Supracristal Ventricular Septal Defect Treatment & Management

  • Author: Ira H Gessner, MD; Chief Editor: Stuart Berger, MD   more...
 
Updated: Nov 15, 2011
 

Approach Considerations

Once the diagnosis of supracristal ventricular septal defect (VSD) has been made, carefully monitor patients for the development of aortic insufficiency. This necessitates not only periodic physical examination with auscultation but also serial and Doppler echocardiograms, because these diagnostic studies are more sensitive than auscultation in detecting valve regurgitation.

Because spontaneous closure is uncommon in supracristal VSDs and aortic insufficiency is a common complication, surgical closure is recommended in most cases. Aortic insufficiency in supracristal VSD is usually progressive and warrants an aggressive approach with early intervention to avoid aortic valve deformity and replacement.

Aortic insufficiency caused by supracristal VSD must be differentiated from that caused by an abnormal aortic valve (usually a bicuspid valve). Surgical intervention is usually delayed in the latter disorder, because the abnormal aortic valve typically requires replacement rather than repair in cases of aortic insufficiency.

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Surgical Treatment

Because of the orientation of the right ventricular outflow tract, a surgical approach from the right atrium may not allow adequate visualization of the ventricular septal defect (VSD).[19, 20] Incision into the main pulmonary artery, which exposes the defect through the pulmonic valve, has proved successful.

Repair may be achieved with patch or suture closure, depending on the size of the defect. Aortic valvuloplasty is often, but not always, necessary, and incision through the aortic root can allow adequate visualization for valve repair (Trusler technique). The approach through the main pulmonary artery avoids the need for incision into the right ventricle. Care should be taken to avoid capturing the aortic cusp into one of the patch sutures.

Intraoperative transesophageal echocardiographic monitoring can be extremely helpful in precisely defining aortic valve prolapse and the severity of valve insufficiency, which determine the necessity of valvuloplasty. Follow-up intraoperative assessment should be used to rule out residual insufficiency.[21]

More extensive damage to the aortic valve from long-standing prolapse and distortion may require valve replacement.

Follow-up care after supracristal ventricular septal defect (VSD) repair and aortic valvuloplasty is essential to ensure that the aortic insufficiency has been corrected completely.

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Activity

Activity level is determined by the age at which signs or symptoms develop. Infants with large left-to-right shunts, particularly with complex left heart obstruction, may show a delay in the development of gross motor skills. Patients with small left-to-right shunts without aortic valve insufficiency or with only trivial aortic valve insufficiency generally should be allowed full activity without restriction.

Older patients with more significant aortic valve insufficiency should be restricted from competitive athletics and from sustained isometric types of activities (eg, weightlifting, rope pulls, sustained heavy lifting on the job).

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Contributor Information and Disclosures
Author

Ira H Gessner, MD  Professor Emeritus, Pediatric Cardiology, University of Florida College of Medicine

Ira H Gessner, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Pediatric Society, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Coauthor(s)

Edward J Bayne, MD  Assistant Professor, Division of Pediatric Cardiology, Emory University School of Medicine; Consulting Staff, Sibley Heart Center Cardiology, Children's Healthcare of Atlanta

Edward J Bayne, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Cardiology, American Heart Association, and American Society of Echocardiography

Disclosure: Nothing to disclose.

Chief Editor

Stuart Berger, MD  Professor of Pediatrics, Division of Cardiology, Medical College of Wisconsin; Chief of Pediatric Cardiology, Medical Director of Pediatric Heart Transplant Program, Medical Director of The Heart Center, Children's Hospital of Wisconsin

Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, and Society for Cardiac Angiography and Interventions

Disclosure: Nothing to disclose.

Additional Contributors

Juan Carlos Alejos, MD Clinical Professor, Department of Pediatrics, Division of Cardiology, University of California, Los Angeles, David Geffen School of Medicine

Juan Carlos Alejos, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Medical Association, and International Society for Heart and Lung Transplantation

Disclosure: Actelion Honoraria Speaking and teaching

Hugh D Allen, MD Professor, Department of Pediatrics, Division of Pediatric Cardiology and Department of Internal Medicine, Ohio State University College of Medicine

Hugh D Allen, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Pediatric Society, American Society of Echocardiography, Society for Pediatric Research, Society of Pediatric Echocardiography, and Western Society for Pediatric Research

Disclosure: Nothing to disclose.

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

References

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  22. Levine HJ, Gaasch WH. Vasoactive drugs in chronic regurgitant lesions of the mitral and aortic valves. J Am Coll Cardiol. Nov 1 1996;28(5):1083-91. [Medline].

 
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Parasternal long-axis echocardiogram view showing supracristal ventricular septal defect (arrow) with buckling and prolapse (***) of the right coronary leaflet of the aortic valve.
Parasternal short-axis echocardiogram view with color Doppler showing proximity of ventricular septal defect jet to the aortic and pulmonic valves. The patient is an infant with neither aortic valve prolapse nor aortic insufficiency.
Subcostal "right ventricular inflow/outflow" view showing the close relationship between the aortic and pulmonic valves in the presence of supracristal ventricular septal defect. Turbulent shunt flow is shown directed into the main pulmonary artery. The patient is an infant with neither aortic valve prolapse nor insufficiency.
Transesophageal horizontal view of aortic root and right ventricle, showing sinus of Valsalva aneurysm leaking through a supracristal ventricular septal defect (VSD)(>
 
 
 
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