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4.3.5 Follow-up recommendations Lifelong regular follow-up of all patients with an AVSD, operated and unoperated, is recommended, including evaluation in specialized ACHD centres. Particular attention should be paid to residual shunt, AV valve malfunction, LV and RV enlargement and dysfunction, PAP elevation, LVOTO, and arrhythmias.164 The frequency of outpatient visits depends on the presence and severity of residual abnormalities. A patient with a surgically repaired AVSD without significant residual abnormalities should be seen at least every 23 years. In the case of residual abnormalities, the intervals should be shorter.

Indications for reoperation for residual abnormalities are comparable with the indications for primary surgery. In operated patients, the most frequently occurring problem is left-sided AV valve regurgitation.165,166 It has to be emphasized that these valves are different from mitral valves and more difficult to repair. Left-sided AV valve stenosis (most often a result of previous repair) that causes symptoms should be operated on.

4.3.6 Additional considerations

• Exercise/sports: for most patients with uncomplicated, repaired AVSD, physical activity does not need restriction. Many will, however, have subnormal exercise performance when measured objectively. Patients with important residual problems require individual recommendations (see section 3.5.5). • Pregnancy: well tolerated in patients with complete repair and no significant residual lesions. An unoperated partial AVSD increases the risk of paradoxical embolization. Patients with pre-capillary PH should be counselled against pregnancy. As a rule, patients with residual left-sided AV valve regurgitation, who have no indication for surgery, tolerate pregnancy relatively well, although arrhythmias and worsening of AV valve regurgitation may occur167 (see section 3.5.7). • IE prophylaxis: recommended only for high-risk patients (see section 3.4.6).

4.4 Patent ductus arteriosus

4.4.1 Introduction and background Patent ductus arteriosus (PDA) is the persistent communication between the proximal left PA and the descending aorta just distal to the left subclavian artery. It can be associated with a variety of CHD lesions, however, in adults, it is usually an isolated finding. PDA originally results in LR shunt and LV and LA volume overload. In moderate and large PDA, PAP is elevated. In patients who reach adulthood with a moderate PDA, either LV volume overload or PAH may be predominant. Adult patients with a large PDA have, in general, developed Eisenmenger physiology.

4.4.2 Clinical presentation and natural history Presentations of adult patients with PDA include: • Small duct with no LV volume overload (normal LV) and normal PAP (generally asymptomatic). • Moderate PDA with predominant LV volume overload: large LV with normal or reduced function (may present with left heart failure). • Moderate PDA with predominant PAH: pressure-overloaded RV (may present with right heart failure). • Large PDA: Eisenmenger physiology with differential hypoxaemia and differential cyanosis (lower extremities cyanotic, sometimes left arm too); see sections 3.4.3 and 3.4.8. Aneurysm formation of the duct is a very rare complication.

Recommendations for intervention in atrioventricular septal defect

Recommendations Classa Levelb

Complete AVSD Surgical repair is not recommended in patients with Eisenmenger physiology, and patients with PAH (PVR >_5 WU) presenting with desaturation on exercise.c III C For recommendations on intervention see also recommendations for intervention in VSD (section 4.2). Partial AVSD (primum ASD) Surgical closure is recommended in patients with significant RV volume overload and should only be performed by a congenital cardiac surgeon. I C For further details see recommendations for intervention in ASD (section 4.1). AV valve regurgitation Valve surgery, preferably AV valve repair, is recommended in symptomatic patients with moderate to severe AV valve regurgitation and should be performed by a congenital cardiac surgeon. I C In asymptomatic patients with severe leftsided AV valve regurgitation, valve surgery is recommended when LVESD >_45 mmd and/or LVEF <_60% provided other causes of LV dysfunction are excluded. I C In asymptomatic patients with severe leftsided AV valve regurgitation, preserved LV function (LVESD <45 mmd and/or LVEF >60%), high likelihood of successful valve repair, and low surgical risk, intervention should be considered when atrial fibrillation or systolic PAP >50 mmHg is present. IIa C Left ventricular outflow tract obstruction See recommendations for intervention in SubAS (section 4.5.3).

ASD = atrial septal defect; AV = atrioventricular; AVSD = atrioventricular septal defect; LV = left ventricle/ventricular; LVEF = left ventricular ejection fraction; LVESD = left ventricular end systolic diameter; LVOTO = left ventricular outflow tract obstruction; PAH = pulmonary artery hypertension; PAP = pulmonary artery pressure; PVR = pulmonary vascular resistance; RV = right ventricle/ventricular; SubAS = subaortic stenosis; VSD = ventricular septal defect; WU = Wood units. aClass of recommendation. bLevel of evidence. cThere are limited data available for a precise cut-off, but by clinical experience, this would be given by a fall of arterial oxygen saturation <90%. dCut-off refers to average-sized adults and may require adaption in patients with unusually small or large stature. Downloaded from academic.oup.com/eurheartj/article/42/6/563/5898606 by guest on 07 October 2026