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............................................................................................................................................................................. clotting factors, and bleeding. Caution is therefore needed if prescribing antiplatelet therapy or LMWH. The evidence base for using advanced therapies is less developed. However, sildenafil (and other phosphodiesterase inhibitors such as tadalafil and vardenafil) is often prescribed, with the addition of prostanoids in patients who remain symptomatic.147 Care should be exercised if prescribing drugs that may lead to sudden systemic vasodilation or a risk of paradoxical air embolism (i.v. therapies). Advanced therapies for Eisenmenger patients should only be prescribed by experienced pregnancy heart teams including a PH expert. The principles guiding delivery are as per other forms of PH as above.

4.2.3 Cyanotic heart disease without pulmonary hypertension 4.2.3.1 Maternal risk Cyanotic congenital heart disease is usually repaired before pregnancy, but some balanced, inoperable, or palliated cases do reach childbearing age.148 Maternal complications (HF, thrombosis, arrhythmias, and endocarditis) occur in >_15% of cyanotic pregnant patients. Maternal outcome will be determined by the underlying condition and the ventricular function rather than the saturation level.

4.2.3.2 Foetal risk If oxygen saturation is >90%, then there is usually a better foetal outcome (10% foetal loss). If oxygen saturation is <85%, foetal growth restriction, prematurity, and foetal death are common and pregnancy should be discouraged (live birth rate of only 12%).149

4.3 Specific congenital heart defects

4.3.1 Left ventricular outflow tract obstruction The principles for managing supravalvular or subvalvular LV outflow tract obstruction are the same as those for valvular aortic stenosis (AS) (section 5). However, balloon valvuloplasty is not a therapeutic option.

4.3.2 Atrial septal defect 4.3.2.1 Maternal risk Pregnancy is well tolerated by most women with repaired atrial septal defect (ASD) (WHO risk class I). In unrepaired ASDs, thromboembolic complications have been described (5%). Atrial arrhythmias occur, especially when the ASD is unrepaired or closed at an older age.150

4.3.2.2 Obstetric and offspring risk In women with unrepaired ASD, pre-eclampsia and growth restriction may occur more frequently.

4.3.2.3 Management For a secundum defect, catheter device closure can be performed during pregnancy but is rarely indicated. If device closure is performed, antiplatelet therapy will be required. Closure for the prevention of paradoxical emboli is not indicated. In women with a residual shunt, prevention of venous stasis (compression stockings and minimizing bed rest) is important and extra care should be taken to avoid air in i.v. lines. 4.3.3 Ventricular septal defect 4.3.3.1 Maternal risk Small or repaired ventricular septal defects (VSDs) (without left heart dilatation or ventricular dysfunction) have a low-risk of complications during pregnancy (mWHO I and II).

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

4.3.3.3 Management Patients should usually be reviewed once or twice during pregnancy with surveillance for PH.

4.3.4 Atrioventricular septal defect 4.3.4.1 Maternal risk After ASD repair, pregnancy is usually well tolerated (WHO risk class II–III). However, arrhythmias and worsening atrioventricular (AV) valve regurgitation have been described. The risk of HF is low, and only exists in women with severe regurgitation or impaired ventricular function.

4.3.4.2 Obstetric and offspring risk Offspring mortality has been reported in 6% of cases, primarily due to the recurrence of congenital heart disease.

4.3.4.3 Management Follow-up is advisable at least once each trimester. This should be increased to monthly or bimonthly in patients with significant valve regurgitation or impaired ventricular function.

4.3.5 Coarctation of the aorta 4.3.5.1 Maternal risk Pregnancy is often well tolerated in women after repair of coarctation of the aorta (CoA) (WHO risk class II). In women with unrepaired CoA and those repaired who have systemic hypertension, residual CoA or aortic aneurysms have an increased risk of complications including dissection. Other risk factors include aortic dilatation and bicuspid aortic valve.

4.3.5.2 Obstetric and offspring risk An excess of hypertensive disorders, including pre-eclampsia and miscarriages, has been reported.

4.3.5.3 Management Close surveillance of BP is warranted and follow-up, at least every trimester, is indicated. Hypertension should be treated and care should be taken to avoid placental hypoperfusion in those with residual coarctation. Percutaneous intervention for re-CoA (using a covered stent) is possible during pregnancy, but should only be performed for refractory hypertension or maternal or foetal compromise.

4.3.6 Pulmonary valve and right ventricular outflow tract disease 4.3.6.1 Maternal risk Pulmonary (valve) stenosis (PS) is generally well tolerated. However, severe stenosis may result in complications including RV failure and Downloaded from academic.oup.com/eurheartj/article/39/34/3165/5078465 by guest on 07 October 2026