Endocardial Cushion Defects (Atrioventricular Canal Defects, Atrioventricular Septal Defects) Guidelines

Updated: Dec 28, 2020
  • Author: Mary C Mancini, MD, PhD, MMM; Chief Editor: Yasmine S Ali, MD, FACC, FACP, MSCI  more...
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Guidelines

Guidelines Summary

2018 American Heart Association/American College of Cardiology (AHA/ACC) guidelines

The 2018 ACC guidelines published in 2019 recommend the following for atrioventricular (AV) septal defect (AVSD). [10]

Cardiac catheterization should be used to assess for the presence of pulmonary hypertension and to assess pulmonary vasoreactivity.

Left AV valve repair or replacement should be undertaken for stenosis and/or regurgitation if it causes symptoms, atrial or ventricular arrhythmia, progressive increase in left ventricular (LV) dimensions or deterioration in LV function. Care should be taken to be sure that pulmonary systolic pressures are less than 50% systemic.

Surgical repair should be undertaken for LV outflow tract obstruction (OTO) with a mean gradient of at least 50 mmHg or peak instantaneous gradient above 70 mmHg or a gradient below 50 mmHg associated with significant mitral regurgitation (MR) or aortic regurgitation (AR).

Surgical repair should be undertaken for residual or recurrent atrial septal defect (ASD) or ventricular septal defect (VSD) with a significant left-to-right shunt. Repair should not be attempted in the face of a right-to-left shunt.

Primary operation is recommended in adults with a left-to-right shunt and pulmonary artery systolic pressures less than 50% systemic and pulmonary vascular resistance (PVR) ess than one third systemic. 

2020 ESC guidelines

The European Society of Cardiology (ESC) updated their 2010 guidelines on the management of adult congenital heart disease (ACHD) in 2020. [11, 12]  Their class I and III recommendations for ASVD are outlined below.

Surgical repair is not recommended in those with Eisenmenger physiology and patients with pulmonary arterial hypertension (PAH) (PVR ≥5 Wood units) who present with exercise desaturation.

Surgical closure performed by a congenital cardiac surgeon is recommended in patients with significant right ventricular (RV) volume overload. Valve surgery, preferably AV valve repair, performed by a congenital cardiac surgeon is recommended in symptomatic patients with moderate to severe AV valve regurgitation.

In asymptomatic patients with severe left-sided AV valve regurgitation, valve surgery is recommended when LV end systolic diameter (ESD) is ≥45 mm and/or LV ejection fraction (EF) is ≤60% after ruling out other causes of LV dysfunction.