Maladie de Roger (Roger’s Disease) : Definition, Etiology, Symptoms, & Treatment
- Maladie de Roger (Roger’s Disease) is a small ventricular septal defect (VSD) in muscular portion, presenting in the older children as a loud pansystolic murmur without other hemodynamic changes. Such defects usually close spontaneously.
- Bruit de Roger (Roger’s murmur) is a loud pansystolic murmur of a ventricular septal defect.
Maladie de Roger (Roger’s disease) & Bruit de Roger (Roger’s murmur) named after a french pediatrician Henri-Louis Roger.
Ventricular septal defect (VSD)
- VSD implies a defect in the interventricular septum.
- Most common congenital heart disease overall.
Interventricular septum has a membranous & a muscular portion.
- Membranous (Perimembranous) ⇒ Most common type
- Muscular ⇒ > 90% cases close spontaneously by 2 year of age
- Supracristal (Rare, but severe) ⇒ Associated with aortic regurgitation
Silent in neonates because pressure gradients needed for L→R are not stable
Usual age of presentation: 4-6 weeks
1. Congestive cardiac failure
- Failure to thrive
- Sweating on forehead while feeding
- Suck-rest-suck cycle
2. Recurrent pneumonia
- S1 & S2: Usually inaudible due to loud murmur
- Sometimes S2: Wide, variable split
- Pan systolic
- Flow murmur ±
- Cardiomegaly with pulmonary plethora
- RA : Normal
- RV : Enlarged (Early)
- LA, LV : Enlarged (Later on once pulmonary hypertension develops
Exception: The cause of RV enlargement in pre-membranous VSD is due to failure of fetal RV enlargement to regress.
- Right axis deviation (Early stages)
- Axis may become normal once biventricular enlargement happens.
- Infective endocarditis
- Pulmonary hypertension
- Eisenmenger’s syndrome (Reversal of shunt due to pulmonary hypertension)
- Aortic regurgitation (AR) in supracristal VSD
- Infective endocarditis prophylaxis
- Closure of VSD
- Any supracristal VSD
- VSD with associated aortic regurgitation or pulmonary stenosis
- Large VSD with failure of medical management of congestive heart failure (CCF)
- Large VSD with Qp/Qs ≥ 2.1
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