The Plan and Baby P's Prognosis

The Plan:

The plan for baby P after birth is to place him in NICU and begin an IV line of prostaglandins to keep the PDA open and his heart functioning. Basically, we are all born with a Patent Ductus Artery (PDA) attached to our heart...it is a normal fetal blood vessel that closes shortly after birth and begins the normal blood flow through the heart, to the lungs, back to heart, and to the rest of the body. Due to the anatomy of baby P's heart, if this were to close he would suffocate as no blood would reach his lungs, therefore they must keep this artery open by administering the prostaglandins. They will then perform another ECHO to confirm his diagnosis (diagnoses often change after birth), wait a week for baby to acclimate to the world, then surgically place a BT shunt, or artificial Goretex tube, in the PDA to keep it open permanently. He will recover for 1-2 additional weeks in the CICU/NICU and then come home. We will then wait 4-8 months for baby to grow before beginning the major surgery(ies) to "fix" the issues...

As of right now we have two options to fix the heart:

1) Create a normal flow of blood through the heart by moving the major arteries to their correct positions and closing the VSD to maintain a 4 chambered heart. This is the preferred first choice as it creates a normal heart that will function near full capacity. It also should only be one open heart procedure barring any complications. The only problem with this option right now is that it may not be possible due to how far to the right the aorta is currently from the left ventricle where it belongs. They will be monitoring this as he grows and doing measurements to see if it is a feasible option. Otherwise, option two. 

2) Create a single ventricle pathway using the Bidirectional Glenn procedure around 10 months of age and the Fontan procedure at 3-4 years of age. Timing of these interventions all depend on his growth...as soon as he stops growing, that is a signal that his body is nearing the max size for his heart to support and surgery is needed before any damage is caused. 

Prognosis:

This information is very difficult to find. Based on reputable sources online, the prognosis depends on the defect, however most children end up leading normal lives. Mortality rate also depends on the complexity of the heart defects and if other health or physical issues are present in a patient, ranging anywhere from 5-60%. Luckily, Baby P's defects are limited to his heart and no other defects have been suspected or confirmed. According to our Pediatric Cardiologist, despite the complexity of Baby P's heart defects she is optimistic that so long as he is healthy enough at birth and tolerates the surgeries well, he will have a normal life with very few limitations. He won't be a weightlifter or track athlete, but he can most certainly participate in and even excel in most sports if that ends up being his thing! Talk about motivation to remain positive, keep stress levels low, and keep him cooking as long as possible!!

D-Day

Official diagnosis: Double Outlet Right Ventricle with remote Ventricular Septal Defect, D-Malposed Great Vessels with Pulmonary Atresia, Bilateral SVC, and a Right Aortic Arch.

This diagram is the closest I could find to our baby's diagnosis. Heart defects are the most common of birth defects, nearly 8 of every 1,000 births. However, our specific combination of defects is extremely rare. The Pediatric Cardiologist didn't have numbers for us and did not have an accurate diagram either. She actually had to draw on the closest one she had to accurately depict his diagnosis. This diagram is missing the Bilateral SVC, the ventricular defect being remote (meaning the aorta is further from the left ventricle than shown), and instead of pulmonary stenosis (narrowing of the aorta), our baby's heart has pulmonary atresia (complete blockage or underdevelopment of the aorta).

To summarize, baby P has 3 major heart defects and 2 minor abnormalities.

The 3 defects are:

1) DORV with remote VSD
2) D-malposed Great Vessels
3) Pulmonary Atresia. 

The 2 minor abnormalities, the right aortic arch and bilateral SVC, were also noted but were stated as more of an anatomical anomaly than an issue and don't necessarily pose a risk of any kind. Hallelujah!

For the curious here is a detailed breakdown:

(d-TGA) Dexter-Transposition of the Great Arteries [transposition=switch the order, great arteries=the aorta & the pulmonary artery, dexter=on the right side] – the positions of the great vessels are switched so that the aorta (which carries oxygen-rich blood to the body) is closer to the oxygen-poor blood AND the pulmonary artery (which carries oxygen-poor blood to the lungs) is closer to the oxygen-rich blood. It is usually accompanied by a hole in the wall between the ventricles (ventricular septal defect or VSD).

(DORV) Double Outlet Right Ventricle [outlet=passage for exit, ventricles=lower chambers of the heart] – both vessels (aorta & pulmonary artery) carrying blood away from the heart come out of the right ventricle. (Normally the aorta carries oxygen-rich blood from the left ventricle and the pulmonary artery carries oxygen-poor blood from the right ventricle).

Pulmonary Atresia (pa) [pulmonary=having to do with the lungs, atresia=without openings] a complete blockage of the pulmonary artery (which carries blood from the heart to the lungs) caused by a missing or fused-shut pulmonary valve.

(VSD) Ventricular Septal Defect [septum=wall between the chambers of the heart, ventricles=lower chambers of the heart] – holes in the inner walls of the heart allowing extra blood flow between the two lower chambers of the heart (ventricles). This causes the oxygen-rich and oxygen-poor blood to mix before leaving the heart.

