General Pregnancy Guidelines on Common Defects
IMPORTANT! Anyone with congenital heart disease, repaired or non-repaired, should consult with their cardiologist prior to becoming pregnant to review the risks.
Anomalous Pulmonary Venous Return, Total
Pregnancy is considered to be high-risk for individuals with cyanotic heart diseases such as unrepaired Total Anomalous Pulmonary Venous Return or with pulmonary vein stenosis after repair and is not recommended for individuals with severe forms. There may be a danger of the formation of blood clots, causing strokes in extreme cases, as a woman's blood coagulates more easily during pregnancy. Pulmonary vein stenosis can result in severe pulmonary hypertension. This may lead to further complications that pose risks to both mother and child.
If the patient chooses to proceed with pregnancy, careful monitoring by health professionals is essential. Minimal activity and bed rest are usually recommended, and treatment with anticoagulants and oxygen therapy may be prescribed. Cesarean section may be selected as the safest form of delivery.
This defect can be inherited as a dominant trait in many families with, theoretically, 50% of a patient's offspring having the anomaly, though variation in its expression reduces the incidence to somewhat less than this.
Aortic Valve Stenosis
While women with mild aortic stenosis may be able to bear children without unusual difficulty, pregnancy is not recommended for those with severe forms of this defect. All women with obstructive (stenotic) valve lesions should be evaluated by an ACHD Center before entering pregnancy. Also, it is important that the stenosis be repaired before pregnancy occurs because patients with mild or no symptoms before conception may show serious and even life-threatening effects as pregnancy progresses, especially during the second and third trimester. Women with prosthetic aortic valves should be aware of the risk of warfarin for the fetus. Warfarin has been demonstrated to cause birth defects.
Aortopulmonary Window
Aortopulmonary windows which were successfully treated in childhood without the development of other symptoms pose no significant problems for pregnancy. However, pregnancy is dangerous for patients with aortopulmonary windows, with significant risks for both mothers and their unborn children.
Atrial Septal Defect
Women with atrial septal defects are usually able to bear children without difficulty. It is common that ASDs are first diagnosed during pregnancy because of the development of heart murmurs or other symptoms, such as breathlessness during exertion (dyspnea).
It is recommended that ASDs be repaired before pregnancy to avoid possible complications caused by the formation of blood clots (embolisms), which may cause strokes, or heart failure.
Atrioventricular Canal Defect
Women with repaired AVC may be able to bear children without significant risk, as long as there is normal mitral valve function. Women with mitral insufficiency or stenosis should be carefully evaluated to determine their risk for pregnancy. Women with mitral insufficiency/stenosis can be high risk for pregnancy.
Coarctation of the Aorta
Pregnancy is considered to be high-risk for individuals with this defect. The increased blood supply and cardiac output may cause upper body hypertension, which heightens the risks of heart failure, rupture of the aorta, and stroke.
Even those women who have had the repair operation for this defect should be closely monitored during pregnancy because of the risks of aneurysms forming at the point of repair, enlargement of the aorta, and other symptoms. Those patients whose coarctation was repaired with a patch are more susceptible to aneurysms than those who received a stent or balloon angioplasty.
Ebstein's Anomaly
Pregnancy is considered to be high-risk for individuals with cyanotic heart disease such as Ebstein's and is not recommended for individuals with severe forms. There may be a danger of the formation of blood clots, causing strokes in extreme cases, as a woman's blood coagulates more easily during pregnancy.
Other changes in circulation may increase right to left shunting in untreated cases, which reduces even further the oxygen supply to the body tissues. This may lead to further complications that pose risks to both mother and child. Babies born to women with cyanosis are usually lower in weight than normal, and there is an increased risk of miscarriage and premature births.
If the patient chooses to proceed with pregnancy, careful monitoring by health professionals is essential. Minimal activity and bed rest are usually recommended, and treatment with anticoagulants and oxygen therapy may be prescribed. Cesarean section may be selected as the safest form of delivery.
The prospects for successful pregnancy are reduced when arrhythmias, right-sided congestive heart failure, and/or cyanosis are present. However, untreated Ebstein's patients with mild or absent symptoms and those who have been treated successfully often have successful pregnancies.
Eisenmenger Complex
Pregnancy is considered to be very high-risk for individuals with Eisenmenger Complex and is to be avoided. Mortality rates for mothers with this disease are as high as 30-50%. If there is a sudden drop in blood pressure or blood loss during pregnancy, sudden death may occur. In addition, there is a heightened risk of premature births, miscarriages, and stillbirths.
