Maladie de Roger (Roger’s disease) is a historical term for a small ventricular septal defect (VSD) that produces a disproportionately loud heart murmur but causes no haemodynamic disturbance and no symptoms. It was described by the French physician Henri-Louis Roger in 1879. The loud murmur arises because even a small hole between the ventricles generates significant turbulence as blood jets through the narrow defect during systole; the smaller the hole, the more turbulent and audible the flow.
The VSD in Maladie de Roger is typically located in the muscular ventricular septum rather than the membranous (perimembranous) part, and because it is small, the shunt of blood from the left to the right ventricle is minimal. The right heart and pulmonary circulation are not overloaded, so the heart remains structurally normal in size and function, and pulmonary pressures remain normal. The murmur, however, can be startlingly loud and was historically alarming to those unfamiliar with its benign nature.
In practice, many small VSDs close spontaneously during childhood without any intervention. For those that persist into adulthood, a small, haemodynamically insignificant VSD typically requires no treatment beyond periodic cardiology review and awareness of the slightly increased risk of infective endocarditis. NICE no longer recommends routine antibiotic prophylaxis before dental or surgical procedures for most congenital heart conditions, but individuals should discuss their specific situation with their cardiologist.
For survivors of sudden cardiac arrest who are found to have a small VSD on echocardiography, the VSD itself is very unlikely to have caused the cardiac arrest, and investigation for other arrhythmia substrates should not be curtailed.