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Saxena et al. Vessel Plus 2022;6:15 https://dx.doi.org/10.20517/2574-1209.2021.96 Page 13 of 17

aorta are at risk of aortic dissection and pregnancy is contraindicated if ascending aorta diameter is
> 50 mm.

Activities should be restricted during pregnancy. Severely symptomatic patients not responding to medical
therapy may have to undergo balloon valvuloplasty to relieve their symptoms. Elective caesarean delivery
under general anesthesia is preferred in severe symptomatic aortic stenosis patients, while vaginal delivery is
favored in asymptomatic severe and in non-severe cases.

Coarctation of aorta
Women with unrepaired coarctation of aorta (CoA), and those with residual lesions (persistent
hypertension, re-coarctation, aortic aneurysms, aortopathy, and aortic valve disease) have an increased risk
of dissection, rupture of aorta, and cerebral aneurysms during pregnancy and delivery. The most common
complication even in women with well repaired CoA is hypertension and is seen in approximately 30% .
[51]
Therefore, these patients should be under close supervision throughout pregnancy for high BP.

In very severe cases with refractory hypertension and maternal or fetal compromise during pregnancy,
balloon dilatation can be considered, but, due to high risk of aortic dissection, covered stent may be
required.

Post-Fontan surgery
Women who have undergone Fontan surgery come under mWHO risk category III/IV due to unique
hemodynamic abnormalities after this surgery. Even women with ideal Fontan circuit (mWHO III) are at
increased risk of fertility issues, atrial arrhythmias, and worsening of functional class but may tolerate
pregnancy. Occurrence of arrhythmias and thrombosis of Fontan circuit due to prothrombotic pregnancy
state are major issues which can jeopardize fetal and maternal outcome. In mothers with a Fontan
circulation, the offspring risk includes premature birth, small for gestational age, and fetal death in up to
50% of cases. Those with resting oxygen saturation < 85%, depressed ventricular function, moderate to
severe atrio-ventricular valve regurgitation, arrhythmias, and protein losing enteropathy are at particularly
[1]
high risk of maternal and fetal adverse outcomes and are best counseled against pregnancy .

Generally, vaginal delivery is possible and is preferred. Therapeutic anticoagulation should be considered
since these patients are at significant risk of thromboembolism. Atrial arrhythmias need to be treated
promptly and often need electrical cardioversion.

Repaired transposition of great arteries
The type of surgical correction and residual abnormalities are the major determinant of risks during
pregnancy. Women with atrial switch operation (Mustard/Senning repair) often have systemic right
ventricular dysfunction, tricuspid regurgitation (TR), and brady/tachyarrhythmias which often worsen
during pregnancy . Failure to increase cardiac output and heart rate adequately during pregnancy may
[52]
occur due to chronological incompetence and rigid conduits, baffles. Generally, pregnancy is tolerated well
in those with no residual defects, normal ventricular function, and in NYHA Class I, but patients with
severe right ventricular dysfunction or severe tricuspid regurgitation are advised against pregnancy .
[1]
Vaginal delivery is recommended in asymptomatic patients with good ventricular function. The risk of
pregnancy after arterial switch operation has not been adequately studied but is likely to be lower than after
atrial switch operation.