Isolated congenital mitral valve regurgitation presenting in the first year of life

Abstract

Our experience suggests closure of a patent ductus arteriosus should be undertaken prior to mitral valve surgery. There may be a poorer prognosis in those under one year of age requiring emergency mitral valve surgery. Those who can be managed conservatively or undergo mitral valve surgery as an elective procedure tend to have a better outcome.

Pathophysiology and clinical presentation of paediatric heart failure related to congenital heart disease

“…Mitral regurgitation (MR) is usually associated with atrioventricular septal defects or VSD and the isolated congenital form is rare but severe in the first year of life. Significant MR leads to volume overload with gradual dilatation of LA and LV, resulting in further progression of MR and clinical presentation with congestive HF 38 . A rare condition characterised by severe atrioventricular valves regurgitation leading to HF within few hours after birth is represented by neonatal Marfan syndrome.…”

“…Although the most frequent symptoms are consequent to bronchial compression due to dilated pulmonary arteries and enlarged LA, the neonates with APV may also develop HF: after PVRs fall, pulmonary blood flow increases because of L-R shunt through VSD and PDA if present 36. The cardiorespiratory distress is related to both the increased RV stroke volume, due to PR, and pulmonary congestion due to L-R.Young patients with repaired Tetralogy of Fallot who present severe residual PR after correction with transannular patch may develop early HF because the valvular regurgitation promotes RV Significant MR leads to volume overload with gradual dilatation of LA and LV, resulting in further progression of MR and clinical presentation with congestive HF 38. A rare condition characterised by severe atrioventricular valves regurgitation leading to HF within few hours after birth is represented by neonatal Marfan syndrome.…”