The decisions you make for your child can greatly improve the baby's chances of doing well. The number one indicator of survival is the choice of hospital. Therefore, it is very important that you research and compare at least 2 different facilities to see which one would be best for you.
According to the Society of Thoracic Surgeons (STS), there are approximately 120 hospitals in the US that perform pediatric heart surgeries. However, not all may perform the 3-stage procedure or heart transplant surgeries, since these are very complex procedures. In addition, it is well documented that there is huge variation in results between hospitals. Most of these studies show that the higher the volume (number of surgeries performed) the higher the survival rate. For example, one study found that hospitals that perform only 2 Norwoods per year have double the mortality (death) rate than those that perform 20 procedures per year. The most experienced hospitals that specialize in complex congenital heart conditions may perform 50-60 Norwoods per year, and achieve approximately a 95% survival rate. In addition, most hospitals that have higher volume (do more surgeries) have been doing the HLHS surgeries for a longer time, and thus, have more experience. Make sure you compare hospitals and ask how many Norwoods they do each year, how long they have been doing these surgeries, and what their survival rate is for “standard” cases and “high-risk” cases. (See our "questions to ask")
2) KNOW WHAT YOU ARE COMPARING
There is no centralized database of child heart surgeries in the US. It is therefore critical to get information on your own, and to understand what you are comparing. Some hospitals take sicker (“high-risk”) patients, and will therefore, have a higher mortality (death) rate – this does not mean that they aren’t good hospitals. In fact, usually the best hospitals are the ones that take on the most complex cases. The mortality rate is usually based upon 30-day mortality, or sometimes “in-hospital” mortality – including those who die before being discharged from the hospital. Some hospitals break out their survival/mortality statistics into “standard” risk and “high” risk cases. Mortality for high risk cases (which includes those with genetic conditions, prematurity or other factors) will be higher than those that are considered “standard” HLHS cases. (Some cite survival rate instead of mortality – if there is a 90% survival rate, then there is a 10% mortality rate.)
3)UNDERSTAND PHYSICIAN BIASES
We all have biases, or opinions, about things. It is important that you understand where your doctor is “coming from” and see if it matches with your values and beliefs.
• Studies have shown that physicians are biased towards the surgical option that their hospital provides. So, if the hospital primarily does heart transplants, the doctors there may not discuss the 3-stage surgery (and vice versa). Make sure you understand WHY the doctor recommends one procedure over another.
•One study showed that even though the survival rates have improved dramatically over the years, many infants with HLHS do not get surgical care. That is because if they are born at or diagnosed at a hospital that does not perform the surgeries, they are often not referred to a surgical facility - simply because that hospital is a competitor. It is our opinion that that is highly unethical, but apparently it is legal to withhold information from parents and not refer them to a surgical hospital, allowing the baby to die. In addition, some physicians may not believe that children with HLHS should undergo the life-saving surgeries – either because they aren’t aware of the vastly improved survival rates, or perhaps, they think that the medical costs are too high for these children. •Some physicians believe in aborting (“terminating the pregnancy”) if diagnosed prenatally with a child with HLHS. Some parents, therefore, feel pressured to terminate. Do not make a decision on this until you have received at least one second opinion from an experienced surgeon to make sure you are getting accurate information. •Not all physicians have up-to-date information on HLHS, or first-hand experience with older survivors. Many physicians, even pediatric cardiologists, may not be aware that there are now many adults with HLHS. Studies have shown that the more experience physicians have with survivors, the more positive they are on the outlook for those with HLHS (because they have seen successful cases for themselves.) Many physicians may not be aware that two young women with HLHS have successfully given birth to their own children – something that was thought unheard of just a few years ago. The point is, things have improved dramatically and quickly with HLHS in just the last decade or so and it is important that you make sure the physicians you are dealing with have accurate information. 4)YOU DECIDE
Don’t let a physician discourage you from getting a second opinion. As you can see from above, there is a huge variation in outcomes depending upon what hospital you choose for surgeries, and huge variation in physician’s optimism/pessimism level for those with HLHS. So please make sure you are evaluating any decisions based upon accurate and up-to-date information. It is also helpful to find support groups and talk to parents who have actually lived it. We parents often have very different views and experiences than physicians who only see the children when they are sick. As parents, we see the whole child – the ups and downs, the joys and triumphs, and even just the plain old normal baby/kid stuff. Please get as much information as possible. The decisions you make now will have great impact on you and your child.