Atypical forms of isolated partial atrioventricular septal defect increase the risk of initial valve replacement and reoperation

Abstract

The morbi-mortality of the isolated partial AV septal defect is primarily perioperative and is linked with the presence of an atypical form of the lesion. This atypical form was the main reason for reoperation for AV valve regurgitation. The AV valve replacement was associated with a high mortality and with the occurrence of complete AV block. Using a standardized technique, the AV septal defect can be repaired with excellent long-term clinical and echographic results.

Partial and Transitional Atrioventricular Septal Defect Outcomes

“…Murashita and colleagues [3] reported 30% of patients with partial/transitional AVSD (median repair age 5.3 years) had at least grade II (scale range, I to IV) LAVVR at hospital discharge that increased to 43% at mean follow-up of almost 9 years. Data from Aubert and colleagues [4] showed LAVVR grade II or higher in 26% of repaired partial AVSD patients (median repair age 5.8 years), with 2.9% having repeat valvuloplasty within 30 days, comparable to our early reoperation rate of 3.4%.…”

“…Some investigators postulate that despite individualizing each case, alterations in geometry and rotation of the axis of closure combined with deficient and dysplastic subvalvar components may leave some of these valves incompetent, despite the advances in valvuloplasty that have been achieved in the recent era [2, 4, 21]. Because valve regurgitation is progressive, the effects of relatively longstanding significant LAVVR may be another contributing factor.…”

Factors Associated with Moderate or Severe Left Atrioventricular Valve Regurgitation within 30 days of Repair of Incomplete Atrioventricular Septal Defect

“…Despite different ages, different weight at repair, and different physiology, patients with both complete and incomplete AVSD present a similar risk of reoperation for LAVVR [ 1 , 3 - 6 , 9 - 14 ] . What both variations of the same disease have in common are the typical anatomical landmarks of AVSD (common AV junction, a common 5-leaflet AV valve, distinct papillary muscle displacement and a narrow and elongated left ventricle outflow tract) [ 15 , 16 ] , as well as a high prevalence of individuals with Down syndrome [ 17 ] .…”

“…A non negligible number of patients who undergo surgical repair of incomplete atrioventricular septal defect (AVSD) are discharged from hospital with residual left atrioventricular valve regurgitation (LAVVR) [ 1 , 2 ] . Reoperation rates for LAVVR are still relatively high, varying between 7% and 22% [ 1 , 3 - 6 ] .…”

“…A non negligible number of patients who undergo surgical repair of incomplete atrioventricular septal defect (AVSD) are discharged from hospital with residual left atrioventricular valve regurgitation (LAVVR) [ 1 , 2 ] . Reoperation rates for LAVVR are still relatively high, varying between 7% and 22% [ 1 , 3 - 6 ] . Many factors associated to reoperation have been proposed [ 1 , 2 , 4 , 6 ] , but there is a lack of data reporting factors associated with immediate postoperative LAVVR, although the reports of valve replacement and of in-hospital deaths due to significant residual LAVVR within 30 days of operation [ 1 , 5 , 7 ] .…”