Congenital heart conditions and pregnancy

Congenital heart defects are the most common heart problems that
affect women of childbearing age. These include shunt lesions,
obstructive lesions, complex lesions and cyanotic heart disease.

Shunt lesions

Shunt lesions are the simplest and most common congenital heart

defects. Shunts include atrial septal defect (ASD), which is a hole
between the upper chambers of the heart; ventricular septal defect
(VSD), which is a hole between the lower chambers of the heart;
and patent ductus arteriosus (PDA), which means there is abnormal
blood flow between the aorta and pulmonary artery. If the hole is
large, a fair amount of blood from the left side of the heart will flow
back into the right side of the heart. The blood gets pumped back to
the lungs again and causes strain on the heart. This can lead to an
enlarged heart, abnormal heart rhythms and increased pressure in the
lungs (pulmonary hypertension). Pulmonary hypertension, when
severe, can cause the blood flow across the shunt to move in reverse.
This can cause low levels of oxygen in the blood (cyanosis). In such
cases, pregnancy is not recommended due to the high risk of the
mother dying.

Obstructive Lesions

Obstructive lesions reduce the amount of blood flow to the heart and
the body’s major blood vessels. One such lesion, aortic
coarctation is a narrowing in the descending aorta, which is the
largest artery in the body. Aortic coarctation can cause a pregnant
woman to have high blood pressure. The condition can also keep the
placenta (the collection of blood vessels that supplies the baby with
blood) from getting enough blood. Depending on how severe the
narrowing is, you may need a procedure before or during pregnancy
to keep you and the baby safe during pregnancy.

Complex lesions
Complex lesions include transposition of the great arteries. This
means the aorta and pulmonary arteries are attached to the wrong
ventricles (bottom chambers of the heart). Surgery to repair the
problem can cause problems with the heart chambers, especially if the
right ventricle pumps blood out to the body (this is usually the job of
the left ventricle). In this case, the problem can cause heart failure and
leaky heart valves, and the conditions can become worse during
pregnancy. If you have this condition, you will need to be closely
followed during pregnancy.

Cyanotic heart disease includes tetralogy of Fallot. This is a

condition that includes a VSD, narrowing of the pulmonary valve and
abnormal configuration of the aorta. Treatment usually keeps cyanosis
from recurring. However, the repair can cause a leaky pulmonary
valve, and that problem can lead to heart failure and heart rhythm
disturbances. If you have a leaky pulmonary valve, you may need to
have it corrected before you become pregnant.

In general, most women with congenital heart defects, especially

those who have had corrective surgeries, can safely become
pregnant. However, the outcome of the pregnancy and risk of
complications depends on the type of heart defect you have, how
severe your symptoms are, and whether you have heart muscle
dysfunction, heart rhythm disturbances or pulmonary hypertension
with related lung disease. Your pregnancy can also be affected if you
have had particular types of heart surgery.
Valve disease and pregnancy

Aortic valve stenosis means the aortic valve (the valve between the
left ventricle and the aorta) is narrowed or stiff. If the narrowing is
severe, the heart has to work harder to pump the increased blood
volume out of the narrowed valve. This, in turn, can cause the left
ventricle (the major pumping chamber of the heart to enlarge – a
condition called hypertrophy). Over time, symptoms of heart
failure can occur or become worse and increase the risk of long-term
complications for the mother.
One common cause of aortic valve stenosis is bicuspid aortic valve
disease. This is a congenital heart condition in which there are only
two leaflets (also called cusps), instead of the normal three leaflets
inside the valve. The leaflets open and close to keep blood flowing in
the right direction and prevent backflow. Without the third leaflet, the
valve can become narrowed or stiff.

Women with bicuspid aortic valve disease or any type of aortic valve
stenosis need to be evaluated by a cardiologist before planning a
pregnancy. In some cases, surgery is recommended to correct the
valve before pregnancy.

Mitral valve stenosis means the mitral valve (the valve between the
left atrium and left ventricle) is narrowed. This condition is often
caused by rheumatic fever.

The increased blood volume and increased heart rate that occur
during pregnancy can make symptoms of mitral stenosis get worse.
The left atrium can become bigger and cause a rapid, irregular heart
rhythm called atrial fibrillation. In addition, the problem can
cause heart failure symptoms (shortness of breath, irregular heart
beat, fatigue and swelling/edema). This can increase the risk to the
mother. If you have mitral valve stenosis, you may need to take
medications while you are pregnant. Your doctor may also
recommend an catheter-based procedure, called percutaneous
valvuloplasty, to correct the narrowed valve while you are pregnant. It
is important to have mitral stenosis evaluated before you become
pregnant. In some cases, surgery or valvuloplasty to correct the valve
will be recommended before pregnancy.

Mitral valve prolapse is a common condition that usually doesn’t

cause symptoms or require treatment. Most patients with mitral valve
prolapse tolerate pregnancy well. If the prolapse causes a severe
leak, you may need treatment before you become pregnant. Be sure
to talk to your doctor if you plan to become pregnant and follow any
recommendations.
Pregnancy in women with prosthetic (artificial) valves
Women who have artificial heart valves may experience complications
during pregnancy because:

 Women who have an artificial heart valve need to take lifelong

anticoagulant medication, and certain anticoagulant medications
can be harmful to the baby. There is controversy about which
anticoagulant medication regimen is best during pregnancy.*
 During pregnancy, there is an increased risk of blood clots.

