APPROACH TO CYANOTIC CONGENITAL HEART DISEASE

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 • 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