Early diagnosis of congenital cardiac

defects and insurance of the necessary

treatment before the transfer to a
specialized centre
Paola Cogo
Ospedale Pediatrico Bambino Gesù
Rome
 Outlines
• Fetal circulation physiology and newborn transitional phase
• Fetal circulation physiology in congenital heart diseases (CHD)
• Comorbidities that could interfere with the newborn outcome
• CPR in newborns with CHD
• Prostaglandin E1
• Dose & when to start
• Side effects
• Clinical management
Fetal circulation
Transitional phase
Prenatal diagnosis in newborns with CHD

1. Improves the preoperative clinical

stability, thus improving clinical and
neurological outcome.
2. Gives more information on the fetal
circulation and on the consequences that
could impair the transitional phase.
 Fetal circulation physiopathology in CHD
Flow alteration (volume & pattern) through cardiac
chambers and vessels
Left ventricular outflow tract obstruction (LVOTO)
Right ventricular outflow tract obstruction (RVOTO)
Alteration on oxygen delivery to the body organs
Parallel circulation (Transposition of the great arteries)
Increased central venous pressure
Ebstein anomaly
Systemic flow obstruction (LVOTO)
Hypoplastic Left Heart Syndrome (HLHS)

IUGR

Microcephaly
 HLHS

At birth

Duct dependent systemic circulation->the systemic

flow depends on the duct diameter and on the ratio
between systemic and pulmonary vascular resistance

Patent foramen ovale (PFO) for the mixing of the

venous return in the right atrium
 RVOTO (Duct dependent pulmonary circulation)
Pulmonary atresia

Ductus arteriousus is usually narrow,

dysplastic and tortuous
 Anatomia e Fisiopatologia
Pulmonary atresia

At birth:

Pulmonary dependent duct circulation with

narrow duct

PFO for the mixing of the pulmonary

venous return
Alteration on oxygen delivery to the body organs
Parallel circulation: Transposition of the great arteries

IUGR

Microcephaly

Restrictive PFO

Persistent Fetal Circulation

 Alteration on oxygen delivery to the body organs
Transposition of the great arteries
At birth

1. PFO to allow the blood mixing between the two atria

2. PFO can be restrictive

3. Persistent ductus arteriousus

4. If inadequate mixing between the atria

5. PERSISTENT FETAL CIRCULATION

Increased central venous pressure: Ebstein anomaly
1. Restrictive PFO or narrow PDA,
2. Tricuspid insufficiency,
3. Miocardial dysfunction HYDROPS
4. arrhytmia

• Low plasma albumin concentration

• Capillary leak
• Prolonged refill time
• Reduced lymphatic drainage
 Fetal circulation physiopathology in CHD
Flow alteration (volume & pattern) through cardiac
chambers and vessels
Left ventricular outflow tract obstruction (LVOTO)
Right ventricular outflow tract obstruction (RVOTO)
Alteration on oxygen delivery to the body organs
Parallel circulation (Transposition of the great arteries)
Increased central venous pressure
Ebstein anomaly
Cardiac emergencies during the first few days
of life
• Delivery room (or within one hour after birth)
• Associated with comorbidities
• Associated with arterious duct closure
Cyanosis &/or LCOS
 Neonatal emergencies in the delivery room

• Transposition of the great arteries with intact ventricular septum and

restrictive PFO
• Emergent atrial septostomy
• HLHS with intact atrial septum
• Emergent atrial septostomy
• Obstructed total anomalous pulmonary venous return (O-TAPVR)
• Emergent cardiac surgery
Tetralogy of fallot with absent pulmonary valve
• Emergent intubation
• Congenital AV block
• Isoproterenol and emergent pacemaker
Neonatal CPR
•10% of newborns requires neonatal
assistance at birth
•1% CPR
•Same percentage for CHD
•Same neonatal guidelines
Neonatal CPR sequence

• Airways aspiration
• O2
• Mask ventilation (Neopuff-Ambu)
• Intubation
• Chest compression
• Adrenaline
• Volume if hypovolemic shock
 Neonatal resuscitation
1.Ventilation on room air
2.Monitor transcutaneous saturation
3.95% after 12 minutes in newborns with no
CHD
4.80-85% in newborns with univentricular
hearts
Cardiac emergencies during the first few days
of life
• Delivery room (or within one hour after birth)
• Associated with comorbidities
• Associated with arterious duct closure
Comorbidities that can interfere with the clinical outcome

• Cesarean section
• Prematurity
• Twins
• Meconium inhalation syndrome
• Sepsis
• Associated malformation
• Brain
• GI tract
• Asphyxia
Comorbidities
 Cesarean section
Newborns
• Disadvantages Newborn with CHD
Respiratory distress
CS=6.2%
Physiol Delivery=3.3%
• Respiratory distress
• Advantages increases the risk of
Clinical plan hypoxia and PPHN
Reduced incidence of asphyxia expecially if cyanosis is
trauma and meconium inhalation
syndrome associated
 Prematurity
Preterm delivery
•Mortality (12% <32 SG) Newborn with CHD

