The most difficult you could ever have is CVS..And pediatric CVS conditions are a challeng in ER.. So just an overview of CHD for EM physicians..

1. Overview Of Congenital Heart Disease
Runal Shah
MEM-PGY1
KDAH, Mumbai

2. Fetal Cardio-vascular circulation

3. • Congenital heart disease (CHD) is the most common congenital
disorder in newborns.
• Critical CHD, defined as lesions requiring surgery or catheter based
intervention in the first year of life,
• One of the leading causes of infant mortality.
• Urgent consultation/referral to a pediatric cardiologist
• In patients with ductal-dependent cardiac lesions and profound
cyanosis, Prostaglandin E1 (Alprostadil) Infusion To Maintain Patency
of Ductus Arteriosus
 Neonates <28 days old: 0.05-0.1 mcg/kg/min IV initially; usual
maintenance ranges from 0.01-0.4 mcg/kg/min
 Preferably administer via large vein; alternatively, administer through
umbilical artery catheter (S/E Apnea)

4. Duct – dependent lesions
• The affected neonate may not be symptomatic during the birth
hospitalization because the Ductus Arteriosus has not yet closed
prior to discharge.
• The lesions that were not diagnosed prior to discharge were primarily
ductal dependent and included :
 Coarctation of the aorta (COA)
 Interrupted aortic arch
 Aortic stenosis
 Hypoplastic left heart syndrome (HLHS)
 Transposition of the great arteries

5. • with ductal-dependent lesion, may appear normal with either
no or very subtle signs and symptoms during the birth
hospitalization.
• As a result, the diagnosis of critical CHD may be missed prior
to discharge.
• In these infants, closure of the Ductus arteriosus may
precipitate rapid clinical deterioration that may be life-threatening.

6. Duct Dependent Circulation
 To Supply Systemic Circulation :
• Interrupted Aortic Arch
• CoA
• HLHS
 To Supply Pulmonic Circulation :
• Critical PS
• Pulmonary Atresia
• Severe Ebstein Anomaly
 For Mixing of Circulation :
• TGA with Intact IVS

7. Shock
• In Left heart obstructive lesions (e.g., HLHS, Critical AS, CoA, and
Interrupted AA), systemic perfusion is lost.
• In Right-sided obstructive lesions (e.g., TAPVC, TA, and Mitral Atresia),
restricted pulmonary blood flow results in reduced systemic blood
flow, which may result in shock.
• In lesions with parallel pulmonary and systemic circulations (e.g.,
TGA with intact ventricular septum), mixing between the two
circulations is decreased, leading to hypoxia and metabolic acidosis,
which results in failure and shock.

8. Cyanosis
• Cyanosis is the bluish discoloration of the skin that occurs from the
presence of deoxygenated hemoglobin (which is blue) in capillary
beds.
• For cyanosis to be clinically apparent, 3 to 5 milligrams/dL of
Deoxyhemoglobin must be present, corresponding to an oxygen
saturation of 70% to 80% on room air.
• Congenital heart defects that present with cyanosis include TGA, TOF,
TA, Truncus arteriosus, and TAPVR.
• These lesions have in common the mixing of Oxygenated and
Deoxygenated blood, circulation of Desaturated hemoglobin, and a
cardinal manifestation as Cyanotic heart disease.
• Another condition resulting in cyanosis is persistent fetal circulation,
which can be caused by structural heart disease or Noncardiac
disease, including Meconium aspiration, pneumonia, sepsis, and
pulmonary hypertension.

9. Cyanosis in Nonductal-dependent congenital
heart defects :
 Total anomalous pulmonary venous connection (TAPVC).
 Truncus arteriosus.
• Lesions may or may not be ductal dependent depending upon the
degree of outflow tract obstruction including tetralogy of Fallot and
tricuspid atresia.
• Other lesions may exhibit differential cyanosis, such as critical
Coarctation of the aorta or interrupted arch, where the deoxygenated
flow through the ductus supplies the lower half of the body's
circulation, but oxygenated blood flow from the left heart supplies
the upper body via the vessels proximal to the site of arch
obstruction.

10. Cyanosis

11. Severe Pulmonary Edema
• Pulmonary edema, resulting in tachypnea and increased work of
breathing, can occur when there is a massive, rapid increase in
pulmonary blood flow associated with a fall in pulmonary vascular
resistance at delivery.
• In conditions such as Truncus arteriosus or PDA in premature infants,
or pulmonary venous circulation obstruction in total anomalous
pulmonary venous connection with obstruction.

13. Vitals
• Pulse : Abnormal heart rate — In infants with heart rates that are
higher or lower than the normal range of 90 to 160 beats per minute
for neonates up to six days of age, electrocardiography is initially
performed to determine whether there is an arrhythmia.
• Respiratory rate : Tachypnea (>40/min)
 Screening according to AAP,AHA,ACCF (1 out of 3) :
1) SpO2 measurement <90 percent
2) SpO2 measurement <95 percent in both upper and lower extremities
on three measurements, each separated by one hour
3) SpO2 difference >3 percent between the upper and lower extremities

14. On Examination
 CVS Auscultation
• Split S2
• Early systolic clicks
• Mid-systolic clicks
• S3 gallop
• Pericardial friction rubs
• Murmurs  Innocent / Pathologic/ Absent
 Peripheral Pulses Examination & BP diff > 10 mm of Hg
 Coughing & Wheezing : Pulm. Vs. Cardiac involvement

15. History
• Maternal and prenatal history
 Preterm infants (gestational age <37 weeks): CHD is two to three
times that found in term infants
• Maternal conditions that increase the risk of neonatal CHD
include the following:
 Diabetes, obesity, hypertension, CHD – refer to family history, Thyroid
conditions
 Epilepsy and mood disorders
 Maternal fever or influenza
 Smoking in the first trimester
 Congenital complete heart block in offspring of mothers with
connective tissue disorders and anti-Ro/SSA and anti-
La/SSB antibodies.
 Congenital infections such as cytomegalovirus, herpesvirus, rubella,
or coxsackie virus.

16. • Drugs taken in pregnancy such as
 Hydantoin  PS, AS
 Lithium  Ebstein's anomaly
 Alcohol  ASD, VSD
• Assisted reproductive technology (ART) increases the risk for
congenital heart disease, particularly for malformations of the
outflow tracts and ventriculo-arterial connections. It is unclear if this
risk is related to the underlying etiology of infertility in the couple or
the ART per se.
• Family history — There is an overall threefold increased risk for CHD
when a first degree relative has CHD. The familial risk of specific
malformations is even greater, suggesting a stronger genetic effect
in these conditions

17. Reference:
 http://www.uptodate.com/contents/congenital-heart-disease-chd-in-the-
newborn-presentation-and-screening-for-critical-chd#H3215187
 Tintinalli’s Emergency Medicine A Comprehensive Study Guide, 7th
Edition

18. ...Thank You...