There is a sort of a quadruple entendre in the title of this post: ECMO still exists; ECMO is needed if you are ‘not yet dead’ and have a chance of surviving; ECMO decreases mortality if you need it; but, unfortunately, using ECMO may sometimes just delay death. I haven’t written about ECMO before on this blog, even though I have been very involved at certain times in my past, the frequency of needing ECMO for neonatal respiratory disease has become vanishingly rare in my practice (I haven’t referred a meconium aspiration for ECMO in 5 years). ECMO for diaphragmatic hernias and for postoperative cardiogenic shock, however, is still sometimes life-saving. I have put together a number of very recent publications.
Tabbutt S, Ghanayem N, Ravishankar C, Sleeper LA, Cooper DS, Frank DU, et al. Risk factors for hospital morbidity and mortality after the Norwood procedure: A report from the Pediatric Heart Network Single Ventricle Reconstruction trial. J Thorac Cardiovasc Surg. 2012;144(4):882-95. Not surprisingly, if you need ECMO after a Norwood, then you are more likely to die. Of those in this large multicenter cohort of infants with hypoplastic left heart syndrome (n=nearly 600) who had post-op ECMO the transplant free survival at 12 months was only 26% compared to 75% if you didn’t need ECMO (and didn’t get CPR either). Still 26% compared to zer0… I guess it depends on your viewpoint, and your resources.
Another study in a similar vein (or should I say with a similar heart) is an analysis of ELSO registry data of over 700 babies who had a Norwood and then needed ECMO. The survival overall (to hospital discharge) was 31%. Sherwin ED, Gauvreau K, Scheurer MA, Rycus PT, Salvin JW, Almodovar MC, et al. Extracorporeal membrane oxygenation after stage 1 palliation for hypoplastic left heart syndrome. The Journal of Thoracic and Cardiovascular Surgery. 2012;144(6):1337-43. The authors note that complications of ECMO are a bad risk factor. The median duration of ECMO among survivors was very close to 72 hours. They show that there was an increased duration of ECMO among non-survivors, and that the Odds Ratio for mortality for every additional day of ECMO is 1.2. Useful information for counselling parents. They also note that there were 28 babies who went back on ECMO for a second time, after the first decannulation, only 1 survived. Probably if you re-satisfy criteria to go back on ECMO, unless there is a surgically correctable reason, you should not offer a second course of ECMO.
But what if you have congenital heart disease and a diaphragmatic hernia? Is ECMO a reasonable option? Dyamenahalli U, Morris M, Rycus P, Bhutta AT, Tweddell JS, Prodhan P. Short-Term Outcome of Neonates With Congenital Heart Disease and Diaphragmatic Hernia Treated With Extracorporeal Membrane Oxygenation. The Annals of Thoracic Surgery. 2013;95(4):1373-6 This report from the ELSO registry has surprisingly good outcomes, and some surprises in the results. For infants with a diaphragmatic hernia who needed ECMO, survival to hospital discharge for those who also had hypoplastic left heart syndrome and single-ventricle physiology was 55% (33 of 60), 48% (43 of 89) for ventricular septal defect, and 40% (24 of 60) for coarctation of the aorta patients. Which is a little weird, infants who had the most severe heart disease had better survival than those with less severe heart disease. I can think of a couple of possible reasons for this. But if you need ECMO for your diaphragmatic hernia (CDH) a VSD is probably not very relevant; it just means that you have a really bad CDH. Whereas if you have a hypoplastic left heart, it is much easier to satisfy ECMO criteria, so you might have less pulmonary hypoplasia, and a better prognosis for short term survival. How you might do after the second stage of the hypoplastic left heart repair is not clear.
