During her first pregnancy, Lena’s mom learned at 20 weeks that her baby had a large, high-risk CPAM. After two courses of prenatal steroids and a CVR that peaked at 2.6, the lesion’s growth stalled, and Lena was born asymptomatic at 39 weeks.
***Lena is a pseudonym.***

The diagnosis

This was my first pregnancy. When my husband and I arrived at our 20 week anatomy scan we were eager to find out whether we were having a girl or a boy. Instead, we left the appointment with such heavy hearts and so many questions. We were told something was wrong with our baby's chest and we would need to be seen immediately at an MFM clinic in a specialist hospital to get an exact diagnosis.

The next day at the MFM clinic we were told our baby girl had a CPAM. Her heart was completely shifted to under her armpit, and the CVR was 1.55. They told us that hydrops can occur if the CVR goes above 1.6 and, since ours was basically there already, I would need to be seen twice a week. I left the clinic hysterical, running out of that hospital as fast as I could, trying to escape what I was just told.

Later that day I spent hours researching anything and everything I could find about CPAM, not really finding much information. The only place I found that reassured me was a CPAM group on Facebook.

The CPAM grows even larger

I continued to be seen for ultrasounds twice a week at the high risk clinic. The CVR continued to climb, and, at 22 weeks, it got up to 1.9. I was given the first course of steroids and told I now needed to be seen three times a week. At this point I was referred to a children’s hospital where I had a fetal MRI, fetal echocardiogram, and another ultrasound.

My husband and I were told things did not look good. Our baby girl had a CVR of 2.6 and we were told to prepare for the worst. The specialist doctors planned for me to have a C-section at Boston Children’s hospital and that most likely she would be born symptomatic. They would then take her for surgery immediately.

I had the second course of steroids at 25 weeks. By 26 weeks the CVR was down to 1.22. I thought the tech didn’t know what she was doing and was measuring wrong! We had never seen a CVR that low. Every week after that the numbers started dropping to the point that, at 27 weeks, some techs couldn’t even measure a CVR, as it was getting harder and harder for them to make out anything on the ultrasound. My husband and I were so thankful things were finally looking up.

The birth

Fast forward to delivery, I was recommended to have an induction at 39 weeks so the NICU team and everyone could be in the room when I gave birth. After a long 72 hour induction, our baby girl was born asymptomatic and didn’t need any NICU time. She had an X-ray at birth which showed the CPAM. We went home 24 hours later and we were so thankful everything had turned out ok.

Surgery and recovery

She had a CT scan at 2 months old which showed her CPAM was pretty big and there was still some mediastinal shift. She had surgery at 4 months which went really well. The recovery was a long 9 days in the hospital due to complications with her chest tube. We were so happy to be home after those long 9 days in the hospital, but she recovered so fast you would have never known. She is now a happy thriving 1 year old, meeting all of her milestones and amazing us every single day. She truly is our miracle baby and a true fighter.

2/ Lena’s story

3/ JJ’s story

1/ Robbie’s story