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CONGENITAL HEART DISEASES
(CYANOTIC)
GUIDE – DR. L. S. PATIL
PRESENTER – DR. DEEPAK R. CHINAGI
BLDE UNIVERSITY'S SBMPMC , ...
Topics will be dealt as follows
• Embryology of the heart
• Gross Classification of congenital heart
disease with special ...
Embryology of the heart
Embryology of the heart
1. Formation of cardiogenic area from
angiogenic plexus
2. Formation of endocardial tubes (heart t...
6. Formation of inter-ventricular septum,
7. Formation of interatrial septum (septum
primum, foramen primum, septum
secund...
Gross classification of congenital heart
disease
• Lesions without shunts
Left heart malformations Right heart malformatio...
• Lesions with left to right shunts (Acyanotic)
Atrial level Ventricular level Others
ASD (ostium
primum, ostium
secundum,...
• Lesions with right to left shunts (Cyanotic)
discussed in detail
With increased pulmonary
blood flow
With normal or decr...
Complete transposition of great
arteries (D-transposition)
• Here the aorta arises from morphologic right
ventricle and li...
• It may also be associated with other
abnormalities like VSD and PDA
• S1 = Normal, S2 = single aortic component
heard. A...
• Treatment –
– Primary – surgical
– Supportive treatment –
• PGE1 infusion to keep PDA open
• Balloon atrial septostomy t...
Jatene procedure
Mustard Senning procedure
Double Outlet Right Ventricle(DORV)
• In this type of cono-truncal anomaly, both the
great vessels arise from right ventri...
• Associated anomalies
– Trisomy 13, trisomy 18, Coarctation of aorta, right
sided aortic arch, TAPVC/PAPVC, tracheo-
esop...
• Clinical features
– Cyanosis
– Systolic thrill and holosystolic murmur due to VSD
• ECG – Right axis deviation with coun...
Truncus Arteriosus
• It is and uncommon congenital anomaly with
single vessel forming outflow tract for both
ventricle, du...
– Type 2 – Right and left pulmonary arteries arise
sepeartely from posterior wall of truncus
– Type 3 – right and left pul...
• ECG – features suggestive of LV volume
overload + RV pressure overload
• CXR-PA – Cardiomegaly + Pulmonary Plethora
(Cli...
Total Anomalous Pulmonary Venous
Connection
• Pulmonary veins normally drain into left atrium,
but in patients with TAPVC,...
• Clinical Features
– Cyanosis
– Continuous murmur along left sternal borderdue
to flow through anomalous pulmonary venous...
• Natural history of TAPVC
– 50% infants dies by 6 months
– 80% infants die by 1 year
– Symptoms start appearing by 1st mo...
• Darling’s classification
Type Also known as Abnormal
connection
Type 1 Supracardiac PV join SVC
Type 2 Cardiac PV join R...
• ECG – is suggestive of RVH with right axis
deviation
• CXR-PA –
– Snow man appearance or figure of eight
appearance
Eisenmengers syndrome (VSD)
• It is the condition in which L>R shunt get
reversed (R>L shunt) with the development of
pulm...
Tetralogy of Fallot
• It is the most common congenital cyanotic
heart disease.
• It has 4 components
– Large VSD
– RV outf...
• Variability of RV outflow tract obstruction and
systemic-pulmonary pressure difference
contributes to occurrence of epis...
• Clinical features-
– S1 – Normal, S2 – Single , loud S2 is present
– Ejection Systolic murmur at Left 3rd and 4th ICS
– ...
• Complications
– Infective Endocarditis
– Embolisation and Cerebral Abscess
– Pulmonary Tuberculosis
– Secondary Polycyth...
• Surgical procedure
– Blalock – Taussig Shunt(left pulmonary artery to
left subclavian artery)
– Pott’s procedure(left pu...
Tricuspid Atresia
• In this condition triscuspid valve is absent, the
floor of RA is intact. It is always accompanied by
V...
Future scope
SIGNALLING PATHWAYS ARE DISCOVERED AT EACH STEP OF CARDIAC
EMBRYOGENESIS.
Thank You
Congenital heart diseases (Cyanotic CHD)
Congenital heart diseases (Cyanotic CHD)
Congenital heart diseases (Cyanotic CHD)
Congenital heart diseases (Cyanotic CHD)
Congenital heart diseases (Cyanotic CHD)
Congenital heart diseases (Cyanotic CHD)
Congenital heart diseases (Cyanotic CHD)
Congenital heart diseases (Cyanotic CHD)
Congenital heart diseases (Cyanotic CHD)
Congenital heart diseases (Cyanotic CHD)
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Congenital heart diseases (Cyanotic CHD)

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A detailed discussion on embryogenesis of heart and ennumeration of all congenital diseases and description of cyanotic congenital heart disease , each disease in detail.

