Carefully monitor pregnant women with heart defects

Patrizia Presbitero (© Private)

Patrizia Presbitero is one of Europe’s foremost authorities on heart disease and pregnancy. She has followed hundreds of women with congenital heart defects through pregnancy and childbirth.

“Women with congenital heart defects are definitely more anxious than other pregnant women,” says Patrizia Presbitero. “Not only are they worried about their child, they also have to consider their own heart problems. Their biggest worries tend to arise after giving birth. They ask, ‘Can I handle this? Will I get to see my child grow up?’ Added to that is the fear that the child will have a heart defect too.” Nevertheless, Patrizia strongly urges her patients to have children.

Double the fears

Patrizia believes the medical profession is to blame for many of these women’s worries. She says doctors are not used to dealing with congenital heart defects, and transfer their own worries to the patient. “That’s why GPs are so important,” says Patrizia. “These women have lived with their heart disease all their lives. They are used to the symptoms, evaluate them constantly, and actually have a tendency to minimise them,” she points out.

Ignorance leads to abortions

Patrizia Presbitero works at the Humanitas Institute in Milan, Italy. She frequently sees patients who have been pregnant once or twice but were convinced by their doctor to have an abortion. “This is an unnecessary burden on the women, and can be attributed to ignorance on the part of the doctors,” says Patrizia. Another common piece of misinformation is that these women should deliver before their pregnancy has run its full term. “If, for example, a women has a mechanical valve, the doctor expects her to deliver after seven months. But that’s completely wrong. If everything is OK, most such pregnancies can, and should, go full term. We should prevent premature children whenever we can,” says the cardiologist.

Pregnancy and childbirth in women with congenital heart defects must be followed up at a hospital with an interdisciplinary GUCH team. “Unfortunately, many countries are poorly organised in this regard, particularly those in southern and eastern Europe,” says Patrizia.

Ideal: Young and planned

The vast majority of women with congenital heart defects can have children, whether their defect is a major or minor one, and regardless of whether they have undergone many operations, one, or none. “This is a direct result of surgical advances. But these hearts do bear scars, and surgeries do not fix everything. That’s why we have to watch them carefully,” says Patrizia. This means monitoring risk factors and measuring physical performance capacity – because giving birth is hard work. “There are periods in which double the normal amount of blood is circulating. Ideally, all women should be examined thoroughly before they become pregnant, but things don’t work that way in practice. We cardiologists should encourage women with congenital heart defects to get pregnant while they are young. That reduces a host of risk factors, such as high blood pressure and cholesterol, and lowers the danger of arrhythmia.”

Inheritability and foetal damage

Certain heart defects increase the risk that the foetus will be damaged or will fail to survive to birth. Examples of such defects include cyanotic states involving low oxygen supply, which can result in the foetus getting too little blood and oxygen. The risk of losing the child is reduced substantially if the hospital follows up properly.
“Most of my patients are also concerned about inheritability. We offer ECGs, and can provide good answers. After six months we have a good understanding of any foetal heart defects that are present. If the child does have a heart defect, it will rarely be the same one the mother has,” says Patrizia. She also stresses that inheritability has not been shown for some types of heart defects, although their incidence is higher in certain families.

Complications

The risk of complications during pregnancy is not necessarily proportional to the degree of severity of the woman’s heart defect. “You could say that the more complicated the name of the defect one has, the greater the danger of complications. But experience shows that heart complications can occur even when the defect is moderate,” says Patrizia. This is attributable to the major changes in blood circulation that occur during pregnancy.

The two diagnoses that probably pose the greatest risk to pregnancy are Pulmonary vascular obstructive disease and Eisenmenger's syndrome. These entail an increased risk of sudden death, particularly during the interval from childbirth to one week after giving birth. Women who have undergone a Fontan operation and those with Marfan’s syndrome are also among those with the highest risk, although such patients are few in number. Pregnant women with Marfan’s syndrome once had a high mortality rate, but major strides have been made in this area as well. Women with very large aortic roots should nevertheless avoid becoming pregnant, according to Patrizia. “Many Marfan patients must undergo surgery after pregnancy, but there are just as many follow-ups among patients who have never been pregnant. Pregnancy thus does not affect the disease,” she says.

Surgery on pregnant women

Mitral stenosis and aortic stenosis are other diagnoses that can pose challenges. Mortality during pregnancy for women with aortic stenosis was once 7%. Today this figure is nearly zero. But 4% of pregnant women with aortic stenosis must undergo surgical treatment during the course of their pregnancy (valvoplasty). Of those with severe cases, 41% must undergo an operation after giving birth. “Interventions are common among patients with stenosis, and we achieve good results,” assures Patrizia. The exception is coronary stenosis. “Here we see mixed results, mainly because the patients come in too late. This emphasises the importance of following up all the way along.”