Pregnancy and Birth with Long QT
Like many pregnancies, I started out excited, nervous, wanting this baby and hoping I would be a good mother. I was diagnosed with Long QT Type 2 at 7 weeks. Doctors vaguely asked me if I wanted to continue the pregnancy knowing that it was risky to myself. My Electrophysiologist (EP) told me the greatest risk would be during the post partum period. He told me that estrogen prolongs the QT interval, which means that the hormone changes that occur in the post partum period is why this time is so risky because the levels of estrogen and progesterone change from pregnancy levels. The doctors told me that the medication that protects Type 2 the best is, nadolol, a class C. One if the big risks of this medication is intrauterine growth restriction. My doctor decided it would be best if I stayed off of the medication until 33 weeks and he would gradually increase the dose so that I would be most protected during the postpartum period.
My pregnancy had a lot of the typical complications: nausea, vomiting, round ligament pain, abdominal separation, gestational hypertension, anemia. My body also threw in a few of it’s own such as preterm labor and preventricular contractions (PVC) every other beat from 32 weeks until 3 years postpartum.
With pregnancy, a person is very limited on what medications they can take. With Long QT, you’re even more limited. Most women take Unisom and Zofran for nausea. Those are QT prolonging medications so I couldn’t take those. I lost 6 pounds in the first month from all the vomiting. My doctor gave me a count of how many vomiting episodes could happen before I needed fluids. I never needed the fluids but I got in the car several times thinking I would need to head to urgent care until the vomiting finally stopped for the day. I had the regular discomforts of being pregnant and the thrill of feeling my baby move. But, at 32 weeks, I felt like I couldn’t breathe. Everyone said it was because the baby was high and putting pressure on my diaphragm. At 36 weeks, he visibly dropped but the symptoms were still there. I told my EP and he signed me up for holter monitoring at 38 weeks because I wouldn’t stop complaining about it. A holter monitor is a 5 lead cardiac monitor that you wear for a couple of days, write down on a piece of paper the time and date of any symptoms and then turn the box into the doctor’s office after the allotted period of time. My doctor never told me the results and I went in for induction the next week. I was on cardiac monitoring at the hospital and that is where I was told I had bigeminy, which means I had a PVC every other beat. This would explain why I felt like I couldn’t breathe. I wasn’t getting great perfusion and this symptom didn’t stop until 3 years postpartum. The other complication I had was at 34 weeks. I woke up at 4 am with cramping in my back. It felt like menstrual cramps and was a constant pain. I called the family birth place that my doctor was associated with and they told me to come in. They put me on the fetal heart monitor and said I was having Braxton-hicks contractions with an average of 28 a minute. Normal is about 8 an hour. They gave me a dose of nifedipine to stop the contractions and I was told to report if symptoms returned. B y 10 am symptoms came back. I went back in, was given another dose, and my doctor put me on bedrest for the remainder of the pregnancy.
I was seen by and OBGYN, an EP, and a high risk OB during my pregnancy, so I basically had an appointment every 3 weeks. The high risk OB determined that I should be induced at 39 weeks so that I would not go into natural labor and the situation could be controlled. At midnight on week 39, we checked into the family birth unit and my Pitocin drip was started. Because of my LQTS, induction had to be mild. They started me on “low dose” and were only able to titrate up a little. After 8 hours, I had a mild contraction that had all the alarms buzzing. A group of staff came in, threw an oxygen mask on my face and told me to get on my hands and knees. My poor husband was sitting in the corner, not understanding what was going on. The doctor came in and explained that the baby’s heart rate dropped from 122 to 72 with that minor contraction. I was only dilated to 2 at this point and he didn’t believe that the baby would tolerate real labor. He suggested a C-section before things got worse. They considered it “urgent” but not “emergent” so my husband could still come in the operating room (OR) with us. Someone took him into another room to get him sterile while they took me into the OR to get ready. I remember leaning on the nurse so that the anesthesiologist could start the spinal block. I felt a “zing” and flinched. He stated he touched the nerve and had to reposition. They laid me on the table, strapped my arms down, and told me that my heart rate was on the verge of being too high. It was 103. He told me if it got into the 1-teens he would have to use general anesthesia. I wanted to avoid this at all costs so I used every calming technique in my arsenal.
You see, I spent a majority of this pregnancy with the fear in the back of my mind that I was going to die, or that my baby would, or that my husband would come home completely empty handed because we both died. I remember looking around my living room at Christmas thinking this could be the last one I ever had. I remember hugging my sister goodbye when we dropped off our dog before induction, thinking this might be the last moment I would ever see her. So, in my mind, general anesthesia meant I may never wake up.
I made it through the entire procedure. My heart rate stayed at 103. When my baby cried I remember tears running down the side of my face. They brought him too us and set him on my chest. I cried more because I wasn’t sure if I would actually get to see his face.
I asked them to take a blood sample from his chord blood so that we could test him for LQTS as well and avoid a major poke and blood draw. They wheeled us into the recovery room and they placed him on me again. Something felt off. He was too quiet, too calm. I said this to the nurse and she said “some babies cry a lot and some don’t.” I said, “This just feels off. He’s too quiet.” She said she would check his blood sugar. It was 32 which is very low for a new born. He was born 5 pounds and 10 ounces at term, which is very small. Because of this, he had no brown fat to burn to help regulate his temperature. He was taken to the NICU for basically a sugar water drip until he could maintain his own sugar with food. He would stay in the NICU for 3 days. I found out later that the theory was that he was small because of the nadolol. They said they weren’t sure. However, studies show that nadolol can cause low birth weight in infants, but it could also be due to hypertension during pregnancy. (See resources). Later, we also learned that my son had an undescended testicle that had to be surgically repaired. He also developed asthma (which has been difficult to maneuver all these years and I will discuss in a separate post.) Both of these complications can be linked to having a baby with low birth weight for gestational age.
When our son was 3, I talked to my various doctors about the possibility of having more kids. My husband wasn’t really for it but he was willing to see what the doctors would say. The whole experience was very traumatic for him. Doctors told me that given my history during the pregnancy and the fact that I had PVCs for 3 years, it would not be wise to have more children because there was a good likelihood that my heart would tolerate pregnancy even worse the next time and a cardiac arrest was a possibility. We decided that our son needed his mother more than he needed a sibling.
Other people I know with LQTS have been able to have multiple pregnancies with little to no complications. This is a personal decision that requires a conversation between the patient, their loved ones, and their physicians. In my case, I am happy with my one child. He does ask about having another sibling once in a while, until he sees what siblings are like for others and is uninterested. He says he likes the cousin concept better, where he can send the other kids home at the end of the play date.
Long QT is something you live with for the rest of your life and it will impact every health care decision you have but with close monitoring of physicians and open and honest conversations, you can still find the quality of life that you desire.