classification of congential heart diseases and cyanotic heart disease
DESCRIPTION
Cyanotic Heart Disease, Congenital, Paediatrics, Medicine, Cardiology, Chan En Ze,TRANSCRIPT
Cyanotic congenital heart disease
Classification of congenital heart diseases
Group I : Left to right shuntsGroup II: Right to lefts shuntsGroup III: Obstructive lesions
Left to right shunts• Atrial Septal Defect• Ventricular Septal Defect• Patent Ductus Arteriosus
Right to Left Shunts1) Tetralogy of Fallot2) Tricuspid atresia3) Ebstein’s anomaly
4) Transposition of Great Vessels5) Truncus Arteriosus6) Total Anomalous Pulmonary Venous
Return (TAPVR)
Obstructive Lesions• Aortic stenosis• Coarctation of the Aorta• Pulmonic Stenosis
Cyanotic heart disease
Right to Left Shunt
Who is this guy?
ÉTIENNE-LOUIS ARTHUR FALLOT!
• a French physician, 1888 Fallot accurately described in detail the four anatomical characteristics of tetralogy of Fallot.
Tetralogy OF Fallot• Most common cyanotic heart
disease!
75%!
TOF4 component!
Imagine this is a HEART!
TOF1) Vetricular Septal Defect
TOF1) Vetricular Septal Defect2) Pulmonic Stenosis
TOF1) Vetricular Septal Defect2) Pulmonic Stenosis3) Overriding of dextroposed aorta
TOF1) Vetricular Septal Defect2) Pulmonic Stenosis3) Overriding of dextroposed aorta4) Right Ventricular hypertrophy
TOF1) Vetricular Septal Defect2) Pulmonic Stenosis3) Overriding of dextroposed aorta4) Right Ventricular hypertrophy
Concentric R ventricular hypertrophy without cardiac enlargement
TOF1) Vetricular Septal Defect2) Pulmonic Stenosis3) Overriding of dextroposed aorta4) Right Ventricular hypertrophy
Concentric R ventricular hypertrophy without cardiac enlargement
Increase in right ventricular pressure*
RV and LV pressures becomes identical
RV and LV pressures becomes identical
There is little or no L to R shunt
RV and LV pressures becomes identical
There is little or no L to R shunt
Hence, VSD is silent
RV and LV pressures becomes identical
There is little or no L to R shunt
Hence, VSD is silent
Right ventricle into pulmonary artery across pulmonic stenosis producing ejection systolic murmur
Hence, the more severe the pulmonary stenosis
Hence, the more severe the pulmonary stenosis
The BIGGER the Left to RIGHT shunt
Hence, the more severe the pulmonary stenosis
The BIGGER the Left to RIGHT shunt
Less flow into the pulmonary artery
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
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!
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
Congestive failure never occur* because…
Right ventricle is effectively decompressed because of the ventricular septal defect.
* exception
Congestive failure never occur* because…
Right ventricle is effectively decompressed because of the ventricular septal defect.
* exception
1)Anemia2)Infective Endocarditis3)Systemic hypertension4)Unrelated myocarditis
complicating TOF5)Aortic or pulmonary valve
regurgitation
Pulmonary obstruction results in delayed P2
Pulmonary obstruction results in delayed P2
Pulmonary artery pressure reduce
Pulmonary obstruction results in delayed P2
Pulmonary artery pressure reduce
P2 become soft or inaudible
Pulmonary obstruction results in delayed P2
Pulmonary artery pressure reduce
P2 become soft or inaudible
(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!
• Ascending aorta in TOF is large, results aortic ejection click
• Diastolic interval is clear• No S3• No S4
Concentric right ventricular hypertrophy reduce the distensibility of the right ventricle during diastole
Concentric right ventricular hypertrophy reduce the distensibility of the right ventricle during diastole
“a” waves become prominent in JVP*
*but not too tall
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
“tet spell”• lethal, • unpredictable episodes • The mechanism
– spasm of the infundibular septum,which acutely worsens the RV outlet obstruction.
