Neonatal Diagnosis

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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)

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Flow from RV to descending Aorta via ductus,
PAs tiny
Ductal flow reverses, Branch PAs open up
Ductus closes,
Then PVR drops (2mos)

• Fetal Circulation

• 8 Hours old

• 24 hrs

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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)

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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 cant be repaired- shortened life

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Neonatal Diagnosis

• When to get consult on a newborn
• Pathological Murmur
• Cyanosis (sats less than 95)
• Poor pulses/perfusion
• Add where

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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

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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
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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

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Neonatal Surgery

• Who gets it
• Mortality
• Long-term neurological outcomes

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Neonatal Surgery

• Who gets it
• Ductal dependant lesions
• Iaa
• HRHS
• HLHS
• Single Ventricle PA
• Coarctation/arch hypoplasia
• TAPVR
• TGA
• Truncus Arteriosus

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Neonatal Surgery

• Mortality
• Congenital heart surgery moving into era of
  outcomes research-Cant 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
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Neonatal Surgery

• Risk Categories
• 1 ASD, PDA (30 d) , coarct (30 d)
• 2ASD/VSD, TOF, Glenn, sub AS
• 3AVR, Ross procedure, MVR
• 4arterial switch, Truncus arteriosus
• 5truncus and interrupted arch
• 6 Norwood, Damus-Kaye-Stansel

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Neonatal Surgery
RACHS surgical risk groups and OHSU
congenital heart surgery outcomes (1/01-9/04)
Mortality
(0/104)
(2/304)
(5/274)
(8/92)
(4/35)
Risk Category
(940 patients)
(JTCVS 2000, 123110-8)
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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

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Neonatal Surgery

• New study compares TCA vs low flow
  cardiopulmonary bypass (with short CA) in
  neonates having arterial switch

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CardioPulmonary Bypass
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CPB Circuit
IVC and SVC Cannulae
Aortic Cannula
oxygenator
pump
Rtn to Body
Heater/Cooler
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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.

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Neonatal Surgery

• TCA effect not noticed if less than 40 min.
  After 41 min worse outcome longer TCA.
• At 8 years oldBoth 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

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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
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Neonatal Surgery

• Advantages
• One surgery
• Less hypoxia
• Maybe shorter LOS

• Disadvantages
• Perhaps worse long-term neuro outcome

• Because we can should we?

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Treatment in Cath Lab

• Atrial Septostomy
• Stent Ductus
• Ductal Closure
• Pulmonary and Aortic Balloon valvuloplasty
• ASD, VSD closure
• Stent pulmonary arteries

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Treatment in Cath Lab

• Atrial Septostomy
• Can be done at bedside
• Use a cutting blade and balloon or stent

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Treatment in Cath Lab

• Patent Ductus Arteriosus
• Surgery vs. Coiling

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Treatment in Cath Lab

• Critical Aortic Stenosis

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Treatment in Cath Lab

• Critical Pulmonary Stenosis

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Treatment in Cath Lab

• ASD Device Closure

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Treatment in Cath Lab

• VSD Closure
• In Phase II trials

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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

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The End
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References

• Siu et al. Prospective Multicenter Study of
  Pregnancy Outcomes in Women with heart Disease.
  Circulation 104
• Kumar RK Comparison of outcome when hypoplastic
  left heart syndrome and transposition of the
  great arteries are diagnosed prenatally versus
  when diagnosis of these two conditions is made
  only postnatally. - Am J Cardiol 1999 83
• Mahle et al. Impact of prenatal diagnosis on
  survival and early neurological morbidity in
  neonates with Hypoplastic Left Heart Syndrome
  Pediatrics 107

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References

• Hoehn et al. Assessment of Parental Stress and
  Decision making in critical cong heart disease
  prenatal vs Postnatal diagnosis In publication
• Kuehl et al. Failure to Diagnose Congenital heart
  Disease in Infancy. Pediatrics April 1999.
• Koppel et al. Effectiveness of Pulse Oximetry
  Screening for Congenital Heart disease in
  Asymptomatic Newborns. Pediatrics March 2003
• Bellinger et al. Boston Circulatory Arrest Study.
  J Thoracic Cardiovascular Surgery 2003

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• Jenkins et al. Consensus-based method for risk
  adjustment for surgery for congenital heart
  disease Journal of Thoracic and Cardiovascular
  Surgery, Volume 123, January 2002