Madderom MJ, Toussaint L, van der Cammen-van Zijp MHM, Gischler SJ, Wijnen RMH, Tibboel D, et al. Congenital diaphragmatic hernia with(out) ECMO: impaired development at 8 years. Archives of Disease in Childhood – Fetal and Neonatal Edition. 2012. This study was a very complete evaluation of 8 year old survivors of CDH repair. The children who needed ECMO in the neonatal period had lower IQ scores than those who survived without. Dutch children are obviously very intelligent, the CDH children who did not need ECMO had a mean IQ of 112. It must be all that cheese or more likely the raw herring ‘Hollandse nieuwe haring’, yum yum. Or maybe living below sea level with their higher PaO2 helps brain development, it certainly makes them tall. The CDH children who had needed ECMO didn’t do quite as well (mean IQ 91) and both groups had troubles with concentration, but overall their quality of life was really good.
If we look at the ELSO registry data for acute neurologic complications (Polito A, Barrett CS, Wypij D, Rycus PT, Netto R, Cogo PE, et al. Neurologic complications in neonates supported with extracorporeal membrane oxygenation. An analysis of ELSO registry data. Intensive care medicine. 2013:1-8) we find that they are common, occurring in about 20% of supported infants, and associated with increased mortality. As always in database information, we don’t know if that might be because the medical care changed as a result of the complication. Is the increased mortality because care was redirected after a complication?…
Madderom MJ, Reuser JJ, Utens EM, van Rosmalen J, Raets M, Govaert P, et al. Neurodevelopmental, educational and behavioral outcome at 8 years after neonatal ECMO: a nationwide multicenter study. Intensive care medicine. 2013:1-10. 135 Dutch babies required ECMO in a 5 year period. Eight years later the survivors are doing really well. The mean IQ is 100 ( I know for a Dutch child that is probably rather low, but it still sounds quite good for the rest of the world). They did have some problems, with some attention difficulties, and a few more having learning disability that will probably require specific educational attention. But these outcomes are very encouraging, and in addition, they aren’t dead yet!
Neonatal Research 
Thank you for your research summary. It is very enlightening. My son was placed on the ECMO twice; once two days after he was born, and then again after his TAPVD surgery. He is still in the ICU, though. I hope you do not mind if I put a link to your post from my blog.
Please feel free to put a link. I wish for the very best outcome for your son. Hope he gets out of ICU soon.
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What a stupid research topic and conclusion. A heart surgeon told me 21 years ago that statistics start and stop at many different times to benefit different studies.Yours are clearly leaning towards your misconception of HLHS. The term “..they aren’t dead yet” is a comment that probably offended many people. Although 1 person 2 years ago replied, which amazes me they did. My son has had 4 open-heart, 2 closed-heart surgeries and many procedures. He isn’t dead yet is an understatement to the father of my grandchild. By November 2014, when you wrote this, the medical field had already became much more advanced with technology then in 1994 when we started his surgeries. You should have been able to find more information that was also positive, although it appears that is not what you were after.
I help people all over the world through support groups online and try to help educate them to help them deal with needing the Norwood procedure, or having a baby born with Hypoplastic Left Heart Syndrome. Comments like “How you might do after the second stage of the hypoplastic left heart repair is not clear.” is another ignorant statement that you did no research before posting it. The second stage has the highest survival rate and had the quickest surgery, the quickest recovery and quickest discharge from the hospital versus stage 1 and stage 3, the Fontan.
I just seen more of your comments and articles and realize you hope people either respond positive to your articles, or respond like me, gaining more attention to your articles. I haven’t read any of your other articles but I do hope you write useful positive information sometimes and that you are not always writing things JUST to piss people off.
I’m sorry but you have completely misinterpreted my post. My blog is mostly to provide a summary of recent articles and sometimes to critique their methods. The comments were about the use of ECMO, and I noted that it might be sometimes life-saving after cardiac surgery, but that the survival rates are low. The comment about the 2nd stage of the HLHS repair was about those infants who also had a diaphragmatic hernia and had survived ECMO, unless you know something I don’t (and I doubt it from your tone), we have no idea how such a baby might do after the later stages of their repair. As for your issues with my having written “,,,they aren’t dead yet’ if you took the time to actually read what I wrote it was that the babies outcomes (not babies who had HLHS by the way, but dutch babies who had ECMO for any reason) were very encouraging, and that ECMO had saved their lives.