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Congenital heart diseases (Cyanotic CHD)

  1. 1. CONGENITAL HEART DISEASES (CYANOTIC) GUIDE – DR. L. S. PATIL PRESENTER – DR. DEEPAK R. CHINAGI BLDE UNIVERSITY'S SBMPMC , VIJAYAPURA 21-03-2017
  2. 2. Topics will be dealt as follows • Embryology of the heart • Gross Classification of congenital heart disease with special emphasis on non shunt lesions and shunt lesions • Description about cyanotic congenital heart disease, each disease in particular
  3. 3. Embryology of the heart
  4. 4. Embryology of the heart 1. Formation of cardiogenic area from angiogenic plexus 2. Formation of endocardial tubes (heart tubes) 3. Fusion of heart tubes cephalo caudally as the embryo bends forward 4. Formation of cardiac loop (bulbus cordis + truncus arteriosus) 5. Deepening of bulbo-ventricular groove
  5. 5. 6. Formation of inter-ventricular septum, 7. Formation of interatrial septum (septum primum, foramen primum, septum secundum and foramen secundum) 8. Division of truncus arteriosus with aorticopulmonary septum. In 180 degree rotation.
  6. 6. Gross classification of congenital heart disease • Lesions without shunts Left heart malformations Right heart malformations Mitral stenosis Ebsteins anomaly Mitral valve prolapse Pulmonic stenosis Double orifice mitral valve Pulmonary regurgitation Parachute mitral valve Idiopathic dilatation of pulmonary valve Aortic stenosis (supravalvular, valvular, subvalvular) Pulmonary artery branch stenosis Aortic regurgitation Coarctation of aorta
  7. 7. • Lesions with left to right shunts (Acyanotic) Atrial level Ventricular level Others ASD (ostium primum, ostium secundum, sinus venosus type) VSD Coronary AV fistula ASD with acquired mitral stenosis VSD with aortic regurgitation PDA Partial anomalous pulmonary venous connection VSD causing LV > RA shunting, Gerbode defect Anomalous origin of left coronary artery from pulmonary artery
  8. 8. • Lesions with right to left shunts (Cyanotic) discussed in detail With increased pulmonary blood flow With normal or decreased pulmonary blood flow Complete transposition of great arteries Tetralogy of Fallot Double outlet Right Ventricle Tricuspid Atresia Truncus Arteriosus Ebsteins Anomaly with R>L atrial shunt (ASD) Total Anomalous Pulmonary Venous Connection Pulmonary Atresia with intact ventricular septum Eisenmengers syndrome (VSD) Pulmonary AV fistula
  9. 9. Complete transposition of great arteries (D-transposition) • Here the aorta arises from morphologic right ventricle and lies anterior to the pulmonary artery, which originates from morphologic left ventricle. • Not compatible with life; however, this abnormality may survive with the simulataneous prescence of an interatrial communication (foramen ovale or ASD) • It is common in male babies. (esp. with diabetic mother)
  10. 10. • It may also be associated with other abnormalities like VSD and PDA • S1 = Normal, S2 = single aortic component heard. Associated with holosystolic murmur of VSD, continuous murmur of PDA or ejection systolic murmur of Pulmonic stenosis • ECG – suggests right ventricular hypertrophy • CXR-PA – “Egg on Stalk” OR “Egg shaped heart” appearance
  11. 11. • Treatment – – Primary – surgical – Supportive treatment – • PGE1 infusion to keep PDA open • Balloon atrial septostomy to keep ASD open • Surgical procedures- – Arterial Switch operation (Jatene procedure) – Atrial Switch operation (Mustard / Senning procedure)
  12. 12. Jatene procedure
  13. 13. Mustard Senning procedure
  14. 14. Double Outlet Right Ventricle(DORV) • In this type of cono-truncal anomaly, both the great vessels arise from right ventricle. It is associated with VSD(Subaortic or subpulmonic) • In DORV with subaortic VSD, oxygenated blood passes LV and flows through VSD across RV into aorta • IN DORV with Subpulmonic VSD, blood from LV flows to pulmonary artery and blood from RA to RV flows to aorta(Taussig – Bing anomaly)
  15. 15. • Associated anomalies – Trisomy 13, trisomy 18, Coarctation of aorta, right sided aortic arch, TAPVC/PAPVC, tracheo- esophageal fistula, dextrocardia • Natural history of DORV with subaortic VSD resembles that of VSD, and Natural history of Subpulmonic VSD resmebles that of TGA
  16. 16. • Clinical features – Cyanosis – Systolic thrill and holosystolic murmur due to VSD • ECG – Right axis deviation with counter- clockwise rotation(near V2)
