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Cyanotic congenital heart disease
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Classification of congenital heart diseases
Group I : Left to right shunts Group II: Right to lefts shunts Group III: Obstructive lesions
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Left to right shunts Atrial Septal Defect Ventricular Septal Defect
Patent Ductus Arteriosus
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Right to Left Shunts Tetralogy of Fallot
Tricuspid atresia Ebstein’s anomaly Transposition of Great Vessels Truncus Arteriosus Total Anomalous Pulmonary Venous Return (TAPVR)
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Obstructive Lesions Aortic stenosis Coarctation of the Aorta
Pulmonic Stenosis
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Cyanotic heart disease
Right to Left Shunt
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Cyanotic heart disease
bluish or purplish tinge to the skin and mucous membranes. 5 g/dL of unoxygenated hemoglobin in the capillaries This usually correspond to oxygen saturation of 70-80%
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Who is this guy?
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ÉTIENNE-LOUIS ARTHUR FALLOT!
a French physician, 1888 Fallot accurately described in detail the four anatomical characteristics of tetralogy of Fallot.
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Tetralogy OF Fallot Most common cyanotic heart disease! 75%!
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TOF 4 component! Imagine this is a HEART!
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TOF Vetricular Septal Defect
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TOF Vetricular Septal Defect Pulmonic Stenosis
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TOF Vetricular Septal Defect Pulmonic Stenosis
Overriding of dextroposed aorta
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TOF Vetricular Septal Defect Pulmonic Stenosis
Overriding of dextroposed aorta Right Ventricular hypertrophy
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TOF Vetricular Septal Defect Pulmonic Stenosis
Overriding of dextroposed aorta Right Ventricular hypertrophy Concentric R ventricular hypertrophy without cardiac enlargement
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TOF Vetricular Septal Defect Pulmonic Stenosis
Overriding of dextroposed aorta Right Ventricular hypertrophy Concentric R ventricular hypertrophy without cardiac enlargement Increase in right ventricular pressure*
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RV and LV pressures becomes identical
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There is little or no L to R shunt
RV and LV pressures becomes identical
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Hence, VSD is silent There is little or no L to R shunt RV and LV pressures becomes identical
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Right ventricle into pulmonary artery across pulmonic stenosis producing ejection systolic murmur
Hence, VSD is silent There is little or no L to R shunt RV and LV pressures becomes identical
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Hence, the more severe the pulmonary stenosis
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Hence, the more severe the pulmonary stenosis
The BIGGER the Left to RIGHT shunt
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Hence, the more severe the pulmonary stenosis
The BIGGER the Left to RIGHT shunt Less flow into the pulmonary artery
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Hence, the more severe the pulmonary stenosis
The BIGGER the Left to RIGHT shunt Less flow into the pulmonary artery Shorter the ejection systolic murmur
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Hence, the more severe the pulmonary stenosis
The BIGGER the Left to RIGHT shunt Less flow into the pulmonary artery Shorter the ejection systolic murmur More cynosis because of less flow to the lung!
