>> WHAT WE WANT TO DO AFTER A BABY IS BORN IS WE WANT TO
STABILIZE THE CHILD, MAKE SURE THAT THERE ARE NO OTHER
ORGANS INVOLVED, BECAUSE THERE ARE OTHER ASSOCIATED
ANOMALIES IN SOME CHILDREN.
WE WANT TO MAKE SURE THAT THERE ARE NO GENETIC ISSUES
THAT WE NEED TO ADDRESS.
WE WANT TO MAKE SURE ALL THE OTHER ORGAN SYSTEMS ARE AS
GOOD AS THEY CAN BE AND ASSESS THEM IF WE HAVE TO.
AND THEN ONCE THAT IS DONE, THEN IT'S MORE A MATTER
OF SCHEDULING THE SURGERY.
>> FOR MANY CHILDREN WITH COMPLEX HEART DISEASE,
THERE'S BLOCKAGE OF THE BLOOD,
EITHER GOING TO THE BODY OR TO THE LUNGS.
AND THERE'S A BLOOD VESSEL CALLED THE DUCTUS ARTERIOSUS
WHICH CONNECTS THOSE TWO ARTERIES.
EVERYBODY HAS ONE, AND IT USUALLY CLOSES AFTER BIRTH.
>> YOU CAN KEEP THAT BLOOD VESSEL OPEN WITH
A MEDICATION CALLED PROSTAGLANDINS.
AND SO, THEREFORE, BY GIVING A BABY PROSTAGLANDINS,
YOU'RE ACTUALLY REPLICATING THE SAME PHYSIOLOGY THAT IT
HAD IN THE UTERUS, WHEN IT WAS VERY STABLE,
TO WHEN IT'S OUTSIDE THE UTERUS.
>> SO INSTEAD OF HAVING A CHILD COME IN CRITICALLY ILL,
WHERE THERE WAS NOTHING WE COULD DO,
WE CAN STABILIZE THESE CHILDREN,
DO DIAGNOSTIC PROCEDURES, AND THEN HAVE A STABLE CHILD
WHO GOES FOR SURGERY.
>> IF YOU JUST LEAVE A CHILD WITH THIS CIRCULATION
FOR A PERIOD OF TIME, THEN THEY END UP GOING INTO HEART
FAILURE BECAUSE SO MUCH BLOOD GOES TO THE LUNGS
IN A CIRCLE THAT THEY END UP IN HEART FAILURE.
SO YOU HAVE TO WALK A BALANCE BETWEEN THE TWO.
YOU HAVE TO HAVE THE DUCTUS OPEN AND EVENTUALLY CONTROL
HOW MUCH BLOOD GOES TO THE LUNGS.
THAT'S WHY THE OPERATION IS DONE USUALLY IN THE FIRST
WEEK OF LIFE.
>> THROUGH OUR EXPERIENCE WITH HYPOPLASTIC LEFT HEART
SYNDROME IN THE FETUS, WE'VE BEEN ABLE TO IDENTIFY
A VARIETY OF FACTORS WHICH FUNCTION AS RISK FACTORS
FOR OUTCOME FOR THESE PATIENTS.
>> ONE OF THE THINGS THAT CAN CAUSE A REAL PROBLEM
IS WHEN THERE'S BLOCKAGE TO BLOOD FLOW FROM THE LUNGS
GETTING BACK TO THE HEART.
>> THERE ARE RARE CHILDREN WHO HAVE WHAT'S CALLED
AN INTACT ATRIAL SEPTUM, AND THESE CHILDREN ARE THE MOST
DIFFICULT TO DEAL WITH WITH HYPOPLASTIC LEFT
HEART SYNDROME.
>> IN HYPOPLASTIC LEFT HEART SYNDROME,
THE BLOOD THAT COMES BACK FROM THE LUNGS COMES
TO THE LEFT SIDE OF THE HEART.
BUT BECAUSE THERE'S ONLY ONE PUMPING CHAMBER,
IT HAS TO CROSS OVER TO THE RIGHT SIDE OF THE HEART
INSIDE THE HEART TO BE ABLE TO PUMP OUT.
