Critical congenital heart disease (CCHD) refers to severe heart defects that require intervention, such as surgery or catheter procedures, in the first year of life. CCHD accounts for about 25% of all congenital heart disease cases. Common types include those dependent on ductal flow, such as hypoplastic left heart syndrome, as well as defects affecting the right ventricle outflow tract like pulmonary atresia. Diagnosis is based on symptoms like cyanosis and screening involves pulse oximetry of the right hand and foot in newborns. Treatment options include pharmacological therapy with prostaglandin E1 to keep the ductus arteriosus open, as well as surgical or catheter-based procedures.

1. CRITICAL CONGENITAL HEART DISEASE
Department of Paediatrics
Dr Tanvi Mehta
Dr Bhadra

2. CONTENTS
1. Definition
2. Fetal circulation
3. Classification
4. Clinical features
5. Diagnosis
6. Treatment
7. Screening
Sources – Cloherty , UpToDate, Article on Screening- link
attached

3. What is Critical CHD?
• Lesions requiring surgery or catheter based
intervention in the first year of life.
Critical CHD= ~25% of all CHD.
https://www.uptodate.com/contents/newborn-screening-for-critical-congenital-heart-disease-using-
pulse-
oximetry?search=newborn%20screening%20for%20cyanotic%20congenital%20heart%20disease&sourc
e=search_result&selectedTitle=3~150&usage_type=default&display_rank=3

5. Concepts
Streaming Mixing
High Pressure to Low Pressure
Uni-Directional
LV to RV Sys to Pul Aor to Pul Art
Near Equal Pressures
Bi-directional
LA & RA Large VSD

6. Start from the beginning – Fetal Circulation
o Heart
 Pumping
 Separation of circulation
 Fetal Circulation
 Source of O2
 High oxygen and glucose demand for brain and
cardiac structures
 Slow growing structures

7. Fetal
circulation

8. Points
1. Oxygen Source – Placenta – SpO2 – 80-90%
2. UV (50-60%)→ DV → IVC
3. IVC = DV ( high in O2) + Lower Limbs ( 25-40%)
4. @ RA – Eusthachian Valve – Oxy blood directed to PFO → LA
( SpO2 – 60-65%)
5. Remaining RA + SVC→ RV → MPA → PDA/DecAo
6. Stroke Volume ( LV & RV ) Vs Combine Cardiac Output
7. Control of circulation- a & b – receptors , local mediators,
circulating catecholamines
 PDA and LUNGS
 Lungs – fluid filled, poor perfusion, less oxy - Vasoconstriction
 PDA – responds to PGE1 and low PaO2
 PGE1 – produced from placenta and metabolized in lungs
 With breathing – PVR – 1/15 ; SVR X 2

9. Classification of critical CHD
1. Duct dependent left heart lesions
2. Duct dependent right heart lesions
3. Other circulations

10. Duct dependent left heart lesions
• Isolated or Spectrum – Involving mitral valve , left
ventricle, aortic valve , aorta
• Hypoplastic left heart syndrome.
• Critical aortic valve stenosis
• Coarctation of aorta
• Interrupted aortic arch

11. Hypoplastic left heart syndrome
Valve Atresia Valve Stenosis

12. Coarctation of aorta

13. Duct dependent right heart lesions
• Component of right ventricle outflow – tricuspid
valve, pulmonary valve , branch pulmonary
arteries
• Critical pulmonary valve stenosis
• Tetralogy of Fallot - stenosis / atresia
• Pulmonary atresia

15. Critical PS

17. Other circulations
• Admixture: TAPVC
• Parallel circulation: d-TGA

18. TAPVC
Total Anomalous Pulmonary Venous Return

21. Clinical Features
• Cyanosis
• Tachycardia
• Tachypnoea
• Feeble distal pulses
• Delayed capillary refill
• Hepatomegaly

22. Features of shock:
• Mottling of skin
• Cool periphery
• Feeble to absent distal pulses
• Prolonged CRT
• Decreased urine output
• Tachycardia
• Hypotension

23. AORTIC ARCH

31. Treatment:
• Pharmacological – PGE1
• Catheterization
• Surgical

32. • PROSTAGLANDIN E1:
• Indication: ductal-dependent congenital heart
defect.
• Always given as continuous IV infusion.
• Start at 0.05-0.1 mcg/kg/min, can be reduced
to 0.005-0.01 mcg/kg/min once duct is
opened. (^SaO2).
• Trade name: Alpostin/ Prostin
• One vial will last 2-3 days for a 3 kg baby.

33. • Start PGE1 infusion: 1 ml vial of 500 mcg/ml
• Reconstitute in 50 ml 10% Dextrose
• 0.6*wt= _ml/hr drip rate for a dose of 0.1
mcg/kg/min
• Cost: 5820

34. Adverse effects of PGE1:
•Apnea
•Hypotension and tachycardia
•Necrotizing enterocolitis (NEC)

36. Balloon
Atrial
Septectomy
Femoral Venous Access
Sonography Guided
Cath Lab or ICU

40. Screening is recommended in the right hand (preductal) and either foot (postductal).
Screening at both locations can occur simultaneously or in direct sequence.

42. Dhoni’s Records – 2004 - 2019
Only captain to win three ICC tournaments
WT20 – 2007, ICC WC – 2011, Champions
Trophy - 2013
Most international matches as a captain
332 Total ( 200 ODI, 60 Tests, 72 T20)
Most final wins as a captain (ODIs)
4/6 multinational ODI
Most Not Outs in ODIs
Unbeaten in 84 ODIs
Most stumpings in international cricket
123 stumping in 350 matches