent follow-ups.


   Discussion Top


Congenital obstruction of the LV outflow tract (LVOT) comprises a heterogeneous group of disorders, with obstruction potentially occurring below, above, or at the level of the aortic valve. Valvar aortic stenosis constitutes the most common type of congenital LVOT obstruction, accounting for approximately 80%–85% of cases.[1]

The second most common type of LVOT obstruction in children is fixed subvalvular stenosis, which accounts for approximately 15% of the total cases. Supravalvular aortic stenosis is least common. In addition to pressure load physiology similar to other causes of LVOT obstruction, coronary abnormalities can occur in supravalvular aortic stenosis. Unlike valvular and subvalvular aortic stenosis, the coronary arteries are exposed to the higher pressure generated by the supravalvular obstruction. Impaired coronary perfusion may occur because of fibrotic thickening in the area immediately surrounding the coronary ostia or because of varying degrees of aortic valve leaflet adhesion to the narrowed sinotubular junction.[3],[4],[5],[6] The left coronary artery is most frequently involved.

Other associated cardiac anomalies include coarctation of the aorta (10%–12%), patent ductus arteriosus, patent foramen ovale or atrial septal defect, ventricular septal defect, and mitral valve abnormalities.[3],[4],[7] Supravalvular aortic stenosis is usually a progressive problem with a progressive increase in LV systolic pressure resulting in exertional symptoms, and if the stenosis is severe, eventual decrease in the LV systolic function.[7]

Surgical treatment of supravalvar aortic stenosis by patch aortoplasty is well established and gives good postoperative results. Residual abnormalities may remain, however, including aortic valve insufficiency and residual outflow tract obstruction. It also carries procedure-related morbidity and mortality.[3],[4] Balloon dilatation is a viable treatment modality for membranous obstructions with considerable fall in the systolic gradient. Tyagi et al.[8] and Jacob et al.[9] have each treated three cases (5 hourglass, one membrane) of supravalvular aortic stenosis by balloon dilatation. Pinto et al. treated two cases of supravalvular stenosis by balloon.[10] In these case series, an excellent result was obtained in the membranous type of supravalvular stenosis, possibly related to the tearing of the membrane by the balloon. Our patient had a membranous type of obstruction. There was a significant decrease in gradient after balloon dilatation.


   Conclusions Top


Balloon dilatation may be an alternative to surgery in selected patients with supravalvular stenosis. Excellent results are obtained in the membranous type of supravalvular stenosis, possibly related to the tearing of the membrane by the balloon. Follow-up of successfully dilated patients shows sustained relief of stenosis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient's parents have given their consent for his images and other clinical information to be reported in the journal. The patient's parents understand that his name and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Campbell M. The natural history of congenital aortic stenosis. Br Heart J 1968;30:514-26.  Back to cited text no. 1
    
2.
Metcalfe K, Rucka AK, Smoot L, Hofstadler G, Tuzler G, McKeown P, et al. Elastin: Mutational spectrum in supravalvular aortic stenosis. Eur J Hum Genet 2000;8:955-63.  Back to cited text no. 2
    
3.
Stamm C, Kreutzer C, Zurakowski D, Nollert G, Friehs I, Mayer JE, et al. Forty-one years of surgical experience with congenital supravalvular aortic stenosis. J Thorac Cardiovasc Surg 1999;118:874-85.  Back to cited text no. 3
    
4.
Scott DJ, Campbell DN, Clarke DR, Goldberg SP, Karlin DR, Mitchell MB, et al. Twenty-year surgical experience with congenital supravalvar aortic stenosis. Ann Thorac Surg 2009;87:1501-7.  Back to cited text no. 4
    
5.
Thistlethwaite PA, Madani MM, Kriett JM, Milhoan K, Jamieson SW. Surgical management of congenital obstruction of the left main coronary artery with supravalvular aortic stenosis. J Thorac Cardiovasc Surg 2000;120:1040-6.  Back to cited text no. 5
    
6.
Sun CC, Jacot J, Brenner JI. Sudden death in supravalvular aortic stenosis: Fusion of a coronary leaflet to the sinus ridge, dysplasia and stenosis of aortic and pulmonic valves. Pediatr Pathol 1992;12:751-9.  Back to cited text no. 6
    
7.
Wren C, Oslizlok P, Bull C. Natural history of supravalvular aortic stenosis and pulmonary artery stenosis. J Am Coll Cardiol 1990;15:1625-30.  Back to cited text no. 7
    
8.
Tyagi S, Arora R, Kaul UA, Khalilullah M. Percutaneous transluminal balloon dilatation in supravalvular aortic stenosis. Am Heart J 1989;118:1041-4.  Back to cited text no. 8
    
9.
Jacob JL, Coelho WM, Machado NC, Garzon SA. Initial experience with balloon dilatation of supravalvar aortic stenosis. Br Heart J 1993;70:476-8.  Back to cited text no. 9
    
10.
Pinto RJ, Loya Y, Bhagwat A, Sharma S. Balloon dilatation of supravalvular aortic stenosis: A report of two cases. Int J Cardiol 1994;46:179-81.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]



 

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