Bilateral Superior Vena Cava (b-SVC) [bilateral=both sides, superior=upper, vena cava=large vein] There is an extra upper vena cava on the left side of the body that drains into the right ventricle through an enlarged coronary artery (one of the blood vessels that normally supply the heart muscle with oxygen-rich blood).

(RAA) Right Aortic Arch [aorta=main vessel carrying oxygen-rich blood from the heart to the body] the part of the aorta that leaves the heart, arches to the owner’s right-side , and then turns downward.  (In a normal heart, the aorta arches to the left.)

Thank you to HeartBabyHome.com  for the images and information. 

Waiting Game

Tuesday May 20th, 2014: As soon as we confirmed that there was indeed a heart defect at our 20 week ultrasound we were referred to Cardinal Glennon Childrens Hospital for an ECHO on baby P's heart and a formal diagnosis by a Pediatric Cardiologist.

Since we would be driving over 2 hours from Columbia to St. Louis, they wanted to be sure we had ample time with specialists to discuss diagnosis, the plans for birth, treatment options for Baby P, prognosis, and also have any questions answered. Because of the coordination of appointments required the soonest we were able to get in was June 11th. WHAT?? I desperately needed answers sooner than that. I had already waited a month to confirm the defect, I did not want to wait another day to find out what was going on with my little guy! 

In the meantime, I did as much reading as I could while caring for a one year old...I researched Pediatric Cardiology departments and their ratings, common heart defects, best pediatric cardiothoracic surgeons, read blogs, etc. While it was stressful and overwhelming, it was actually in some ways therapeutic and readied me for the impending news.

After a full day of research, chatting, calls, and emails, I decided it would be in our best interest to contact St Louis Childrens Hospital to arrange a second opinion. Fortunately a local mom got me in touch with a nurse coordinator there that same day. I laid our story out for her and asked if she could be of any help. She went above and beyond moving schedules around and coordinating appointments for us just two days later with a high risk OB and Pediatric Cardiologist. Finally, the answers were coming....

From the beginning....

I suppose I should start at the beginning:

*****February 20th: Suspected and confirmed pregnancy with a home pregnancy test. At this time I was still exclusively breast feeding Evan and so pregnancy was not even on my radar prior to this date. I was physically active doing hot yoga twice a week, still taking pre-natals, and eating healthy to support Evans hefty appetite.

*****March 27th: First prenatal appointment with our midwife. Heart rate was clearly audible with the doppler and was strong in the low 150's. BP was low, 90/50, which is rather normal for me compared to my first pregnancy. Weight Gain: 3lbs

*****April 14th: I noticed my milk supply was extremely low and that my uterine height was measuring 16ish weeks instead of the expected 12 weeks so I contacted the midwife and made an appointment. She confirmed the height was definitely high and recommended an ultrasound to rule out multiples and also confirm a better conception and due date. I had to give up breastfeeding and started to wean Evan to Goats milk and then cows milk. It was bittersweet but I truly believe it was my body's way of preparing for the new baby.

*****April 17th: Met with Dr. Grant at Women's Wellness Center for our ultrasound reading. Heartbeat was strong at 157 beats/min and baby P was measuring 15 weeks and 4 days...a full 3 weeks ahead of what we thought initially! And unlike the first pregnancy, we found out what we are having...its a BOY! Everything looked normal and on track for the baby's size however they raised some concerns about a possible heart defect as they could only see 3 chambers of the heart at the time. Immediately, I became very emotional. As parents you want the best for your child and certainly want them to above all be healthy and thrive! We were handed several chromosomal testing options and then sent home to let the baby grow a couple more weeks before they would diagnose or confirm anything as the baby was still pretty small at this point. This was tough because we had so many questions we wanted answered but simply had to wait patiently. We prayed a lot, I researched all I could about congenital heart defects, asked family about any history we weren't aware of, and waited for baby to grow. We prayed that whatever they saw or didn't see would resolve itself naturally or that perhaps they just weren't able to get a full look due to baby's size and position. We also continued to meet with the midwife, clinging onto the idea that a natural home birth was still a possibility once we completed the 20 weeks scan so long as I was not risked out. Weight gain 5 lbs.

*****May 16th: 20 week ultrasound was here. Heartbeat strong: 163 beats/min, measuring right on for 20 weeks still, physical anatomy right on, BUT.....deep breath......we can confirm there is indeed a heart defect. From what Dr Grant could tell from ultrasound, he suspected transposed great vessels (the main arteries to and from the heart are switched) and a ventricular septal defect (a hole between the walls of the heart). I held it together the best I could, but the wells were filling quickly. What does this mean? How serious is this? Home birth?? Please tell me this is still feasible...I can't even describe the amount of questions I had at that time but was too overwhelmed to even ask them. Remain composed, remain composed, this news is not from left field....

So hospital birth?, I asked.

"Yes." Okay, not ideal but I can still do this.

"Hospital birth, highly managed...probably an induction or C section, and it will have to be in St. Louis at a tertiary or higher facility. This is highly likely to not be an unmedicated, natural, intervention free, baby on your chest, breastfeeding right away, experience...but that's depending on the official diagnosis and also up to the experts to decide."

The flood gates officially open. Complete heartbreak.