>> OUR CURRENT STRATEGY FOR RECONSTRUCTION OF THE HEART
FOR HLHS INVOLVES THREE STAGES,
ONE DONE INITIALLY AT BIRTH, ONE AT APPROXIMATELY THREE
TO SIX MONTHS OF AGE, AND THE THIRD STAGE
AT APPROXIMATELY TWO TO THREE YEARS OF AGE.
>> THIS IS NOT AN OPERATION THAT CREATES A NORMAL HEART.
>> WE 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.
>> WE FOUND A WAY TO RECONSTRUCT THIS VERY
DIFFICULT SUBSET OF PATIENTS WHO HAD 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 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 BLOOD STREAM 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 IN 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 BREAST BONE.
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 OK,
THAT THE BLOOD PRESSURE IS OK,
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 TO 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 IS STABLE,
THE BABY WILL COME BACK UP TO THE INTENSIVE CARE UNIT,
AND THE OPERATION USUALLY TAKES ABOUT THREE
AND A HALF TO FOUR 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 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 THREE
AND SIX 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 BI-DIRECTIONAL 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 BREAST BONE 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 ON 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 THREE
AND A HALF YEARS OLD.
>> THIS LAST ONE WAS DEFINITELY TOUGHER.
SHE WAS WELL AWARE OF WHAT WAS GOING ON,
WHAT WAS COMING TO HER, AND 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 EXTRA CARDIAC 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 REMNANTS 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 THIS 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 PLURAL 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.