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Cyanotic Congenital Heart Disease Dr. Ahmad Rustam bin Mohd Zainudin MD, MMed (UKM) Paediatric Cardiologist Hospital Pulau Pinang
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Lecture Outline Overview and Causes of Cyanosis Cyanotic Congenital Heart Disease –Types –Clinical Features –Management
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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%
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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
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Heart Disease in Children AcquiredCongenital AcyanoticCyanotic
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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
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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
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Most common cyanotic congenital heart disease 5Ts TGATOFTAPVD Tricuspid Atresia Truncus
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Tetralogy of Fallot
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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
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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
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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
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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
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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)
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Palliative procedures RVOT stentingModified BT shunt
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Thomas-Blalock-Taussig Shunt Helen TaussigAlfred Blalock Vivien Thomas
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Transposition of Great Arteries
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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
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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
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Total Anomalous of Pulmonary Venous Drainage
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Types of TAPVD
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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
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Management Digitalis & diuretics- control HF Correction of metabolic acidosis Ventilatory support (high PEEP) Corrective surgery: –Obstructed: Urgent (ASAP) –Unobstructed: 4-6 months old
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Tricuspid Atresia
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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
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Management Management of Tricuspid Atresia PG infusion +/- BAS Restrictive PBFUnrestrictive PBF PDA stenting/PA banding BT shunt Glenn shunt Fontan (TCPC)
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Truncus Arteriosus
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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
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Truncus arteriosus
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Truncus arteriosus repair
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Thank You
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