THAT'S NORMALLY DONE THROUGH A HOLE IN THE WALL BETWEEN
THE TWO UPPER CHAMBERS OF THE HEART CALLED A FORAMEN
OVALE.
SOME CHILDREN WE SEE WHERE THERE'S A VERY TINY HOLE
AND THE HOLE IS RESTRICTIVE.
SOMETIMES WE CAN SEE WHERE THERE IS NO HOLE,
AND THE ATRIAL SEPTUM IS COMPLETELY CLOSED.
>> THIS COMPLETE CLOSURE OF THE PARTITION BETWEEN
THE UPPER CHAMBERS OF THE HEART MEANS THAT BLOOD THAT GETS
BACK TO THE LEFT SIDE OF THE HEART CAN'T GO ANYWHERE.
>> THAT RESULTS IN AN INCREASED BACK-UP PRESSURE
ON THE PULMONARY VEINS.
>> SO RIGHT AFTER BIRTH, WE HAVE TO DO SOMETHING VERY
QUICKLY TO OPEN UP THAT PARTITION.
>> AND THE WAY THAT'S DONE IS BY PASSING A CATHETER
ACROSS THE ATRIAL SEPTUM, BLOWING A SMALL BALLOON UP--
>> AND YOU PULL IT BACK VERY QUICKLY,
AND YOU LITERALLY RIP A HOLE BETWEEN THE TWO
UPPER CHAMBERS.
THAT ALLOWS THE BLUE AND THE RED BLOOD TO MIX NOW,
AND THEN THEY GET-- THE CHILDREN BECOME MUCH PINKER.
THEY HAVE OXYGEN IN THEIR BLOODSTREAM,
AND THEY'RE STABILIZED SO THAT THEY CAN BASICALLY
SURVIVE UNTIL THEY CAN HAVE SURGERY.
>> AND THAT CAN ALLOW THE RESISTANCE IN THE LUNGS TO
DROP ENOUGH TO CONSIDER THE STAGE I OPERATION,
USUALLY AFTER FIVE OR SIX DAYS.
>> OFTENTIMES, EVEN WHEN THESE BABIES ARE BORN
AND WE IMMEDIATELY OPEN THE ATRIAL SEPTUM,
THERE CAN ALREADY BE DIFFICULTIES WITH THE WAY
THE LUNGS HAVE FORMED.
AND THAT CAN ADD A RISK TO THESE PATIENTS.
WE KNOW ALSO FROM OUR EXPERIENCE THAT IF THERE
ARE ANY EXTRA CARDIAC ABNORMALITIES BESIDES
THE HEART DISEASE ITSELF, THAT CAN ADD RISK TO
THE RECONSTRUCTION OF THE HEART IN HLHS.
AND WE ALSO KNOW THAT IF THERE'S A KNOWN GENETIC
ABNORMALITY, THAT CAN ADD RISK.
AND THEN FINALLY, THERE'S THE ISSUE OF PREMATURITY.
ALTHOUGH WE'RE NOW ABLE TO CARE FOR BABIES WITH HLHS
VERY, VERY WELL THAT ARE VERY SMALL,
IF THERE IS SIGNIFICANT PREMATURITY AND IMMATURITY,
OR LACK OF NORMAL DEVELOPMENT OF THE LUNGS
AND OTHER ORGAN SYSTEMS, THAT CAN ADD SIGNIFICANT RISK
TO THE OVERALL OPERATION.
>> SO IF AN AMNIOCENTESIS TEST HASN'T BEEN DONE
OR A CHORIONIC VILLUS SAMPLING TEST HASN'T BEEN DONE PRIOR
TO THE PATIENT BEING REFERRED,
THEN WE CAN HELP TO COORDINATE THAT,
EITHER WITH THE REFERRING DOCTOR,
OR WE CAN TAKE PRIMARY RESPONSIBILITY FOR GETTING
THAT TESTING DONE HERE.