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Key Concepts

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  • Epidemiology

    • Ventricular septal defect (VSD) is the most common noncyanotic cardiac anomaly (20 percent of all malformations); it is present in over 50 percent of children with complex congenital heart disease. VSDs occur in 0.5 of 1000 live births, and 5 percent are related to chromosomal syndromes.

  • Morphology

    • VSDs vary in size, number, and location along septum. They are classified as (1) perimembranous (PM, most common), (2) inlet type, (3) nonmuscular outlet, and (4) muscular. The conduction system and aortic valve leaflets are in anatomic proximity and are at risk during repair.

  • Pathophysiology

    • Degree and direction of shunting between the right and left ventricles depend on size (restrictive or nonrestrictive) and balance between the systemic vascular resistance (SVR) and pulmonary vascular resistance (PVR). Aortic regurgitation can ensue as a consequence of leaflet prolapse through the defect.

  • Clinical features

    • Small, restrictive defects are often asymptomatic. Symptoms of overcirculation are present in unrestrictive VSDs; with the development of irreversible pulmonary vascular changes in untreated large shunts, fixed pulmonary hypertension and cyanosis develop (Eisenmenger syndrome).

  • Diagnosis

    • Echocardiography accurately defines the anatomy of VSD and associated lesions. Cardiac catheterization is rarely required.

  • Treatment

    • Timing of closure is indicated by the degree of shunting and aortic valve involvement, and is most typically performed in infancy. Transatrial or transventricular approaches are most commonly utilized, with minimal morbidity and mortality. Percutaneous or periventricular device closure is still experimental, while a staged approach (with pulmonary arterial banding and delayed complete repair) is now considered only in selected cases.

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Definition and Epidemiology

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A ventricular septal defect (VSD) is a deficiency in the ventricular septum that can vary in size, number, or location on the septum. All three determine its physiology, while the location alone determines its nomenclature. This is the most commonly recognized congenital heart defect excluding bicuspid aortic valve. Approximately 20 percent of patients with cardiac malformations have isolated VSDs and, if one includes VSDs in combination with other defects, VSDs are diagnosed in 50 percent of all patients with congenital heart disease.1 VSDs occur at a rate of 0.5 per 1000 live births and in 4.5 to 7 of 1000 premature infants,1,2 with a slightly higher prevalence in females (56 percent).3 About 5 percent of VSDs are related to chromosomal syndromes (such as 22q11 deletion and trisomy 21), in which VSD is the most common cardiac defect identified.4

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Historical Note

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In 1891, Dupren coined the term Maladie de Roger in honor of Henry-Louis Roger, who first described the clinical and pathologic findings of a VSD in 1879.5 Eisenmenger then chronicled the natural history of an unrestrictive VSD by his account of the postmortem findings in a cyanotic patient who died at age 32 with a large VSD, a severely hypertrophied right ventricle (RV), pulmonary valve (PV) and tricuspid valve (TV) insufficiency, and thickened pulmonary arteries (PAs).6...

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