I too am not dead yet, but I will be one day, as will all the babies in these articles.
I am glad your son did well, and that you are now a grandfather.
My apologies for my emotion of a sensitive subject and writing what I did.
I did not see anywhere in paragraphs two and three, including the links that you referenced, diaphragmatic hernia. I did see “Not surprisingly, if you need ECMO after a Norwood, then you are more likely to die.” and “The authors note that complications of ECMO are a bad risk factor. ”
I see where you discuss “But what if you have congenital heart disease and a diaphragmatic hernia?” in paragraph 4. I apologize for relating the comment in paragraph 4 to paragraphs 2 and 3. At that point I was cloudy apparently and was speed reading to the end.
I was also reading http://cdn.intechopen.com/pdfs/37833.pdf and I guess I was confused.
……”Hypoplastic left heart syndrome (HLHS) is rarely associated with CDH (Nishimura,
Taniguchi et al. 1992) despite many infants with CDH noted to have left heart hypoplasia.
This is thought to result from compression by an enlarged right ventricle, but with
structurally normal aortic and mitral valves and aortic arch with normal volume. (Lin, Pober et al. 2007)These infants are at increased risk of a low left ventricular mass which has been confirmed at post mortem.(Schwartz, Vermilion et al. 1994) Left ventricle mass measured on two-dimensional echocardiogram may predict outcome in infants with CDH, Schwartz et al concluded that left ventricular mass, indexed to patient weight was significantly diminished in patients with left sided CDH, (n=31) and this tool may be useful in determining suitability for Extracorporeal membrane oxygenation (ECMO) prior to surgical repair and possibly to help predict survival. (Schwartz, Vermilion et al. 1994) Case reports of infants with CDH and congenital heart disease exist with individual good outcomes…. ”
I was in contact with a parent today who’s baby had the Norwood Procedure yesterday and she mentioned they have the baby on ECMO. I was researching real quick, while at work, information that could help her. I ran across your article and I was confused on the relation that I was reading, or apparently misreading.
I will delete my post and I appreciate you taking the time to reply. We have been researching HLHS for 21 years and I would really want to know if ECMO was a problem or a short-term fix to an existing problem that was not repairable.
No worries, I agree with a lot of what you say, I think that if a center is going to do Norwood procedures (or the newer variants, I tend to still call it the Norwood) then they should have ECMO available and should be prepared to use it when there is no better alternative. They should also get informed consent from parents who should know that survival is only 26% in large series, when you follow the kids out to 1 year. But as I said, 26% is better than 0%.
ECMO was first designed as a treatment for refractory pulmonary hypertension, which is usually a temporary problem. Using ECMO for severe cardiac dysfunction came afterwards, and such cardiac dysfunction after pediatric heart surgery is not always reversible. The hope that it will improve with time is why babies may be placed on ECMO. It is certainly a short term fix, but in the proportion of kids who do improve during that short term it may be life saving.
For many years I worked in PICU, so I have personally looked after kids getting all 3 stages (or sometimes more) of HLHS repair (or should I say palliation). What I think is actually most pressing is that societies limit the number of centers so that volumes in each center are larger. Every children’s hospital wants to do major cardiac surgery, but having multiple small centers increases mortality. It should be considered to be the moral duty of physicians to direct babies to low mortality centers, rather than treat them in low volume centers where more of them will die. In Canada that could and should be done by governments, in different societies that may be more difficult, but health insurers, medical groups themselves, parent groups and all other interested parties should be working to that goal.
First stage mortality varies between 50 and 20%, in a few places even lower. The centers with higher mortality should be shut down.