*Use of warfarin, heparin, aspirin, and combinations of these

anticoagulant medications have been suggested and compared. The
most recent recommendations from the European Heart Association
are to use heparin during the first trimester, followed by warfarin up to
the 36th week of pregnancy, and subsequent replacement with
heparin until delivery OR to use oral anticoagulation medication
throughout pregnancy, until the 36th week, followed by heparin until
delivery.

The use of warfarin is less harmful if the dose is kept to less than 5
mg. In addition, other specialists have recommended the addition of
low-dose aspirin to treat women who are at high risk.

If you have a prosthetic valve and are taking an anticoagulant

medication, it is very important to be evaluated by a cardiologist
before planning a pregnancy. The cardiologist will talk to you about
your potential risks and determine the best anticoagulant therapy
routine for you.

In addition, ask your doctor what precautions you should continue to

follow to prevent endocarditis.
Aorta Disease and pregnancy

Women who have conditions that affect the aorta, such as aortic
aneurysm, dilated aorta, or connective tissue disorders such
as Marfan syndrome, are at increased risk during pregnancy.
Pressure in the aorta increases during pregnancy and when bearing
down during labor and delivery. This extra pressure increases the risk
of an aortic dissection or rupture, which can be life-threatening.

It is very important for women who have aorta disease to be evaluated

by a cardiologist before planning a pregnancy. A thorough evaluation
of your condition will provide the physician with information about the
potential risks of pregnancy. It is also important to note that some
conditions, such as Marfan syndrome, are genetic and can be passed
down to children, so genetic counseling may be recommended.
After you become pregnant

Congratulations on your pregnancy! During pregnancy, it’s important

to:

 Continue following a heart-healthy diet.

 Exercise regularly, as recommended by your cardiologist.
 Quit smoking!

In addition to keeping your follow-up appointments with your obstetric

provider throughout pregnancy, schedule regular follow-up visits with
your cardiologist and follow the recommendations carefully. Your
cardiologist can evaluate your heart condition throughout your
pregnancy so symptoms and/or potential complications can be
detected and treated early. This will help ensure a safe outcome for
you and your baby.

Some conditions may require a team approach that involves you and
your obstetrician, cardiologist, anesthesiologist and pediatrician.
Depending on your heart condition, special arrangements may be
needed for labor and delivery.
Cardiovascular disorders that may develop during pregnancy

Peripartum cardiomyopathy

Peripartum cardiomyopathy is a rare condition. It is when heart

failure develops in the last month of pregnancy or within five months
after delivery. The cause of peripartum cardiomyopathy remains
unknown. Certain patients, including those with multiple pregnancies
and those of African descent, are at greatest risk. Women with
peripartum cardiomyopathy have symptoms of heart failure. After
pregnancy, the heart usually returns to its normal size and function.
But, some women continue to have poor left ventricular function and
symptoms. Women with peripartum cardiomyopathy have an
increased risk of complications during future pregnancies, especially if
the heart dysfunction continues.

Hypertension (high blood pressure)

About 6% to 8% of women develop high blood pressure, also called

hypertension, during pregnancy. This is called pregnancy-induced
hypertension (PIH) and is related to preeclampsia, toxemia, or
toxemia of pregnancy. Symptoms of PIH include high blood pressure,
swelling due to fluid retention, and protein in the urine. Pregnancy-
induced hypertension can be harmful to the mother and the baby. To
learn more about who is at risk for PIH, symptoms of PIH, and how
PIH is diagnosed and treated, click on the following links:

 Cleveland Clinic - Pregnancy-Induced Hypertension

 Cleveland Clinic - Preeclampsia and Eclampsia
 American Heart Association - Pregnancy and High Blood
Pressure

Myocardial infarction

Heart attack (myocardial infarction) is fortunately a very rare but

potentially deadly complication that can occur during pregnancy or
during the first few weeks afterwards. A heart attack can be caused by
many things. Patients with coronary artery disease (“hardening of the
arteries”) can have a myocardial infarction if the plaque inside their
arteries ruptures. This problem is becoming more common, since
many women wait until later in life to become pregnant. Other causes
of a heart attack include a spontaneous blood clot inside a coronary
vessel (because pregnancy increases the risk of blood clots) and
coronary dissection (a weakening of the vessel wall that leads to a
spontaneous tear and clotting). If you have a heart attack, it is critical
to get emergency help. Treatment will be focused on ensuring your
survival.

Heart Murmur

Sometimes, the increase in blood volume during pregnancy can cause

a heart murmur (an abnormal “swishing” sound). In most cases, the
murmur is harmless. But in rare cases, it could mean there’s a
problem with a heart valve. Your doctor can evaluate your condition
and determine the cause of the murmur.

Arrhythmias and pregnancy

Abnormal heartbeats (arrhythmias) during pregnancy are common.

Women who have never had an arrhythmia or heart problem may first
develop an arrhythmia during pregnancy. When an arrhythmia
develops during pregnancy, it can be a sign of a heart condition you
didn’t know you had. Most of the time, the arrhythmia causes little in
the way of symptoms and does not require treatment. If you have
symptoms, your doctor may order tests to determine the type
arrhythmia you have and attempt to determine its cause.