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Cyanotic heart disease
Cyanotic heart disease
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  1. 1. Introduction to Congenital Heart Disease ALI AHMED MAHAREAK, MD Lecturer Of Anesthesiology And Intensive Care , Al-Azhar University Ali_m7areak@yahoo.com
  2. 2. Learning Objectives Applied anatomy of the Heart Embryo-fetal circulation and Changes at birth Classification of CHD Pathophysiology of CHD The concepts of shunting, single ventricle physiology, and intercirculatory mixing Pulmonary vascular occlusive disease Specific CHD Defects
  3. 3. Heart anatomy  Four chamber muscular organ  The overall shape of the heart is that of a three-sided pyramid  Located in the mediastinum Behind sternum Between 2nd and 6th ribs Between T5-T8  Apex of heart Located at the 5th intercostal space
  4. 4. Heart position in thorax
  5. 5. Chambers of the Heart  Atria – two superior chambers “Receiving chambers” Blood from veins enters atria Ventricles – two inferior chambers “pumping chambers” Thick muscular walls to increase force of pumping action Left > right Separated by interventricular septum
  6. 6. Valves of the Heart Tricuspid” valve  RA to RV  three leaflets septal, anterosuperior, and inferior Mitral valve LA to LV Two leaflets ,The aortic (or anterior) leaflet and Posterior leaflet Pulmonary valve RV to pulmonary trunk Aortic valve LV to aorta three leaflets, the right coronary cusp, the left coronary cusp, and the non-coronary cusp.
  7. 7. Heart anatomy  The surgical anatomy of the heart is best understood when the position of the cardiac chambers and great vessels is known  One third of the cardiac mass lies to the right of the midline, and two thirds to the left.  The long axis of the heart is orientedfrom the left epigastrium to the right shoulder
  8. 8. Heart anatomy •The short axis, which corresponds to the plane of the atrioventricular groove, is oblique and is oriented closer to the vertical than to the horizontal plane careful study of this short axis,  First, the atrial chambers lie to the right of their corresponding ventricles.  Second, the right atrium and ventricle lie anterior to their left-sided counterparts  The septal structures between them are obliquely oriented
  9. 9. Embryo-fetal circulation The placenta provides gas exchange in the fetus. Changes at birth are directly related to the exclusion of the placenta and the establishment of the lungs as the unit of gas exchange Oxygenated blood flows from the placenta To the fetus via the umbilical vein
  10. 10.  After reaching fetus the blood flows through the inferior vena cava  IVC Empties into the right atrium of the heart  The blood then passes to the left atrium through the foramen ovale  Only about one-third of this blood reaches the lungs (due to high flow resistance since the lungs are not yet expanded, and due to hypoxic vasoconstriction Embryo-fetal circulation Three shunts are present in fetal life: 1. Ductus venosus: connects the umbilical vein to the inferior vena cava 2. Ductus arteriosus: connects the main pulmonary artery to the aorta 3. Foramen ovale: anatomic opening between the right and left atrium.
  11. 11. Flow Chart of Fetal Circulation
  12. 12. Changes at birth o Clamping the cord shuts down low- pressure system o Increased atmospheric pressure causes lungs to inflate with oxygen o Lungs now become a low-pressure system o Pressure from blood flow in the lt side of the heart foramen ovale closure o Closure of the ductus arteriosus : o functional closure within 1st day of life and permanent anatomic closure after 2-3 weeks
  13. 13. Fetal vs. Infant Circulation Fetal 1. Low pressure system 2. Right to left shunting 3. Lungs non functional 4. Increased pulmonary resistance 5. Decreased systemic resistance Infant 1. High pressure system 2. Left to right blood flow 3. Lungs functional 4. Decreased pulmonary resistance 5. Increased systemic resistance
  14. 14. Classification of CHD A cyanotic Heart Disease Cyanotic Heart disease Increase pulmonary blood flow Obstruction of blood flow Decrease pulmonary blood flow Mixed blood flow
  15. 15. A Cyanotic Heart Defect A Cyanotic Increased in pulmonary blood flow 1. ASD 5-10% 2. VSD 25 % 3. AVC 4. PDA Obstruction of blood flow form ventricle 1.Pulmonary stenosis 2.Aortic stenosis 3.Coarctation of the Aorta
  16. 16. Cyanotic Heart Defect Cyanotic Decreased pulmonary blood flow Tetralogy of Fallot (TOF) 10% Tricuspid atresia (TA) Pulmonary atresia (PA) / critical PS Mixed blood flow Transposition of the great Arteries 5% Total Anomaly pulmonary venous return Truncus Arteriosus Hypo plastic left Heart Syndrome Double outlet right ventricle (DORV)
  17. 17. Pathophysiology of CHD  The presence of shunts is the hallmark of CHD  Anesthetic management of the patient with CHD is dependent on a clear understanding of:  The concepts of shunting , single ventricle physiology, and intercirculatory mixing.  The types and the dynamics of the anatomic shunting process.  The effects of congenital lesions on the development of the pulmonary vascular system.  The factors that influence pulmonary and systemic vascular resistance.  The factors responsible for producing myocardial ischemia.
