>> OUR CURRENT STRATEGY FOR RECONSTRUCTION OF THE HEART
FOR HLHS INVOLVES THREE STAGES, ONE DONE INITIALLY AT BIRTH,
ONE AT APPROXIMATELY 3 TO 6 MONTHS OF AGE,
AND THE THIRD STAGE AT APPROXIMATELY 2 TO 3 YEARS
OF AGE.
>> THIS IS NOT AN OPERATION THAT CREATES A NORMAL HEART.
>> YOU CAN'T FIX THE HEART.
>> WE CANNOT MAKE ANOTHER PUMPING CHAMBER.
>> WHAT WE DO SURGICALLY IS REARRANGE THINGS,
CLOSE HOLES, MAKE CONNECTIONS--
>> --SO THAT YOU CAN HAVE BLOOD GOING TO THE LUNGS AND BLOOD
GOING TO THE BODY WITH ONLY ONE PUMPING CHAMBER.
>> BUT THAT ISN'T THE SAME AS A NORMAL HEART.
>> BY REROUTING THE PLUMBING, WE NORMALIZE THE CIRCULATION.
>> AND THAT PROCEDURE THAT DOES THAT IS CALLED
THE FONTAN OPERATION.
>> THE PRINCIPLE OF THE FONTAN OPERATION WAS REALLY THAT IF YOU
HAD LOW RESISTANCE IN THE LUNGS, THAT IS,
IF BLOOD COULD PASS THROUGH THE LUNGS WITHOUT MUCH DIFFICULTY,
THEN YOU DIDN'T HAVE TO HAVE A PUMPING CHAMBER PUSHING BLOOD
THROUGH THE LUNGS.
>> HOWEVER, YOU CAN'T DO IT IN A NEWBORN,
THE LUNGS ARE TOO IMMATURE, THE BABIES ARE TOO SMALL.
>> ESSENTIALLY THE PRESSURE IN THE LUNGS IS TOO HIGH--
>>--TO ALLOW BLOOD FLOW TO GO THROUGH WITHOUT SOMETHING
PUSHING IT.
SO WE ALREADY KNEW HOW TO DO A FINAL OPERATION FOR HYPOPLASTIC
LEFT HEART SYNDROME.
THE PROBLEM WAS THE FIRST OPERATION.
>> THE PRINCIPAL ADVANCE, WITH RESPECT TO HYPOPLASTIC LEFT
HEART SYNDROME, TOOK PLACE HERE AT CHOP WITH DR. NORWOOD,
WHO FOUND A WAY TO RECONSTRUCT THIS VERY DIFFICULT SUBSET
OF PATIENTS WHO HAVE SINGLE VENTRICLE,
THOSE WITH HYPOPLASTIC LEFT HEART SYNDROME.
>> AND THAT'S CALLED EITHER THE NORWOOD PROCEDURE OR STAGE I
RECONSTRUCTION.
>> WE LIKE TO DO THAT SURGERY IN THE FIRST WEEK AFTER BIRTH,
BUT IT CAN BE A DAY, TWO DAYS, THREE DAYS,
IT JUST DEPENDS ON HOW STABLE THE BABY IS.
AND THE PRINCIPLES OF THE THAT OPERATION ARE TO CONNECT
EVERYTHING SO THAT THE ONE GOOD PUMPING CHAMBER--
WHICH IN HYPOPLASTIC LEFT HEART SYNDROME IS THE RIGHT VENTRICLE--
THAT VENTRICLE HAS TO PUMP TO THE BODY.
SO YOU HAVE TO CONNECT THE ARTERIES TO THE LUNGS AND THE
BODY IN SUCH A WAY THAT ALL THE BLOOD GOES OUT TO THE BODY
WITHOUT ANY OBSTRUCTION.
>> IN HLHS THE AORTA IS USUALLY TOO SMALL.
SO WE CORRECT THAT BY PUTTING A PATCH ON TO ENLARGE IT.
>> AND THAT'S DONE BY CONNECTING THE PULMONARY ARTERY,
WHICH COMES FROM THE RIGHT VENTRICLE,
TO THE AORTA AND THEN USUALLY PUTTING A PATCH ON TO FINISH THE
CONNECTION OF THOSE TWO BLOOD VESSELS AND ENLARGE THE AORTA.
