Friday, August 7, 2009

The best people

This week I have continued to be the lucky beneficiary of assistance and advice from my various friends and the people they know.

Careful readers may remember that today I was supposed to meet up with the friend-of-a-friend UCSF pediatric cardiology fellow. That didn't come to pass because of scheduling conflicts, but I'll probably talk to him on Monday instead or some other time next week.

However, I did get a lot of helpful information from another source today. My high school friend Rachel is now a doctor at a prestigious hospital back East, and she wrote me this week to say that she'd contacted a cardiac surgeon at her institution who does these surgeries and that he'd kindly offered to meet with her and discuss HLHS. So, this morning, she took him up on that offer, and sent me a lengthy e-mail based on their discussion, which I will excerpt for you here.

A lot of it was stuff I already knew, but even hearing what I already knew was helpful, because while I've talked to a few cardiologists now, this was the first surgeon I'd heard from, so it was interesting to hear his take on things. So, here is what Rachel wrote with my own notes asterisked (by the way, I was very impressed by her note-taking skills here! I can see why she did so well in medical school!):

What you can do right now while pregnant

- stay pregnant as long as possible, and get that baby boy as big as possible. Apparently low birth weight is a poor prognostic factor, so ideally he should go to full term and be a good sized little guy if at all possible.

- He says there is nothing really prognostic to find on the ECHO right now, as much as people have tried to look.

- There is nothing that can be done in utero in terms of treatment. (I wondered if there might be growth factors or something)

- He can’t think of anything that could relate the bright spots in the bowel to HLHS. He had no idea what that might mean.

The post-natal period

- OK, so as I’m sure you are already aware, this is where things get very busy, and I imagine quite stressful for all involved. There are three stages, or surgeries. Apparently there used to be a lot of debate in the field about whether these three surgeries were preferable versus heart transplant, but as the data have matured, the staged surgeries have been shown to be clearly superior. He said the way they are done has been standardized for about 15-20 years, so any surgeon should be doing about the exact same thing. Only a couple places still do transplants.

- Once the baby is born they can look at the heart more, mostly by ECHO is sounds like. If there are other malformations (coronary arteries, etc) or the single heart valve is leaky, those are poor prognostic signs.

- Stage 1: done in the first week of life to minimize the stress on the single ventricle, thereby minimizing the chance of heart failure. The most difficult surgery with the highest mortality rate. National average is about 70% survival* of this stage. Dangers are peri-op things like bleeding, arrythmias, stroke, etc. I’m sure the surgeons will go into a lot of detail about this if they haven’t already.

- Post-stage 1: in the ICU 10-14 days, then at least another week in the hospital; all depending on the individual course your little guy takes of course.

- Then you go home, and again I’m sure others will give you more detail on this. But it sounds like oxygen monitoring at home at all times, perhaps some feeding difficulties, as the recurrent laryngeal nerve can be damaged in the initial surgery and that can affect swallowing. But he said mostly these little babies eat fine, gain weight, and do what a baby would do (sleep, poop, win you over with their adorableness)

- There is a small risk for what he called “interstage mortality” – he estimated 5% or so. This is primarily due to sudden cardiac death. But he stressed that this is unusual, and more likely between stages 1 and 2, than between 2 and 3.

- Stage 2: occurs around 4-5 months. Over time they have been trying to do this stage earlier and earlier to reduce the strain on the heart until the second procedure is done, and reduce the chance for the interstage mortality. This procedure is generally much more well-tolerated with a mortality of <5%.

- Stage 3: Doesn’t happen until the little guy is about 3 years old. Also very well tolerated, mortality <5%. After this stage the intensity of the medical visits and procedures goes down significantly for most children.

- Of course all along there will be very very close follow up with your doctors, and perhaps ancillary services and therapies depending on his exact situation.

- There may be some small cognitive deficits, but he thinks these are so minor as to be mostly un-noticeable. You can be aggressive about testing and therapy in early childhood to try to make up for anything that might be there. I guess it’s largely thought to stem from early surgery, time in the ICU, oxygen effects, less close people contact, you name it --- really, they have no idea.


- Basically, as you are probably aware, the longest term survivors are now in their 20’s, since it’s about that long since the surgery has improved.

- Overall, he feels that if they get through the first three stages, their quality of life is generally very very good. They can do many sports – probably not competitively – but for fun. They go through school normally, etc. Depending on their situation they may need other valve surgeries, etc. But so far, there are no clear boundaries or medical worries. They’ve even helped a couple of female HLHS patients through successful pregnancies, though they don’t recommend it due to the stress on the heart**.

- What is the long long-term situation? Unknown. But as he pointed out, cardiology is an advanced and advancing field. He feels that congenital heart surgery is pretty mature, but adult heart procedures are always advancing.

Overall, he said this is going to have a huge impact on your life, obviously much more than a baby already does, as I’m sure you realize. But he said parents of HLHS babies make very close bonds with, and really treasure their children. It can sometimes be a struggle later, as they grow older to not be too over-protective of them. But kids that have HLHS are wonderful children who live very normal lives, and parents he knows who have gone through this feel it was absolutely worth all the worry and work.

She then added a few more bits of info in a follow-up e-mail:

I forgot to mention in my last email that after stage 1 and even stage 2, the expected oxygen saturations are about 80%, with a partial arterial oxygen pressure of 40. These numbers perhaps won't mean much to you now, but later you'll have this to reference, and I'm sure Dr Azakie will talk to you about all of this too. These will improve to near normal after the stage 3 surgery. I can explain more about why this is, physiologically, if you want me to, though you may already know all about all this (basically the blood supplies are mixed and going in parallel until the 3rd stage is completed). I also forgot to mention that one thing long term survivors have is elevated central venous pressure (CVP). As babies and older children (likely even their whole life) HLHS survivors are usually on lasix to decrease fluid load (which stresses the heart), and vaso-dilators to bring down the CVP. Over time there is a question (no data yet) of whether elevated CVP will damage their kidneys or livers. Again, given that survivors are still quite young, the long-term sequelae of this are unknown.
Something that was interesting to me: Usually the wall of the right heart is thin, and the wall of the left heart is thicker and much more muscular, given it's job of pumping blood to the body. It made sense to me that perhaps in folks with HLHS their right ventricle might become thicker and more like a left ventricle. I asked him, and he said yes, that is what happens. And by the time the third stage is complete, unoxygenated blood never returns to the heart, but is pushed through the pulmonary system by the elevated CVP I mentioned above. The oxygenated blood then comes back to the single ventricle to be pumped out to the body. So in the end, basically, physiologically there is no right heart.

So, yet again, I am deeply indebted to my wonderful friends who have done so much to help! I know I already said thank you a bunch of times today, Rach, but again, this is really great information and I can't tell you how much I appreciate you and the surgeon taking the time to do this.

*My understanding is that survival rates are quite a bit higher for this surgery at centers where they do a lot of these. The UCSF cardiologist told me 85-90% survival and the one at Stanford said about 97% survival in the last year. But I'm not sure if these statistics take into account babies with low birth weight or other risk factors for a poor prognosis.

**I did not know this, although I'd heard of other women with related but less severe heart defects having babies.

1 comment:

  1. Wow, this was fascinating to read and I can only imagine how valuable this information must be for you. How awesome of Rachel to do this!