24 Weeks, 4 Days

This morning started out a little crazy. I woke up from a deep sleep around 9:30 when Blaine just walked out the door for work. I just wanted to lay there, but had a hunch to look at my phone, I had a bunch of text messages, two missed calls, and one voicemail, from the cardiologists office. I called her back immediately as she said the doctors thought they could squeeze us in today, a week earlier than expected before our appointment. She said they could get us in at 11:00, but had to arrive around 10:45 to get registered, it was now about 9:45. Carsyn was still in her bed playing. I had about 30 minutes to get her dressed, fed breakfast, me dressed, the dog out to potty, and us packed in the car to leave. As I'm running around the house trying to gather everything, and not forget all of Carsyn's snacks since I knew we would need something to keep her quiet, I kept thanking God that we will find out some answers. Answers that I have been very anxious about knowing. Throughout this journey I have had so many highs and lows emotionally. I know in my heart God has put us here for a reason, and I have kept my faith, even through the worst.

We did make it to the office just in time!

We met our pediatric cardiologist, and another pediatric cardiologist who would be running our fetal echocardiogram with the technician. I couldn't see much of anything, but could catch words and phrases here and there of what he was looking at. We then went into a conference room so he could explain his findings to us more in detail. He explained that Kennedy had HLHS with a Double Outlet Right Ventricle (DORV) and a possibility of an intact or highly restrictive atrial septum. This either made things better, or worse.


Double Outlet Right Ventricle (DORV)

Double Outlet Right Ventricle (DORV) is also a rare congenital heart defect, just like HLHS. In a normal heart, the aorta outlets from the left ventricle and pumps blood to the body and the pulmonary artery outlets from the right ventricle to pump blood to the lungs. In hearts with DORV, both of the arteries are connected to the right ventricle. In typical DORV cases, there is also a ventricular septal defect present. Kennedy's ventricular septum is intact, but with her HLHS, the left ventricle is too underdeveloped and small to hold and pump blood to the body. Best case scenario, if her aorta is big enough and strong enough, she will just need a pulmonary band to help regulate the blood flow to the lungs, but the right side would be the main pumping side that would be responsible for pumping blood to both the lungs and the body. This would replace the first stage surgery called the Norwood procedure. If her aorta is not efficient enough, she will still have to undergo the Norwood, the surgeons wouldn't know which one she would need until she had surgery and after she has been monitored after birth.

Double Outlet Right Ventricle

Intact or Highly Restrictive Atrial Septum

In fetuses, the atrial septum naturally has a defect or hole that allows the blood to freely flow between the atriums. This helps regulate the pressure in the lungs. Once a baby is born, the atrial septum usually closes on it's own due to pressure changes within the heart and lungs to create two separate atriums and four total chambers within the heart. If a fetus has a restrictive atrial septum, the pressure in the atrium becomes too great. Babies diagnosed with a restrictive septum have a higher mortality rate (50% survival rate).

After our discussion on the newest details, he still wasn't positive on Kennedy having a restrictive septum. He rushed the images to the other cardiologists in Ann Arbor to take a look so we could get some answers ASAP. If she did have a restrictive atrial septum, we had three options: the first of which is not an option in my book, to terminate the pregnancy; the second, to have a fetal intervention (surgery in-utero to open up the atrium wall using a needle through my belly wired with a balloon on the end, like an angioplasty); or the third, wait until she is born and rush her into surgery immediately. Once again, we walked out of the doctors with so many more questions and more concerns for our baby's future.

A couple hours later, the doctor calls to confirm his findings. I can still hear his voice over the phone, "The doctors in Ann Arbor confirmed what I thought. Intervention can happen between 28-29 weeks, and they said there's no need to rush in here this week. The nurse practitioners will call you with an appointment for sometime next week." Once we got off the phone, there I was again...blank. No emotions, feelings, or thoughts. How has something already so fragile gone through so much? The answers I needed to hear, were not the ones I wanted to hear. Another day with too many tears and more heartbreaking news.

I have been praying for guidance, for all the decisions I make, and pray they are the best for us. We have weighed the options for fetal intervention vs. postnatal surgery, and researched as much as we possibly could. The wait still sits between us and what's next, but I have a lot of faith we will do what's best. As I sit here tonight, I still feel all the love and support we have been blessed with, between family, friends, and acquaintances. Although, many of those people don't know our situation yet, it's amazing the people God places in your life, even those that you may have only said hi to once or twice in passing. I really do feel all the support we have been given thus far, and I pray we get our miracle for Kennedy.