>> IN ADDITION, YOU HAVE TO PREVENT BLOOD FROM BACKING UP
INSIDE THE LEFT SIDE OF THE HEART,
SO YOU HAVE TO CUT OUT THE PARTITION BETWEEN THE TWO UPPER
CHAMBERS OF THE HEART.
AND THEN THE THIRD PRINCIPLE OF THE NORWOOD OPERATION,
OR THE FIRST STAGE OPERATION, IS TO PROVIDE SOME RESTRICTED BLOOD
FLOW TO THE LUNGS.
>> WHAT WE CALL PULMONARY BLOOD FLOW.
AND WE DO THAT BY PLACING A SHUNT, OR A SMALL TUBE,
FROM ONE OF THE SYSTEMIC ARTERIES, OR ARTERIES THAT GOES
FROM THE HEART TO THE BODY,
TO THE PULMONARY ARTERY.
>> THAT IS ENOUGH FLOW THAT YOU CAN GET ENOUGH OXYGEN IN THE
BLOODSTREAM BUT NOT SO MUCH FLOW THAT YOU HAVE RESISTANCE
PROBLEMS.
BECAUSE THAT RESISTANCE IS WHAT PREVENTS YOU FROM DOING ANY
FURTHER SURGERY.
>> IT'S VERY, VERY IMPORTANT THAT THE FAMILY FEELS
COMFORTABLE WITH YOU AS AN INDIVIDUAL TAKING THEIR CHILD
FROM THEM AND THAT THE CHILD RECOGNIZES THAT NOTHING BAD IS
GOING TO HAPPEN TO THEM, THAT THEY CAN DO THIS WITHOUT BEING
AWAKE FOR ANY PAINFUL EXPERIENCE.
>> THEY'LL GO TO THE OPERATING ROOM.
THE ANESTHESIOLOGIST WILL PUT THEM TO SLEEP.
WE'LL THEN CLEAN, PREPARE EVERYTHING, THEIR CHEST
AND ABDOMEN STERILELY SO THAT WE CAN DO THE OPERATION
WITHOUT INFECTION.
IT'S PERFORMED THROUGH AN INCISION IN THE FRONT OVER THE
BREASTBONE, AND WE PUT THEM ON THE HEART-LUNG MACHINE,
AND COOL THEM DOWN TO TAKE CARE OF THEIR BRAIN AND OTHER ORGANS
WHILE WE DO THE REPAIR.
>> WE'RE BYPASSING THE HEART AND THE LUNGS.
SO THAT THE OPERATIVE FIELD, THE AREA WHERE WE'RE WORKING,
IS CLEAN, AND WE CAN SEE WHAT'S GOING ON.
THE HEART'S GENERALLY NOT BEATING,
ALTHOUGH OCCASIONALLY IT IS, BUT AT LEAST IT'S DECOMPRESSED,
THERE ISN'T BLOOD FLOWING THROUGH IT.
>> WE'LL THEN DO THE OPERATION, WARM THEM UP,
BRING THEM OFF THE HEART-LUNG MACHINE.
WE'LL MAKE SURE THAT THE HEART IS WORKING OKAY,
THAT THE BLOOD PRESSURE IS OKAY.
THAT THERE'S ENOUGH OXYGEN IN THE BLOOD,
THAT THERE'S NO BLEEDING.
AND THEN USUALLY WE LEAVE A COUPLE LITTLE TUBES INSIDE
THE HEART THAT LET US MEASURE PRESSURES IN THE HEART
AND GIVE DRUGS.
THESE COME OUT THROUGH THE SKIN.
THERE'S ALSO USUALLY TWO, LITTLE BLUE PACING WIRES WHICH LET US
CHANGE THE HEART RHYTHM.
WE THEN LEAVE A DRAINAGE TUBE, AND ONCE EVERYTHING'S STABLE,
THE BABY WILL COME BACK UP TO THE INTENSIVE CARE UNIT,
AND THE OPERATION USUALLY TAKES ABOUT 3 1/2 TO 4 HOURS.
>> WE ANTICIPATE THE HOSPITAL STAY AFTER SURGERY FOR
HYPOPLASTIC LEFT HEART SYNDROME TO BE APPROXIMATELY TWO WEEKS.
IT CAN BE LONGER SOMETIMES THREE OR FOUR WEEKS OR EVEN LONGER
IF THERE ARE SIGNIFICANT COMPLICATIONS.
>> SHE PULLED THROUGH THAT WITH FLYING COLORS.