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Hence, Severity of cyanosis is directly proportional to the severity of pulmonic stenosis Intensity of the systolic murmur is inversely related to the severity of pulmonic stenosis
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Congestive failure never occur* because…
Right ventricle is effectively decompressed because of the ventricular septal defect. * exception
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Congestive failure never occur* because…
Right ventricle is effectively decompressed because of the ventricular septal defect. Anemia Infective Endocarditis Systemic hypertension Unrelated myocarditis complicating TOF Aortic or pulmonary valve regurgitation * exception
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Pulmonary obstruction results in delayed P2
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Pulmonary artery pressure reduce
Pulmonary obstruction results in delayed P2
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P2 become soft or inaudible
Pulmonary artery pressure reduce Pulmonary obstruction results in delayed P2
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Since P2 is inaudible, hence S2 = A2 + P2 [S2 is single sound]
(Second Sound) S2= A2 + P2 Since P2 is inaudible, hence S2 = A2 + P2 [S2 is single sound] Aorta is displace anteriorly too, A2 become LOUD! P2 become soft or inaudible Pulmonary artery pressure reduce Pulmonary obstruction results in delayed P2
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Ascending aorta in TOF is large, results aortic ejection click
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Diastolic interval is clear
No S3 No S4
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Concentric right ventricular hypertrophy reduce the distensibility of the right ventricle during diastole
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Concentric right ventricular hypertrophy reduce the distensibility of the right ventricle during diastole “a” waves become prominent in JVP* *but not too tall
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Clinical Picture Symptomatic any time after birth
Paroxysmal attacks of dyspnea Anoxic spells Predominantly after waking up Child cry Dyspnea Blue Lose conscious Convulsion Frequency varies from once a few days to many attack everyday
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“tet spell” lethal, unpredictable episodes The mechanism
spasm of the infundibular septum, which acutely worsens the RV outlet obstruction.
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Dyspnea on exertion Exercise intolerance
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Sitting posture – squatting
Compensatory mechanism Squatting increases the peripheral vascular resistance, which diminishes the right-to-left shunt increases pulmonary blood flow.
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Cyanosis during feeding
Poor feeding fussiness, tachypnea, and agitation. Birth weight is low. Growth is retarded. Development and puberty may be delayed.
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Rarely, patient remain asymptomatic into adult life.
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Physical examination Clubbing + Cyanosis (Variable) Squatting position
Scoliosis – Common bulging left hemithorax
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Prominent “a” waves JVP Normal heart size Systolic trill (30%)
Mild parasternal impulse Systolic trill (30%)
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S1 normal S2 single Murmur Ejection aortic click only A2 heard
P2 soft & delayed: INAUDIBLE Murmur Shunt murmur (VSD) absent Flow murmur: Ejection systolic, the smaller the flow the shorter the murmur Ejection aortic click
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Retinal engorgement Hemoptysis
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ECG ECG
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ECG ECG
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ECG wiLLiam moRRow ECG
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ECG Right axis deviation (+120° to +150°)
Right or combined ventricular hypertrophy Right atrial hypertrophy Partial or complete right bundle branch block (especially true of patients after surgical repair)
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Coeur en sabot (boot-shaped heart) secondary to uplifting of the cardiac apex from RVH and the absence of a normal main pulmonary artery segment
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Normal heart size due to the lack of pulmonary blood flow and congestive heart failure
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Decreased pulmonary vascularity
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Right atrial enlargement
Right-sided aortic arch (20-25% of patients) with indentation of leftward-positioned tracheobronchial shadow
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Echocardiography Reveals a large VSD overriding aorta
variable degrees of right ventricular outflow tract (RVOT) obstruction
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Course and Complication
Each anoxic spell is potentially fatal Polycytemia Cerebrovascular thrombosis Anoxic infaction of CNS Neurological complication
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4) LUNG is an awesome filter.