  18. 18. Shunting ,intercirculatory mixing  Shunting is the process whereby venous return into one circulatory system is recirculated through the arterial outflow of the same circulatory system.  Effective blood flow is the quantity of venous blood from one circulatory system reaching the arterial system of the other circulatory system.  Effective pulmonary blood flow and effective systemic blood flows are the flows necessary to maintain life.
  19. 19. Classification of anatomic shunts Simple shunts a communication (orifice) exists between pulmonary and arterial vessels or heart chambers  In a simple shunt, there is no fixed obstruction to outflow from the vessels or chambers involved in the shunt. the shunt orifice may be small (restrictive shunt) or large (nonrestrictive) Complex shunts In complex anatomic shunt lesions there is obstruction to outflow in addition to a shunt orifice The obstruction may be at the valvular, subvalvular, or supravalvular level  The obstruction may be fixed (as with valvular stenosis) or variable (as with dynamic infundibular obstruction).
  20. 20. Simple shunts
  21. 21. Single ventricle physiology  Single ventricle physiology is used to describe the situation wherein complete mixing of pulmonary venous and systemic venous blood at the atrial or ventricular level  then distribute output to both the systemic and pulmonary beds.  Ventricular output is the sum of pulmonary blood flow (Qp) and systemic blood flow (Qs).  Distribution of systemic and pulmonary blood flow is dependent on the relative resistances to flow  Oxygen saturations are the same in the aorta and the PA.
  22. 22. Single ventricle physiology
  23. 23. Pulmonary vascular occlusive disease (PVOD) PVOD produces obstruction to pulmonary blood flow due to structural changes in the pulmonary vasculature The morphologic changes have three components: increased muscularity of small pulmonary arteries, small artery intimal hyperplasia, scarring, and thrombosis and reduced numbers of intra-acinar arteries Causes of PVOD :  Exposure to systemic arterial pressures and high pulmonary blood flows large (nonrestrictive) VSD.  Exposure to high blood flow large ASD  Obstruction of pulmonary venous drainage TAPVR, mitral atresia, congenital aortic stenosis, severe coarctation of the aorta
  24. 24. A Cyanotic Heart Defect A Cyanotic Increased in pulmonary blood flow 1. ASD 2. VSD 3. AVC 4. PDA Obstruction of blood flow form ventricle 1.Pulmonary stenosis 2.Aortic stenosis 3.Coarctation of the Aorta
  25. 25. Defect with increased pulmonary blood flow 1. VSD (ventricular septal defect ) 2. ASD (Atrial septal defect ) 3. AVC (Atrioventricular canal defect ) 4. PDA ( patent ductus arteriosus)
  26. 26. Ventricular Septal Defect  Single most common congenital heart malformation,30% of all CHD  Defects can occur in both the membranous and muscular portion s of the septum
  27. 27. Ventricular Septal Defect Types Size Small VSDs < 3 mm in diameter Moderate VSDs 3-5 mm in diameter Large VSDs with normal PVR 6-10 mm in diameter Site Perimembranous (or membranous) Infundibular (subpulmonary VSD) – involves the RV outflow tract. inlet VSD, almost always involves AV valvular abnormalities Muscular VSD – can be single or multiple.
  28. 28. Ventricular Septal Defect Indications for Surgical Closure  Large VSD w/ medically uncontrolled .  Ages 6-12 mo w/ large VSD & Pulm. HTN  Age > 24 mo w/ Qp:Qs ratio > 2:1.