SHE WAS IN AND OUT OF THE HOSPITAL IN A WEEK AFTER
HER SURGERY.
HER SECOND SURGERY WAS WHEN SHE WAS THREE AND A HALF MONTHS OLD.
>> ONCE A BABY HAS HAD THE NORWOOD PROCEDURE,
THEN EVENTUALLY WE'RE GOING TO GO TO THE FONTAN OPERATION.
AND IN THE FONTAN OPERATION WHAT WE DO IS WE CONNECT UP THE BLOOD
FLOW COMING BACK FROM THE BODY DIRECTLY TO THE LUNGS.
YOU CAN DO IT IN ONE OPERATION, BUT WE'VE LEARNED THAT BABIES DO
MUCH BETTER IF WE SPLIT IT UP, AND DO IT IN TWO OPERATIONS.
>> WHEN YOU HAVE THIS SINGLE VENTRICLE MALFORMATION,
ALL THE BLOOD THAT GOES TO THE LUNGS COMES BACK TO THE HEART
GOES OUT TO THE BODY AND A PORTION TO THE LUNGS AGAIN
AND THE AMOUNT THAT IS GOING TO THE LUNGS IS AN EXTRA AMOUNT
OF BLOOD THAT THE HEART HAS TO PUMP.
THE SECOND STAGE OPERATION, DONE SOMEWHERE BETWEEN 3 AND 6 MONTHS
OF AGE GENERALLY, TAKES AWAY THAT EXTRA VOLUME THAT THE HEART
HAS TO PUMP, AND IT DOES THAT BY GETTING RID OF THE SHUNT
OR GETTING RID OF THE BLOOD FLOW TO THE LUNGS DIRECTLY FROM THE
HEART AND CONNECTING THE VEINS FROM THE UPPER PART OF THE BODY
DIRECTLY TO THE ARTERIES TO THE LUNGS.
>> YOU HAVE TO WAIT UNTIL THE LUNGS ESSENTIALLY MATURE ENOUGH
THAT YOU CAN PROVIDE A DIFFERENT SOURCE OF PULMONARY BLOOD FLOW.
>> THIS IS CALLED EITHER A BIDIRECTIONAL GLENN SHUNT
OR A HEMI-FONTAN OPERATION, HALF OF THE FONTAN OPERATION.
>> WE GO BACK THROUGH THE SAME INCISION.
AND THIS IS SORT OF THE RISKY PART OF A REDO OPERATION
BECAUSE THERE'S SCARRING FROM THE FIRST OPERATION,
AND SO WE HAVE TO BE VERY CAREFUL WHEN WE GO THROUGH
THE BREASTBONE, THAT WE DON'T DAMAGE THE HEART,
THE LUNGS, THE AORTA.
AND EVERY NOW AND THEN THERE CAN BE BLEEDING FROM THE HEART JUST
BECAUSE OF THE SCAR TISSUE AND WE HAVE TO GO IN THE HEART-LUNG
MACHINE THROUGH OTHER BLOOD VESSELS EITHER IN THE NECK
OR IN THE GROIN.
IT'S RARE TO NEED TO DO THAT, BUT WE'RE ALWAYS READY TO DO
THAT, IF WE HAVE TO.
>> ONCE WE GET TO THAT SECOND OPERATION,
THE HEART IS IN A MUCH BETTER CONDITION AND USUALLY THE RISK
OF THAT SURGERY IS EXTREMELY LOW AND THE RISK AFTER THAT SURGERY
BECOMES QUITE LOW ALSO OF ANY SUDDEN EVENT.
SO I ALWAYS BREATHE A SIGH OF RELIEF WHEN WE GET TO THAT
SECOND OPERATION OR THAT SECOND STAGE PROCEDURE BECAUSE IT'S
A MUCH BETTER PHYSIOLOGIC SITUATION FOR THE HEART.
>> SHE DID WELL WITH THAT PROCEDURE AS WELL.
SHE WAS IN AND OUT OF THE HOSPITAL IN FIVE DAYS.
IT WAS A LITTLE EASIER IN THAT YOU KNOW WHAT TO EXPECT OF THE
ROUTINE OF THE HOSPITAL THE STAFF THAT YOU'RE WORKING WITH.
>> THE THIRD STAGE OPERATION IS A VARIANT OF THE FONTAN
OPERATION.
>> AND THAT GENERALLY TAKES PLACE SOMEWHERE AFTER TWO YEARS.
