Pregnancy is often described as one of the most transformative periods in a woman’s life. However, for women living with connective tissue diseases (CTDs) such as lupus, scleroderma, or mixed connective tissue disease (MCTD), pregnancy can present additional challenges—especially when complicated by pulmonary hypertension (PH). CTD-associated pulmonary hypertension (CTD-PH) is a serious condition that affects the lungs and heart, and pregnancy, in these cases, requires careful planning and management.
What Is CTD-Associated Pulmonary Hypertension?
Pulmonary hypertension is a type of high blood pressure that affects the arteries in the lungs and the right side of the heart. When a woman has CTD-associated pulmonary hypertension (CTD-PH), the arteries in her lungs become narrow or blocked, making it harder for blood to flow through. As a result, the right side of the heart has to work harder to pump blood, which can lead to heart failure over time. CTD-PH is most commonly associated with connective tissue diseases like:- Systemic Sclerosis (Scleroderma) – A condition that causes thickening and hardening of the skin and connective tissues, which can affect the lungs.
- Systemic Lupus Erythematosus (SLE) – An autoimmune disease that can affect multiple organs, including the lungs and heart.
- Mixed Connective Tissue Disease (MCTD) – A rare autoimmune disorder with features of scleroderma, lupus, and polymyositis.
Why Pregnancy and CTD-PH Is High Risk
Pregnancy causes significant changes in a woman’s cardiovascular system. The body’s blood volume increases, and the heart has to work harder to pump the extra blood. For a healthy woman, this is generally not a problem. However, for a woman with CTD-PH, the added strain on the heart and lungs can be dangerous.Why is it risky?
- Increased cardiovascular demand – During pregnancy, blood volume can increase by up to 50%, placing additional strain on the already overworked heart in women with pulmonary hypertension.
- Risk of heart failure – Because CTD-PH causes the heart to struggle with pumping blood through narrowed lung arteries, the increased cardiovascular load during pregnancy can lead to heart failure.
- Higher maternal mortality rate – Studies have shown that women with pulmonary hypertension face a higher risk of complications during pregnancy, including preeclampsia, preterm birth, and even maternal death if the condition is not carefully managed.
Managing Pregnancy with CTD-PH
While the risks associated with pregnancy in women with CTD-PH are high, some women still choose to pursue pregnancy. In such cases, a multidisciplinary team of healthcare providers, including a rheumatologist, pulmonologist, cardiologist, and high-risk obstetrician, is essential for monitoring and managing both the mother and the baby. If you’re considering pregnancy, here are some key steps to take:Pre-Pregnancy Counseling and Planning
If you have CTD-PH and are considering pregnancy, the first and most crucial step is to seek pre-pregnancy counselling. This involves a detailed assessment of your health, including the severity of your pulmonary hypertension and overall heart function. Your healthcare team can help you understand the risks and outline potential options, such as assisted reproductive techniques or surrogacy, if pregnancy is deemed too risky.Close Monitoring
For women with CTD-PH who proceed with pregnancy, close monitoring throughout the pregnancy is critical. This includes frequent check-ups with specialists who can track the progression of your pulmonary hypertension and heart function.- Echocardiograms – Regular heart ultrasounds may be performed to assess how well your heart is coping with the increased blood volume.
- Lung Function Tests – These tests monitor how well your lungs are functioning and ensure that oxygen is reaching your bloodstream.
Medication Management
Managing medications during pregnancy can be challenging, as many drugs used to treat CTD and pulmonary hypertension may not be safe for the baby. Your doctors may adjust your medication regimen to balance the need for treating your condition while minimising risks to your pregnancy. For example:- Immunosuppressive drugs – Commonly used to treat autoimmune diseases like lupus may need to be modified, as some can pose risks during pregnancy.
- Pulmonary vasodilators – These medications help open the arteries in the lungs but must be carefully monitored during pregnancy for potential effects on the fetus.
