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Muscular Ventricular Septal Defect Clinical Presentation

  • Author: Michael D Taylor, MD, PhD; Chief Editor: Stuart Berger, MD  more...
 
Updated: Jan 04, 2016
 

History

Murmur

Most patients with small muscular ventricular septal defects (VSDs) are asymptomatic and come to medical attention due to the discovery of a systolic murmur. Most murmurs have a delayed presentation in the newborn period, occurring in the first few days to weeks following birth.

At birth, PVR is high, which maintains an elevated right ventricular pressure equal to the left ventricular pressure. As the PVR falls, the developing pressure gradient from the left ventricle to the right ventricle allows high-velocity left-to-right shunting across the muscular VSD, producing the typical holosystolic murmur.

Progression of symptoms

Patients with an isolated, large muscular VSD are typically asymptomatic in the immediate newborn period. As PVR falls, the degree of left-to-right shunting is proportional to the size of the defect and the relative degree of PVR. The larger the VSD and the lower the PVR, the greater is the degree of left-to-right shunting.

Typically, infants with large VSDs present with signs and symptoms of CHF at age 6-8 weeks or later as PVR continues to fall and the degree of left-to-right shunting increases. CHF signs include inadequate weight gain and growth, along with recurrent lower respiratory tract infections in patients with a large VSD without evidence of CHF but with elevated pulmonary artery pressure (>50% systemic pressure) or or a pulmonary artery – to – systemic flow ratio greater than 2:1.

Signs and symptoms of CHF also include poor feeding, tachypnea, tachycardia, sweating (especially after feeding), and lethargy.

Chromosomal anomalies

VSD is the most common congenital heart lesion (20-30%) in infants with chromosomal anomalies or syndromes. Defects may be discovered in the first days of life due to additional diagnostic evaluation to exclude multiple congenital defects.

Next

Physical Examination

Typical physical examination findings are influenced to a significant degree by the size of the ventricular septal defect (VSD) and the degree of left-to-right shunting.

Small VSD

Symptoms of a small VSD (defined as a VSD with a dimension less than half the size of the aortic annulus diameter) include the following:

  • Vital signs and weight gain are normal
  • The precordium is quiet, with a normal apical impulse
  • The first heart sound is normal
  • The second heart sound is typically narrowly split; the pulmonary component may be accentuated
  • A third heart sound is generally not present
  • A palpable thrill may be observed at the middle to lower left sternal border
  • A grade III-VI/VI holosystolic murmur, which widely radiates throughout the precordium, is present along the left sternal border

The intensity of the murmur is inversely proportional to the size of the defect, the left ventricle–to–right ventricle pressure gradient, and the degree of left-to-right shunting. In general, smaller defects produce the loudest murmur.

Systolic murmurs are usually holosystolic but may occasionally be crescendo or crescendo-decrescendo. No diastolic murmur is typically present.

Large VSD

The symptoms of a large VSD (defined as a defect size equal to the diameter of the aortic annulus) are as follows:

  • Poor growth and poor weight gain are common
  • Signs and symptoms of CHF may be present, including tachypnea, tachycardia, sweating, and pallor
  • Hyperdynamic precordium, with or without a precordial bulge secondary to underlying cardiomegaly
  • Abnormal apical impulse with or without right ventricular tap is present; a thrill is uncommon with large VSDs
  • A normal first heart sound and a narrowly split second heart sound with a loud pulmonary component are evident
  • A prominent third heart sound is typically present at the apex, producing a gallop rhythm
  • A II-III/VI holosystolic murmur is maximal at the left sternal border, with wide precordial radiation
  • A diastolic flow rumble may be present at the cardiac apex; this diastolic murmur is caused by a significant left-to-right shunt (at least a 2:1 left-to-right shunt), with excessive flow across a normal-sized mitral annulus
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Contributor Information and Disclosures
Author

Michael D Taylor, MD, PhD Director, Advanced Imaging Innovation, Cincinnati Children's Hospital Medical Center; Assistant Professor, Department of Pediatrics, University of Cincinnati College of Medicine

Michael D Taylor, MD, PhD is a member of the following medical societies: American College of Cardiology, American Heart Association, Society for Cardiovascular Magnetic Resonance

Disclosure: Nothing to disclose.

Coauthor(s)

Benjamin W Eidem, MD, FACC, FASE Professor of Pediatrics and Medicine, Departments of Pediatrics and Medicine, Divisions of Pediatric Cardiology and Cardiovascular Diseases, Mayo Medical School

Benjamin W Eidem, MD, FACC, FASE 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, Society of Pediatric Echocardiography

Disclosure: Nothing to disclose.

Chief Editor

Stuart Berger, MD Medical Director of The Heart Center, Children's Hospital of Wisconsin; Associate Professor, Department of Pediatrics, Section of Pediatric Cardiology, Medical College 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, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

Acknowledgements

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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