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  • • A congenital heart lesion that increases pulmonary arterial blood flow

    • Results in left-to-right shunt, results in lung infection, pulmonary vascular congestion, pulmonary artery (PA) hypertension, right heart failure, pulmonary vasoconstriction, pulmonary vascular obstructive disease

    Eisenmenger syndrome: Increased pulmonary hypertension such that left-to-right shunt ceases and shunt becomes right-to-left, requiring heart-lung transplant

    • Inhaled nitric oxide, oxygen, or IV tolazoline reverses PA vasoconstriction

    • PA band is palliative and can reduce PA flow to alleviate RV failure and progression of pulmonary hypertension

    • Defects occur in 4 anatomic positions

    • -Perimembranous septum (85%), anterior to crista supraventricularis, beneath leaflet of tricuspid valve, or muscular septum

    • Often associated with more complex defects: Truncus arteriosus, AV canal defect, tetralogy of Fallot, transposition of great arteries

    • Isolated perimembranous defects may have associated patent ductus arteriosus (PDA), aortic coarctation

    • Supracristal defects may have aortic regurgitation

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Epidemiology

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  • • VSD accounts for 25% of congenital cardiac anomalies

    • In infants, 33% of defects are small, many close spontaneously by age 8

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Symptoms and Signs

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  • • If defect small, asymptomatic

    • If large, heart failure with dyspnea, frequent respiratory infections, poor growth

    • Pansystolic murmur at left lower sternal border

    • Loud S2 with apical diastolic flow murmur and biventricular enlargement

    • Dyspnea on exertion, poor weight gain, easy fatigability

    • Chronic respiratory difficulties, poor feeding

    • Pulmonary vascular disease may develop

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Imaging Findings

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  • Chest film: Enlarged heart, congested lung fields

    Echocardiography: Diagnostic

    Catheterization: If older defect or young child: Assess pulmonary artery pressure and vascular resistance

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  • • May be a part of a more complex defect (truncus arteriosus, AV canal defect, tetralogy of Fallot)

    • Isolated perimembranous defects may also have PDA or aortic coarctation

    • Supracristal defects occasionally have aortic valve regurgitation

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  • • Physical exam

    • Echocardiography

    • Cardiac catheterization selectively

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  • • 33% have small defects, often close spontaneously by age 8

    • 33% have large defects (symptomatic) require aggressive treatment

    • Synthetic patch closure, sutures away from conduction system

    • May require deep hypothermic circulatory arrest

    • Percutaneous device closure recently used

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Surgery

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Indications

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  • • Medically refractory symptoms in infant

    • Elevated PA pressure

    • Canal-type defect or malaligned conoventricular defect

    • Aortic insufficiency (supracristal defect)

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Complications

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  • • Risk of damage to conduction system

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Prognosis

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  • • Operative mortality rare

    • Severe preoperative failure: Mortality, 3-5%

    • Symptomatic improvement dramatic

    • Pulmonary resistance: No change

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References

Anderson H et al: Is complete heart block after surgical closure of ventricular septal defects still an issue? Ann Thorac Surg 2006;82:948.  [PubMed: 16928514]
Carminati M et al: Transcatheter closure of congenital ventricular septal defects: results of the European registry. Eur Heart ...

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