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............................................................................................................................................................................. arrhythmias. Severe pulmonary regurgitation has been identified as an independent predictor of maternal complications, especially in patients with impaired RV function.

4.3.6.2 Obstetric and offspring risk There is no evidence of increased obstetric risks.

4.3.6.3 Management Mild and moderate PS are low-risk lesions (WHO risk classes I and II) and two or three follow-up sessions are sufficient. In patients with severe PS, monthly or bimonthly cardiac evaluations are advised, focusing on RV function. In severely symptomatic PS, which is unresponsive to medical therapy and bed rest, percutaneous valvuloplasty can be appropriate.

4.3.7 Congenital aortic stenosis AS, aortic dilatation, and bicuspid aortic disease are discussed in sections 5 and 6.

4.3.8 Tetralogy of Fallot 4.3.8.1 Maternal risk Women with repaired tetralogy of Fallot usually tolerate pregnancy well (WHO risk class II). Cardiac complications have been reported in 8% of repaired patients, especially in those taking cardiac medication prior to pregnancy.151 Arrhythmias and HF are the most common complications. Thrombo-embolism and endocarditis are rarer. Dysfunction of the RV and/or moderate to severe pulmonary regurgitation are risk factors. Previous pregnancy may be associated with a persisting increase in RV size and long-term cardiac events.

4.3.8.2 Obstetric and offspring risk The risk of offspring complications is increased, in particular foetal growth restriction.152 Maternal screening for 22q11 deletion should be undertaken prior to pregnancy.

4.3.8.3 Management Follow-up every trimester is sufficient in most patients. In women with severe pulmonary regurgitation, monthly or bimonthly cardiac evaluation is indicated. If RV failure occurs during pregnancy, treatment with diuretics should be started and bed rest advised. Early delivery or, rarely, transcatheter valve implantation could be considered in those who do not respond to conservative treatment.

4.3.9 Ebstein’s anomaly 4.3.9.1 Maternal risk In women with uncomplicated Ebstein’s anomaly, pregnancy is often tolerated well (WHO risk class II). Symptomatic patients with cyanosis and/or HF should be counselled against pregnancy. The haemodynamic problems seen largely depend on the severity of tricuspid regurgitation (TR) and on RV function. Cyanosis (due to ASD/patent foramen ovale) and arrhythmias due to accessory pathways are common. There is also an increased risk of HF and pre-term delivery.153

4.3.9.2 Obstetric and offspring risk Foetal and neonatal outcomes are related to maternal oxygen saturation and CO. 4.3.9.3 Management Even severe TR with HF can usually be managed medically during pregnancy. Women with interatrial shunting can develop progressive cyanosis during pregnancy and be at increased risk of paradoxical emboli, and these should be assessed at each visit.

4.3.10 Transposition of the great arteries 4.3.10.1 Maternal risk In patients with transposition of the great arteries (TGA), the risks associated with pregnancy are mainly attributable to women with a previous atrial (Senning and Mustard) switch, not an arterial switch. Though many women with an atrial switch operation tolerate pregnancy relatively well, there is an increased risk of developing arrhythmias (sometimes life-threatening) and HF (WHO risk class III). An irreversible decline in RV function and worsening TR are also described.154,155 Patients with more than moderate impairment of RV function or greater than moderate TR should be advised against pregnancy.

4.3.10.2 Obstetric and offspring risk The risk of low birth weight and pre-term delivery is 38%.

4.3.10.3 Management Monthly or bimonthly review focusing on systemic RV function and arrhythmia is required. Diuretics and other HF therapies may be required.

4.3.10.4 Arterial switch operation The risk of pregnancy seems low in these patients with good clinical condition pre-pregnancy and preserved ventricular function. Women with a dilated neo-aorta will require closer surveillance. Although this is now the most common operation for TGA, few data are available on pregnancy outcomes.

4.3.11 Congenitally corrected transposition of the great arteries 4.3.11.1 Maternal risk In patients with congenitally corrected TGA (also called AV and ventriculoarterial discordance) risk depends on functional status, ventricular function, and the presence of arrhythmias and associated lesions (such as a VSD and pulmonary valve stenosis). Complications include arrhythmias and HF (WHO risk class III). These patients are also predisposed to developing AV block. Some 10% of patients have an irreversible decline in RV function.148,156 Patients in New York Heart Association (NYHA) classes III or IV, with ventricular dysfunction [ejection fraction (EF) <40%], or severe TR should be counselled against pregnancy.

4.3.11.2 Obstetric and offspring risk The rate of foetal loss is increased, especially if there is cyanosis.

4.3.11.3 Management For follow-up, it is recommended that patients have frequent echo surveillance of systemic RV function (every 4–8 weeks) and assessment of symptoms and rhythm. Downloaded from academic.oup.com/eurheartj/article/39/34/3165/5078465 by guest on 07 October 2026