Neonatal Diagnosis
Nursery is the ideal time to diagnose congenital
heart disease (if not prenatally diagnosed) in
order to assure early appropriate care
• Many problems very subtle in early NB period
– Some present after ductus closes (8-48? Hours)
– Some present when PulmonaryVascular Resistance
drops (2-6 weeks)
– Some very minor findings won ’t be obvious for
years (minor coarct, ASD, bicusp AV)
Flow from RV to
descending
Aorta via ductus,
PA’s tiny
Ductal flow
reverses, Branch
PA’s open up
Ductus closes,
Then PVR
drops
(2mos)
Fetal Circulation
8 Hours old
24 hrs
Neonatal Diagnosis
English study: 1590 total pts
• 45  diagnosed prior to d/c
• 20  presented before 6 weeks of age
• 10  at 6wk NB exam
• 24  diagnosed later in 1st year of life
• 1  die of heart disease undiagnosed (2
 Baltimore)
Neonatal Diagnosis
• 50  of babies with murmur in first few days of life
have CHD
• 25  of babies with murmur at 6 weeks have CHD
• Diagnoses most likely to lead to death soon after
discharge: HLH, IAA, Coarctation (they look pink until
ductus closes)
• Some get irreversible pulmonary vascular disease and
can’t be repaired- shortened life
Neonatal Diagnosis
When to get consult on a newborn
• Pathological Murmur
• Cyanosis (sats less than 95)
• Poor pulses/perfusion
– Add where
Neonatal Diagnosis
NY study: Screening for CHD with Pulse Oximetry
• Current newborn screening looks for diseases much rarer
than CHD
• Post ductal saturations on all babies at two hospitals at
time of NB screen. 11,281 babies/1 yr
• If sat 95 echocardiogram done
• Results: 4 abnormal sats
3 CHD (2 TAPVR, 1 Truncus)
1 Pulmonary Htn,
• 1/3760 incidence
Neonatal Diagnosis
• This method of screening will only catch cyanotic
lesions such as…
–
–
–
–
–
Hypoplastic Left Heart
Pulmonary Atresia
D-Transposition of Great Arteries
Total Anomalous Pulmonary Veins
Tricuspid Atresia
• Will not catch coarctation or Aortic Stenosis,
VSD, ASD, pulmonic stenosis
Koppel et al. Pediatrics 2003
Neonatal Diagnosis
• Hospitals locally starting to set up program
– Requires O2 sat screening after 24 hours
– REQUIRES method to do echo (tech, training, and
pediatric Cardiologist to read echo)
• Probably more valuable at facilities where few
patients get fetal echos
Neonatal Surgery
• Who gets it
• Mortality
• Long-term neurological outcomes
Neonatal Surgery
• Who gets it
– Ductal dependant lesions
•
•
•
•
•
•
•
•
Iaa
HRHS
HLHS
Single Ventricle PA
Coarctation/arch hypoplasia
TAPVR
TGA
Truncus Arteriosus
Neonatal Surgery
Mortality
• Congenital heart surgery moving into era of
outcomes research-Can’t just ask for
institutional mortality for CH surgery. Need to
ask what is mortality by risk category for
particular type of patient and surgery.
• 2 systems out there, RACHS and Aristotle score
Jenkins et al. Journal of Thoracic and Cardiovascular Surgery, 2002
Neonatal Surgery
Risk Categories
1= ASD, PDA (>30 d) , coarct (>30 d)
2=ASD/VSD, TOF, Glenn, sub AS
3=AVR, Ross procedure, MVR
4=arterial switch, Truncus arteriosus
5=truncus and interrupted arch
6= Norwood, Damus-Kaye-Stansel
Neonatal Surgery
Mortality
RACHS surgical risk groups and OHSU congenital heart surgery
outcomes (1/01-9/04)
50.00%
45.00%
40.00%
35.00%
30.00%
25.00%
20.00%
15.00%
10.00%
5.00%
0.00%
47.70%
PCCC
OHSU
19.40%
11.40%
8.50%
3.80%
0.40% 0.00%
(0/104) Lowest
(2/304)
(JTCVS 2000, 123:110-8)
8.60%
1.80%
0.60%
Low
Moderate
(5/274)
(8/92)
Risk Category
High
(4/35)Highest
(940 patients)
Neonatal Surgery
Long-term neurological outcomes
• Cognitive and adaptive behavior abnl and
lower than expected at school age for
heart transplant HLHS pts
• Similar for Norwoodglennfontan
Neonatal Surgery
• New study compares TCA vs low flow
cardiopulmonary bypass (with short CA)
in neonates having arterial switch
CardioPulmonary
Bypass
CPB Circuit:
IVC and SVC Cannulae
Aortic Cannula
oxygenator
pump
Heater/Cooler
Rtn
to
Body
Neonatal Surgery
• Total Circulatory Arrest- the body is
cooled by the CPB pump to enable the
body to withstand no blood flow. The
heart is stilled with cardioplegia, the
pump is turned off and the pump
catheters are removed.
Neonatal Surgery
• TCA effect not noticed if less than ~40 min. After
41 min worse outcome longer TCA.
• At 8 years old—Both groups had academic, fine
motor, visual spatial, attention and higher order
thinking than expected for general population. 1/3
in special ed or remedial education
Neonatal Surgery
• TCA-worse manual dexterity, apraxia, V-M
tracking, Handwriting
• Low flow bypass--impulsiveness, worse
behavior
• These results appear to be worse than surgeries
done at greater than 30 days of age… why?
– Neonates have more seizures
– Immature neurons
Bellinger et al. J Thoracic Cardiovascular Surgery 2003
Neonatal Surgery
Advantages
• One surgery
• Less hypoxia
• Maybe shorter LOS
Disadvantages
• Perhaps worse longterm neuro outcome
•Because we can should we?
Treatment in Cath Lab
•
•
•
•
•
•
Atrial Septostomy
Stent Ductus
Ductal Closure
Pulmonary and Aortic Balloon valvuloplasty
ASD, VSD closure
Stent pulmonary arteries
Treatment in Cath Lab
Atrial Septostomy
• Can be done at
bedside
• Use a cutting blade
and balloon or stent
Treatment in Cath Lab
Patent Ductus Arteriosus
Surgery
vs. Coiling
Treatment in Cath Lab
Critical Aortic Stenosis
Treatment in Cath Lab
Critical Pulmonary Stenosis
Treatment in Cath Lab
ASD Device Closure
Treatment in Cath Lab
VSD Closure
• In Phase II trials
Pulmonary Artery Stents
• Inserted for branch
pulmonary artery stenosis
(often a complication of
other surgeries).
• Device positioned then
opened up to allow better
flow distal to device
The End