Hypoplastic Left Heart Syndrome (HLHS) is a known killer. It is 100% fatal if left untreated, and before 1981 there was no treatment. For several years after that there were very few survivors as doctors learned that a surgical correction could not be done in one step, but rather took two (and eventually three) separate operations. Infant Heart Transplantation was a dream until 1984.
So doctors have only been able to combat HLHS for 30 years, and there is still a lot that is unknown. The hows and the whys of the defect are still only being guessed at. The operations are delicate: not only are they demanding on a newborn’s Circulatory System, but so much depends on blood flow and pressures in the heart. All of the blood now flows through the Tricuspid Valve, which wasn’t designed to move that much blood. The proper “balance” often depends on the patient and there is no “standard” series of operations for HLHS. Each one must be customized to the individual.
Which leads to this interesting report from the March 2010 issue of the medical journal Pediatrics. 749 Pediatric Cardiologists were surveyed concerning their management recommendations when giving a diagnosis of Hypoplastic Left Heart Syndrome. 99.7% of all Pediatric Cardiologists recommended the three surgery approach. 67% of the doctors discussed a heart transplant with the new parents, and 62.2% discussed Compassionate Care without a surgical intervention. (The high numbers in each category shows that the doctors were not limited to one response.) However, only a small percentage – 14.9% discussed all three options.
Also, the doctors were asked what they would recommend when confronted with a prenatal diagnosis of HLHS. Almost all of them (98.8%) recommended continuation of the pregnancy with surgery after birth. 53.5% argued for continuation of the pregnancy with Compassionate Care/no surgery; and a very high 74.3% discussed termination of the pregnancy. Only 36.5% discussed all three options.
Interesting numbers, and much to think about. The bottom line is that most Cardiologists would tell you to fight for your child. But why would so many of them recommend ending the pregnancy when HLHS is diagnosed prenatally?
UPDATE: A reader has brought to my attention (See comments) that the research report states that the Cardiologists/Surgeons discuss these options rather than recommend them. A discussion, of course, is not a recommendation. Some patients may treat it as such (“The doctor will probably know best…”) but it does answer my closing question much better – and reflect the experiences of my readers who have chosen to comment.
I have stated before that I am not immune to mistakes, that is why I try to include a link to anything that is not in my personal experience – so you can check my work for yourself.
And I would like to publicly thank the commenter for bringing this error to my attention.