BUT THE CHILD'S PHYSIOLOGY REALLY TELLS US THE BEST TIME
TO DO THAT.
>> HER THIRD SURGERY WAS WHEN SHE WAS ALMOST 3 1/2 YEARS OLD.
>> THIS LAST ONE WAS DEFINITELY TOUGHER.
SHE WAS WELL AWARE OF WHAT WAS GOING ON--WHAT WAS COMING
TO HER.
AND, I MEAN, SHE STILL TALKS ABOUT IT TODAY.
>> SO IT WAS HARD FOR US LEADING UP TO THAT THIRD SURGERY.
>> THERE ARE TWO TYPES OF FONTAN OPERATIONS.
ONE IS CALLED THE LATERAL TUNNEL FONTAN IN WHICH A BAFFLE IS
PLACED INSIDE THE HEART TO REDIRECT THE BLOOD FLOW
FROM THE LOWER PART OF THE BODY TO THE LUNGS.
THE OTHER IS CALLED THE EXTRACARDIAC FONTAN WHERE WE
ACTUALLY USE A TUBE THAT BRINGS THE BLOOD OUTSIDE THE HEART UP
TO THE PULMONARY ARTERIES.
>> BUT THE FONTAN OPERATION IS BASED ON CONNECTING THE VEINS
FROM THE BODY DIRECTLY TO THE ARTERIES TO THE LUNGS.
>> NORMALLY, THE INFERIOR VENA CAVA, WHICH DRAINS
THE BOTTOM OF THE BODY, ATTACHES TO THE HEART AT THE INFERIOR,
OR BOTTOM PORTION.
AND WHAT WE DO IS TO LITERALLY DIVIDE THIS FROM THE HEART.
WE SEW UP THE PORTION OF THE HEART WHERE THE INFERIOR VENA
CAVA ENTERED, AND THEN WE SEW A TUBE END TO END TO THE REMNANT
OF THE INFERIOR VENA CAVA.
WE TAKE THIS TUBE AND LOOP IT AROUND THE SIDE OF THE HEART
AND SEW IT INTO A HOLE WE MAKE INTO THE PULMONARY ARTERY.
SO WE ESSENTIALLY BYPASS THE ENTIRE HEART.
>> IT'S A MORE EFFICIENT CONNECTION BECAUSE THE TUBE
IS A VERY SPECIFIC SIZE AND THE BLOOD GOING THROUGH THERE
IS NOT TURBULENT.
TURBULENCE LOSES POWER.
AND POWER LOSS IS A PROBLEM IF YOU DON'T HAVE ANYTHING PUSHING
BLOOD THROUGH THE LUNGS.
IT CANNOT WORK UNLESS THERE'S LOW RESISTANCE IN THE LUNGS.
AND THE KEY IS YOU HAVE TO HAVE A GOOD PUMPING CHAMBER ON THE
OTHER SIDE WHICH ESSENTIALLY SORT OF SUCKS BLOOD THROUGH
THE LUNGS.
>> NOW THE ONE MODIFICATION IS THAT WE PUT A SMALL HOLE IN THE
SIDE OF THE HEART AND IN THE SIDE OF THE GRAFT AND SEW IT
TOGETHER WITH WHAT'S CALLED A FENESTRATION.
>> AND THE REASON WE DO THAT IS THAT IT'S BEEN SHOWN THAT HAVING
THAT LITTLE HOLE, WHILE IT DOES ALLOW SOME BLUE BLOOD TO MIX
WITH THE RED BLOOD, IT ALSO DECREASES THE RISK OF DEVELOPING
FLUID AROUND THE LUNGS AFTER SURGERY--SOMETHING CALLED A
PLEURAL EFFUSION.
EFFUSIONS USED TO BE THE BIGGEST PROBLEM AFTER THE FONTAN
OPERATION.
CHILDREN WOULD HAVE THE SURGERY AND DO QUITE WELL,
BUT THEY WOULD DEVELOP A LOT OF FLUID THAT COLLECTED AROUND
THE LUNG, AND THEY'D OFTEN HAVE TO HAVE A TUBE IN THE CHEST
FOR WEEKS OR EVEN MONTHS TO DRAIN THAT FLUID.
NOWADAYS, WITH THE USE OF THIS FENESTRATION,
THAT IS A VERY UNCOMMON EVENT, WHEREAS IT USED TO BE ROUTINE.