Pulmonary Hypertension and CHD

Female patient listening to doctor

What is it?

High blood pressure in the arteries that supply the lungs is called pulmonary hypertension (PH) or pulmonary arterial hypertension (PAH). The blood pressure measured by a cuff on your arm isn't directly related to the pressure in your lungs. The blood vessels that supply the lungs constrict and their walls thicken, so they can't carry as much blood. As in a kinked garden hose, pressure builds up and backs up. The heart works harder, trying to force the blood through. If the pressure is high enough, eventually the heart can't keep up, and less blood can circulate through the lungs to pick up oxygen. Patients then become tired, dizzy and short of breath.

PH - Causes and Repairs

If a pre-existing disease triggered the PH, doctors call it secondary pulmonary hypertension. That's because it's secondary to another problem, such as a left heart or lung disorder. However, congenital heart disease can cause PH that's similar to PH when the cause isn't known, i.e., idiopathic or unexplained pulmonary arterial hypertension. In this case, the PAH is considered pulmonary arterial hypertension associated with congenital heart disease, such as associated with a VSD or ASD (either repaired or unrepaired). The problem is due to scarring in the small arteries in the lung.

It's important to repair congenital heart problems (when possible) before permanent pulmonary hypertensive changes develop. Intracardiac left-to-right shunts (such as a ventricular or atrial septal defect, a hole in the wall between the two ventricles or atria) can cause too much blood flow through the lungs. This situation is sometimes called Eisenmenger complex. In the past, the holes can't be repaired due to increased stress on the heart produced by the high pressures in the scarred lung blood vessels. This situation is changing with the availability of new medicines. Heart valve conditions, such as mitral stenosis (a narrowing of the mitral valve), can also cause secondary PH. Fixing the valve usually reverses the PH.

Pulmonary Hypertension in Children

Some children with congenital heart disease have high blood pressure in their lungs. This is called pulmonary hypertension. Surgical repair of a heart defect will often lead to improvement and normal blood pressure in the lungs. In other cases the pressure may remain higher than normal, making it harder for the heart to pump. In rare cases the pressure may be high enough that surgery becomes too risky.

Oxygen or medications may be used to try to relax the blood vessels and reduce the blood pressure in the lungs after surgery. These treatments may also be used if surgery isn't possible. 

Cyanosis due to Pulmonary Hypertension with Congenital Heart Disease

When there is a communication between the right and left heart (ASD, VSD and atrioventricular septal defect) or circulations (PDA), blood goes from the low oxygen chamber or vessel to the high oxygen chamber or vessel. An example would be shunting from the right atrium to the left atrium in patients with atrial septal defect (ASD) and pulmonary arterial hypertension. The patient who was once acyanotic becomes cyanotic. One way the body compensates is to increase the ability of the blood to carry oxygen by increasing the number of red blood cells, a condition called secondary erythrocytosis, sometimes referred to as polycythemia. It is now recognized that removing blood with a procedure called phlebotomy is only rarely needed. Symptoms that may prompt a doctor to recommend it are headaches, excessive fatigue and worsening exercise tolerance. Before this is performed, it is important that the doctors tests for iron deficiency. Phlebotomy is preferably performed in a center with experience in adult congenital disease as special precautions are required.

Treating Pulmonary Hypertension

Once pulmonary hypertension has been diagnosed, often more medical therapy is needed. You'll require regular follow-up with a cardiologist or pulmonologist trained in caring for patients with this condition.

As long as the underlying disease exists, it will keep causing PAH. Once you have PAH (especially if you've had it for some time), curing the disease that caused it may not make the high blood pressure go away. In that case, the PAH will usually need special treatment. While PH is a serious illness, treatments are available. You may be treated with oxygen, agents to help your heart pump better, diuretics, anticoagulants (*blood thinners) and medications to lower your PAH and reverse the scarring in the blood vessels in the lung. Sometimes lung transplants also are done for PAH, but in congenital heart disease, usually heart and lung transplantation is required.

Physical Activity and Pulmonary Hypertension

A diagnosis of PAH doesn't necessarily mean you can't have an active, fulfilling life. Still, you should consider certain measures and precautions. PAH is a lifelong illness that can be made worse by a variety of factors, such as smoking or traveling to high altitude.

Once PAH has been diagnosed, you must seek advice about physical activity. If you have PAH, you should be as active as physically possible. Strenuous physical activity; however is associated with serious increases in pulmonary artery pressure. Therefore, avoid isometric exercises and activities like heavy lifting, or bench press that can produce dangerous symptoms, such as chest pain or dizziness. A supervised cardiopulmonary rehabilitation program may help promote conditioning. Many patients with PAH report having "good and bad days." If you need to rest, do so.


Most medications often used to treat coexistent illnesses are safe for people with PAH. Still, care should be taken with prescription medications and over-the-counter drugs. Any anesthetics or sedatives can be very hazardous; ask your physician which medications are safe. Discuss the use of decongestant cold medicines with your doctor.

Pregnancy and Contraception

Pregnancy is not recommended in women with PH. The changes associated with pregnancy and delivery produce changes that can seriously endanger the life of the mother and baby. Therefore it is important for women with PH to use a more permanent but safe form of contraception. Because estrogen can aggravate PH, it's important to avoid any contraception containing estrogen. Progesterone forms of contraception are preferable. It is generally recommended that women with PH have tubal ligation or use the Mirena IUD. See the section on Birth Control and Pregnancy for more information.

Additional Precautions

Additional precautions are often taken with PAH patients. These include supplemental oxygen during air travel, antibiotic therapy for significant respiratory tract infections, pneumococcal pneumonia vaccine and yearly flu vaccines (since pneumonia can be very serious with PH patients). Also avoid conditions in which the ambient oxygen concentration may be decreased, such as high altitude and travel in unpressurized airplane cabins. Before starting an exercise program, ask your physician what activities are appropriate for you. Finally, if you've begun medical treatment for your PAH, stopping any of your medicines without your physician's approval can be extremely dangerous. Medical therapy has significantly improved the outlook for most patients with pulmonary hypertension, but it doesn't "cure" it. Don't stop medical therapies unless your physician (trained in caring for PAH patients) recommends doing so.

Preventing Endocarditis

People with PH and certain underlying congenital heart defects may need antibiotics before certain dental procedures to prevent endocarditis. As the endocarditis recommendations have changed, please discuss with your cardiologist.

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* Some medications are commonly called blood thinners because they can help reduce a blood clot from forming. There are two main types of blood thinners that patients commonly take: anticoagulants such as warfarin, dabigatran (Eliquis) and rivaroxaban (Xarelto), and antiplatelet drugs such as aspirin or clopidogrel. Each type of medication has a specific function to prevent a blood clot from forming or causing a blocked blood vessel, heart attack or stroke.