• Dyspnea on exertion• Exercise intolerance
• Sitting posture – squatting– Compensatory mechanism– Squatting increases the peripheral
vascular resistance, – which diminishes the
right-to-left shunt – increases pulmonary
blood flow.
• Cyanosis during feeding– Poor feeding– fussiness, tachypnea, and agitation.– Birth weight is low.– Growth is retarded.– Development and puberty may be
delayed.
• Rarely, patient remain asymptomatic into adult life.
Physical examination• Clubbing + Cyanosis (Variable)• Squatting position• Scoliosis – Common• bulging left hemithorax
• Prominent “a” waves JVP• Normal heart size
– Mild parasternal impulse• Systolic trill (30%)
• S1 normal• S2 single
– 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
• Retinal engorgement• Hemoptysis
ECG• ECG
ECG• ECG
ECG• ECG wiLLiam
moRRow
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)
• 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
• Normal heart size due to the lack of pulmonary blood flow and congestive heart failure
• Decreased pulmonary vascularity
• Right atrial enlargement
• Right-sided aortic arch (20-25% of patients) with indentation of leftward-positioned tracheobronchial shadow
Echocardiography• Reveals a large VSD • overriding aorta • variable degrees of right ventricular
outflow tract (RVOT) obstruction
Course and Complication1) Each anoxic spell is potentially fatal2) Polycytemia
1) Cerebrovascular thrombosis3) Anoxic infaction of CNS
1) Neurological complication
4) LUNG is an awesome filter. 1) Bypassing it may not be a good idea!2) TOF, venous blood from gut, peripheral
system by pass the lung and re-enter circulation
3) Hence TOF can cause:1) Brain Abcess2) Infective endocarditis3) Paradoxical embolism
Management of anoxic spell1) Knee chest position2) Humified O23) Be careful not to provoke the child
1) Especially you are bad at gaining IV access2) Ask for help from someone more experience3) Permit the baby to remain with mother
4) Morphine 0.1 -0.2 mg/Kg Subcutaneous5) Correct acidosis – Sodium Bicarb IV
6) Propanolol1) 0.1mg/kg/IV during spells2) 0.5 to 1.0 mg/kg/ 4-6hourly orally
7) Vasopressors: Methoxamine IM or IV drip
8) Correct anemia9) GA is the last resort
Palliative Surgery• Blalock-Taussig shunt• Pott procedure• Waterston shunt
Blalock Taussig Shunt• Subclavian artery – Pulmonary
artery anastomosis
Modified Blalock Taussig Shunt
• Goretex graft
Surgical Palliation
• Palliative operation prolong life• Increase exercise tolerance
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%
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
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
TGA• Exam :
• cyanosis in an otherwise healthy looking baby
• Loud S2 ( aorta is anterior )
• CXR : • Egg on side• Narrow
mediastinum
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 septostomyLife saving procedure in the presence of
inadequate atrial septal defect
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
Truncus Arteriosus• The presence of a
common trunk that supply the systemic, pulmonary and coronary circulation
• Almost always associated with VSD
• 1.2-2.5% of all congenital heart disease
Truncus Arteriosus
• There are different anatomical tupes of truncus arteriosus
• This is relevant for surgical repair
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)
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
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
Trcuspid Atresia• Complete absence
of communication between the right atrium and right ventricle
• About 3 % of congenital heart disease
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
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
Tricuspid Atresia- EKG
Very characterestic : Left axis deviation
ManagementPBF
Decreased Increased
PGE-1, and minimal supplemental O2 to maintain ductal patency
No O2Afterload reductionDiuretics
Surgical ManagementSingle 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
Total Anomalous Pulmonary Venous Return (TAPVR)
TAPVR- Infracardiac
Radiography
Infracardiac type
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