Evaluation of cyanotic child & management of cyanotic

Cyanosis diagnosis and management

1. Evaluation of Cyanotic child & Management of Cyanotic spell DR DHANESH KUMAR

2. CYANOSIS Definition Clinically determined by- History & Examination Saturation probe!! Types : Central, Peripheral, Abnormal pigments, mixed or differential Cause- Cardiac, pulmonary , hematologic , Infectious or High altitude ?? The cyanotic neonates: By Suzanne Roberts , D. O ., MPH, and Jahn Avarello , M.D.

3. Clinical Diagnosis % of arterial blood that is desaturated Hb concentration!! If art O2 saturation is 60% Cyanosis is detectable if Hb >12.5gm/dl ! But not if Hb <10gm/dl ! i.e. 4gm/dl is insufficient for detection of cyanosis ! Cyanosis is recognizable at a higher arterial oxygen saturation in patients with polycythemia and at a lower arterial oxygen saturation in patients with anemia

4. Diagnosis Hyperoxia Test: Confirm Place infant on 100% O2 hood for 10 min. PaO2 >100 mmHg = Parenchymal lung disease PaO2=50-100 mmHg= Parenchymal lung disease or cardiovascular disease PaO2 <50 mmHg=Fixed R-L shunt Cyanotic congenital heart disease of PPHN

5. Unusual causes of cyanosis Without murmurs! Surviving to adolescent/ adult life Left SVC to LA IVC to LA Rt.SVC to LA Pulmonary AV Fistulae(Osler Rendu Weber syndrome)

6. Approach to a child with cyanotic CHD History: H/o. cyanotic spell(TOF physiology) Cyanosis increased during defecation, feeding H/o squatting Easy fatigability Failure to thrive Syncope-TOF Hemoptysis : Eisenmenger’s Convulsions: Cerebral abscess Cough, breathlessness & repeated chest infection due to increased PBF-TAPVC, TGA, Truncus arteriosus

7. Some points in clinical examination & History Prenatal History Consanguinity Age of Parents(esp. mother) Maternal medication during pregnency Sod. Valproate -TOF,VSD Lithium-ASD, Tricuspid atresia , Ebstein’s Marijuana- Ebstein’s Clomiphene -TOF Sex hormones-VSD,TGA,TOF

8. Approach to a child with cyanotic CHD Cyanosis at birth TGA with intact IVS HLH Syndrome Hypoplastic RV with pulmonary atresia Truncus arteriosus Obstructive TAPVC TOF with pulmonary atresia Cyanosis in 1 st week Pulmonary atresia Tricuspid atresia HLH Syndrome Ebstein’s anomalies Cyanosis after 1 month TOF TGA TAPVC

9. Sex of Patient Males-more common D-TGA Hypoplastic left heart Single ventricle Females-more Common OS-ASD PDA Equal in both sexes Ebstein’s anomaly Pulmonary atresia Tricuspid atresia Truncus atreriosus TOF Congenital venacaval to LA communication

10. Marfan’s and Down syndrome : TOF Noonan & Maternal rubella syndrome: TOF Cat cry syndrome : Tricuspid Artesia Di George syndrome : Truncus arteriosus ( hypoplastic mandible, defective ears and short philtrum ) Anomalies of 16-18 chromosomes :DORV Eisenmenger’s syndrome : Differential clubbing only in toes, shortstature , kyphoscoliosis , arthropathy , dental anomalies, growth retardation

11. Approach to a child with cyanotic CHD Pulse : Collapsing: Truncus arteriosus TOF with collaterals/AR AV Malformation Decreased L carotid and left brachial pulses: HLHS

12. Jugular Examination Enlarged Tricuspid Artesia Hypo plastic left heart TAPVR TGA with ↑PBF DORV with ↑PBF Truncus arteriosus Normal jugulars Fallot’s tetrology TGA with PS DORV with PS

13. Precordium Examination Normal TOF DORV Pulmonary atresia with intact ventricular septum Tricuspid atresia Precordial Bulge TAPVC TGA Hypoplastic Lt. Ventricle PS with intact IVS

14. Approach to a child with cyanotic CHD Splitting of S1: Epstein's anomaly Loud P2: TAPVC,DORV with increased PBF, Eisenmenger syndrome Single S2: TOF, TOF With PS,DORV with PS, Tricuspid atresia , Truncus arteriosus Muffled S2: Ebstein’s anomaly Continuous murmur: TOF with collateral, Truncus arteriosus

15. Chest X-ray Heart size Cardiac silhouette Right aortic arch? Midline Liver?

16. Radiology Boot shape heart: TOF Enlarged heart: TOF with collaterals, associated anemia or hypertension Concavity in pulm . Artery area: PS Rt. Sided aortic arch: 25% cases of TOF Rib notching: TOF with collaterals Egg on side appearance: TGA Figure of 8 appearance: TAPVC

17. ECG

18. Other non invasive tests Echocardiography Holter monitoring Kadle et al. Estimating PG by auscultation :How tecnology (echocardiography) can improve clinical skills. World J Cardiol 2017

19. Invasive methods Cardiac catheterization Angiography Contrast CT 3-D Printing Temel et al. Prevalence and characteristics of coronary artery anomalies in children with congenital heart disease diagnosed with coronary angiography. Turk Kardiyol Dern Ars 2017

20. Keys to clinical diagnosis Work in order Pulses? Colour -cyanosis, pallor, polycythemia Inspect for chest form and pulsations Palpate to determine which ventricle? Forget the murmur!! Listen first to S1 then S2 Can you split the S2?? Then concentrate on components Finally the murmurs- systolic, ejection or pan systolic Is there a diastolic murmur??

21. Cyanotic Spell Progressive increase in rate & depth of respiration & culminates in paroxysmal hyperapnea , deepening of cyanosis, limpness, syncope & ocasionally convulsion or death.

22. Cyanotic Spell Cause: TOF DORV,VSD,PS VSD,PS TGA,VSD,PS ASD,PS Tricuspid Atresia 1b/ IIb Triggering factors Spontaneous Crying Feeding Bowel movement Sudden wake up

23. Cyanotic Spell Medical emergency Early hours Mechanism: Exact cause-Not Known Wood’s Theory : Local intracardiac production of catecholamine's increased due to stress-which increase infundibular spasm leading to more R to L shunt Vulnerable resp. centre( Guntheroth & Morgan ) Paroxysmal attack of arrhythmias: Atrial Tachycardia increased R-L shunt( Young )

24. Cyanotic Spell Supportive Treatment: 1.Knee chest position 2.O2 inhalation 3.I.V access 4.Sodium bicarbonate(1-2meq/Kg/dose) for acidosis

25. Intubation and ventilation Emergency BT Shunt If ductal dependent-PGEI/Duct stenting If TGA= Baloon atrial septostomy

26. Prevention Propronalol (oral) :1-4mg/Kg Iron supplement:1mg/kg/day Avoid precipitating cause –Vasodilators Treat URTI promptly Blood letting Plan surgical management

27. Polycythemia -Management 1.Avoid dehydration 2.Iron therapy 3.Phlebotomy-if Hct . > 65-70%

28. THANK YOU !