1. APPROACH TO CYANOTIC CONGENITAL HEART DISEASE
Dr. R. Suresh Kumar
Head, Department of Pediatric Cardiology

2. Cyanosis
Bluish discolouration of skin & mucous membranes due to increased concentration of reduced Hb >3g% ABG; >5g%CBG
Causes— CNS, Musculoskeletal system, Airways, Alveolar-capillary membrane, Hb AND CVS.
Central vs peripheral.
Cardiac vs Respiratory. Peaceful cyanosis is cardiac

3. Evaluation of the cyanotic baby
Is the baby cyanosed? SpO2 will confirm.
Is the cyanosis central/peripheral? Keep the baby warm.
Is cyanosis cardiac?
1)Respiratory distress
2)Response to oxygen
ABG - low pH, low pO2, Low pCo2
CxR-heart size & configuration, vascularity
ECG Axis RV/LV dominance

4. Cyanotic CHD-classification
Reduced Pulmonary blood flow
Tetralogy of Fallot/pulmonary atresia
TOF-like physiology (complex congenital heart disease, large VSD PS/PA)
Critical PS/ pul.atresia intact IVS
Ebstein’s anomaly
Low pAO2, oligaemic lungs, neonates respond to PGE1.

5. Classification - Cyanotic CHD, Increased PBF
Transposition of Great Arteries
Total Anomalous Pulmonary Venous connection
Single Ventricle
Truncus Arteriosus
DORV

6. Clinical findings CCHD with decreased pulmonary blood flow
Quiet precordium
Single S2
Murmurs
Ejection systolic murmur at the base
No murmur
Continuous murmur of PDA / collaterals
ECG – RAD, RVH
LV dominance in tricuspid atresia
LAD in tricuspid atresia, AVCD with PS, Single ventricle with PS

7. Clinical findings - 2 CCHD with increased pulmonary blood flow TGA
Mild cyanosis
Active precordium
Epigastric pulsations
Congestive heart failure
Single S2 (except in TAPVC)
TGA
Profound cyanosis
Single S2
No murmur
Normal / mildly increased lung vascularity on CXR

8. Tetralogy of Fallot/P.Atresia
Large VSD/ RVOT obstruction/atresia
Cyanosis depends on degree of Obstruction, PDA/collaterals
Neonates may need PGE1 to maintain saturation – needs urgent BT shunt.
Progressive cyanosis/spells
Total correction-around 1 yr.

11. Cyanotic Spells
Rapid decrease in systemic oxygen saturation with hyperventilation  Convulsions, coma, death.
Age : 6mon – 6 yrs.
Cyanosis on crying is not a spell.
Crying/exercise may trigger spells.
Drop in SVR/ Inf.spasm  R to L shunt  acidosis  hyperventilation :vicious cycle

13. Cyanotic Spell- Treatment
Knee chest position , Oxygen
Morphine 0.1 mg/kg iv (sedation,resp dep.);Soda bicarb iv 1-2 ml/kg
Increase SVR : Propranolol 0.1 mg/kg iv; Methoxamine 0.1 mg/kg iv; Phenylephrine 1-5mic.g/kg /min iv infusion.
Ventilation. Paralyse
Emergency surgery
Propranolol for prophylaxis

15. Complex CHD,TOF-like physiology
DORV PS/PA Presents like TOF. Needs neonatal BT shunt, if ductus-dependent. Total correction around 1 year, may need a homograft.
Single Ventricle PS/PA. Presents like TOF. But 2-ventricle Repair not possible.Neonatal BT shunt, if needed. SVC to RPA anastomosis (Bidirectional Glenn) at 3-6months. IVC to PA connection (Lateral tunnel or Extra cardiac Fontan) at 2-5 yrs.

17. Tricuspid Atresia
Pulmonary blood flow depends on ASD, VSD, Pulmonary stenosis. May be increased / decreased / balanced. Cyanosis or heart failure predominant presentation accordingly.
LV dominance and left axis in ECG helpful.
Management in Fontan track.Neonatal BT shunt (for severe cyanosis) or PA banding (for heart failure) may be needed.

