Pediatric Subvalvar Aortic Stenosis Workup

  • Author: Douglas J Schneider, MD; more...
 
Updated: Mar 29, 2011
 

Approach Considerations

No specific laboratory blood tests are required in the workup of SAS. Echocardiography is the principal diagnostic study.

Go to Imaging in Aortic Stenosis for more complete information on this topic.

Next

Echocardiography

In SAS, echocardiography enables evaluation of the following:

  • Definition and location of the stenosis
  • Extent of involvement of the LVOT
  • Early aortic valve closure from its involvement and its associated lesion
  • Aortic regurgitation
  • Mitral regurgitation
  • LV hypertrophy
  • Poststenotic aortic dilatation
  • Diagnostic features of associated congenital heart defects
  • LV systolic performance
  • Filling characteristics

Echocardiography helps in defining and localizing SAS (see the image below). It reveals the extent of LVOT involvement, the degree of LV hypertrophy, the indices of LV performance, and the parameters of diastolic function of the LV. Secondary effects, such as the degree of aortic insufficiency, mitral regurgitation, or poststenotic dilatation of the aorta, may be assessed. Finally, associated congenital heart defects and their influence on the hemodynamic effects of SAS may be evaluated.

Echocardiogram of membranous subaortic stenosis. AEchocardiogram of membranous subaortic stenosis. AO = aortic; LA = left atrium; LVOT = left ventricular outflow tract.

Two-dimensional echocardiography with color Doppler imaging is the current modality of choice to establish the diagnosis of SAS. This noninvasive method allows for serial evaluation of the progression of the lesion and of the results of intervention.

M-mode echocardiography provides indirect evidence of SAS by revealing early closure (from the Venturi effect of the jet formed by the SAS) and the coarse flutter of the aortic valve leaflets.

Two-dimensional echocardiography, and now 3-dimensional (3D) echocardiography, reveals and defines the position of lesions, the extent of involvement of the LVOT (in tunnellike SAS), and the associated defects. Apical views reveal the relationship of the SAS to surrounding structures (eg, mitral valve), and parasternal and subcostal long-axis views reveal the proximity of SAS to the aortic valve (see the image below).

Three-dimensional echocardiography can produce images of SAS with depictions of the morphology and extent remarkably similar to those observed during surgical visualization.

Multiplanar transesophageal echocardiography (TEE) provides superior definitions of the lesion, making it an ideal tool for intraoperative evaluation of the lesion to guide surgical resection and to evaluate the immediate results at the time of surgery.[14] TEE is also useful for diagnostic purposes in patients with a poor acoustic window in whom transthoracic imaging results are not definitive.

A peak instantaneous and a mean pressure gradient across the LVOT estimated during continuous wave Doppler interrogation provide measures of the severity of LVOT obstruction.

When used as a guide to cardiac intervention, Doppler interrogation does not permit the clinician to accurately estimate the pressure gradient in the presence of multiple obstructive LVOT lesions in series, a large ventricular septal defect (VSD), or a tunnel-like obstruction.

Color Doppler evaluation reveals the presence and severity of aortic and mitral regurgitation.

Previous
Next

Cardiac Catheterization

Cardiac catheterization is not routinely indicated in SAS. However, it is helpful in patients with LVOT obstruction in series and tunnellike obstruction to delineate the site and severity of LVOT obstruction. Cardiac catheterization is also used for preoperative hemodynamic evaluation in SAS associated with other congenital heart defects.

Careful pullback pressure measurements performed with an end-hole or high-fidelity manometer-tipped catheter from the LV to the aorta allow delineation of the pressure gradient and the exact site of obstruction in the LVOT.

Previous
Next

Electrocardiography

In SAS, even if mild, ECG reveals a variable degree of LV hypertrophy in 50-80% of patients. ECG findings are occasionally normal in patients with severe SAS. A prominent Q wave in the left precordial leads may be present from septal hypertrophy. Strain pattern is visible on the ECG in approximately 25% of patients and indicates severe obstruction.

Previous
Next

Cineangiography

Cineangiography is usually not necessary to define the anatomy of SAS, but it may be helpful if catheterization is being performed to better delineate multilevel LVOT obstruction and to evaluate other associated cardiac defects. A left ventriculogram obtained in a cranially angulated left anterior oblique orientation delineates the LVOT and the anatomy of the SAS, and it reveals aortic valve stenosis, if present. The degree of mitral valve regurgitation and anatomy of any ventricular septal defects, if present, are also well demonstrated.

Previous
Next

Histologic Findings

Histologic findings in SAS are the same in lesions of the fibromuscular ridge or collar and in tunnellike lesions. A composite of different tissue cells, which varies from patient to patient, is present.

Abundant amounts of irregularly oriented and dense collagen fibers and thin, short elastic fibers are visible. Also visible are sparsely scattered fibroblasts with elongated nuclei and smooth muscle cells. Vascularity is generally absent.

Previous
 
 
Contributor Information and Disclosures
Author

Douglas J Schneider, MD  Associate Professor of Pediatrics, Clinical Tract, Division of Pediatric Cardiology, Department of Pediatrics, University of Kentucky College of Medicine; Director, Pediatric Cardiac Catheterization Laboratory, Kentucky Children's Hospital

Disclosure: Nothing to disclose.

