1. Cyanotic Congenital Heart Disease Dr. Ahmad Rustam bin Mohd Zainudin MD, MMed (UKM) Paediatric Cardiologist Hospital Pulau Pinang

2. Lecture Outline Overview and Causes of Cyanosis Cyanotic Congenital Heart Disease –Types –Clinical Features –Management

3. Cyanosis "Blue discolouration of the skin & mucous membrane" Deoxygenated Hb reaches 4 to 6 gm/dl, cyanosis may be seen. Normally approximately 80-87% oxygen saturation would give rise to clinically apparent cyanosis. However, in cases of anemia where haemoglobin levels are low, lower oxygen saturation may show up as cyanosis. Hb level of 6 g/dl for example shows cyanosis only when oxygen saturation has dropped below 60%

8. Hyperoxia test 100% O2 via HBO2 for 15 minutes ABG from right radial artery Cyanotic CHD: –pO2 <100mmHg; or –rise in pO2 <20 mmHg Shortfalls: –Massive intrapulmonary shunts (eg: PPHN, AVM): pO2 <100 mmHg –Cyanotic CHD with large pulmonary blood flow (eg: TAPVD): pO2 may significantly rise with O2

10. Heart Disease in Children AcquiredCongenital AcyanoticCyanotic

11. Differential Diagnosis of CCHD CXR Increased Pulmonary Vascularity ECG RVH: TGA, TAPVD LVH or BVH: Truncus, Single ventricle, TGA & VSD Decreased Pulmonary Vascularity ECG RVH: TOF LVH: Pulmonary Atresia, Tricuspid Atresia BVH: Single ventricle & PS, TGA & PS RBBB: Ebstein anomaly

12. Differential Diagnosis of CCHD Increased Pulmonary Vascularity  TGA  TAPVD  Truncus Arteriosus  Single Ventricle Decreased Pulmonary Vascularity  Pulmonary Atresia  Tricuspid Atresia  Single ventricle with PS  TGA with PS  Ebstein anomaly

13. Most common cyanotic congenital heart disease 5Ts TGATOFTAPVD Tricuspid Atresia Truncus

14. Tetralogy of Fallot

15. 5-10% of CHD Criteria: –Large VSD –Overriding of Aorta –Pulmonary stenosis –Right Ventricular Hypertrophy Clinical features: –Cyanosis, Murmur –Hypercyanotic spells Investigations: –CXR: "boot-shaped" heart –ECG: RAD, RVH –Echo: confirmed diagnosis

18. Hypercyanotic spell Also known as ‘tet’ spell (commonly seen in Tetralogy of Fallot) Characterized by: –Period of uncontrollable cry/ panic –Rapid & deep breathing (hyperpnea) –Deepening of cyanosis –Decreased intensity of heart murmur –Limpness, convulsions & rarely, death Peak age: 2-24 months –Taksande et al, J MGIMS, 2009

19. How does it happen? Imbalance between pulmonary & systemic vascular resistance  decreased pulmonary blood flow & increased right-to-left shunting. Results in fall of arterial PaO2 Fall in pH  stimulate respiratory centre  hyperpnea Presence of fixed resistance at the RVOT  more shunting  vicious cycle of hypoxic spell –Taksande, J MGIMS, 2009

20. Medical Emergency!!! Knee-chest/ squatting position O2 100% IV/IM/SC Morphine 0.1-0.2 mg/kg IV Propanolol 0.05-0.1 mg/kg slow bolus over 10 min (alternative: IV Esmolol/ IV Metoprolol) IV NaHCO3 1mEq/kg Vasopressor +/- Inotropes Fluid challenge Heavy sedation, intubation & ventilation

21. Management of TOF Medical Palliative Oral propanolol procedure/ surgery 0.2-1.0 mg/kg bd/tds RVOT stenting BT shunt Corrective Surgery VSD closure & RVOT resection (ideally at 6-12 months old)

22. Palliative procedures RVOT stentingModified BT shunt

23. Thomas-Blalock-Taussig Shunt Helen TaussigAlfred Blalock Vivien Thomas

24. Transposition of Great Arteries

25. dTGA: 5-7% of CHD; Boys 3:1 Most common cause admission for cyanotic CHD during 1st 2 weeks of life Criteria: –Aorta arises anteriorly from RV –PA arises posteriorly from LV Other associated defect: –50% no other defect (other than small PFO/PDA) –30-40% VSD, 5% PS, 10% VSD with PS Clinical features: –Cyanosis (usually noted at birth) –Heart failure Investigations: –CXR: Cardiomegaly, "egg on side" –Echo: confirmed diagnosis

29. Management Simple TGA (intact ventricular septum) –Prostaglandin E infusion 5-60ng/kg/min –Balloon Atrial Septostomy (BAS) –Atrial Switch Operation (ASO) at 2-4 weeks TGA with VSD –ASO & VSD closure at 1-3 months TGA with VSD & PS –BT shunt during infancy –Rastelli repair at 4-6 years

30. Total Anomalous of Pulmonary Venous Drainage

31. Types of TAPVD

32. Total Anomalous of Pulmonary Venous Drainage 1% CHD, Boy 4:1 (infracradiac type) Types: –Supracardiac (50%) –Intracardiac (20%) –Infracardiac (20%) –Mixed (10%) Clinical features: –Obstructed: Cyanosis, respiratory distress, profound desaturation, acidosis, pulmonary HPT, FTT –Unobstructed: Cyanosis, HF Investigations: –CXR: "snowman" sign –Echo: Large RA,RV. Small LA, PHT, abnormal pulmonary venous return

33. Management Digitalis & diuretics- control HF Correction of metabolic acidosis Ventilatory support (high PEEP) Corrective surgery: –Obstructed: Urgent (ASAP) –Unobstructed: 4-6 months old

34. Tricuspid Atresia

37. 1-3% CHD Criteria: –Absent tricuspid valve, hypoplastic RV –most common (50%): normally related great arteries, small VSD, PS Associated defect: –ASD, VSD, PDA (necessary for survival) –30% TGA, COA, IAA, subaortic stenosis Clinical features: –Cyanosis at birth –Hepatomegaly Investigations: –CXR: reduced pulmonary vascularity –Echo: TA, relation of great arteries, pulmonary arteries assessment

38. Management Management of Tricuspid Atresia PG infusion +/- BAS Restrictive PBFUnrestrictive PBF PDA stenting/PA banding BT shunt Glenn shunt Fontan (TCPC)

40. Truncus Arteriosus

41. Truncus arteriosus 1% CHD Criteria: –single arterial trunk with a truncal valve leaves the heart & gives rise to the pulmonary, systemic & coronary circulations. Types: –Type 1: MPA present –Type 2: No MPA –Type 3: RPA & LPA distant –Type 4: RPA & LPA from descending aorta Clinical features: –Cyanosis, HF Investigations: –CXR: cardiomegaly, increased pulmonary vascularity –Echo: confirmed diagnosis

42. Truncus arteriosus

44. Truncus arteriosus repair

45. Thank You