21. Critical PS PAIVS
Cyanosis on day 1. Pulmonary circulation ductus dependent. RV normal or in a range of hypoplasia.
If RV is adequate,catheter or surgical pulmonary valvotomy is the treatment. If RV is small, BT shunt. May need both.
Further management may be - Glenn + Pulm. Valvotomy (1½ ventricle) or staged Fontan, when 2-ventricle repair not possible.
Myocardial sinusoids / RV dependent coronary circulation - adverse prognosis.

24. Ebstein’s anomaly
Inferior displacement of septal and inferior tricuspid leaflets. Thin RV. May have PS.
Neonatal cyanosis may be severe. High PVRTR  R to L atrial shunt. Usually transient.
Course:May be asymptomatic. Cyanosis, Rt heart failure and SVT may be problems.
Surgery for severe cyanosis/heart failure.

25. Ebstein’s anomaly History of SVT Generally good effort tolerance
Cardiomegaly
Multiple heart sounds (split S1, split S2, S3, S4)
Scratchy systolic murmur at LLSB
Cardiomegaly with huge RA on CxR.
ECG-abnormal axis, RAE, polyphasic QRS. May have WPW syndrome

28. Cyanotic CHD, increased PBF. Transposition of GA
Commonest cyanotic CHD on day 1.
Aorta from RV, PA from LV. Presentation depends on Intact IVS, VSD, PS.
TGA intact IVS. Cyanosis on Day1. No murmur. CxR- egg on side heart,normal vascularity. Untreated 90% die in first month, if no ASD.
Management : PGE1 till Balloon Atrial Septostomy. Arterial Switch Operation in first month. Good long-term result.

32. TGA with VSD
No PS: Cyanosis minimal. CHF in a few weeks. Needs Arterial Switch operation in 3 months. Rapid progression of PVD, if untreated.
TGA/VSD/PS: Cyanosis depends on degree of PS. BT shunt if cyanosis is severe. Rastelli operation at 3-5 yrs.

33. TAPVC
All 4 pulm veins join a common chamber which eventually reaches RA.
Supracardiac- common chamber drains to innominate vein or to SVC.
Cardiac- drains to coronary sinus or RA
Infracardiac (Infradiaphragmatic). Descending vertical vein  portal vein  ductus venosus  IVC. Obstructed.

35. TAPVC-2
Obstructed venous return: Presents with pulmonary oedema. Infradiaphragmatic on Day1, supracardiac in a fewdays. Emergency surgery life saving. Normal life span.
Unobstructed: Cardiac, some supracardiac. Slow onset of CHF, minimal cyanosis. Clinical features of ASD Figure of 8 cardiac silhouette on CxR. Good surgical results.

38. Persistent Truncus Arteriosus
A single arterial trunk leaves the heart above a VSD. PA arises with a common trunk or as separate branches.
CHF in a few weeks of life, mild cyanosis. Bounding pulses,systolic click and murmur. EDM, if truncal valve is incompetent. CxR - CE, High origin of PA branches,increased vascularity.
Surgical Repair in first month preferred.

40. Cyanotic CHD in older children and adults
Tetralogy of Fallot
Commonest CCHD with reduced pulmonary blood flow surviving into adulthood
Exertional dyspnoea and squatting
Varying degrees of cyanosis
Eisenmenger syndrome
History of recurrent respiratory infections in early childhood
Good effort tolerance till late in the nature history
Mild cyanosis
Findings of severe pulmonary artery hypertension

41. Eisenmenger VSD Presents in second decade Single S2
Normal heart size on CXR
Dilated central pulmonary arteries with peripheral pruning

42. Eisenmenger PDA Presents in second or third decade
Differential cyanosis and clubbing
Normally split S2 with loud P2
Normal heart size on CXR
Dilated central pulmonary arteries with peripheral pruning

43. Eisenmenger ASD Presents in fourth or fifth decade Cardiomegaly
Widely split S2
Atrial fibrillation

44. Thank You