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Maladie de Roger (Roger’s Disease) : Causes, Symptoms, & Treatment

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.

Types

Interventricular septum has a membranous & a muscular portion.

  1. Membranous (Perimembranous) ⇒ Most common type
  2. Muscular ⇒ > 90% cases close spontaneously by 2 year of age
  3. Supracristal (Rare, but severe) ⇒ Associated with aortic regurgitation

Clinical Features

Silent in neonates because pressure gradients needed for L→R are not stable

Usual age of presentation: 4-6 weeks

Presents as

1. Congestive cardiac failure

  • Failure to thrive
  • Sweating on forehead while feeding
  • Suck-rest-suck cycle
  • Hepatomegaly

2. Recurrent pneumonia

Heart sound

  • S1 & S2: Usually inaudible due to loud murmur
  • Sometimes S2: Wide, variable split

Murmur

  • Pan systolic
  • Flow murmur ±

Chest X-ray

  • 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.

ECG

  • Right axis deviation (Early stages)
  • Axis may become normal once biventricular enlargement happens.

Complications

  • Infective endocarditis
  • Pulmonary hypertension
  • Eisenmenger’s syndrome (Reversal of shunt due to pulmonary hypertension)
  • Aortic regurgitation (AR) in supracristal VSD

Treatment

A. Medical

  • Infective endocarditis prophylaxis

B. Surgical

  • Closure of VSD

Indications

  • 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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