Excerpts from the American Heart Association regarding Closed Fetal Cardiac Intervention (closed FCI) and Fetal HLHS with Intact or Highly Restrictive Atrial Septum:

Closed Fetal Cardiac Intervention

In the present review, “closed FCI” indicates mechanical interventions in which the uterus is not opened or accessed with a port ≥3 mm in diameter. In practice, closed FCI consists primarily of percutaneous interventions in which an 18- to 19-gauge needle is used to gain uterine and fetal access. The first reported case of closed FCI was a balloon aortic valvuloplasty performed in 1989.⁶³ Since then, FCI has been reported in human fetuses with aortic stenosis (AS), HLHS with atrial septal restriction, pulmonary atresia or stenosis, and AV block.^3–6,62 In 2000, we began a program for FCI, initially to treat fetal AS with evolving HLHS, then expanding to include HLHS with an intact or highly restrictive atrial septum, pulmonary atresia with an intact ventricular septum (PA/IVS) and evolving hypoplastic right heart syndrome, and structural anomalies causing hydrops.^5–9,17,18 Since our first procedure, we have attempted FCI in more than 120 fetuses.

Typically, we perform FCI using a percutaneous ultrasound-guided approach with maternal and fetal anesthesia; in a minority of cases, laparotomy without hysterotomy is used to facilitate fetal imaging or access. All of the procedures we perform consist of opening an atretic or restrictive valve or septum, and instrumentation is limited and simple, with an access cannula, a guidewire, an angioplasty balloon and/or stent, and sometimes an additional needle to enter the heart or perforate an atretic valve or septum. With the exception of an 18-gauge curved-tip cannula that was developed specifically for FCI (SHARC Access Needle Set, ATC Technologies, Wilmington, Mass), we use off-label equipment that was designed for other applications and may limit technical options and potentially procedural feasibility.

Fetal HLHS With Intact or Highly Restrictive Atrial Septum

Although neonatal survival in infants with HLHS continues to improve, outcomes among certain subsets of patients remain poor; one of the strongest risk factors for early mortality is an intact or highly restrictive atrial septum.^68,69 Although limited pulmonary venous egress may be well tolerated in utero, neonates with major septal restriction are at substantially higher risk of death than those without.^67,68There are 2 primary problems associated with this condition: (1) Profound hypoxemia after birth due to restricted outflow from the pulmonary veins, which results in little effective pulmonary blood flow, and (2) chronic pulmonary venous hypertension in utero due to restriction to left atrial outflow, which results in pulmonary venous thickening⁶⁸ and perioperative morbidity and mortality. Thus, even if postnatal opening of the atrial septum is rapid and effective, damage to the pulmonary vasculature may contribute to further mortality in the first few weeks or months of life.

In fetuses with HLHS and an intact atrial septum, FCI may improve both of the major problems posed by the restriction of pulmonary venous outflow. If the left atrium can be decompressed before birth, the profound perinatal hypoxemia and acidosis and their associated morbidities may be prevented. If left atrial decompression can be achieved sufficiently early in gestation, adverse pulmonary venous remodeling may also be prevented. Physiologically, the earliest possible treatment of left atrial hypertension on diagnosis of atrial septal restriction should maximize the benefit of normalizing the developmental conditions for the pulmonary vasculature. In our experience, however, there are technical limitations to creating a large interatrial defect in the second trimester. Thus, for logistical reasons, we have focused on creating a large atrial communication. Given the currently available technological options, this can be pursued more aggressively in older and larger fetuses, and we thus tend to wait until the early-to-mid third trimester to perform this procedure. At the same time, we continue to search for technical and technological innovations that will allow earlier effective atrial septal opening.

We recently reported our experience with FCI in 21 fetuses with HLHS and an intact or highly restrictive atrial septum.⁹ Prenatal atrial septoplasty for this condition has also been reported by others with various monitoring and interventional approaches.^10,11 In our experience, the atrial septum was crossed and an interatrial communication created in all cases, either with balloon dilation or placement of a stent. Fetal demise occurred in 2 cases; in both of these, a significant hemopericardium was noted at the conclusion of the procedure. Among neonates delivered after FCI for HLHS with significant atrial septal restriction, surgical survival remains quite poor (58%); however, in utero creation of an atrial septal defect does appear to have some benefit in terms of preoperative management, because neonates with an interatrial defect ≥3 mm after FCI had higher oxygen saturation at birth and were less likely to need urgent postnatal left atrial decompression.¹⁹ Although assessment of any postnatal survival benefit of FCI in patients with HLHS and an intact atrial septum will require more extensive experience, the potential to avert immediate postnatal deterioration has been demonstrated, and we consider FCI indicated for the comprehensive management of these high-risk fetuses.