Bypassing it may not be a good idea! TOF, venous blood from gut, peripheral system by pass the lung and re-enter circulation Hence TOF can cause: Brain Abcess Infective endocarditis Paradoxical embolism
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Management of anoxic spell
Knee chest position Humified O2 Be careful not to provoke the child Especially you are bad at gaining IV access Ask for help from someone more experience Permit the baby to remain with mother Morphine mg/Kg Subcutaneous Correct acidosis – Sodium Bicarb IV
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Vasopressors: Methoxamine IM or IV drip Correct anemia
Propanolol 0.1mg/kg/IV during spells 0.5 to 1.0 mg/kg/ 4-6hourly orally Vasopressors: Methoxamine IM or IV drip Correct anemia GA is the last resort
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Palliative Surgery Blalock-Taussig shunt Pott procedure
Waterston shunt
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Blalock Taussig Shunt Subclavian artery – Pulmonary artery anastomosis
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Modified Blalock Taussig Shunt
Goretex graft
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Surgical Palliation
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Palliative operation prolong life
Increase exercise tolerance
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Definitive operation Closing the VSD Resecting infundibular
90% can return almost normal life after operation Complication: RBBB Residual VSD Residual Pulmonary stenosis Pulmonary regurgitation (pulmonary valve excised) Risk 5%
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Transposition of Great Areries (TGA)
Aorta originating from the right ventricle, and pulmonary artery originating from the left ventricle Accounts for 5-7% of all congenital heart disease
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TGA Survival is dependent on the presence of mixing between the pulmonary and systemic circulation Atrial septal defect is essential for survival 50% of patients have a VSD Usually presents in the first day of life with profound cyanosis More common in boys
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TGA Exam : cyanosis in an otherwise healthy looking baby
Loud S2 ( aorta is anterior ) CXR : Egg on side Narrow mediastinum
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TGA .. Acute Management PGE-1 with no supplemental O2
Maintain ductus arteriosus patency, this will increase the effective pulmonary blood flow, and thence increase the left atrial pressure, therefore inhance the left to right shunt at the atrial level Balloon atrial septostomy Life saving procedure in the presence of inadequate atrial septal defect
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TGA .. Surgical Management
Arterial switch with re-implantation of the coronary artery to the new aortic site. Atrial switch : the old style surgery Redirecting the pulmonary and systemic venous return to result in a physiologically normal state The right ventricle remains the systemic ventricle Rarely needed
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Truncus Arteriosus The presence of a common trunk that supply the systemic, pulmonary and coronary circulation Almost always associated with VSD % of all congenital heart disease
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Truncus Arteriosus There are different anatomical tupes of truncus arteriosus This is relevant for surgical repair
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Truncus Arteriosus Generally patients have increased pulmonary blood flow Degree of cyanosis is mild and may not be evident clinically until late stage with pulmonary vascular disease Presenting feature is congestive heart failure (tachypnia, hepatomegally)
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Truncus Arteriosus Exam is significant for
Single S2 Ejection click of the abnormal truncal valve Systolic murmur of truncal valve stenosis if present Diaastolic murmur of truncal valve insufficiency Gallop CXR : Cardiomegally , increased pulmonary circulation
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Managment Acute management
No O2 to minimize pulmonary blood flow Diuretics Afterload reduction to inhance systemic blood flow Surgical management: complete repair with VSD closure and conduit placement between the right ventricle and pulmonary arteries Long term problems : truncal valve dysfunction RV conduit obstruction
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Trcuspid Atresia Complete absence of communication between the right atrium and right ventricle About 3 % of congenital heart disease
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Tricuspid Atresia There is an obligate interatrial communication
Usually associated with VSD The pulmonary blood flow is dependent on the size of the VSD Pulmonary blood flow can be increased or decreased causing variable presenting symptoms If there is no VSD ( also called Hypoplastic right ventricle) the pulmonary blood flow is dependent on the PDA
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Tricuspid Atresia- presentation
The presentation will depend on the amount of pulmonary blood flow If the PBF is decreased, the main presenting symptom is cyanosis If the PBF is increased the presentation is that of congestive heart failure CXR will also reflect the amount of pulmonary blood flow
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Tricuspid Atresia- EKG
Very characterestic : Left axis deviation
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Management PBF Decreased Increased No O2
Afterload reduction Diuretics PGE-1, and minimal supplemental O2 to maintain ductal patency
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Surgical Management Single ventricle paliation
First stage : to establish a reliable source of PBF Aorta to pulmonary artery shunt ( BT shunt) Pulmonary arterial banding in cases of increased PBF Second stage: Glenn Anastomosis ( superior vena cava to pulmonary artery Third stage : Fontan anastomosis ( Inferior vena cava to pulmonary artery
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Total Anomalous Pulmonary Venous Return (TAPVR)
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TAPVR- Infracardiac
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Radiography
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Infracardiac type
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Thank You
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