December 14, 2009

Maren went to Rady's Childrens Hospital today for a sedated echocardiogram so the doctors could get an updated picture of the size and dimensions of her right ventricle. She had not been seen by her doctor since her surgery on October 23, 2009. At that time, it seemed likely that heart surgery would be set for around February 2010 - whether as part of a 2 ventricle repair or a 1.5 ventricle repair. We were bracing for this surgery and expecting the type of surgery to be dictated by the results of today's test.

But, Maren is on her own calendar, and apparently has her own heart plan in mind which she had not yet decided to share with us. The test today showed that she has improved just enough - yet again - to push off the surgery decision as to 2 ventricle/1.5 ventricle. The 1.5 ventricle plan appears more probable than not. Her ventricle is a little bit bigger - "but still not big enough" according to her doctor. Because her oxygen levels are still 95%, and the pressure gradient in her right ventricle and across the pulmonary valve is improved (which was a major goal of the October 23, 2009 procedure), we are still not under the gun to make a surgery decision right now.

So, rather than surgery in February, we are now scheduled for a routine checkup in February. If there are no surprises between here and there (namely, a decrease in Maren's oxygen levels), the plan is to schedule Maren for an MRI in an effort to get a clearer picture of her ventricle. This MRI could be scheduled for approximately late March 2010. We are encouraged by the fact that Maren's progress supports the risks and costs of the MRI - which requires general anesthesia to insure Maren does not move at all - and is the cutting edge of right ventricle mapping. (See

http://www.pvrireview.org/article.asp?issn=0974-6013;year=2009;volume=1;issue=3;spage=180;epage=185;aulast=Blyth

(And Scroll down to cardiovascular magnetic resonance imaging)

December 6, 2009

December 6, 2009

Maren is now 7 months old. She is 50th percentile in weight (17.6 lbs) and in length. She laughs, can roll over and sit up, and loves to track her brothers' play. She loves to look at herself in the mirror. She is an angel. What else is there to say?

Maren Comes Home For The First Time

May 21, 2009 Through December 3, 2009:

Maren came home from the Rady's Children's Hospital on May 21, 2009. With the stent in her heart providing additional blood to the lungs, we started on the wait and see part of her recovery. The stent procedure is palliative. This means it alleviates the situation temporarily but is not a permanent cure. The purpose of the stent is to allow for (1) the baby to grow and gain some sturdiness, which is necessary for whatever surgeries come next, and (2) the right ventricle, which is too small (hypertrophic) and underdeveloped (hypoplastic) to grow and develop. During this time, the doctors can get a better picture of the attributes of the right ventricle. When the valve is blocked it is difficult to predict what the true characteristics of the right ventricle and the valves around it are. The heart is a muscle. When it is trying its hardest to pump blood out of the ventricle, and is unable to, the walls of the ventricle become thick and muscular. Further, the lack of blood flow impedes development, so that where there should be three parts to the ventricle, there could be just two. So a PAIVS baby will have a thick, small ventricle, which then lacks the ability to pump the volume of blood necessary.

However, with removal of the blockage at the pulmonary valve, the blood starts to flow, and the walls of the right ventricle literally can thin out as they relax. This increases the volume available in the ventricle, and it can heal. Still, there are limitations on how much the right ventricle can heal during this time and there is no way of knowing what the baby's ventricle will be capable of, except by waiting. Of course, in the interim, there is always the chance the flow could be insufficient - even with the stent - and Maren would not get enough oxygen, could turn blue, and we would head back to the NICU. Thankfully, this has not been the case. Maren has been as pink as can be, and has grown and developed beautifully during this time. The stent succeeded in bringing the oxygen levels in her blood to approximately 95% - which is basically normal.

In late June 2009 and again in late August 2009, the cardiologists conducted sedated echocardiograms to obtain information on how Maren's heart was progressing. While there is no way for the doctors to predict with certainty what would happen if the stent were blocked off (i.e. would Maren's heart pump enough blood) the doctors can measure the sizes of Maren's valves, measure the diameters of her arteries and veins, obtain pressure measurements, and eyeball the size of the right ventricle itself. This allows them to categorize and classify babies with PAIVS as either mild, moderate or severe. These classifications ultimately are used to select the heart treatment plan and what type of surgeries will occur in the future for the PAIVS baby, as follows:

Mild = 2 Ventricle repair: This means varying degrees and types of surgeries to augment the size and function of the right ventricle; the plan is to have a baby with 2 fully operating ventricles.

Moderate = 1.5 Ventricle Repair: This heart plan is premised on using the right ventricle to do just half of its job. Thus, half of the blue blood returning to the right ventricle is bi-passed via a shunt (called a Glenn Shunt or a Bi-Directional Cavopulmonary Shunt) such that it is piped directly into the lungs.

