Surgical Ventricular Septal Myectomy for XXXX With Noonan Syndrome and Symptomatic Left Ventricular Outflow Tract Obstruction.

@entity1 @entity384 @entity4346 @entity204 @entity1121 @entity12697
UNASSIGNED: Approximately 20% to 30% of @entity1 with @entity12697 ( @entity12697 ) have asymmetric @entity4346 ( @entity4346 ) and LV outflow tract obstruction (LVOTO). The role of surgical myectomy in such @entity1 is unknown. We sought to compare clinical features and outcomes of @entity1 with @entity12697 and LVOTO with age- and gender-matched @entity1 with nonsyndromic, obstructive @entity1121 ( @entity1121 ) after myectomy. Two cohorts were selected and retrospectively analyzed using Mayo Clinic databases from 1996 to 2014. Subjects included @entity1 with @entity12697 with @entity4346 and LVOTO and nonsyndromic controls with obstructive @entity1121 . Twenty-three @entity1 with @entity12697 and @entity4346 were identified, of whom 12 (8 males) underwent myectomy (10 septal and 2 combined septal/apical) for severe LVOTO (10 pediatric and 2 adults; 13    10  year old [range 1 to 39]). Similar echocardiographic improvements were noted in both groups. There were no perioperative @entity204 . Residual gradients were slightly higher in @entity1 with @entity12697 . No improvement was noted in left atrial volume after myectomy in @entity1 with @entity12697 . At early follow-up, the majority showed improvement in the New York Heart Association class (88% in @entity12697 vs 82% in @entity1121 , median of 6 and 2  months, respectively). At late follow-up (median of 7  years), the survival rate was 92% in @entity12697 and 100% in @entity1121 . In @entity1 with @entity12697 with @entity4346 and symptomatic LVOTO, myectomy reduces both gradient and the New York Heart Association class, similar to @entity1 with @entity1121 . Residual gradients were slightly higher, and left @entity384 persisted in @entity1 with @entity12697 . In conclusion, myectomy should be considered in @entity1 older than 1 year with @entity12697 and symptomatic LVOTO.
