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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)
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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)
Flow from RV to descending Aorta via ductus, PA’s tiny Ductal flow reverses, Branch PA’s open up 24 hrs Fetal Circulation 8 Hours old Ductus closes, 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 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 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, 123:110-8)
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
IVC and SVC Cannulae CPB Circuit: Aortic Cannula oxygenator pump Rtn to Body Heater/Cooler
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
Advantages One surgery Less hypoxia Maybe shorter LOS Disadvantages Perhaps worse long-term neuro outcome Neonatal Surgery • 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