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>> HELLO, MY NAME IS DR. SHARI WELLEN.
I'M A PEDIATRIC CARDIOLOGY FELLOW AT
THE CHILDREN'S HOSPITAL OF PHILADELPHIA.
THE RESEARCH I'M PRESENTING AT CARDIOLOGY 2012
IS ON TWIN-TWIN TRANSFUSION SYNDROME.
TWIN-TWIN TRANSFUSION SYNDROME IS A DISEASE
OF MONOCHORIONIC, DIAMNIOTIC TWIN GESTATIONS IN WHICH
THERE'S A SHARE OF PLACENTAL MASS WITH VASCULAR
COMMUNICATIONS THAT ALLOW FOR AN IMBALANCE
OF BLOOD VOLUME BETWEEN THE TWIN PAIR.
THIS RESULTS IN A SMALLER DONOR TWIN WITH OLIGURIA
AND OLIGOHYDRAMNIOS AND A LARGER RECIPIENT TWIN WITH POLYURIA
AND POLYHYDRAMNIOS.
IN ADDITION TO THE IMBALANCE OF BLOOD VOLUME,
IN RESPONSE TO THE OLIGURIA, THE DONOR TWIN RELEASES
A SERIES OF HORMONAL AND VASO-ACTIVE MEDIATORS
THAT GET TRANSFERRED ALONG WITH THE BLOOD VOLUME
TO THE RECIPIENT TWIN.
AND THESE COLLECTIVELY RESULT IN A SERIES
OF CARDIAC CHANGES IN THE RECIPIENT TWIN.
ANYTHING FROM RIGHT VENTRICULAR HYPERTROPHY
AND SYSTOLIC DYSFUNCTION TO MORE SEVERE CARDIOMYOPATHY.
AND IN A SUBSET OF PATIENTS WE SEE THE DEVELOPMENT
OF ABNORMALITIES IN THE PULMONARY VALVE ITSELF,
WITH EITHER ABNORMAL GREAT VESSEL SIZE RELATIONSHIP
WITH THE PULMONARY VALVE SMALLER IN SIZE OR EQUAL
TO THE AORTA.
OR ACTUAL RIGHT VENTRICULAR OUTFLOW TRACT OBSTRUCTION
WITH THE DEVELOPMENT OF PULMONARY STENOSIS OR ATRESIA.
IF UNTREATED, THE MORTALITY WITH THIS DISEASE PROCESS
IS GREATER THAN 80%, BUT THERE'S BEEN THE DEVELOPMENT
OF SELECTIVE FETOSCOPIC LASER PHOTOCOAGULATION,
MAKING THIS ONE OF THE FEW FETAL DISEASE STATES
IN WHICH WE CAN PERFORM A SUCCESSFUL
THERAPEUTIC INTERVENTION.
AND THAT INVOLVES A DIRECT INTERRUPTION OF THE VASCULAR
COMMUNICATIONS WITHIN THE PLACENTA.
AND SO IN THIS STUDY WE LOOKED SPECIFICALLY
AT THE SUBSET OF PATIENTS THAT HAD PULMONARY VALVE
ABNORMALITIES BEFORE LASER THERAPY TO SEE HOW THEY
RESPONDED AFTER LASER THERAPY,
AND DETERMINE HOW TO FURTHER FOLLOW THESE PATIENTS
AND PLAN POSTNATALLY.
AND WE FOUND 28 PATIENTS OVER A TWO-YEAR PERIOD THAT
HAD ABNORMALITIES IN THE PULMONARY VALVE AND LOOKED
AT THEM AS SEPARATE GROUPS.
THE GROUP WITH THE PULMONARY VALVE SMALLER THAN THE AORTA,
EQUAL TO THE AORTA, OR ACTUAL RIGHT VENTRICULAR
OUTFLOW TRACT OBSTRUCTION WITH PULMONARY STENOSIS
OR ATRESIA.
AND IN THE GROUP IN WHICH THERE WAS A PULMONARY VALVE
SIZE DISCREPANCY WHERE IT WAS SMALLER OR EQUAL
TO THE AORTA, AFTER LASER THERAPY THERE WAS COMPLETE
NORMALIZATION OF THE PULMONARY VALVE TO AORTIC SIZE RATIO.
AND IN THE GROUP WITH RIGHT VENTRICULAR OUTFLOW TRACT
OBSTRUCTION THERE WAS NEVER NORMALIZATION.
SO WHAT THIS SHOWS IS THAT WE DO HAVE A SUCCESSFUL
THERAPEUTIC STRATEGY AND THAT A SUBSET OF PATIENTS
WILL RESPOND TO LASER THERAPY WITH NO FURTHER
INTERVENTION NEEDED BEYOND FOLLOW UP
IN THE PRENATAL PERIOD.
BUT THAT PATIENTS THAT HAVE PULMONARY STENOSIS
OR ATRESIA, THERE MAY BE THE NEED FOR--
THERE'S NEED FOR CONTINUED PRENATAL SURVEILLANCE
AND POSTNATAL EVALUATION AND POSSIBLE EARLY INTERVENTION.
THANK YOU.