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Management Strategies
Preconception counseling/Contraceptive methods
Successful pregnancies are reported in women with coarctation; however, preconception risk stratification is important. The severity of the coarctation gradient, the size of the aorta, the presence of an aneurysm at the repair site, the presence of hypertension and the severity of bicuspid aortic valve disease, if present, are important issues to consider when determining an individual’s risk. There are also other cardiac characteristics, which can have an impact on outcomes (see General Considerations).
Ideally, a comprehensive cardiovascular examination should be undertaken before embarking on pregnancy. This includes a careful history and physical examination, an electrocardiogram and an echocardiogram. All women should have cardiac magnetic resonance imaging to assess the entire aorta including the repair site. Catheterization may be indicated for women requiring surgery prior to pregnancy or if there are other unaddressed hemodynamic issues.
Repair of significant coarctation should occur prior to pregnancy. Pregnancy should be postponed in women in whom blood pressure is not well controlled.
Transmission of congenital heart disease to offspring should be discussed. The risk of transmission of congenital heart disease is approximately 5-10%, compared to a background risk of approximately 1%.
A discussion about contraceptive methods is appropriate in all women with coactation. Combined oral contraceptives containing estrogen/progestin should be used with caution in women with repaired coarctation with dilated aortas, patch aneurysms or hypertension. (see Contraception)
Women treated with angiotensin converting enzyme inhibitors or angiotensin receptor blockers need to have these medications stopped prior to pregnancy. Medication use should be reviewed if a woman is contemplating pregnancy or is pregnant. The MOTHERISK website is an excellent resource.
Ante-partum Care
Coordinated care with a congenital heart disease specialist and a high-risk obstetrician should be implemented. The frequency of clinical and echocardiographic assessments during pregnancy should be determined on an individual basis.
Women with minimal coarctation gradients, normal aortic root sizes and no significant associated bicuspid aortic valve disease have the best chance for an uncomplicated pregnancy.
Treatment of hypertension is important during pregnancy. However, antihypertensive medications can exacerbate hypotension distal to the coarctation site and result in diminished placental perfusion. Optimal blood pressure targets are not defined in this population. Because of their potential role in protecting the diseased aorta, beta blockers should be considered first line agents in women with aortic dilatation.
Women should be offered fetal echocardiography at approximately 20 weeks gestation.
Labour and Delivery
Labour and delivery should be planned with a multidisciplinary team well in advance. It is important to communicate the delivery plan to the woman and to other physicians involved in her care. The best delivery plan is not useful if information is not readily available when needed.
Generally, vaginal deliveries are recommended unless there are obstetric indications for a cesarean delivery. Good pain management for labour and delivery is very important in order to minimize maternal cardiac stress and help to control blood pressure. To decrease maternal expulsive efforts during the second stage of labour, forceps or vacuum delivery is often utilized. Uterine contractions are utilized to facilitate the initial descent of the presenting part in order to decrease potential harmful complications from difficult mid cavity-assisted delivery,
The need for maternal monitoring at the time of labour and delivery is dictated the severity of the coarctation, the degree of ventricular dysfunction, and the existence of associated lesions. Invasive blood pressure monitoring may be required for women with poorly controlled blood pressure.
In general, endocarditis prophylaxis at the time of labour and delivery is not recommended in women with coarctation. However, some experts continue to administer antibiotics because they feel that the risks of adverse reactions to antibiotics are small and the risk of developing endocarditis has major health consequences.
Post-partum Care
The hemodynamic changes of pregnancy may take up to six months to normalize. Women should be seen early after pregnancy (usually within 6-8 weeks). The frequency of additional follow up visits should be dictated by the clinical status of the women.