Posts Tagged ‘Sano Shunt’

Which HLHS surgery works better?

November 18, 2009

Update: My friend is out of surgery and doing well!

The three operation Norwood Procedure was developed in the 1980’s as an answer to Hypoplastic Left Heart Syndrome (HLHS). Since infant heart transplant was developed at about the same time, HLHS was 100% fatal before the invention of the Norwood.

But the results from those early Norwood Procedures varied wildly, especially in the early days when the Norwood consisted of only two surgeries. When the Sano Shunt – a direct Right Ventricle to Pulmonary Artery connection, using a “tube” made of Goretex – was introduced in early 2003, matters improved greatly. The Sano Shunt provided the heart with much more stability and increased survivability rates.

But while we know that the Sano works better, there hasn’t been much research into how much better it works…. until now.

The Pediatric Heart Network studied the cases of 555 infants with HLHS. Through random selection, roughly half of the infants received the Stage I Norwood with a Sano Shunt, the other half received the Stage I Norwood with a Modified Blalock-Taussig Shunt (MBTS).

Followup with the children showed that 26% of the Sano Shunt children required a heart transplant before the age of 1; 36% of the children with the MBTS version needed a transplant. But after two years, the number of children who needed a heart transplant was within four percentage points of each other. Good Pulmonary Artery growth helps in the success of Stage II and Stage III of the Norwood, and the MBTS seems to encourage Pulmonary Artery growth.

Meanwhile, the Sano Shunt had more complications that required a balloon or a stent to keep the shunt open. True, this is not as serious procedure as open heart surgery, but the fewer, the better. After all, minor surgery is any surgery that happens to someone I don’t know – when it is YOU, your child, or a family member, things quickly become very serious!

Overall, it seems that the Sano Shunt gives an HLHS patient better results over the first two years – after that, the odds even out. These children will have to be followed for years to determine the long term success rate of either the Sano Shunt or the Modified Blalock-Taussig.

The Dirty Little Secret of HLHS

October 1, 2009

She didn’t even have a name for it.

When Dr. Helen Taussig wrote Congenital Malformations of the Heart in 1947, she described one malformation as “Atresia or marked hypoplasia of the aortic orifice prevents the expulsion of blood from the left ventricle in the normal manner.” Taussig described several variations of the defect – actually different defects, later grouped together under the same deadly name – but could offer no treatment suggestions. Her Tetralogy of Fallot (ToF) patients could at least squat and get some temporary relief; children cursed with this malady died in less than one week. The left side of the heart wasn’t damaged as much as it just wasn’t there.

It wasn’t until 1958 that Dr. Jacqueline Noonan and Dr. Alexander Nadas named the group of defects Hypoplastic Left Heart Syndrome (HLHS). Hypoplastic comes from the root word hypoplasia, which means “small”.

But having a name for it didn’t make it any less deadly as HLHS continued to claim 100% of its victims. This sad story continued until 1985, when the first successful infant heart transplant took place.

At about the same time the Norwood Procedure was developed. Originally designed as one operation (which consistently failed) the procedure was soon split into two heart operations – and eventually three – which seemed to work. HLHS children now had a chance. Obviously long term survival rates aren’t known yet, but approximately 70% survive the three surgery protocol.

And thats when the dirty little secret of HLHS showed itself: While survival rates for the three operation procedure may be in the 70% range, getting from Stage I to Stage II is the hardest step.

The goal of the Stage I operation is to make the Right Ventricle do the job of the defective Left Ventricle – getting the blood to the body. With all of the blood flowing through the right side of the heart, that side is subject to higher flow pressures than it usually receives. A lot higher. In a defect in which the entire left side of the heart is damaged, a successful outcome usually depends on the Tricuspid Valve – located on the right side of the heart.

The inital results were confusing. The Norwood worked – then it didn’t. There didn’t seem to be any rhyme or reason to it, no way to predict outcomes. The surgery itself worked, but too often the results¬† seemed to be as random as a roll of the dice. The answer was the Sano Shunt, invented by Japanese surgeon Shunji Sano – a direct connection from the Right Ventricle to the Pulmonary Artery through a Gortex conduit. (this sounds redundant, but the Pulmonary Artery is normally disconnected from the ventricle in the Stage I operation). Studies showed that survival chances with the Modified Norwood was 11 times greater than with the Classic Norwood.

And now it is time to turn the tables – we’ve got a nasty little trick of our own. Heart defects have to start somewhere… at some point in fetal development, there must be one isolated problem that seems to “snowball” into something bigger as time passes. If we could find that one little problem and repair it then, maybe we can stop the snowball before it gets too big. A pretty cool idea, and maybe one day…

Someday…

A study released Monday (September 28, 2009) revealed that for seven years, doctors at several hospitals in Boston and Harvard Medical School have been detecting Aortic Stenosis in fetuses. Fetal Aortic Stenosis is a snowball; it usually becomes HLHS as the fetus develops.

70 future HLHS patients underwent surgery while still in the womb – a needle was inserted into the mother’s abdomen, passed into the fetus, and into the heart of the unborn child. A small balloon was used to enlarge the abnormally small Aortic Valve. 51 of the procedures were considered successful… and 30% (17 children of the 51) were born with two functional ventricles. That’s 17 children who won’t have HLHS.

Mark September 28¬† on your calendar… that was the day that the CHD world changed forever.