Severe = 1 Ventricle Repair: This heart plan occurs where the right ventricle is so atrophied it cannot do even half of its job. This repair requires the Glenn shunt surgery initially, and then later, another surgery called the Fontan operation, at which point all blue blood is bi-passed directly into the lungs.

At her discharge on May 21, 2009, Maren was considered moderate to severe. In late June she was solidly moderate, and in late August she was at the top end of moderate, on the edge of mild. Her improvement has led to some difficult decisions. First, it made it an option to perform a second pulmonary valvuloplasty (ballooning open the pulmonary valve). Since her original surgery on May 7, 2009, by late August, Maren's pulmonary valve was showing some obstruction to flow. It was not clear that another surgery of this type would produce benefits justifying the risks of the surgery. Ultimately however, based on the recommendation of the doctors, we went ahead and had this third surgery on October 23, 2009. Fortunately, it was successful. And again, because this is a catheter surgery (no knives), recovery is swift. We went in at 7am on a Friday and Maren was home around 3pm on a Saturday.

Second, Maren's improvement makes the decision less clear as to whether she is best suited for a 1.5 Ventricle repair or a 2 Ventricle repair. (And there is still an outside chance of a 1 ventricle repair ). There is no medical agreement on exactly where to draw the line between mild and moderate for PAIVS babies. The assessment is very difficult as to what defines mild, moderate and severe, and it is controversial and basically unknown whether better results will come from attempting two ventricle repairs or from performing 1.5 ventricle repairs in babies in Maren's spot. It all goes back to the uniqueness of each PAIVS baby. Under either scenario however, there is likely to be open chest surgery in connection with these repairs.

On December 14, 2009, Maren is scheduled for a sedated echocardiogram. Shortly thereafter, with those test results in hand, a decision likely will have to be made as to what heart plan and surgery is next. Maren's current circulation is not sustainable, as it mixes red and blue blood. Over time, this has other negative effects on the heart. The timetable for Maren's next surgery, to remove the stent and perform whatever other repairs may be required, is roughly anticipated to be January-March 2010.

Maren In Intensive Care

May 7, 2009 - May 21, 2009

Maren was anesthetized and wheeled into catheter surgery around her 24th hour of life. The surgery, a pulmonary valvotomy and valvuloplasty, was to open up her pulmonary valve so that blood could flow from her right ventricle to her lungs. This required two steps: (1) using a radiofrequency wire, the doctors burned a pinhole through the sealed valve; and (2) using an inflatable balloon, they force open the valve. This surgery went well, and after 3 hours, Maren was wheeled backed to the NICU for recovery.

With a PAIVS baby, there is a broad spectrum; the morphology of each baby's heart and subsequent treatment plan is unique and highly individualized. Some babies will have the valve pierced, and flow will return through the ventricle in an amount sufficient such that the baby can survive without any further intervention. This is the case in maybe 10 percent of PAIVS babies. In the vast majority, after opening the valve, the flow is slow to return through the right ventricle. Accordingly, the baby still needs an alternate source of blood to the lungs in order to survive. The infusion of the drug PGE, by keeping open the PDA connection from the Aorta to the Pulmonary Artery, accomplishes this task in the short run. However, PGE infusion is not a sustainable approach, as it has many side effects. After Maren's initial surgery, the next week or so was spent closely monitoring Maren and attempting to wean her off of PGE, allowing her PDA to close naturally, and measuring how much oxygen she had in her blood as the PDA closed, which stat gives an indication of whether there is flow to the lungs through the ventricle.

In Maren's case, she was among the majority that could not survive with the additional blood supply through the PDA. Accordingly, two options were available: (1) open surgery for insertion of a shunt (known as a Blalock-Taussig Shunt); or (2) a closed catheter surgery insertion of a wire-mesh stent into the existing PDA. A shunt is literally the insertion of a separate extra pipe to connect two blood vessels. Here, it would be a pipe connection into the pulmonary artery to provide additional blood to the lungs. The PDA could then close up, as it wanted to, and this pipe would provide blood to the lungs. The stent accomplishes the same thing, but it uses the existing connection of the PDA by just propping it open. Which path is the right choice is a very difficult assessment decision for the doctors. Here, Maren was a good candidate for option 2, the stent, and the stent was inserted by Maren's cardiologist via the catheter procedure on May 19, 2009.

Maren recovered from the stent surgery quickly and on May 21, 2009, we were checked out of the NICU and sent directly home for some much needed Family Time!

