Complex congenital heart disease

Perinatal meeting Dr Jancy Dr Eman

Dr Prakash.I Dr Sameer

Perinatal management of congenital heart

defect

Birth History Delivered by elective section

Cried at birth

APGAR Score 7 and 8 at 1 and 5 min respectively

Needed intubation at 30 minute of life in NICU

On admission to NICU Growth Parameters: Weight: 2.85kg, Height:48 cm, Head

Circumference: 33.5 cm

Vital signs: Temperature: 36.6’C, Pulse Rate: 168bpm, VRR: 55/min, Blood pressure: 88/47 mmHg ,MAP: 60 mmHg, Oxygen Saturation: pre ductal 60%, post ductal 35%

General Examination: Cyanosed, poor perfusion Examination of Systems: Chest: good air entry bilaterally

CVS: systolic murmur +nt, hyper dynamic precordium

Abdomen: Soft, no organomegaly

Baby on mechanical ventilation, with guarded general condition

Preductal saturation 60%, Postductal saturation 40%

Abnormal movement within 2 hrs, so anticonvulsant was given.

IV antibiotics started (septic screening done)

Investigations: WBC: 16.8, Hb: 17.2 gm%, HCT: 52.4, PLT: 227Blood culture: SterileCXR :……….

Pediatric cardiology consult was taken

2D ECHO done

Inj Prostaglandin E1 started

Transferred to SKMC for further management

At SKMCOn day 2 –surgical repair was done

SpO2: 60-70s

BP: borderline

Deteriorated further on day 3 and died on day 3

Congenital heart defect

Prevalence of CHD: at birth -6 to 13 /1000 live birth

Most common: Acyanotic: muscular and perimembranous

VSD, secundum ASD. Cyanotic: TOF

Preterm > Term babies (2-3 times )

Critical CHD: require surgery or invasive intervention in the 1st yr of life

Critical CHD: 25% of those with CHD

Time of presentation Prenatally During birth hospitalization

Maternal risk factors

Maternal diabetes Maternal obesity Maternal hypertension Maternal CHD(family history) Maternal thyroid conditions Maternal drugs intake Smoking in 1st trimester

Congenital infections: CMV, herpes virus, rubella, coxsackie virus

Drugs in pregnancy: hydantoin, lithium alcohol

Assisted reproductive technology (outflow tracts and ventriculoarterial connections)

Congenital complete heart block: maternal connective tissue disorder, anti-Ro/SSA and anti- La/SSB

Family history 1st degree relative has CHD: 3 fold increase

risk

Familial risk of specific malformations is even greater, suggesting stronger genetic effect in these conditions

Relative risk for monozygotic twins:15.2 Dizygotic twins: 3.3

Risk in singletons birth with 1st,2nd or 3rd degree relatives with CHD was 3.2,1.8 or 1.1 respectively

PRENATAL USSSensitivity of prenatal screening range from

0-80% detection rates

Sensitivity depends on: GA, maternal weight, fetal position and type of defect

Early serious or life threatening presentation Shock : differentiate with sepsis as a cause. Cardiomegaly is

common finding Cyanosis

Reduced Hb 4-5gm% Associated with metabolic acidosis leading to cardiac

dysfunction and cardiogenic shock. Severe pulmonary edema

Tachypnea ↑ work of breathing

Physical findings Heart rate: 90-160bpm(upto 6 days of age)

SVT VT etc.

Precordial activity Heart sounds (S2,gallop rhythm, clicks) Murmurs (innocent, pathological) Peripheral arterial pulses Cyanosis

Investigations ECG

Chest X ray

Blood gas

Pulse oxymetry ECHO

What we do for screening in LH?

Pulse oxymetry (if abnormal)4 limb BP Blood gas Chest X ray ECG

ECHO (as required)

Pulse oxymetry Cutoff SpO2 <95%: sensitivity of 75% and

specificity of ≥90%

Do not detect Non cyanotic heart disease Left sided obstructive lesions with PDA

GA ≥35 weeks

Timing of screening: <24 hrs Vs ≥24 hrs of life. False positive more in < 24 hr group.

Factors affecting the test :ambient light, probe detachment, poor perfusion, dyshemoglobinaemias, crying, moving.

Decreases the cost

Criteria for positive screenFulfilling one of the following three criterion:

SpO2 <90 percent

SpO2 <95 percent in both upper and lower extremities on three measurements, each separated by one hour

SpO2 difference >3 % between the upper and lower extremities

Patient Reassessment CriteriaIf the saturation is < 95% in the right hand or

foot or >3% difference in right hand or foot repeat the screening after 1 hour.( first attempt)

If the saturation is < 95% in the right hand or foot or >3%difference in right hand or foot after the first attempt repeat the screening again after another 1 hour.(second attempt)

If the saturation remains <95% in the right hand or foot or >3%difference in right hand or foot after the second attempt the baby is considered to have a positive screen.

Newborn screening is directed for following lesions

Hypoplastic left heart syndrome Pulmonary atresia TOF TAPVR TGA Tricuspid atresia Truncus arteriosus

Management General supportive care

Antibiotics

Prostaglandin E1(specific cases)

Cardiac catheterization

Surgical intervention

Thank you