eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology

Ventricular Septal Defect, Perimembranous: Differential Diagnoses & Workup

Author: Michael D Taylor, MD, PhD, Assistant Professor, Departments of Pediatrics (Division of Cardiology) and Radiology, Baylor College of Medicine, Texas Children's Hospital
Coauthor(s): Benjamin W Eidem, MD, FACC, FASE, FAAP, Associate Professor, Divisions of Pediatric Cardiology and Cardiovascular Diseases, Department of Pediatrics, Mayo Clinic College of Medicine
Contributor Information and Disclosures

Updated: Nov 25, 2008

Differential Diagnoses

Aortic Stenosis, Subaortic
Double Outlet Right Ventricle, Normally Related Great Arteries
Double-Chambered Right Ventricle
Pulmonary Stenosis, Infundibular
Ventricular Septal Defect, Muscular
Ventricular Septal Defect, Supracristal

Workup

Laboratory Studies

  • For children with small ventricular septal defects (VSDs), no specific laboratory blood tests are indicated.
  • Occasionally, in the evaluation of children with symptomatic large VSD, brain natriuretic peptide (BNP) is measured as a marker of congestive heart failure (CHF) severity.
  • Children who are maintained on diuretics and ACE inhibitors have their electrolyte levels periodically measured.

Imaging Studies

  • Chest radiography
    • Small VSDs show normal cardiac size and normal pulmonary vascularity.
    • Large VSDs demonstrate cardiac enlargement and increased pulmonary vascular markings proportional to the size of left-to-right shunt, left atrial and left ventricular enlargement, posterior displacement of left ventricular apex, and prominence of main pulmonary artery segment.
  • Two-dimensional echocardiography and Doppler
    • Echocardiography is the most reliable noninvasive modality to identify the presence, size, number, and location of the VSD.
    • Perimembranous VSDs are readily identified from the subcostal short and long axis planes, the apical 4-chamber, parasternal long axis, and parasternal short axis scan planes.
    • Small VSDs (defined as VSD dimension less than half the size of the aortic annulus diameter) are usually isolated defects with otherwise normal cardiac anatomy and function.
    • Large VSDs (defined as defect size equal to the diameter of the aortic annulus) typically have left atrial and left ventricular dilation with normal left ventricular systolic function.
    • Dilation of the main and branch pulmonary arteries also is common.
    • Doppler echocardiography can be used to predict the intracardiac pressure gradient from the left ventricle to the right ventricle using the continuous wave Doppler tracing (modified Bernoulli equation = 4 [velocity squared]). If the systolic systemic pressure is known, in the absence of aortic outflow obstruction, right ventricle and pulmonary artery (in the absence of right ventricular outflow obstruction) systolic pressures can be predicted by subtracting the gradient between the ventricles from the aortic systolic blood pressure.
    • Color Doppler is useful to determine VSD location and size as well as the degree of intracardiac shunting.
    • Echocardiography is also essential to rule out other commonly associated congenital heart lesions, including atrial septal defects, patent ductus arteriosus, pulmonary valve stenosis, and complex congenital heart disease with an associated VSD.
  • MRI
    • Cardiac MRI is a useful adjunct in the evaluation of large muscular VSDs. Black blood imaging at end-diastole reliably shows the anatomy of the ventricular septum, ventricular chambers, and great vessels. Bright blood gradient-echo dynamic images are useful for evaluating the anatomy in all segments of the cardiac cycle. Tiny muscular VSDs are not well seen using cardiac MRI.
    • Flow-sensitive phase contrast imaging is the criterion standard for determining the direction and magnitude of shunting. It can alleviate the requirement for cardiac catheterization in some cases.
  • Three-dimensional echocardiography
    • With the development of real-time 3-dimensional echocardiography (RT3DE), this modality can be applied to the characterization of the ventricular septum.
    • RT3DE allows accurate determination of VSD size, shape, and location. The short acquisition time and acceptable reconstruction time make this technique clinically applicable.3

Other Tests

  • ECG findings vary depending on the VSD size and the degree of intracardiac shunting.
  • Patients with small VSDs have normal ECG findings.
  • Large VSDs show left ventricular hypertrophy (LVH) (ie, volume overload), right ventricular hypertrophy (RVH) (ie, pressure overload), and left atrial enlargement.

Procedures

  • Cardiac catheterization
    • Routine diagnostic cardiac catheterization is no longer required for perimembranous VSDs. Older children and adults with an unoperated large VSD usually require cardiac catheterization prior to surgical closure to assess pulmonary vascular resistance (PVR).
    • Indications for cardiac catheterization in patients with VSD include inadequate noninvasive echocardiographic assessment of size, number, or location of VSD as well as complicated associated anatomy.
    • Another indication is the requirement of additional hemodynamic data prior to medical management or surgical repair (eg, determination of pulmonary vascular resistance and its reactivity, quantitation of left-to-right shunting, exclusion of associated congenital heart defects).
  • Angiography: Membranous VSDs are best demonstrated in the long axial oblique orientation.

Histologic Findings

  • No specific histological abnormality is present.

More on Ventricular Septal Defect, Perimembranous

Overview: Ventricular Septal Defect, Perimembranous
Differential Diagnoses & Workup: Ventricular Septal Defect, Perimembranous
Treatment & Medication: Ventricular Septal Defect, Perimembranous
Follow-up: Ventricular Septal Defect, Perimembranous
References
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References

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Further Reading

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Keywords

ventricular septal defect, VSD, perimembranous, membranous ventricular septal defect, ventricular septum, right ventricular outflow obstruction, congestive heart failure, CHF, cardiac lesion, atrial septal defect, ASD, patent ductus arteriosus, prematurity, pulmonary valve stenosis, pulmonary venous obstruction, persistent elevation of pulmonary vascular resistance, mitral stenosis, Eisenmenger syndrome, cardiomegaly

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Contributor Information and Disclosures

Author

Michael D Taylor, MD, PhD, Assistant Professor, Departments of Pediatrics (Division of Cardiology) and Radiology, Baylor College of Medicine, Texas Children's Hospital
Michael D Taylor, MD, PhD is a member of the following medical societies: American College of Cardiology, American Heart Association, and Society for Cardiovascular Magnetic Resonance
Disclosure: Nothing to disclose.

Coauthor(s)

Benjamin W Eidem, MD, FACC, FASE, FAAP, Associate Professor, Divisions of Pediatric Cardiology and Cardiovascular Diseases, Department of Pediatrics, Mayo Clinic College of Medicine
Benjamin W Eidem, MD, FACC, FASE, FAAP is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Society of Echocardiography, Society for Pediatric Research, and Society of Pediatric Echocardiography
Disclosure: Nothing to disclose.

Medical Editor

Juan Carlos Alejos, MD, Associate Clinical Professor, Department of Pediatrics, Division of Cardiology, University of California at Los Angeles
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

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

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.

CME Editor

Gilbert Herzberg, MD, Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College
Gilbert Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics
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.

 
 
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