After the first trimester, changes in circulation will cause cyanosis (blueness caused by low oxygen saturation in the arterial blood supply) and an increase in the right to left shunting of blood through the ventricular septal defect. These problems will become more serious during labor and may become life-threatening.
If the patient chooses to proceed with pregnancy against medical advice, careful monitoring by health professionals is essential. The risk of fetal demise is significant. Minimal activity and bed rest are usually recommended, and treatment with anticoagulants and oxygen therapy may be prescribed. Cesarean section may be selected as the safest form of delivery. Reduced activity and careful monitoring for a couple of weeks after giving birth are necessary to guard against sudden death.
Hypertrophic Cardiomyopathy
Most forms of Hypertrophic Cardiomyopathy (HCM) are inherited, which should be considered by the prospective mother. Pregnancy is usually safe for women with this defect. The increased blood supply that occurs is actually beneficial. However, sudden changes in cardiac function during labor and delivery make close monitoring necessary. Patients for whom calcium-blockers and beta-blockers have been prescribed before conception should continue to take these medications.
Marfan's Syndrome
Marfan syndrome is an autosomal dominant condition, which will be inherited from a parent in 65-75% of cases. This needs to be considered by the prospective parents and consultation from an ACHD Center should be recommended. Genetic counseling may be offered at ACHD Centers. Pregnancy is considered high risk for Marfan patients because of the elevated blood pressure and blood volume and is not recommended for individuals with an aortic root that exceeds 40mm in diameter. Aortic rupture may occur during pregnancy, or as its consequence for up to 6 months after delivery. A good resource for patients and providers is the National Marfan Foundation's web site at www.marfan.org
Mitral Stenosis
Pregnancy is not recommended for individuals with this defect, unless the stenosis and its symptoms are extremely mild. There is a significant risk of heart failure, atrial fibrillation (rapid and erratic heartbeat), and the formation of blood clots, especially during the later stages of pregnancy. If a woman with mitral stenosis chooses to bear children, it is important that she be carefully tested before conception and monitored during gestation and for a time after childbirth. Any obstructive valvular lesion should be carefully monitored through pregnancy and consultation with an Adult Congenital Heart Center should occur.
Patent Ductus Arteriosus
Small PDAs, and those which were successfully treated in childhood without the development of other symptoms, pose no significant problems for pregnancy. There is the risk of heart failure in women with substantial left to right shunts and the enlargement of the left heart. These patients will require careful monitoring during pregnancy and immediately thereafter. It is common for antibiotics to be administered in order to prevent inflammation of the heart's interior lining (bacterial endocarditis) at the time of delivery.
Pregnancy can be dangerous for patients with large untreated PDAs, with high mortality rates for both mothers and their unborn children.
Pulmonary Atresia
The risks inherent in pregnancy for the mother with pulmonary atresia with intact ventricular septum depend on her surgical history and current symptoms. Pregnancy is considered to be high-risk for individuals with cyanosis, which may be present in the patient who received either the "two ventricle" or the "one and one half ventricle" treatments.
"Two ventricle" expectant mothers may experience right heart failure because of the increase in blood supply during pregnancy. Atrial arrhythmias may also occur. These risks are increased if there is significant pulmonary stenosis or regurgitation of the tricuspid valve.
Patients who received the "one and a half" treatment approach who are cyanotic (with resting oxygen saturations of below 85%) will be subject to enhanced risks of circulatory complications, miscarriage, and premature births.
"One Ventricle" patients who become pregnant face greater risks of blood clot formation, congestion of the veins, the development of atrial arrthymias, and a breakdown in ventricular function.
Pulmonary Stenosis
Pregnancy should present no problem for women with mild to moderate forms of pulmonary stenosis or those who have been treated surgically or with a balloon valvuloplasty. However, severe obstruction presents the risk of right heart failure and the development of atrial arrhythmias in response to the increased cardiovascular load that occurs. It is recommended that significant stenosis be treated prior to pregnancy, though valvuloplasty may be performed in the prospective mother if necessary.
Single Ventricle
Contraception with the combined pill is dangerous for women with single ventricle defects because of the risk of blood clot formation. Other birth control methods, including progestogen-only pill (POP), may be used.
The cardiovascular changes involved in pregnancy are especially risky for the single ventricle and post-Fontan patient. For the non-Fontan patient, the amount of risk depends on the degree of cyanosis, aortic valve regurgitation, and/or ventricular dysfunction present. There is also a risk of blood clot formation during pregnancy as well as hemorrhage after delivery.
Expectant Fontan patients also face significant risks, the degree depending on ventricular function and other factors. Arrhythmias may bring complications, and nearly 1/3 of the pregnancies of Tricuspid Atresia and Hypoplastic Left Heart Syndrome women will result in miscarriages.