Naming Maren Blue

Maren was born with a heart defect that causes "blue baby syndrome", because babies are born looking blue. Because of this, the nurses and doctors, and others, sometimes make a mention of her middle name and relate it to her heart condition. But Maren's middle name, Blue, was actually given to her many years before her birth. In 2005, when Jessica was pregnant with our oldest son, Oliver, we went through the naming process at great length for both boys and girls. At that time, Jessica and I agreed that if and when we had a girl, her middle name would be Blue. There is no one defining reason for choosing this name, rather than a family name, as we did for Maren's older brothers, Oliver Ellis and Ethan Christopher. It just felt right. Blue is a word with infinite potential reference points. For us, in no particular order, these include, but are not limited to: the sky; the sea; Joni Mitchell; Miles Davis; all blues music; Dylan's Tangled up in Blue and It's All Over Now Baby Blue; Dali's "Muchacha en la Ventana"; and, at least for me, Laker Blue.

Also, when Jessica was a baby, her family consisted of her mom and their dog named Blue. As legend has it, Blue would watch over baby Jessica on the beach while her mom surfed at Beacon's (surf spot in Leucadia). I guess we chose a family name after all.

Maren Is Born

May 6, 2009


Maren Blue Demian was born on May 6, 2009. She weighed in at 8 pounds 4 ounces, 20 inches in length.

Maren was born with a Congenital Heart Defect (CHD) called Pulmonary Atresia with Intact Ventricular Septum (PAIVS). This is an extremely rare defect - even among CHDs - and a very serious condition for which the prognosis was grim as recent as 10 years ago. In the U.S. approximately 7.5 in 100,000 babies are born with PAIVS; in Great Britain and Ireland the number is approximately 4 in 100,000. This means that approximately 300 of the 4,000,000 babies born in 2009 in the U.S. will have PAIVS. The prognosis for PAIVS is much improved in recent years, which is attributable to improved treatment plans as well as medical advances.

A CHD describes a condition where a baby is born with a heart that did not form properly during the pregnancy. There is no one identifiable cause for a CHD. They are not predicted by either environmental or genetic factors. Rather, they are multi-factorial in origin. CHDs range from minor (requiring no medical intervention), severe (requiring immediate heart transplant), and everything in between.

The heart forms around the 5th week of the pregnancy, or 3 weeks following conception. In a baby with PAIVS, the pulmonary valve does not form properly and seals off completely, blocking flow through the heart. This blockage results in, among other things, an undersized and underdeveloped right ventricle and tricuspid valve. This condition can have many additional impacts, but the former issues are the primary focus, and have been and continue to be the focus with Maren.

Note, the sealed off pulmonary valve while in utero, does not prevent the fetus's heart from beating and circulating blood, because the blood circulation does not involve the lungs, and is mixed at that time via the foramen ovale, which connects the right atrium with the left atrium, and the ductus arteriosus (PDA), which is a small connection between the pulmonary artery and the aorta. Following birth, when the lungs are kicked into operation, these openings close, and blood is thereafter circulated via two separate avenues: as red blood, pumped from the left ventricle and out to the body, and thereafter returning as blue blood to the right ventricle; and blue blood, pumped from the right ventricle into the lungs, where it is oxygenated and becomes red blood. http://www.childrenshospital.org/az/Site636/mainpageS636P0.html


A PAIVS heart can be seen at: http://www.americanheart.org/presenter.jhtml?identifier=1303

A Normal Heart can be seen at: http://www.americanheart.org/presenter.jhtml?identifier=770

See also this one minute British Heart Foundation video: http://www.youtube.com/watch?v=ep4cQrYFL0w

At birth, a baby with PAIVS is blue, as was Maren. (As will be discussed in a separate post, this is not the source of her name, although it turns out to be fitting.) The first breath of oxygen is a little different for a PAIVS baby. Initially, there is mixing of blue blood into the red blood heading out to the body that reduces oxygen and causes a dusky appearance. Maren started to breathe within seconds, and her color quickly returned.

She scored 8-9 APGARs with the only mark down for the color prior to her getting her breathing going. At that point, all was well in the world. We were sent to recovery, where, even as friends and family started to gather, our recovery nurse, Rusty, heard a loud heart murmur and started the ball moving that would save Maren's life that day. Maren quickly escalated through the system, was put in a little oxygen tent, had her oxygen levels tested (they were low 80s; which is cause for concern, but not so low as to cause damage) and was reviewed by the on call cardiologist. Within a few hours Maren was taken away, placed in a rolling pram/incubator and evacuated down the road to the NICU at Rady's Children's hospital for an echocardiogram. Little Maren was administered an IV of PGE, and referred for further testing. At that point, her pulmonary valve being completely sealed, the PDA is the ticket to survival. PGE is a wonder drug that has the effect of keeping the PDA open, which was the lifeline by which our precious Maren would stay alive that first day. I followed after her to Rady's with Jessica stuck in recovery, arriving around midnight to observe a fellow (cardiologist in training) performing an echocardiogram; to get some sketchy results from the fellow on the spot about the structure of Maren's heart; and then went back to Jessica to try to explain, in a daze, around 3 in the morning, that our world was on its head.