  17. 17. Truncus Arteriosus • It is and uncommon congenital anomaly with single vessel forming outflow tract for both ventricle, due to failure of development of aortico-pulmonary septum. It is always associated with large supracristal VSD. • An interesting note- truncus valve is usually tricuspid occasionally quadricuspid • Three types (Collette – Edward Classification) – Type 1 – a short single segment of pulonary artery arises from truncus and later divides into right and left pulmonary artery
  18. 18. – Type 2 – Right and left pulmonary arteries arise sepeartely from posterior wall of truncus – Type 3 – right and left pulmonary arteries arise seperately from lateral wall of truncus • Associated anomalies – Di-George Syndrome • Clinical Features – Normal S1, Loud S2 without splitting – Ejection Systolic murmur heard
  19. 19. • ECG – features suggestive of LV volume overload + RV pressure overload • CXR-PA – Cardiomegaly + Pulmonary Plethora (Clincally Cyanosis) : suggestive of truncus arteriosus • Natural History – – mean age of death – 5 weeks – Only 15%survive till one year, severe pulmonary hypertension develops after 1 year of life • Ideal age for corrective surgery 3 to 6 months
  20. 20. Total Anomalous Pulmonary Venous Connection • Pulmonary veins normally drain into left atrium, but in patients with TAPVC, pulmonary veins may connect to systemic veins within the thorax(supradiaphragmatic) or portal vein in the abdomen(infradiaphragmatic). Thereby draining oxygenated blood into right atrium. • Associated anomalies – Common atrium – Single ventricle – PDA – Pulmonary valve stenosis – Truncus Arteriosus
  21. 21. • Clinical Features – Cyanosis – Continuous murmur along left sternal borderdue to flow through anomalous pulmonary venous channels – Loud P2 and development of pulmonary hypertension gradually – The intensity of continuous murmur decreases as the pulmonary hypertension progresses
  22. 22. • Natural history of TAPVC – 50% infants dies by 6 months – 80% infants die by 1 year – Symptoms start appearing by 1st month of life and progress rapidly in 6 months • Smith’s classification – Supradiaphragmatic – Infradiaphragmatic
  23. 23. • Darling’s classification Type Also known as Abnormal connection Type 1 Supracardiac PV join SVC Type 2 Cardiac PV join RA Type 3 Infracardiac PV joins IVC or below Type 4 Mixed Rare , multiple connections
  24. 24. • ECG – is suggestive of RVH with right axis deviation • CXR-PA – – Snow man appearance or figure of eight appearance
  25. 25. Eisenmengers syndrome (VSD) • It is the condition in which L>R shunt get reversed (R>L shunt) with the development of pulmonary hypertension, central cyanosis, clubbing, secondary polycythemia. • Symptoms of poor exercise tolerance and rarely hemoptysis may occur. Generalised cyanosis, Loud and palpable P2, prominent parasternal heave may appear
  26. 26. Tetralogy of Fallot • It is the most common congenital cyanotic heart disease. • It has 4 components – Large VSD – RV outflow obstruction (Pulmonic stenosis – infundibular type) – Overriding of aorta – Right ventricular hypertrophy
  27. 27. • Variability of RV outflow tract obstruction and systemic-pulmonary pressure difference contributes to occurrence of episodic cyanosis in TOF. • Presence of ASD = Pentology of Fallot • Pulmonic Stenosis + RV hypertrophy + ASD with R>L shunt = Triology of Fallot
  28. 28. • Clinical features- – S1 – Normal, S2 – Single , loud S2 is present – Ejection Systolic murmur at Left 3rd and 4th ICS – Large VSD murmur less produced. • ECG – – Right axis deviation – Large R wave in V1 • CXR-PA – – “Boot shaped heart” or “Couer en Sabot” – Pulmonary Oligemia
  29. 29. • Complications – Infective Endocarditis – Embolisation and Cerebral Abscess – Pulmonary Tuberculosis – Secondary Polycythemia – CCF – rare • Treatment of cyanotic spells – squating, oxygen, morphine, beta- blocker (Propranolol)
  30. 30. • Surgical procedure – Blalock – Taussig Shunt(left pulmonary artery to left subclavian artery) – Pott’s procedure(left pulmonary artery to anterior wall of descending aorta) – Waterston procedure(right pulmonary artery to ascending aorta)
  31. 31. Tricuspid Atresia • In this condition triscuspid valve is absent, the floor of RA is intact. It is always accompanied by VSD • Blood flows from RA to LA across interatrial septum and later into LV and then to RV across VSD and then into Pulmonary artery • Clinical Features- – Cyanosis , JVP a wave prominent, first and second heart sounds may be single, Systolic murmur due to VSD.
  32. 32. Future scope SIGNALLING PATHWAYS ARE DISCOVERED AT EACH STEP OF CARDIAC EMBRYOGENESIS.
  33. 33. Thank You

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