While in the past patients who had undergone coarctation repair were considered to be cured, it is now recognised that even a well-repaired CoA is frequently associated with premature morbidity and mortality. Surprisingly, despite improvements in repair techniques, better medical management of hypertension, comorbidity surveillance for cardiovascular disease and monitoring for aortic complications, this seems to have had a limited impact on late mortality. In early studies, reporting in 1989, survival at 30 years was 72 %; a more recent study by Brown et al. reporting in 2013 showed an almost identical survival of 74 % during long-term follow-up [27, 28]. Older age at the time of the initial repair (>20 years) has been associated with decreased survival, but patients younger than 5 years at the time of the initial operation had the greatest risk of reoperation, while patients older than 9 years had a greater prevalence of residual hypertension [28]. As a consequence, lifelong follow-up care after CoA treatment is required, ideally on an annual basis. Patients with a repaired CoA do not have impaired fertility. The largest study to date on the outcome of pregnancy after repair of aortic coarctation showed that, overall, maternal and neonatal outcome of pregnancy is excellent and without major cardiovascular complications. On the other hand, the rate of miscarriage and preeclampsia was higher than experienced by the general population [29]. Women planning a family should be thoroughly investigated before conception to exclude late problems including systemic hypertension, residual coarctation and re-coarctation and aortic aneurysm formation. Also the physician should investigate for potential clinical signs of premature coronary and cerebrovascular disease [30].

Hypertension is seen in more than 30 % of cases after CoA repair in early childhood and in the absence of restenosis [31]. In general, pregnancy is tolerated well by mother and fetus as long as hypertension is well controlled. Ambulatory blood pressure monitoring (in the right arm) and exercise testing should be performed before pregnancy, and regular blood pressure monitoring is important throughout pregnancy due to the risk of pregnancy-induced hypertension. It is crucial to treat persistent resting hypertension and it appears reasonable to treat women normotensive at rest, but whose systolic blood pressure exceeds 200 mmHg at low levels of exercise on treadmill testing, especially in pregnancy. This is because pregnancy – with its increase in blood volume and cardiac output – can be regarded as a state of mild to moderate exercise [32, 33]. Women with unrecognised and untreated hypertension are at risk of developing superimposed preeclampsia with fetal growth restriction, placental abruption, congestive heart failure and acute renal failure [34], all of which may require preterm delivery. In every day clinical practice, blood pressure should be measured in both arms. If the left subclavian artery has been used as a part of the initial repair, then the left arm blood pressure may be falsely low. Blood pressure monitoring should be placed on the right arm [15]. Antihypertensive treatment in pregnancy generally consists of beta-blocker therapy while angiotensin-converting enzyme (ACE) inhibitors and angiotensin-receptor blockers are contraindicated [35].

Residual coarctation and re-coarctation can be seen after all known surgical techniques (end-to-end anastomosis, prosthetic patch aortoplasty, subclavian flap aortoplasty) with the various incidence rates from 5 to 24 % [36–39]. In re-coarctation gradients are usually small and pregnancy is well tolerated. Sometimes it can be difficult to distinguish between restenosis and enhanced aortic stiffness. In this setting analysing diastolic flow patterns at the isthmus and verifying high flow during diastole, reflecting diastolic runoff, is the most important echocardiographic sign of restenosis. Further, women who have a residual coarctation or re-coarctation are at increased risk of developing systemic hypertension. On the other hand, if re-coarctation is significant, blood pressure in the lower part of the body can be reduced and endanger the fetus. Regular monitoring of mothers’ blood pressure and fetal growth is mandatory. Depending on the severity, re-coarctation should be managed in the same as native coarctation [20, 32].

Late aneurysm formation has been reported after all types of CoA repair, but it occurs most frequently after Dacron patch repair [27, 40]. Dacron patch aortoplasty gained popularity in the mid-1960s because of its excellent haemodynamic result and because it avoided sacrifice of intercostal arteries and minimised restenosis. Due to the high incidence of late aneurysm formation, this technique has been abandoned. Nevertheless, we continue to see adult women of child-bearing age after these procedures highlighting the need to obtain information about previous operation techniques in other to evaluate potential risk factors before pregnancy [41, 42]. Furthermore, late aneurysm formation in the ascending aorta, proximal to the surgical repair site, has also been noted as a late complication [43]. Aneurysm formation can lead to aortic rupture and sudden death. Pregnancy appears to increase the risk of aneurysm rupture. This might be due to hemodynamic, hormonal and vascular changes [44]. The treatment decision depends on various factors, including the size of the aneurysm, gestational age and type of procedure. Current ESC Guidelines on the management of cardiovascular diseases during pregnancy state that depending of the aortic diameter, patients should be monitored by echocardiography every 4–12 weeks while maintaining strict blood pressure control. Pre-pregnancy surgical treatment is recommended when the ascending aorta is ≥45 mm. During pregnancy prophylactic surgery should be considered if the aortic diameter is ≥50 mm and increasing rapidly. When progressive dilatation occurs during pregnancy, before the fetus is viable, aortic repair with the fetus in utero can be considered. When the fetus is viable, caesarean delivery followed directly by aortic surgery is recommended [45]. Caesarean delivery should be considered when the aortic diameter exceeds 45 mm. A cardiac operation with cardiopulmonary bypass of a gravid patient still remains a high-risk procedure, especially for the fetus. Maternal mortality is similar to that of non-pregnant women but fetal mortality is 16–33 % [46]. In the modern era, endovascular repair with stent graft could be a therapeutic alternative (minimally invasive approach) in pregnant patients with late thoracic aortic aneurysms after surgical repair of aortic coarctation [47–49]. However, to our knowledge, there are no literature reports of endovascular stent grafting for aneurysms in patients during pregnancy.

Early discussion with the cardiologist, obstetrician and anaesthetist will facilitate optimal planning for the management of pregnancy and delivery. It appears that vaginal delivery with an effective epidural is the preferred method. It causes fewer and less dramatic changes in haemodynamic parameters and is associated with lower risk of maternal complications such as haemorrhage, infection and thrombosis. Vaginal delivery has also shown neonatal benefits in terms of a later delivery and greater birth weight [26]. During delivery, indication for antibiotic prophylaxis is controversial. We do not generally advocate antibiotic prophylaxis at the time of delivery. However, the only two cases of maternal death with coarctation and pregnancy in the UK mortality statistics from 1997 to 1999 were associated with endocarditis [22].

In the puerperium other antihypertensive drugs, contraindicated during pregnancy, can be considered. Caution is advised with beta-blocker therapy in mothers who are breastfeeding since they can cause neonatal bradycardia and the excretion of particular beta-blockers should be discussed with the obstetricians. In patients with severe aortic coarctation, repair should be planned early after delivery to avoid unnecessary complications.