Supracristal Ventricular Septal Defect Clinical Presentation

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

History

In patients with supracristal ventricular septal defects (VSDs), symptoms and severity are a function of the size and location of the defect, the relative systemic and pulmonary vascular resistances, and the presence of associated abnormalities. Symptoms may range from severe congestive failure and cardiogenic shock in patients with large conal defects and left heart obstruction to complete absence of symptoms in patients with small, isolated defects.

Exercise intolerance and dyspnea suggest progressive aortic insufficiency, although early detection and treatment for valve insufficiency should obviate any significant symptoms.

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Physical Examination

Congestive heart failure does not occur in the patient with an isolated, small supracristal ventricular septal defect (VSD). General examination findings remain normal, with no signs of respiratory distress or growth failure. Infants with larger defects, especially those associated with significant left ventricular outflow obstruction (eg, doubly committed subarterial defect with interrupted aortic arch), may present as early as the first week of life with profound congestive heart failure and cardiogenic shock. Infants with only a large left-to-right shunt usually develop symptoms in the second month of life.

The murmur of an isolated, small supracristal VSD is similar to that of other types of small VSDs. While it may be loudest in the third left intercostal space (ie, more superior than other VSDs), it begins with the first heart sound and has a similar harsh, noisy quality. As with other types, a large defect may produce no murmur from the defect itself. In this case, a murmur may result from turbulent flow through the pulmonic valve, thus becoming crescendo-decrescendo in character. This murmur may radiate laterally and posteriorly because of shunt flow directed into the branch pulmonary arteries.[14]

Second heart sound findings depend on volume of shunt flow as well as pulmonary artery pressure and resistance. With a small shunt, the second heart sound splits and varies normally with respiration, and the pulmonary component is normal in intensity. With a large shunt, and elevated pulmonary artery pressure, the pulmonary component of S2 increases in intensity. Intensity of this sound is further increased if pulmonary resistance is increased, in which case the splitting interval of S2 is decreased. With a large left-to-right shunt, one should hear a short, low-frequency, middiastolic apical murmur due to enhanced, rapid (passive) filling of the left ventricle. With significantly elevated pulmonary vascular resistance, shunt flow decreases and this diastolic murmur does not occur.

When a patient is known to have a supracristal VSD, physical examination should focus on whether aortic insufficiency is present. Blood pressure must be carefully evaluated for pulse pressure (ie, the difference between systolic and diastolic blood pressures) and pulse amplitude, as these increase with increasing aortic insufficiency unless heart failure also occurs. With significant aortic insufficiency, the aortic component of S2 decreases in intensity. If left ventricular end diastolic pressure increases, left atrial pressure increases, thus causing an increase in intensity of the pulmonic component of S2. Aortic insufficiency causes a high-pitched diastolic murmur beginning with the aortic component of the second heart sound. It is best heard along the left sternal border, usually in the third left intercostal space at the sternal edge.

The combined systolic and diastolic murmurs of supracristal VSD with aortic insufficiency may be likened to the sound of sawing wood. This systolic-diastolic murmur combination should not be misinterpreted as a continuous murmur (eg, patent ductus arteriosus, arteriovenous malformation or fistula). Significant aortic insufficiency may cause a late diastolic murmur at the apex resulting from atrial contraction augmenting late ventricular filling. This is the Austin Flint murmur.

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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.

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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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