In addition to the concerns mentioned above, the medications commonly prescribed to control arrhythmias and other problems may have adverse effects on the developing fetus. However, successful pregnancies under careful medical supervision have been reported for patients with various forms of single ventricle, both with and without the Fontan.
Tetralogy of Fallot
Pregnancy is considered to be high-risk for individuals with cyanotic heart diseases such as Tetralogy of Fallot and is not recommended for individuals with severe untreated forms. There may be a danger of the formation of blood clots, causing strokes in extreme cases, as a woman's blood coagulates more easily during pregnancy. Other changes in circulation may increase right to left shunting, which reduces even further the oxygen supply to the body tissues. This may lead to further complications that pose risks to both mother and child.
Accordingly, the untreated Tetralogy of Fallot patient should not undergo pregnancy, especially if oxygen saturations are below 85%. There is a 30% fetal mortality rate for un-operated Tetralogy of Fallot patients, and maternal mortality may be as high as 15%. However, the woman who receives an effective repair operation prior to pregnancy can expect a good chance of success. If the hemodynamics (blood pressures and saturation of oxygen and other gases) are near normal, then the level of risk will approach that of women with normal hearts.
Careful monitoring by health professionals is imperative during pregnancy. In some cases the increased blood volume may cause heart failure or the development of arrhythmias. Other potential difficulties include pulmonary valve regurgitation, right ventricular dysfunction, and right ventricular outflow tract obstruction. In some cases, minimal activity and bed rest may be recommended, and treatment with anticoagulants and oxygen therapy may be prescribed. Echocardiography is often used to monitor fetal development and Cesarean section may be selected as the safest form of delivery.
Transposition of the Great Arteries, D-Type
Patients who have received the arterial switch (Jatene) repair for TGA-D, which is comparatively new, are not expected to have significant cardiac problems during pregnancy. However, those who received the Senning or Mustard atrial switch procedures are at risk of developing right ventricular and tricuspid valve dysfunction as well as arrhythmias, such as atrial flutter. These symptoms will significantly increase the risks of pregnancy. Therefore, these patients require careful evaluation before conception and regular monitoring if they become pregnant.
Rastelli patients are also at risk for the development of arrhythmias and may experience left ventricular dysfunction. Therefore, they should be carefully evaluated before pregnancy and monitored closely during it.
Generally speaking, patients with repaired D-Type Transposition of the Great Arteries may expect to undergo pregnancy without difficulty if they have no history of arrhythmia, an adequately functioning right ventricle, and no signs of congestive heart failure. There is, however, an increased risk of prematurity or low birth weight. Fetal development will often be monitored through echocardiography (producing an echocardiogram).
Transposition of the Great Arteries, L-Type
Women with this defect should be carefully examined before pregnancy to determine the degree of risk involved. If there is evidence of significant ventricular or tricuspid valve dysfunction, then pregnancy is usually not recommended. Patients with cyanosis face an increased chance of complications.
There may be a danger of the formation of blood clots, causing strokes in extreme cases, as a woman's blood coagulates more easily during pregnancy. Other changes in circulation may increase right to left shunting in untreated cases, which reduces even further the oxygen supply to the body tissues. This may lead to further complications that pose risks to both mother and child.
Patients who have undergone surgery in which a conduit was inserted should have it checked before pregnancy. This is to ensure that it is not obstructed and is in sufficiently good shape to withstand the increase in blood flow that will occur.
If the patient chooses to proceed with pregnancy, careful monitoring by health professionals is essential. Minimal activity and bed rest are usually recommended, and treatment with anticoagulants and oxygen therapy may be prescribed. Cesarean section may be selected as the safest form of delivery.
Truncus Arteriosus
Successful pregnancies are possible after the complete surgical repair of Truncus Arteriosus. Careful testing before pregnancy and close medical monitoring throughout are necessary, however.
It should be borne in mind that there is a high incidence of genetic abnormalities in patients with this defect (involving chromosome 22q11). Therefore, genetic testing is recommended for all prospective mothers.
Ventricular Septal Defect
Women with small to moderate sized VSDs and those with successfully closed defects usually have no difficulty bearing children. Occasionally, moderate-sized defects will cause the development of arrhythmias and/or congestive heart failure during pregnancy. Women with large VSDs and high levels of pulmonary vascular resistance, as in Eisenmenger Complex, are exposed to serious risks and should not undergo pregnancy. About 3% of the offspring of women with VSD may be expected to have some form of congenital heart disease. |
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