  29. 29. Atrial Septal Defects Three major types  Ostium secundum o most common o In the middle of the septum in the region of the foramen ovale  Ostium primum o Low position o Form of AV septal defect  Sinus venosus o Least common o Positioed high in the atrial septum o Frequently associated with PAPVR
  30. 30. Treatment o Closure generally recommended when ratio of pulmonary to systemic blood flow (Qp:Qs) is > 2:1 o Operation performed electively between ages 2 and 5 years Atrial Septal Defects
  31. 31. Atrioventricular canal defect (AVC ) Complete AVC Low primum ASD continuous with a posterior VSD. Cleft in both septal leaflets of TV/MV. Results in a large L to R shunt at both levels Partial AVC  ostium primum ASD association with a cleft mitral valve
  32. 32. Patent Ductus Arteriosus  PDA results when the ductus fails to close after birth  More in preterm hypoxic infants  Blood flows from aorta to the pulmonary artery, creating a left to right shunt  Increased pulmonary blood flow can result in pulmonary hypertension and reversal of the shunt  Medical : indomethacin 0.1-0.3 mg/kg BID for 3 doses  May be PDA dependent circulation  Anesthetic Management -Maintain high arterial pressures as low diastolic pressure can result in decreased organ perfusion
  33. 33. Anesthetic management goals in Defect with RT to LT shunt Maintain heart rate, contractility, and preload to maintain cardiac output. Avoid decreases in PVR:SVR ratio. The increase in pulmonary blood flow that accompanies a reduced PVR:SVR ratio necessitates an increase in cardiac output to maintain systemic blood flow  Avoid large increases in the PVR:SVR ratio. An increase may result in production of a right-to-left shunt In instances in which a right-to-left shunt exists, ventilatory measures to decrease PVR should be used. In addition, SVR must be maintained or increased
  34. 34. Cyanotic Heart Defect Cyanotic Decreased pulmonary blood flow Tetralogy of Fallot (TOF) Tricuspid atresia (TA) Pulmonary atresia (PA) / critical PS Mixed blood flow Transposition of the great Arteries Total pulmonary venous return Truncus Arteriosus Hypo plastic left Heart Syndrome Double outlet right ventricle (DORV)
  35. 35. Cyanotic CHD WITH Decreased pulmonary blood flow 1. Tetralogy of Fallot (TOF) 2. Tricuspid atresia (TA) 3. Pulmonary atresia (PA) / critical PS
  36. 36. Cyanotic CHD WITH Mixed blood flow 1. Transposition of the great Arteries 2. Total pulmonary venous return 3. Truncus Arteriosus 4. Hypo plastic left Heart Syndrome 5. Double outlet right ventricle (DORV)
  37. 37. Tetraology of Fallot 1.Large outlet VSD 2.RVOT Obstruction (infundibular or valvular, supravalvular) 3.RVH 4.Overriding aorta Types of TOF: 1. TOF/PS with pulmonary stenosis 2. TOF/PA with pulmonary atresia
  38. 38. Tetraology of Fallot TOF/PA
  39. 39. Tetraology of Fallot Goals of Anesthetic management  Maintain heart rate, contractility, and preload to maintain cardiac output to prevent exacerbation of dynamic RVOT  Avoid increases in the PVR:SVR ratio to avoid increase R–L shunting and reduce pulmonary blood flow  Use ventilatory measures to reduce PVR.  Maintain or increase SVR. This is particularly important when RV outflow obstruction is severe  Aggressively treat episodes of hypercyanosis.
  40. 40. Characterized by Hyperapnea (Rapid and deep respirations) Irritability and prolonged crying  cyanosis and heart murm Pathophysiology  Lower SVR or inc resistance of RVOT can increase the R-L shunt Hypoxic Spell (“TET Spell”) Treatment 1. Hold infant in knee-chest position 2. Morphine 3. Sodium bicarbonate to treat acidosis- decreases resp stimulating effect of acidosis 4. Vasoconstrictor (phenylephrine) 5. Propranolol
  41. 41. Tetraology of Fallot -Surgical therapy Palliative shunts  to increase pulmonary blood flow  systemic-to-pulmonary arterial shunt,  modified Blalock–Taussig shunt (MBTS) is used know Definitive repair between the ages of 2–10 months of age Surgery is aimed at  relieving the outflow obstruction by resection of hypertrophied, obstructing muscle bundles  Trans-annular patch. across the pulmonary valve and into the main PA.  Finally, the VSD is closed
  42. 42. Complete Transposition of the Great Arteries Complete separation of the 2 circuits: • Hypoxemic blood circulating in the body • Hyperoxemic blood circulating in the pulmonary circuit • Defect to permit mixing of 2 circulations- ASD, VSD, PDA Necessary for survival
  43. 43. Truncus Arteriosus  A single trunk leaves the heart Gives rise to pulm, systemic, and coronary circulations  Large VSD
  44. 44.  Tricuspid valve is absent  RV and PA are hypoplastic  Associated defects- ASD, VSD, or PDA (necessary for survival)  Dilation of LA and LV  Essentially single ventricle physiology Tricuspid Atresia
  45. 45. Total Anomalous Pulmonary Venous Return  The pulmonary veins drain into the RA or its venous tributaries rather than the LA  A inter-atrial communication (ASD or PFO) is necessary for survival  Systemic and pulmonary venous blood are completely mixed
  46. 46. Management of single ventricle • The primary goal in the management of patients with single ventricle physiology is optimization of systemic oxygen delivery and perfusion pressure. • Blalock-Taussig shunt in infancy • Bidirectional Glenn : SVC is connected to the pulmonary arteries • Fontan Procedure : Redirects IVC to pulmonary arteries