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......................................................................................................................................................................... 4.2.4 Surgical/catheter interventional treatment Indications for intervention are summarized in the Recommendations for intervention in ventricular septal defect (native and residual) table and in Figure 3.

Surgical closure can be performed with low operative mortality (12%) and good long-term results.163 Transcatheter closure has become an alternative, particularly in residual VSDs, in VSDs that are poorly accessible for surgical closure, and in muscular VSDs that are located centrally in the interventricular septum. In perimembranous VSD, it has been shown to be feasible. Whether the risk of complete AV block and entrapment of TV tissue leading to TR, or the risk of AR that has been observed in children, is relevant in adults undergoing interventional closure of a perimembranous VSD remains to be seen. Patients eligible for VSD closure at adult age are rare. Most patients have either small VSDs with insignificant shunt or have already developed PH. The latter must be evaluated with particular caution. Patients with shunt closure and persistent/progressive PAH appear to have a particularly poor outcome.48 4.2.5 Follow-up recommendations Development of AR or TR, degree of (residual) shunt, LV dysfunction, elevation of PAP, or development of DCRV, should be excluded or assessed if present by echocardiography.

Possible development of complete AV block requires attention (patients who develop bifascicular block or transient trifascicular block after VSD closure are at risk in later years for the development of complete AV block). Patients with more than small residual VSD, valvular lesions, or haemodynamic impairment (LV dysfunction or PAH) should be seen every year, including evaluation in specialized ACHD centres. In patients with a small VSD (native or residual, normal LV, normal PAP, asymptomatic) and no other lesion, 35-year intervals may be reasonable. After device closure, regular follow-up during the first 2 years and then, depending on the results, every 25 years is reasonable. After surgical closure without residual abnormality, 5-year intervals may be reasonable.

4.2.6 Additional considerations

• Exercise/sports: no restrictions are required in patients after VSD closure, or with small VSD without PH, significant arrhythmias, or LV dysfunction. Patients with PAH must limit themselves to low-intensity recreational activity/sports (see section 3.5.5). • Pregnancy: patients with pre-capillary PH (PAH) should be counselled against pregnancy. The risk is low in asymptomatic patients with normal LV and no PAH (see section 3.5.7). • IE prophylaxis: recommended only for high-risk patients (see section 3.4.6).

4.3 Atrioventricular septal defect

4.3.1 Introduction and background An AVSD (AV canal or endocardial cushion defect) is characterized by the presence of a common AV junction. The partial AVSD usually has a defect only at the atrial level (primum ASD) or, in rare cases, only at the ventricular level. The anterior and posterior bridging leaflets are fused centrally, creating separate left- and right-sided orifices. In a complete AVSD, the central fusion is not present and there is only one orifice. A complete AVSD (complete AV canal) has a septal defect in the crux of the heart, extending into both the interatrial and interventricular septum (non-restrictive inlet VSD). The AV node is positioned posterior and inferior to the coronary sinus. The bundle of His and the left bundle branch are displaced posteriorly. This accounts for an abnormal activation sequence of the ventricles (prolongation of AV conduction time, left-axis deviation) and is important to recognize during EP studies and catheter ablation. Most complete AVSDs occur in Down syndrome patients (>75%), and most partial AVSDs occur in non-Down syndrome patients (>90%). AVSD may occur in association with TOF and other forms of complex CHD. An AVSD with unequal position of the common AV valve above the ventricles is accompanied by a variable degree of ventricular hypoplasia (unbalanced AVSD). The following recommendations apply to balanced AVSDs.

Recommendations for intervention in ventricular septal defect (native and residual)

Recommendations Classa Levelb

In patients with evidence of LV volume overloadc and no PAH (no non-invasive signs of PAP elevation or invasive confirmation of PVR <3 WU in case of such signs), VSD closure is recommended regardless of symptoms. I C In patients with no significant LR shunt, but a history of repeated episodes of IE, VSD closure should be considered. IIa C In patients with VSD-associated prolapse of an aortic valve cusp causing progressive AR, surgery should be considered. IIa C In patients who have developed PAH with PVR 35 WU, VSD closure should be considered when there is still significant LR shunt (Qp:Qs >1.5). IIa C In patients who have developed PAH with PVR >_5 WU, VSD closure may be considered when there is still significant LR shunt (Qp:Qs >1.5), but careful individual decision in expert centres is required. IIb C VSD closure is not recommended in patients with Eisenmenger physiology and patients with severe PAH (PVR >_5 WU) presenting with desaturation on exercise.d III C

AR = aortic regurgitation; IE = infective endocarditis; LR = left-to-right; LV = left ventricle/ventricular; PAH = pulmonary arterial hypertension; PAP = pulmonary artery pressure; PVR = pulmonary vascular resistance; Qp:Qs = pulmonary to systemic flow ratio; VSD = ventricular septal defect; WU = Wood units. aClass of recommendation. bLevel of evidence. cLV enlargement with increased stroke volume. dThere 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%. Downloaded from academic.oup.com/eurheartj/article/42/6/563/5898606 by guest on 07 October 2026