p.31 抽出テキスト

.......................................................... pulmonary vein(s) to the left innominate vein, and right upper pulmonary vein(s) connecting high on the SVC. Long-term sequelae of anomalous pulmonary venous connections reflect the impact of right heart volume overload and are similar to the sequelae of ASDs.

Surgical repair can be challenging as low-velocity venous flow imparts risk of thrombosis of the surgically operated vein, particularly in scimitar syndrome; it should only be performed by a congenital cardiac surgeon. Indications for surgery follow the principals of recommendation for ASD closure, but technical suitability for repair and operative risk must be weighed against the potential benefit of intervention. It is unusual for a single anomalous pulmonary venous connection of only one pulmonary lobe to result in a sufficient volume load to justify surgical repair.

4.1.6 Follow-up recommendations Follow-up evaluation should include assessment of a residual shunt, RV size and function, TR and PAP by echocardiography, and assessment of arrhythmias by history, ECG, and  only if indicated  Holter monitoring. Patients repaired at age <25 years without relevant sequelae or residua (no residual shunt, normal PAP, normal RV, no arrhythmias) do not require regular follow-up. However, patients and referring physicians should be informed about the possible late occurrence of tachyarrhythmias.

Patients with residual shunt, elevated PAP, or arrhythmias (before or after repair) and those repaired at adult age (particularly >40 years) should be followed on a regular basis, including evaluation in specialized ACHD centres (intervals depending on the severity of residual problems). After device closure, regular follow-up during the first 2 years and then, depending on results, every 35 years is reasonable. Late post-operative arrhythmias after surgical repair at age <40 years are most frequently IART or atrial flutter, which can be successfully treated with radiofrequency or cryoablation. Without repair or with repair after age 40 years, AF becomes more common. In patients who undergo ASD closure aged >40 years, the prevalence of atrial arrhythmias is up to 4060%. Access to the LA may be restricted after device closure. SVC stenosis may occur after repair of a sinus venosus defect, as well as stenoses of redirected pulmonary veins.

©ESC 2020

Figure 2 Management of atrial septal defect. ASD = atrial septal defect; LR = left-to-right; LV = left ventricle/ventricular; PAH = pulmonary arterial hypertension; PVR = pulmonary vascular resistance; Qp:Qs = pulmonary to systemic flow ratio; RV = right ventricle/ventricular; WU = Wood units. aRV enlargement with increased stroke volume. bProviding there is no PAH or LV disease. cIn elderly patients not suitable for device closure, carefully weigh surgical risk vs. potential benefit of ASD closure. dCarefully weigh the benefit of eliminating LR shunt against the potential negative impact of ASD closure on outcome due to an increase in filling pressure (taking closure, fenestrated closure, and no closure into consideration). Downloaded from academic.oup.com/eurheartj/article/42/6/563/5898606 by guest on 07 October 2026