•Morbidity (cerebral palsy 12-15%) Try to prevent preterm delivery

•Respiratory distress (87%) •Use corticosteroid prophylaxis
•Intracranial hemorrhage (8,9%)
•Increased risk for cardiac surgery and
•Periventricular leucomalacia
(3,4%) interventional cardiology
•Increased risk of infection(18,9%)
•Necrotizing Enterocolitis(4,3%)
•Asphyxia
 Twins Newborn with CHD
Newborn Increased risk of organs’
Increased risk: immaturity
• Severe Prematurity
(at least 2 weeks of gestational
•Low birth weight (IUGR)
age delay
•Malformations
•Mortality Twin-twin transfusion
•Morbidity syndrome
cerebral palsy 5-10 times 5-15% in monochorionic
more frequent
twins
 Sepsis
CHD newborn
Sepsis
•More hemodynamic instability
•Incidence: 0,5-2 /1000 birth
•Increased risk of pulmonary
•mortality 10%
hypertension
•meningitis 10%
•Septic shock •Increased risk of endocarditis
prematurity, IUGR gram negative
sepsis
Increased risk of neurological •Increased risk of neurological
injury complications
 Meconium inhalation syndrome (MAS)

Newborn CHD newborn

•Pneumonia •Increased hemodynamic
incidence 5%
instability
•PPHN (30%)
•Pneumothorax (15- •Prenatal prevention
30% )
•Adequate CPR with
•Sepsis
airways aspiration in the
•Asphyxia
delivery room
 Malformations

Newborn CHD Newborn

•Surgical complications
•Thorax
•Increased risk of NEC
•GI
•Surgical priority
Cardiac emergencies during the first few days
of life
• Delivery room (or within one hour after birth)
• Associated with comorbidities
• Associated with arterious duct closure
 Duct closure
Cyanosis &/or LCOS
 Postpartum closure of ductus arteriosus

In newborn with CHD the PDA closure is delayed

• In RVOTO for hypoxia
• In LVOTO for increased pulmonary pressure

Symptoms of LCOS and Cyanosis in neonates with

Critical obstruction right or left heart (inflow/outflow)
Abnormal origin of great vessels (TAPVR, TGA)
 Criterio
debole
Criterio
Anamnesis and clinical evaluation forte
Criterio
molto forte

• Medical hystory: age newborn, pregnancy, family

hystory
• Heart rate, respiratory rate, blood pressure arms and
legs, SpO2 arms and legs (to assess the difference in
SpO2 before and after the duct)
• Hyperoxic test, increased liver size, weak femoral
pulses
• ABG, chest x-ray, EKG, echocardiogram
Diagnostic criteria
Hyperoxia test Age
FiO2 at 100% for 10 min • Severe cyanosis (SpO2
< 85%) in 1-2 days of
•PaO2 > 150 mmHg on post- age -> duct
ductal ABG -> respiratory dependent
cyanosis( respiratory pulmonary circulation
distress) • Moderate cyanosis
•PaO2 < 150 mmHg on (SpO2 90-95%) within
postductal ABG -> cyanosis one week of age-
on cardiac basis ( + distress) >duct dependent
systemic circulation

DOLBEC, CONGENITAL HEART DISEASE

Emerg Med Clin North Am. 2011 Nov;29(4):811-27
 CHD classification.
• Parallel circuit (Duct • Systemic flow duct dependency
dependent) (Duct dependent)
TGA HLHS
• Pulmonary flow duct Interrupted Aortic Arch
dependency (Duct Aortic Coartation
dependent) Critical Aortic Valve stenosis
PAIS • Obstructed total anomalous
PA + VSD pulmonary venous return
Critical PVS • Ebstein Anomaly (Duct
Tetralogy of fallot dependent)
What shall we do??

IF Congenital Heart Disease is SUSPECTED

•START PGE1 INFUSION
•Transport to a pediatric cardiac-surgical center as
soon as possible
 Prostaglandin E1
• 20-30% of CHD require PGE infusion
• To be infused iv
• peripheral vein
• A central venous catheter( umbilical or CVC placed under
sterile condition)
• Initial dose 50-100 ngr/kg/min
• Maintenance dose 10 ngr/kg/min
• Side effects
• Apnea 19%
• Hypotension 6.5%
• Fever 1.6%
• Cutaneous rush 1.6%
 Prostaglandin and neonatal transport
Carefull evaluation of the respiratory pattern
IF
• Infusion started at least for 30 minutes
• No sedation
• No comorbidities

NO ELECTIVE INTUBATION FOR TRANSPORT

Consider prophylaxix with caffeine 20 mg/kg bolus followed

by 5 mg/kg twice a day
 Take-home message
Critical newborn with
• LCOS
• Cyanosis

Suspect ductus-dependent perfusion!

• Start PGE 1
• ABC

Transfer
GRAZIE!!!
RAZ I E
G