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)

Ductal flow reverses, Branch

PA’s open upDuctus closes,

Flow from RV to descending

Aorta via ductus, PA’s tiny

24 hrsFetal Circulation 8 Hours old

Then PVR

drops (2mos)

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 SurgeryMortality• 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

0.40% 0.00%

3.80%

0.60%

8.50%

1.80%

19.40%

8.60%

47.70%

11.40%

0.00%

5.00%

10.00%

15.00%

20.00%

25.00%

30.00%

35.00%

40.00%

45.00%

50.00%

Lowest Low Moderate High Highest

PCCCOHSU

(0/104) (2/304)

Risk Category(5/274) (8/92) (4/35)

Mor

tali

ty

RACHS surgical risk groups and OHSU congenital heart surgery outcomes (1/01-9/04)

(JTCVS 2000, 123:110-8) (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 Norwoodglennfontan

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 long-

term 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