Coauthor(s)

Gautam K Singh, MD, DCh, MRCP, FACC  Associate Professor of Pediatrics, Division of Cardiology, Director of Noninvasive Imaging Research, Co-director of Echocardiography Laboratory, Washington University School of Medicine; Attending Faculty, Department of Pediatrics, Division of Cardiology, St Louis Children's Hospital

Gautam K Singh, MD, DCh, MRCP, FACC is a member of the following medical societies: American College of Cardiology, American Heart Association, American Society of Echocardiography, and Royal College of Physicians

Disclosure: Nothing to disclose.

Specialty Editor Board

Paul M Seib, MD  Associate Professor of Pediatrics, University of Arkansas for Medical Sciences; Medical Director, Cardiac Catheterization Laboratory, Co-Medical Director, Cardiovascular Intensive Care Unit, Arkansas Children's Hospital

Paul M Seib, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, Arkansas Medical Society, International Society for Heart and Lung Transplantation, and Society for Cardiac Angiography and Interventions

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.

John W Moore, MD, MPH  Professor of Clinical Pediatrics, Section of Pediatric Cardiology, Department of Pediatrics, University of California San Diego School of Medicine; Director of Cardiology, Rady Children's Hospital

John W Moore, MD, MPH is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and Society for Cardiac Angiography and Interventions

Disclosure: Nothing to disclose.

References
  1. Iwata Y, Imai Y, Shin'oka T, Kurosawa H. Subaortic stenosis associated with systolic anterior motion. Heart Vessels. Nov 2008;23(6):436-9. [Medline].

  2. Somerville J, Stone S, Ross D. Fate of patients with fixed subaortic stenosis after surgical removal. Br Heart J. Jun 1980;43(6):629-47. [Medline]. [Full Text].

  3. Bezold LI, Smith EO, Kelly K, et al. Development and validation of an echocardiographic model for predicting progression of discrete subaortic stenosis in children. Am J Cardiol. Feb 1 1998;81(3):314-20. [Medline].

  4. Edwards JE. Pathology of left ventricular outflow tract obstruction. Circulation. Apr 1965;31:586-99. [Medline].

  5. Barkhordarian R, Wen-Hong D, Li W, et al. Geometry of the left ventricular outflow tract in fixed subaortic stenosis and intact ventricular septum: an echocardiographic study in children and adults. J Thorac Cardiovasc Surg. Jan 2007;133(1):196-203. [Medline].

  6. Kleinert S, Geva T. Echocardiographic morphometry and geometry of the left ventricular outflow tract in fixed subaortic stenosis. J Am Coll Cardiol. Nov 1 1993;22(5):1501-8. [Medline].

  7. Newfeld EA, Muster AJ, Paul MH, et al. Discrete subvalvular aortic stenosis in childhood. Study of 51 patients. Am J Cardiol. Jul 1976;38(1):53-61. [Medline].

  8. Wright GB, Keane JF, Nadas AS, et al. Fixed subaortic stenosis in the young: medical and surgical course in 83 patients. Am J Cardiol. Oct 1 1983;52(7):830-5. [Medline].

  9. Suri RM, Dearani JA, Schaff HV, Danielson GK, Puga FJ. Long-term results of the Konno procedure for complex left ventricular outflow tract obstruction. J Thorac Cardiovasc Surg. Nov 2006;132(5):1064-71. [Medline].

  10. Karamlou T, Gurofsky R, Bojcevski A, et al. Prevalence and associated risk factors for intervention in 313 children with subaortic stenosis. Ann Thorac Surg. Sep 2007;84(3):900-6; discussion 906. [Medline].

  11. Rohlicek CV, del Pino SF, Hosking M, Miro J, Côté JM, Finley J. Natural history and surgical outcomes for isolated discrete subaortic stenosis in children. Heart. Dec 1999;82(6):708-13. [Medline]. [Full Text].

  12. Gersony WM. Natural history of discrete subvalvar aortic stenosis: management implications. J Am Coll Cardiol. Sep 2001;38(3):843-5. [Medline].

  13. Brauner R, Laks H, Drinkwater DC Jr, Shvarts O, Eghbali K, Galindo A. Benefits of early surgical repair in fixed subaortic stenosis. J Am Coll Cardiol. Dec 1997;30(7):1835-42. [Medline].

  14. Singh GK, Shiota T, Cobanoglu A, et al. Diagnostic accuracy and role of intraoperative biplane transesophageal echocardiography in pediatric patients with left ventricle outflow tract lesions. J Am Soc Echocardiogr. Jan 1998;11(1):47-56. [Medline].

  15. Brown JW, Ruzmetov M, Vijay P, et al. Operative results and outcomes in children with Shone's anomaly. Ann Thorac Surg. Apr 2005;79(4):1358-65. [Medline].

  16. [Guideline] Bonow RO, Carabello BA, Kanu C, et al. ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease): developed in collaboration with the Society of Cardiovascular Anesthesiologists: endorsed by the Society for Cardiovascular Angiography and Interventions and the Society of Thoracic Surgeons. Circulation. Aug 1 2006;114(5):e84-231. [Medline].

Previous
Next
 
Echocardiogram of membranous subaortic stenosis. AO = aortic; LA = left atrium; LVOT = left ventricular outflow tract.
Tunnel-type of subaortic stenosis (subvalvular aortic stenosis [SAS]). MV = mitral valve.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.